PUBLISHED BY THE BELGIAN SOCIETY OF RADIOLOGY (BSR)

DIAGNOSTIC AND INTERVENTIONAL IMAGING, RELATED IMAGING SCIENCES,AND CONTINUING EDUCATION

WETTEREN 1

1 Volume 98 Page 1-62

January-February

Bimonthly – 2015

P 702083

C1-Cover 1.indd 1 19/02/15 16:38

Sections of the Belgian Radiological Society (BSR):

Abdominal and digestive imaging B. Op de Beeck, E. DanseBone and joints J.F. Nisolle, M. ShahabpourBreast imaging M. Mortier, S. MurgoCardiac imaging N. Mollet, A. NchimiCardiovascular and interventional radiology S. Heye, D. HenroteauxChest radiology B. Ghaye, W. De WeverHead and neck radiology J. Widelec, R. HermansNeuroradiology M. Lemmerling, L. TshibandaPediatric radiology B. Desprechins, L. Breysem

For addresses and particulars, see website at http://www.bsr-web.be

Instructions to authors

The purpose of The Belgian Journal of Radio­logy is the publication of articles dealing with diagnostic radiology and related imag­ing techniques, therapeutic radiology, allied sciences and continuing education. All — new and revised — manuscripts and correspond­ence should be addressed to JBR­BTR Edi to­rial Office, Avenue W. Churchill 11/30, B­1180 Bruxelles, tel.: 02­374 25 55, fax: 32­2­374 96 28.Please note that the following instructions are based on the “Uniform Requirements for manuscripts Submitted to Biomedical Jour­nals” adopted by the International Committee of Medical Journal Editors (Radiology, 1980,135: 239­243). It should however be noted that presentation modifications may be introduced by the Editorial Office in order to conform with the JBR­BTR personal style.Authors should specify to which of the fol­lowing headings their manuscript is intended:Original Article, Review Article, Case Report, Pictorial Essay, Continuing Education, Technical Note, Book Review, Opinion, Letter to the Editor, Comment, Meeting News, in Memoriam, News.Authors should consider the following remarks and submit their manuscripts accord­ingly.All articles must contain substantive and speci fic scientific material.– Original articles are articles dealing with

one specific area of Radiology or allied science related through the personal expe­rience of the author.

– Review articles are special articles reporting the experience of the author considered in

the general perspective of the literature over the topic.

– Case reports are short descriptions of a particular case providing a message directly linked to an individual patient investigated.

– No more than one case should be described in detail and clinical description should be kept to a minimum. Case reports should invest the usual headings of articles but should focus on the particular radiologic procedure that contributed to the diagno­sis. References should be present, though limited in number. Tables and acknowl­edgements are usually omitted.

– Pictorial essays are articles presenting information through illustrations and leg­ends. The presentation remarks stated in the paragraph dealing with case reports apply to pictorial essays.

– Continuing education articles are designed in accordance with the general guidelines for articles published in the JBR­BTR in particular they are divided into introduc­tion, material and methods, results, discus­sion, references, and are provided with an abstract.

However, papers addressing the continuing education may have only additionnally to their contents an introduction (stating the aim of the article and providing any back­ground information useful to understand why the topic is relevant, and describing the subtopics covered by the study), refer­ences, and an abstract.

Tables should be limited to a maximum of one table per 6 pages of manuscript. Illustrations should also be limited to a maximum of one illustration (1010 cm)

(possibly made up of different parts) per 3 pages of manuscript.

All the material should be made available to the JBR­ BTR editorial office (2 copies of the manuscript with 2 sets of illustrations) with the corresponding diskette though there will not be peer review.

– Images in Clinical Radiology are short (max. 1 typed page) case reports designed to illustrate with max. 3 figures a specific enti­ty. The report should not include abstract nor discussion but consist of a synthetic description of the clinical and radiological features as well as the final diagnosis and one major reference. Technical notes are short descriptions of a specific technique, procedure or equipment of interest to radi­ologists. Technical notes may originate from radiologists having experience of the item presented or from commercial firms (these should contact the Editorial Office to obtain specific guidelines for publication). The manuscript length should be inferior to 1 typed page, original language should be English, the manuscript may be accompa­nied by maxi mum 1 b/w figure, and include one major reference.

– Book reviews should be limited to one typed page, mention full references of the book, including number of pages, of illus­trations (when available), and price. The author should specify to whom the book is intended and give a personal apprecia­tion. They will be publish ed with the initial letters of the signature.

(continued on p. vi)

Editor-in-Chief: J. PringotConsulting Co-Editor: Ph. Grenier (Paris)Managing Editor: P. SeynaeveWebmaster: P. Vanhoenacker

Editorial Board: F. Avni, L. Breysem, N. Buls, B. Coulier, B. Daenen, E. Danse, H. Degryse, P. Demaerel, B. Ghaye, J. Gielen, P. Habibollahi, N. Hottat, M. Laureys, F. Lecouvet, M. Lemmerling, B. Lubicz, J.F. Monville, T. Mulkens, A. Nchimi, J.F. Nisolle, B. Op de Beeck, R. Oyen, S. Pans, V.P. Parashar (USA), P. Parizel, P. Peene, H. Rigauts, N. Sadeghi, P. Simoni, S. Sintzoff Jr, A. Snoeckx, J. Struyven, H. Thierens, P. Van Dyck, F. Vanhoenacker, Ph. Van Hover, J. Verschakelen, K. Verstraete.

Board of the Belgian Society of Radiology (BSR):

President: G. Villeirs

Vice-President: D. Henroteaux

Secretary: Ch. Delcour

Treasurer: P. Vanhoenacker

BVAS/ABSYM-Representative: O. Ghekiere

Board Members: P. Aerts, B. De Foer, J.­F. De Wispelaere, M. Grieten, H. Jaspers, J.­P. Joris, R. Oyen, P. Seynaeve, G. Souverijns, D. Tack, B. Vande Berg, G. Vandenbosch, Ch. Van de Velde

Scientific Committee

President: R. Oyen

Secretaries: J. de Mey, B. Vande Berg

Members: R. Achten, D. Bielen, B. De Foer, Y. Lefebvre, M. Lemort, J. Pringot, R. Salgado, D. Tack, J. Ver­schakelen

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JBR-BTR 98/1 2015

Journal Belge de Belgisch Tijdschrift voor RADIOLOGIE

Founded in 1907

A bimonthly journal devoted to diagnostic and interventional imaging,related imaging sciences, and continuing education

published by the Belgian Society of Radiology

Contents

A LETTER FROM THE BSR PRESIDENT. JBR-BTR, A NEW START. G. Villeirs 1

Pictorial review: Thoracic involvement in connective tissue diseases: radiological patterns and follow-upG. Serra, A.L. Brun, P. Ialongo, M.L. Chabi, P.A. Grenier . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3

The efficiency of US elastography in the differential diagnosis of thyroid nodulesN. Çetin, C. Yücel, P. Uyar Göçün, S. Aladag Kurt, F. Taneri, S. Oktar, H. Özdemir. . . . . . . . . . . . . . . . . . . . . . . 20

Gastrointestinal complications of accidental ingestion of foreign objectsJ. Huyskens, E. Van Hedent, L. Trappeniers, W. Simoens, T. Jager . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27

A giant retroperitoneal lipoma presenting as a sciatic hernia: MRI findings S. Duran, M. Cavusoglu, E. Elverici, T.D. Unal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32

Pseudotumoral tophaceous involvement of the Achilles paratenonT. Ryckaert, I. Crevits, S. Brijs, G. Debakker, F. Rosseel, A. Tieleman, R. De Man . . . . . . . . . . . . . . . . . . . . . . . . 34

Report of a rare anatomic variant: left upper lobe partial anomalous pulmonary venous returnY. De Brucker, B. Ilsen, C. Muylaert, L. Goethals, K. Nieboer, A. Fares, T. Jager, J. de Mey . . . . . . . . . . . . . . . 37

Ischiofemoral impingement due to a solitary exostosisJ. Schatteman, F.M. Vanhoenacker, J. Somville, K.L. Verstraete . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39

Macrodystrophia lipomatosa with ulnar distribution in handM. Azfar Siddiqui, M. Ahmad, N. Redhu, I. Ahmad, E. Ullah . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 43

Osteochondroma of the proximal humerus with frictional bursitis and secondary synovial osteochondromatosis

J. De Groote, B. Geerts, K. Mermuys, K. Verstraete . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 45

Cardiac amyloidosisP. Lu, H. Van Acker, P. Waer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49

IMAGES IN CLINICAL RADIOLOGY

Gaucher disease presenting with vertebral compression fractures and vertebral osteonecrosis.G. Clinckemaille, A. Larbi, P. Omoumi, J. Manelfe, B. Dallaudière . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 50

Combination of unusual lesions after blunt trauma.L. Médart, P. Lamborelle, L. Collignon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51

Umbilical artery pseudoaneurysm.S. Tribolet, J. Khamis, M. Lewin, T. Khuc . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52

Extrapulmonary manifestation of lymphangioleiomyomatosis. N. Favoreel, S.F. Van Meerbeeck, R. Gosselin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 53

Arachnoidal cyst arising from the oculomotor cistern.M. Eyselbergs, P. Cheecharoen, A. Bali, C. Venstermans, F. De Belder, Ö. Özarlak, J. Van Goethem, T. Menovsky, M. Lammens, F. Vanhoenacker, P.M. Parizel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54

Contribution of color Doppler sonography to the characterization of an unusual thickening of the common bile duct.

N. Michalakis, D. Van Gansbeke . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 55

Foix-Chavany-Marie or opercular syndromeP. Gillardin, R. Van Eetvelde, T. Kostermans, R. De Potter, D. Dewilde, M. Lemmerling . . . . . . . . . . . . . . . . . . 56

Sclerosing hemangioma of the lungS. Van Petegem, J. De Cock, B. Houthoofd, A. Annicq, K. Verstraete . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 57

Progressive quadriplegia resulting from septic facet joint arthritisS. Raeymaeckers, K. Nieboer, J. De Mey . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 58

01-Contents-2015(1).indd 1 19/02/15 16:49

u The terms used for indexation of subjects were developed by the Radiological Society of North America (RSNA) over a period of years. Their use here is by permission of the RSNA. The terms may not be used in any other index, print or electronic, except by specific permission of RSNA.

uu Indexed in Index Medicus and in Zentralblatt Radiologie. Evaluated for Medline User, EMBASE and CANCERNET. Abstracted in Excerpta Medica Journals.

Serpiginous cholesteatoma mimicling a vascular channelA. Karandikar, S.Ch. Loke, J. Goh, S.B. Yeo, T.Y. Tan . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59

A rare case of ischemic stroke following occlusion of the artery of PercheronH. Dechamps, P. Gillardin, R. De Potter, D. Dewilde, M. Lemmerling. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60

Forthcoming Courses and Meetings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61

Announcements: ESNR Educational Spine Course 2015 – Virtual Colonoscopy Workshop . . . . . . . . . . . . . . . . . . 62

Instructions to Authors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . cii

Advertising index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61

01-Contents-2015(1).indd 2 19/02/15 16:49

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www.bracco.com

DENOMINATION DU MEDICAMENT: MultiHance 0,5 M solution injectable. COMPOSITION QUALITATIVE ET QUANTITATIVE: 1 mL de solution contient : acide gadobénique 334 mg (0,5 M) sous forme de sel de diméglumine. [529 mg de gadobénate de diméglumine = 334 mg d’acide gadobénique + 195 mg de diméglumine]. 5 mL de solution contient : acide gadobénique 1670  mg (2,5 mmol) sous forme de sel de diméglumine. [2645 mg de gadobénate de diméglumine = 1670 mg d’acide gadobénique + 975 mg de diméglumine]. 10 mL de solution contient : acide gadobénique 3340 mg (5 mmol) sous forme de sel de diméglumine. [5290 mg de gadobénate de diméglumine = 3340 mg d’acide gadobénique + 1950 mg de diméglumine]. 15 mL de solution contient : acide gadobénique 5010 mg

(7,5 mmol) sous forme de sel de diméglumine. [7935 mg de gadobénate de diméglumine = 5010 mg d’acide gadobénique + 2925 mg de diméglumine]. 20 mL de solution contient : acide gadobénique 6680 mg (10 mmol) sous forme de sel de diméglumine. [10580 mg de gadobénate de diméglumine = 6680 mg d’acide gadobénique + 3900 mg de diméglumine]. FORME PHARMACEUTIQUE: Solution injectable. Solution aqueuse limpide, incolore, remplie dans des flacons de verre incolore.Osmolalité à 37°C : 1,970 Osmol/kg. Viscosité à 37°C : 5,3 mPa.s. DONNEES CLINIQUES: Indications thérapeutiques: Ce médicament est à usage diagnostique uniquement. Produit de contraste paramagnétique utilisé dans l’imagerie par résonance magnétique (IRM) et indiqué dans : • IRM du foie pour la détection des lésions hépatiques lorsqu’un cancer hépatique secondaire ou primitif (carcinome hépatocellulaire) est suspecté ou connu. • IRM du cerveau et de la moelle épinière où il améliore la détection des lésions et apporte des informations diagnostiques supplémentaires comparativement à une IRM sans produit de contraste. • Angiographie par résonance magnétique (ARM) où il améliore l’exactitude diagnostique pour la détection de la maladie vasculaire sténo-occlusive cliniquement significative lorsqu’une pathologie vasculaire des artères abdominales ou périphériques est suspectée ou connue. •  IRM du sein, pour la détection des lésions malignes chez les patients pour lesquels un cancer du sein est connu ou suspecté, sur la base des résultats disponibles de mammographie ou d’échographie. Posologie et mode d’administration: IRM du foie : La dose recommandée chez l’adulte est de 0,05 mmol/kg de poids corporel, soit 0,1 ml/kg de la solution 0,5 M. IRM du système nerveux central : La dose recommandée chez l’adulte et l’enfant de plus de 2 ans est de 0,1 mmol/kg de poids corporel, soit 0,2 ml/kg de la solution 0,5 M. ARM : La dose recommandée chez l’adulte est de 0,1 mmol/kg de poids corporel, soit 0,2 ml/kg de la solution 0,5 M. IRM du sein : La dose recommandée chez l’adulte est de 0,1 mmol/kg de poids corporel, soit 0,2 ml/kg de la solution 0,5 M. MultiHance doit être introduit dans la seringue immédiatement avant l’injection et ne doit pas être dilué. Tout reliquat éventuel doit être jeté et ne doit pas être utilisé pour d’autres examens IRM. Pour diminuer le risque d’extravasation de MultiHance dans les tissus mous environnants, il est conseillé de s’assurer de la bonne disposition de l’aiguille ou de la canule dans la veine. Foie et système nerveux central : le produit doit être administré par voie intraveineuse soit en bolus soit en injection lente (10 mL/min). ARM : le produit doit être administré par voie intraveineuse en bolus, soit manuellement soit à l’aide d’un injecteur automatique. L’injection doit être suivie d’un bolus de chlorure de sodium à 0,9%. Acquisition des images post-contraste :

Populations particulières: Insuffisants rénaux: L’administration de MultiHance doit être évitée chez les patients présentant une insuffisance rénale sévère (DFG < 30 mL/min/1,73  m²) et en période périopératoire de transplantation hépatique, sauf si les informations diagnostiques sont indispensables et ne peuvent être obtenues au moyen d’une IRM sans rehaussement du contraste. Si l’administration de MultiHance ne peut être évitée, la dose ne doit pas excéder 0,1 mmol/kg de poids corporel dans le cas d’une IRM du cerveau et de la moelle épinière ou d’une angiographie par résonance magnétique (ARM) et 0,05 mmol/kg de poids corporel dans le cas d’une IRM du foie. Ne pas administrer plus d’une dose au cours de l’examen IRM. En raison du manque d’information sur les administrations répétées, les injections de MultiHance ne doivent pas être réitérées, sauf si l’intervalle entre les injections est d’au moins 7 jours. Sujets âgés (à partir de 65 ans): Aucune adaptation posologique n’est nécessaire. Utiliser avec prudence chez les sujets âgés. Enfants: Aucun ajustement posologique n’est nécessaire. L’utilisation dans l’IRM du système nerveux central n’est pas recommandée chez l’enfant de moins de 2 ans. L’utilisation dans l’IRM du foie et l’ARM n’est pas recommandée chez l’enfant de moins de 18 ans. Contre-indications: MultiHance est contre-indiqué chez :• les patients présentant une hypersensibilité à l’un de ses constituants. • les patients ayant des antécédents d’allergie ou d’effet indésirable liés à d’autres chélates de gadolinium. Effets indésirables: Les effets indésirables suivants ont été observés au cours du développement clinique de MultiHance sur 2637 sujets adultes. Aucun effet indésirable de fréquence supérieure à 2 % n’a été rapporté. Manifestations infectieuses. Peu fréquent (≥1/1 000, <1/100) : rhinopharyngite. Manifestations neurologiques. Fréquent (≥1/100, <1/10) : céphalée. Peu fréquent (≥1/1 000, <1/100) : paresthésies, sensations vertigineuses, syncope, parosmie. Rare (≥1/10 000, <1/1  000): hyperesthésie, tremblements, hypertension intra-crânienne, hémiplégie, convulsion. Troubles oculaires. Rare (≥1/10 000, <1/1 000): conjonctivite. Manifestations ORL. Rare (≥1/10 000, <1/1 000): acouphènes. Troubles cardiaques. Peu fréquent (≥1/1 000, <1/100) : tachycardie, fibrillation auriculaire, bloc auriculo-ventriculaire du 1er degré, extrasystoles ventriculaires, bradycardie sinusale. Rare (≥1/10 000, <1/1 000): arythmie, ischémie myocardique, allongement de l’intervalle PR. Manifestations vasculaires. Peu fréquent (≥1/1 000, <1/100) : hypertension artérielle, hypotension artérielle. Manifestations respiratoires, thoraciques et médiastinales. Peu fréquent (≥1/1 000, <1/100) : rhinite. Rare (≥1/10 000, <1/1 000): dyspnée, laryngospasme, sifflements respiratoires, congestion pulmonaire, œdème pulmonaire. Troubles gastro-intestinaux. Fréquent (≥1/100, <1/10) : nausée. Peu fréquent (≥1/1 000, <1/100) : sécheresse buccale, dysgueusie, diarrhée, vomissement, dyspepsie, hypersialorrhée, douleurs abdominales. Rare (≥1/10  000, <1/1 000): constipation, incontinence faecale, pancréatite nécrosante. Manifestations cutanées et sous-cutanées. Peu fréquent (≥1/1 000, <1/100) : prurit, éruption cutanée, œdème de la face, urticaire, hypersudation. Manifestations musculaires, ostéoarticulaires et du tissu conjonctif. Peu fréquent (≥1/1 000, <1/100) : douleurs dorsales, myalgies. Troubles rénaux et manifestations urinaires. Rare (≥1/10 000, <1/1 000): incontinence urinaire, mictions impérieuses. Manifestations générales et au point d’injection. Fréquent (≥1/100, <1/10) : réaction au point d’injection, sensation de chaleur. Peu fréquent (≥1/1 000, <1/100) : asthénie, fièvre, frissons, douleur thoracique, douleurs, douleur au point d’injection, extravasation au point d’injection. Rare (≥1/10 000, <1/1 000): inflammation au point d’injection. Anomalies des examens paracliniques. Peu fréquent (≥1/1 000, <1/100) : examens de laboratoire anormaux, ECG anormal, allongement de l’intervalle QT. Les anomalies biologiques citées ci-dessus, observées chez 0,4 % des patients au maximum après administration de MultiHance, incluent : anémie hypochrome, hyperleucocytose, leucopénie, augmentation des basophiles, hypoprotéinémie, hypocalcémie, hyperkaliémie, hyper- ou hypoglycémie, albuminurie, glycosurie, hématurie, hyperlipidémie, hyperbilirubinémie, augmentation du fer sérique et augmentation des taux sériques des transaminases, des phosphatases alcalines, de la lactico-deshydrogénase et de la créatininémie. Cependant, ces phénomènes ont été essentiellement observés chez des patients présentant une insuffisance hépatique connue ou une maladie métabolique sous-jacente. Dans la plupart des cas, il s’agissait d’événements non-graves, transitoires et qui ont régressé spontanément sans séquelle. Aucune corrélation n’a pu être établie avec l’âge, le sexe ou la dose administrée. Pédiatrie Au cours des essais cliniques chez l’enfant, les événements indésirables les plus fréquemment rapportés incluent les vomissements (1,4%), la fièvre (0,9%) et l’hyperhidrose (0,9%). La fréquence et la nature des événements indésirables étaient similaires à celles observées chez l’adulte. Depuis la commercialisation de MultiHance, des effets indésirables ont été rapportés, chez moins de 0,1 % des patients. Les plus fréquents sont : nausées, vomissements, manifestations cliniques plus ou moins sévères d’hypersensibilité comprenant : choc anaphylactique, réactions anaphylactoïdes, angioœdème, spasme laryngé et éruption cutanée. Des réactions au point d’injection, consécutives à une extravasation du produit de contraste, entraînant localement une douleur ou une sensation de brûlure, voire une tuméfaction, un décollement cutané ou, dans de rares cas de tuméfaction locale sévère, une nécrose ont également été rapportées. Une thrombophlébite locale a également été reportée dans de rares cas. Des cas isolés de fibrose néphrogénique systémique (FNS) ont été rapportés avec MultiHance chez des patients ayant également reçu d’autres produits de contraste contenant du gadolinium. TITULAIRE DE L’AUTORISATION DE LA MISE SUR LE MARCHE: Bracco Imaging Deutschland GmbH, Max-Stromeyer-Straße 116, 78467 Konstanz, Allemagne. NUMEROS D’AUTORISATION DE MISE SUR LE MARCHE: MultiHance 5 ml: BE199963, MultiHance 10 ml: BE199972, MultiHance 15 ml: BE199981, MultiHance 20 ml: BE199997. MODE DE DÉLIVRANCE: Sur prescription médicale. DATE DE MISE A JOUR/D’APPROBATION DU TEXTE: Date de mise à jour : 10/2013.

Des informations sur les rubriques Mises en garde spéciales et précautions d’emploi, Interactions, Fécondité, grossesse et allaitement, Effets sur l’aptitude à conduire des véhicules et à utiliser des machines, Surdosage, Propriétés pharmacologiques et Données pharmaceutiques se trouvent dans le Résumé des Caractéristiques du Produit complet. Pack sizes commercialised in Belgium: Multihance 10 ml, 15 ml and 20 ml - vial.

Foie

Imagerie dynamique Immédiatement après l’injection en bolus

Imagerie retardéeEntre 40 et 120 minutes après l’injection (IRM retardée), en fonction du type d’imagerie nécessaire

Système nerveux central Jusqu’à 60 minutes après administration

ARM

Immédiatement après l’administration, avec un délai d’acquisition calculé sur la base du bolus test ou par la technique de détection automatique du bolus. Si la détection automatique du contraste en séquence pulsée n’est pas utilisée, alors l’injection d’un bolus test de 2 ml de produit au maximum devra être réalisée pour calculer le timing d’acquisition adéquat.

SeinAcquisition dynamique en séquence pondérée T1 après administration d’un bolus et répétée après 2, 4, 6 et 8 minutes.

NAAM VAN HET GENEESMIDDEL: MultiHance, 0.5 M oplossing voor injectie. KWALITATIEVE EN KWANTITATIEVE SAMENSTELLING: 1 ml oplossing voor injectie bevat: 334 mg gadobeenzuur (0,5 M) als het dimeglumine-zout [gadobeendimeglumine 529 mg = gadobeenzuur 334 mg + meglumine 195 mg]. 5 ml oplossing voor injectie bevat: 1.670 mg gadobeenzuur (2,5 mmol) als dimegluminezout. [gadobenaatdimeglumine 2.645 mg = gadobeenzuur 1.670 mg + meglumine 975 mg]. 10 ml oplossing voor injectie bevat: 3.340 mg gadobeenzuur (5 mmol) als dimegluminezout [5.290 mg gadobenaatdimeglumine = 3.340 mg gadobeenzuur + 1.950 mg meglumine]. 15 ml oplossing voor injectie bevat: 5.010 mg gadobeenzuur (7,5 mmol) als dimegluminezout [7.935 mg gadobenaatdimeglumine = 5.010 mg gadobeenzuur + 2.925 mg meglumine]. 20 ml oplossing voor injectie bevat: 6.680  mg gadobeenzuur (10 mmol) als dimegluminezout [10.580 mg gadobenaatdimeglumine = 6.680 mg gadobeenzuur + 3.900 mg meglumine]. FARMACEUTISCHE VORM: Oplossing voor injectie. Heldere waterige oplossing, afgevuld in kleurloze glazen injectieflacons. Osmolaliteit bij 37 ºC: 1,97 osmol/kg. Viscositeit bij 37 ºC: 5,3 mPa.s KLINISCHE GEGEVENS: Therapeutische indicaties: Dit geneesmiddel is uitsluitend bestemd voor diagnostisch gebruik. MultiHance is een paramagnetische contrastvloeistof die wordt gebruikt voor de magnetische resonantie tomografie (MRI) geïndiceerd voor: •  MRI van de lever voor de detectie van focale leverlaesies bij patiënten met bekende of verdachte primaire leverkanker (b.v. hepatocellulair carcinoom) of metastasen. • MRI van de hersenen en het ruggenmerg, waar het de detectie van laesies verbetert en aanvullende diagnostische informatie kan geven op de informatie uit de niet contrast-versterkte MRI. • Contrast-versterkte MR-angiografie (MRA) bij patiënten met verdachte of bekende vasculaire ziekten van de abdominale of perifere arteriën. • MRI van de borst voor het opsporen van kwaadaardige laesies bij patiënten met een gekende of vermoede borstkanker op basis van de resultaten van een voorafgaande mammografie of echografie. Dosering en wijze van toediening: MRI van de lever: de aanbevolen dosis MultiHance bij volwassenen bedraagt 0,05 mmol/kg lichaamsgewicht, hetgeen overeenkomt met 0,1 ml/kg van de 0,5 M oplossing. MRI van de hersenen en het ruggenmerg: de aanbevolen dosis MultiHance bij volwassen en pediatrische patiënten ouder dan 2  jaar is 0,1 mmol/kg lichaamsgewicht, hetgeen overeenkomt met 0,2 ml/kg van de 0,5 M oplossing. MRA: de aanbevolen dosis MultiHance bij volwassenen is 0,1 mmol/kg lichaamsgewicht hetgeen overeenkomt met 0,2 ml/kg van de 0,5 M oplossing. MRI van de borst: de aanbevolen dosis MultiHance bij volwassenen is 0,1 mmol/kg lichaamsgewicht hetgeen overeenkomt met 0,2 ml/kg van de 0,5 M oplossing. MultiHance moet onmiddellijk voor het gebruik in de injectiespuit worden opgezogen en mag niet worden verdund. Eventuele ongebruikte restanten contrastvloeistof moeten worden vernietigd, en mogen niet worden gebruikt voor ander MRI onderzoek. Om de mogelijke risico’s van extravasatie van MultiHance in het spierweefsel te voorkomen dient men erop toe te zien dat de i.v. naald of canule zorgvuldig in de vena wordt aangebracht. Lever en hersenen en ruggenmerg: de oplossing dient intraveneus te worden toegediend als bolus of als langzame injectie (10 ml/min.). MRA: de oplossing dient intraveneus als een bolus injectie te worden toegediend, handmatig of gebruikmakend van een automatisch injecteersysteem. Na de injectie dient een spoeling met fysiologische zoutoplossing plaats te vinden. Post-contrast tomogrammen acquisitie:

Speciale populaties: Nierfunctiestoornis: Het gebruik van MultiHance dient te worden vermeden bij patiënten met een nierfunctiestoornis (glomerulaire filtratiesnelheid (GFR) < 30 ml/min/1,73  m²) en bij patiënten in de perioperatieve levertransplantatieperiode, tenzij de diagnostische informatie essentieel is en niet kan worden verkregen met niet-contrastversterkte MRI. Indien gebruik van MultiHance niet kan worden vermeden, dient de dosis niet groter te zijn dan 0,1 mmol/kg lichaamsgewicht wanneer gebruikt voor een MRI van de hersenen en ruggenmerg of MR angiografie en niet groter dan 0,05 mmol/kg lichaamsgewicht wanneer gebruikt voor een MRI van de lever. Niet meer dan één dosis mag worden gebruikt bij een scan. Wegens het ontbreken van informatie over herhaalde toedieningen dient MultiHance niet herhaald te worden toegediend, tenzij het interval tussen de injecties ten minste 7 dagen bedraagt. Ouderen (van 65 jaar en ouder): en dosisaanpassing wordt niet noodzakelijk geacht. Voorzichtigheid is geboden bij oudere patiënten. Pediatrische populatie: Er is geen dosisaanpassing nodig. Gebruik voor MRI van de hersenen en de wervelkolom wordt niet aanbevolen bij kinderen jonger dan 2 jaar. Gebruik voor MRI van de lever en MRA wordt niet aanbevolen bij kinderen jonger dan 18 jaar. Contra-indicaties: MultiHance is gecontraïndiceerd bij: • patiënten met overgevoeligheid voor het werkzame bestanddeel of voor een van de hulpstoffen. • patiënten die eerder allergische reacties of andere bijwerkingen hebben ondervonden met andere gadoliniumchelaten. Bijwerkingen: Tijdens toediening aan 2637 volwassenen gedurende de klinische ontwikkeling van MultiHance zijn de volgende bijwerkingen gemeld. Er zijn geen bijwerkingen die met een frequentie van meer dan 2% voorkomen. Infecties en parasitaire aandoeningen. Soms (≥ 1/1.000, <1/100): nasofaringitis. Zenuwstelselaandoeningen. Vaak (≥ 1/100, <1/10): hoofdpijn. Soms (≥ 1/1.000, <1/100): paresthesie, duizeligheid, syncope, stoornis van het reukvermogen. Zelden (≥  1/10.000, < 1/1.000): hyperesthesie, tremor, intracraniële hypertensie, halfzijdige verlamming, convulsie. Oogaandoeningen. Zelden (≥ 1/10.000, < 1/1.000): conjunctivitis. Evenwichtsorgaan- en ooraandoeningen. Zelden (≥ 1/10.000, < 1/1.000): oorsuizen. Hartaandoeningen. Soms (≥ 1/1.000, <1/100): tachycardie, atriumfibrilleren, eerste-graads atrioventriculair block, ventriculaire extrasystoles, sinus bradycardie. Zelden (≥ 1/10.000, < 1/1.000): aritmie, myocard ischemie, verlengd PR interval. Bloedvataandoeningen. Soms (≥ 1/1.000, <1/100): hypertensie, hypotensie. Ademhalingsstelsel-, borstkas- en mediastinumaandoeningen. Soms (≥ 1/1.000, <1/100): rhinitis. Zelden (≥  1/10.000, < 1/1.000): dyspnoe, laryngismus, hijgende ademhaling, pulmonale congestie, pulmonaal oedeem. Maagdarmstelselaandoeningen. Vaak (≥ 1/100, <1/10): misselijkheid. Soms (≥ 1/1.000, <1/100): droge mond, smaakverandering, diarree, braken, dyspepsie, overmatig speekselvloed, abdominale pijn. Zelden (≥  1/10.000, < 1/1.000): obstipatie, fecale incontinentie, necrotiserende pancreatitis. Huid- en onderhuidaandoeningen. Soms (≥ 1/1.000, <1/100): pruritus, rash, gelaatsoedeem, urticaria, zweten. Bot-, skeletspierstelsel- en bindweefselaandoeningen. Soms (≥ 1/1.000, <1/100): rugpijn, myalgie. Nier- en urinewegaandoeningen. Zelden (≥ 1/10.000, <  1/1.000): urine-incontinentie, pollakisurie. Algemene aandoeningen en toedieningsplaatsstoornissen. Vaak (≥ 1/100, <1/10): reactie op de plaats van injectie, warmte gevoel. Soms (≥ 1/1.000, <1/100): asthenie, koorts, rillingen, pijn op de borst, pijn, pijn op de plaats van injectie, extravasatie op de plaats van injectie. Zelden (≥ 1/10.000, < 1/1.000): ontsteking op de plaats van injectie. Onderzoeken. Soms (≥ 1/1.000, <1/100): afwijkende laboratoriumwaarden, afwijkingen in het ECG, verlengd QT-interval. Afwijkende laboratoriumwaarden zoals hierboven omschreven bevatten hypochrome anemie, leukocytose, leukopenie, basofilie, hypoproteïnemie, hypocalciëmie, hyperkaliëmie, hyperglykemie of hypoglykemie, albuminurie, glucosurie, hematurie, hyperlipidemie, hyperbilirubinemie, toename van serumijzer, en toename van serum transaminasen, alkalische fosfatase, lactaatdehydrogenase en serumcreatinine werden bij minder dan 0,4% van de patiënten gesignaleerd na toediening van MultiHance. Echter, deze bevindingen hadden voor het grootste deel betrekking op patiënten bij wie voordien al sprake was van een verminderde leverfunctie of een bestaande metabole aandoening. Voor het grootste deel waren deze bijwerkingen niet ernstig, van voorbijgaande aard en verdwenen zij spontaan zonder effecten na te laten. Er werd geen verband gezien met leeftijd, geslacht of dosering. Kinderen Bij pediatrische patiënten opgenomen in klinische onderzoeken omvatten de meest gerapporteerde bijwerkingen braken (1,4%), koorts (0,9%) en hyperhydrose (0,9%). De frequentie en de aard van de bijwerkingen waren gelijk aan die bij volwassenen. In minder dan 0,1% van de patiënten zijn, sinds het product op de markt is, bijwerkingen gemeld. De meest voorkomende bijwerkingen waren: misselijkheid, braken, signalen en symptomen van overgevoeligheidsreacties inclusief: anafylactische shock, anafylactoïde reacties, angio-oedeem, larynxspasme en huiduitslag. Reacties op de plaats van injectie als gevolg van extravasatie van het contrastmiddel leidend tot lokale pijn of branderig gevoel, zwelling, blaarvorming en, in zeldzame gevallen wanneer de lokale zwelling ernstig is, tot necrose zijn gemeld. Lokale tromboflebitis werd ook in zeldzame gevallen gemeld. Geïsoleerde gevallen van Nefrogene Systemische Fibrose (NSF) zijn gemeld met MultiHance bij patiënten die gelijktijdig andere gadoliniumhoudende contrastmedia toegediend kregen. HOUDER VAN DE VERGUNNING VOOR HET IN DE HANDEL BRENGEN: Bracco Imaging Deutschland GmbH, Max-Stromeyer-Straße 116, 78467 Konstanz, Duitsland. NUMMERS VAN DE VERGUNNING VOOR HET IN DE HANDEL BRENGEN: MultiHance 5 ml: BE199963, MultiHance 10 ml: BE199972, MultiHance 15 ml: BE199981 MultiHance 20 ml: BE199997. AFLEVERINGSWIJZE: Op medisch voorschrift. DATUM VAN HERZIENING/GOEDKEURING VAN DE TEKST: Datum van herziening van de tekst: 10/2013.

Informatie over de rubrieken Bijzondere waarschuwingen en voorzorgen bij gebruik, Interacties, Vruchtbaarheid, zwangerschap en borstvoeding, Beïnvloeding van de rijvaardigheid en het vermogen om machines te bedienen, Overdosering, Farmacologische eigenschappen en Farmaceutische gegevens kan u vinden in de volledige versie van de Samenvatting van de Productkenmerken.

Pack sizes commercialised in Belgium: Multihance 10 ml, 15 ml and 20 ml - vial.

Lever

Dynamische tomografie: Onmiddellijk na een bolus injectie.

Vertraagde tomografie:Tussen de 40 en 120 minuten na de injectie, afhankelijk van de individuele tomografische behoefte.

Hersenen en ruggenmerg Tot 60 minuten na de toediening.

MRA

Onmiddellijk na toediening, met scan vertraging die op basis van de testbolus of automatische bolus detectie techniek wordt berekend. Indien een automatische contrastdetectie puls-sequentie niet wordt gebruikt voor bolus timing, dan dient een test bolus injectie ≤2 ml van de oplossing gebruikt te worden om de geschikte scan vertraging te berekenen.

BorstT1-gewogen, dynamische acquisitie onmiddellijk na de bolus injectie en opnieuw na 2, 4, 6 en 8 minuten.

MULTIHANCE® 0,5 mmol/ml : Solution injectable en flacon ou en seringue pré-remplie. COMPOSITION QUALITATIVE ET QUANTITATIVE : 1 ml de solution contient : acide gadobénique 334 mg (0,5 M) sous forme de sel dediméglumine. [529 mg de gadobénate de diméglumine = 334 mg d’acide gadobénique + 195 mg de diméglumine]. Pour tous les excipients, voir rubrique Données pharmaceutiques. FORME PHARMACEUTIQUE : Solutioninjectable en flacon ou en seringue pré-remplie. Solution aqueuse limpide, incolore, remplie dans des flacons de verre incolore ou des seringues de plastique transparent. Osmolalité à 37°C : 1,970 Osmol/kg. Viscosité à 37°C :

5,3 mPa.s. pH : 6,9-7,3. DONNEES CLINIQUES : Indications thérapeutiques : Ce médicament est à usage diagnostique uniquement. Flacons : Produit de contraste parama gnétique utilisé dans l’imagerie par résonancemagnétique (IRM) et indiqué dans : • IRM du foie pour la détection des lésions hépatiques lorsqu’un cancer hépatique secondaire ou primitif (carcinome hépatocellulaire) est suspecté ou connu. • IRM du cerveau et de la moelle

épinière où il améliore la détection des lésions et apporte des informations diagnostiques supplémentaires comparativement à une IRM sans produit de contraste. • Angiographie par résonance magnétique (ARM) où il améliore l’exactitudediagnostique pour la détection de la maladie vasculaire sténo-occlusive cliniquement significative lorsqu’une pathologie vasculaire des artères abdominales ou périphériques est suspectée ou connue. Seringues pré-remplies : MultiHance est un produit de contraste paramagnétique, utilisé dansl’imagerie par résonance magnétique (IRM) indiqué dans le cadre de : • L’IRM du foie pour la détection des lésions hépatiques focales chez les patients chez lesquels un cancer hépatique secondaire ou primitif (hépatocarcinome) est suspecté ou connu. • L’IRM du cerveau et de la moelle épinièrecar il améliore la détection des lésions et apporte des informations diagnostiques supplémentaires comparativement à une IRM sans produit de contraste. Posologie et mode d’administration : • IRM du foie (flacon et seringue pré-remplie) : La dose recommandée chez l’adulte est de0,05 mmol/kg de poids corporel, soit 0,1 ml/kg de la solution 0,5 M. • IRM du système nerveux central (flacon et seringue pré-remplie) : La dose recommandée chez l’adulte et l’enfant de plus de 2 ans est de 0,1 mmol/kg de poids corporel, soit 0,2 ml/kg de la solution 0,5 M. • ARM(flacon) : La dose recommandée chez l’adulte est de 0,1 mmol/kg de poids corporel, soit 0,2 ml/kg de la solution 0,5 M. Flacon : MultiHance doit être introduit dans la seringue immédiatement avant l’injection et ne doit pas être dilué. Tout reliquat éventuel doit être jeté et ne doit pas être utilisépour d’autres examens IRM. Seringue pré-remplie : MultiHance doit être utilisé immédiatement après ouverture et ne doit pas être dilué. Tout reliquat éventuel doit être jeté et ne doit pas être utilisé pour d’autres examens IRM. Pour utiliser la seringue, l’extrémité filetée de la tige du piston doitêtre vissée dans le piston dans le sens des aiguilles d’une montre, puis enfoncée de quelques millimètres pour éliminer toute friction entre le piston et le corps de la seringue. Tout en maintenant la seringue droite (capuchon de la canule vers le haut), retirer aseptiquement le capuchon de lacanule de l’extrémité de la seringue et y fixer soit une aiguille jetable, stérile, soit une tubulure 5/6 compatible aux vis Luer en exerçant une poussée rotative. La seringue toujours en position verticale, appuyer sur le piston pour évacuer l’air jusqu’à ce que le liquide perle à l’extrémité de l’aiguilleou que la tubulure soit complètement remplie. L’injection doit être réalisée en respectant la procédure d’aspiration habituelle. Pour diminuer le risque d’extravasation de MultiHance dans les tissus mous environ nants, il est conseillé de s’assurer de la bonne disposition de l’aiguille ou de la canuledans la veine. Foie et système nerveux central : le produit doit être administré par voie intraveineuse, soit en bolus, soit en injection lente (10 ml/min). ARM : le produit doit être administré par voie intraveineuse en bolus, soit manuellement, soit à l’aide d’un injecteur automatique. L’injection soitêtre suivie d’un embol de chlorure de sodium à 0,9 %. Acquisition des images post-contraste : • Foie (flacon et seringue pré-remplie) : Imagerie dynamique : Immédiatement après l’injection en bolus. Imagerie retardée : Entre 40 et 120 minutes après l’injection (IRM retardée), en fonctiondu type d’imagerie nécessaire. • Système nerveux central (flacon et seringue pré-remplie) : Jusqu’à 60 minutes après admi nis tration. • ARM (flacon) : Immédiatement après l’administration, avec un délai d’acquisition calculé sur la base du bolus test ou par la technique de détection automatiquedu bolus. Si la détection automatique du contraste en séquence pulsée n’est pas utilisée, alors l’injection d’un bolus test de 2 ml de produit au maximum devra être réalisée pour calculer le timing d’acquisition adéquat. Populations particulières : Insuffisants rénaux : L’administration deMultiHance doit être évitée chez les patients présentant une insuffisance rénale sévère (DFG < 30 ml/min/1,73 m²) et en période périopératoire de transplantation hépatique, sauf si les informations diagnostiques sont indispensables et ne peuvent être obtenues au moyen d’une IRM sansrehaussement du contraste (voir rubrique Mises en garde spéciales et précautions particulières d’emploi). Si l’administration de MultiHance ne peut être évitée, la dose ne doit pas excéder 0,1 mmol/kg de poids corporel lorsqu’elle est administrée pour une IRM du cerveau et de la moelle épinièreou une ARM et la dose ne doit pas excéder 0,05 mmol/kg de poids corporel lorsqu’elle est administrée pour une IRM du foie. Ne pas administrer plus d’une dose au cours de l’examen. En raison du manque d’information sur les administrations répétées, les injections de MultiHance ne doiventpas être réitérées, sauf si l’intervalle entre les injections est d’au moins 7 jours. Sujets âgés (à partir de 65 ans) : Aucune adaptation posologique n’est nécessaire. Utiliser avec prudence chez les sujets âgés (voir rubrique Mises en garde spéciales et précautions particulières d’emploi). L’utilisationdans l’IRM du système nerveux central n’est pas recommandée chez l’enfant de moins de 2 ans. L’utilisation dans l’IRM du foie et l’ARM n’est pas recommandée chez l’enfant de moins de 18 ans. Contre-indications : MULTIHANCE est contre indiqué chez - les patients présentant unehypersensibilité à l’un de ses constituants. - Les patients ayant des antécédents d'allergie ou d'effets indésirables liés à d'autres chélates de gadolinium. Mises en garde spéciales et précautions particulières d’emploi : Les patients doivent être maintenus sous surveillance étroite durantles 15 minutes suivant l’injection, puisque la majorité des réactions graves surviennent dans cette période. Le patient devra rester dans un environnement médical pendant l’heure suivant l’injection. Les procédures habituelles de sécurité en imagerie par résonance magnétique doivent êtreappliquées lors de l’emploi de MultiHance, en particulier l’exclusion de tout patient porteur de matériel ferromagnétique (pace-makers ou clips vasculaires par exemple). La prudence est recom mandée chez les patients présentant une pathologie cardiovasculaire. L’utilisation de produits de contrastediagnostiques doit être réservée aux établissements dont le personnel est formé à la prise en charge des urgences et dans lesquels le matériel de réanimation cardio-respiratoire est rapidement disponible. De faibles quantités d’alcool benzylique (< 0,2 %) peuvent être relarguées par le gadobénatede diméglumine durant son stockage. En conséquence, MultiHance ne doit pas être administré aux patients présentant des antécédents d’allergie à l’alcool benzylique. Comme avec les autres chélates de gadolinium, un délai de 7 heures devra être respecté avant toute nouvelle IRM avec produitde contraste afin de permettre l’élimination de MultiHance par l’organisme. • Insuffisance rénale : Avant l’administration de MultiHance, des examens de laboratoire afin de rechercher une altération de la fonction rénale sont recommandés chez tous les patients. Des cas de fibrosenéphrogénique systémique (FNS) ont été rapportés après injection de certains produits de contraste contenant du gadolinium chez des patients ayant une insuffisance rénale sévère aiguë ou chronique (clairance de la créatinine < 30 ml/min/1,73 m²). Les patients devant bénéficier d’unetransplantation hépatique sont particulièrement à risque, car l’incidence de l’insuffisance rénale aiguë est élevée dans ce groupe. Etant donné qu’il est possible que des cas de FNS surviennent avec MultiHance, l’administration de ce produit doit être évitée chez les patients présentant uneinsuffisance rénale sévère et chez les patients durant la période pré ou post-opératoire d’une transplantation hépatique, sauf si le diagnostic ne peut être obtenu par d’autres moyens que l’IRM avec injection de gadolinium. La réalisation d’une hémodialyse peu de temps après l’administration deMultiHance pourrait faciliter l’élimination de ce produit de l’organisme. Il n’est pas établi que l’instauration d’une hémodialyse puisse prévenir ou traiter la FNS chez les patients qui ne sont pas déjà hémodialysés. Sujets âgés : L’élimination rénale de gadobénate de diméglumine pouvant êtrealtérée chez les sujets âgés, il est particulièrement important de rechercher un dysfonctionnement rénal chez les sujets âgés de 65 ans et plus. Interactions avec d’autres médicaments et autres formes d’interaction : Au cours du dévelop pement clinique de MultiHance, aucune étudespécifique d’interaction n’a été menée. Cependant, il n’a pas été rapporté d’interaction entre MultiHance et d’autres médicaments durant les essais cliniques. Effets indésirables : Les effets indésirables suivants ont été observés au cours du développement clinique de MultiHance sur 2 637sujets adultes. Aucun effet indésirable de fréquence supérieure à 2 % n’a été rapporté. Fréquent (≥ 1/100 - < 1/10) : • Manifestations neurologiques : céphalées. • Troubles gastro-intestinaux : nausée. • Manifestations générales et au point d’injection : réaction au point d’injection, sensationde chaleur. Peu Fréquent (≥ 1/1.000 - < 1/100) : • Manifestations infectieuses : rhinopharyngite. • Manifestations neurologiques : paresthésies, sensations vertigineuses, syncope, parosmie. • Troubles cardiaques : tachycardie, fibrillation auriculaire, bloc auriculo-ventriculaire du 1er degré, extra-systoles ventriculaires, bradycardie sinusale. • Manifestations vasculaires : hypertension artérielle, hypotension artérielle. • Manifestations respiratoires, thoraciques et médiastinales : rhinite. • Troubles gastro-intestinaux : sécheresse buccale, dysgueusie, diarrhée, vomissement, dyspepsie,hypersialorrhée, douleur abdominale. • Manifestations cutanées et sous-cutanées : prurit, éruption cutanée, œdème de la face, urticaire, hypersudation. • Manifestations musculaires, ostéo-articulaires et du tissu conjonctif : douleurs dorsales, myalgies. • Manifestations générales et au pointd’injection : asthénie, fièvre, frissons, douleur thoracique, douleurs, douleur au point d’injection, extravasation au point d’injection. • Anomalies des examens paracliniques : examens de laboratoire anormaux, ECG anormal, allongement de l’intervalle QT. Rare (≥ 1/10.000 - < 1/1.000) : •Manifestations neurologiques : hyperesthésie, tremblements, hypertension intracrânienne, hémiplégie. • Troubles oculaires : conjonctivite. • Manifestations ORL : acouphènes. • Troubles cardiaques : arythmie, ischémie myocardique, allongement de l’intervalle PR. • Manifestations respiratoires,thoraciques et médiastinales : dyspnée, laryngospasme, sifflements respiratoires, congestion pulmonaire, œdème pulmonaire. • Troubles gastro-intestinaux : constipation, incontinence fécale, pancréatite nécrosante. • Troubles rénaux et manifestations urinaires : incontinence urinaire, mictionsimpérieuses. • Manifestations générales et au point d’injection : inflammation au point d’injection. Les anomalies biologiques citées précédemment observées chez 0,4 % des patients au maximum, après administration de MultiHance sont les suivantes : anémie hypochrome, hyperleucocytose,leucopénie, augmentation des basophiles, hypoprotéinémie, hypocalcémie, hyperkaliémie, hyper- ou hypoglycémie, albuminurie, glycosurie, hématurie, hyperlipidémie, hyperbilirubinémie, augmentation du fer sérique et augmentation des taux sériques des transaminases, des phosphatases alcalines,de la lactico-deshydrogénase et de la créatininémie. Cependant, ces phénomènes ont été essentiel lement observés chez des patients présentant une insuffisance hépatique connue ou une maladie métabolique sous-jacente. Dans la plupart des cas, il s’agissait d’événements non-graves, transitoireset qui ont régressé spontanément sans séquelle. Aucune corrélation n’a pu être établie avec l’âge, le sexe ou la dose administrée. Pédiatrie : Au cours des essais cliniques chez l’enfant, les événements indésirables les plus fréquemment rapportés incluent les vomissements (1,4 %), la fièvre(0,9 %) et l’hyperhidrose (0,9 %). La fréquence et la nature des événements indésirables étaient similaires à celles observées chez l’adulte. Depuis la commercialisation de MultiHance, des effets indésirables ont été rapportés chez moins de 0,1 % des patients. Les plus fréquents sont : nausées,vomissements, manifestations cliniques plus ou moins sévères d’hypersensibilité comprenant : choc anaphylactique, réactions anaphylactoïdes, angioœdème, spasme laryngé et éruption cutanée. Des réactions au point d’injection, consécutives à une extravasation du produit de contraste, entraînantlocalement une douleur ou une sensation de brûlure, voire une tuméfaction ou un décollement cutané, ont également été rapportées. Des cas isolés de fibrose néphrogénique systémique (FNS) ont été rapportés avec MultiHance, chez des patients ayant également reçu d’autres produits decontraste à base de gadolinium (voir chapitre mises en garde spéciales et précautions particulières d’emploi). PROPRIETES PHARMACOLOGIQUES : Produit de contraste paramagnétique code ATC V 08CA08. DONNEES PHARMA CEUTIQUES : Liste des excipients : Eau pour préparationsinjectables. L’étiquette détachable de traçabilité placée sur les flacons/seringues doit être collée dans le dossier du patient afin de permettre un suivi précis du produit de contraste à base de gadolinium administré. La dose administrée doit également être enregistrée. 3400934741128 : 5 ml en flacon (verre), 25,44 € - 3400934741296 : 10 ml en flacon (verre), 42,55 € - 3400934741357 : 15 ml en flacon (verre), 60,93 € - 3400934741418 : 20 ml en flacon (verre), 77,11 €- 3400938879667 : 10 ml de solution en seringue préremplie, 42,55 € -3400938879728 : 15 ml de solution en seringue préremplie, 60,93 € - 3400938879896: 20 ml de solution en seringue préremplie, 77,11 € - Liste I - Remb. Séc. Soc. 65 % dans toutes les indications de l’AMM (flacons : foie, SNC et ARM - seringue pré-remplie : foie, SNC). Agréé Coll. Datede révision de la monographie : Octobre 2012. Pour une information complète, se référer au dictionnaire Vidal. Titulaire de l’AMM et exploitant : Bracco Imaging France - 7, place Copernic - Courcouronnes - 91023 Evry Cedex.

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Le service commercial dispose de moyens informatiques destinés à gérer plus facilement la relation client. Les informations enregistrées sont réservées à l’usage du service concerné et ne peuvent être communiquées qu’aux services des affaires pharmaceutiques et financiers. Conformément aux articles 39 et suivants de la loi n° 78-17 du 6 janvier 1978 relative àl’informatique, aux fichiers et aux libertés, toute personne peut obtenir communication et, le cas échéant, rectification ou suppression des informations la concernant, en s’adressant au pharmacien responsable de notre société. Vous avez reçu un délégué Bracco Imaging France, conformément aux dispositions de la charte de la visite médicale, vous pouvez nous faire partde votre appréciation de cette visite, notamment sur sa qualité scientifique, par courrier à l’attention du pharmacien responsable.

1- F.M. Cavagna, et al. Gadolinium chelates with Weak Binding to Serum Proteins. A New Class of High Efficiency, General Purpose Contrast Agents for Magnetic Resonance Imaging. Invest Radiol 1997; 32: 780-796. 2- C. de Haën, et al. Gadobenate Dimeglumine 0,5 M Solution for Injection (MultiHance®) : Pharmaceutical Formulationand Physicochemical Properties of a New Magnetic Resonance Imaging Contrast Medium. JCAT 1999; 23 (Suppl. 1): S161-S168. 3- Source Vidal. 4- J.M. Idée, et al. Clinical and biological consequences of transmetallation induced by contrast agents for magnetic resonance imaging: a review. Fundam and clin pharmacol 2006; 20:563-576- Erratum in: Fundam Clin Pharmacol. 2007 Jun; 21(3): 335. 5- K.R. Maravilla, et al. Contrast enhancement of central nervous system lesions: multicenter intraindividual crossover comparative study of two contrast agents. Radiology 2006, 240: 389-400. 6- M. Essig, et al. Enhancing lesions of the brain: intraindividualcrossover comparison of contrast enhancement after gadobenate dimeglumine versus established gadolinium comparators. Acad. Radiol. 2006; 13: 744-751. 7- J. Pintaske et al. Relaxivity of gadopentetate dimeglumine (Magnevist), gadobutrol (gadovist) et gadobenate dimeglumine (MultiHance) in human blood plasma at 0,2, 1,5and 3 Tesla. Invest Radiol 2006; 41: 213-221 et erratum. 8- Giesel et al.Influence of human serum albumine on longitudinal and tranverse relaxation (R1 and R2) of magnetic contrast agents. Invest Radiol 2006; 41: 222-228.

Multichance_bijsluiter_BE.indd 1 7/03/14 18:39

MR Angiography with MultiHance® :

• �MultiHance®� is� also� indicated� for� Contrast-enhanced� MR-angiography� where� it� improves�the� diagnostic� accuracy� for� detecting� clinically� significant� steno-occlusive� vascular� disease� in�patients�with�suspected�or�known�vascular�disease�of�the�abdominal�or�peripheral�arteries.(1)

• The� recommended�dose�of�MultiHance®� injection� in� adult� patients� is� 0.1�mmol/kg�body�weight.� This�corresponds�to�0.2�mL/kg�of�the�0.5�M�solution.(1)

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Multihance_advertentie.indd 1 12/03/14 19:58

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02-Instr.indd 2 19/02/15 16:50

JBR–BTR, 2015, 98: 1.

A LETTER FROM THE BSR PRESIDENT

JBR-BTR, a new start

Dear colleagues,

This first issue of the 98th volume of our Journal is a very special one. It may well become a collector’s item, because it is the last issue that will be distributed in print version. The JBR-BTR hence concludes a long history of radiological publishing, founded in 1907 and spanning more than 100 years, interrupted only by the two world wars.

This by no means implies the disappearance of JBR-BTR! In fact, the Journal is very alive and remains one of the keystones of our Society life. As it proudly announces in each issue, it is essentially a journal devoted to diagnostic and interventional imaging, related imaging sciences and continuing education. Hence, it constitutes an indispensable forum for residents and young investigators, but equally for certified and settled radiologists and their colleagues.

In keeping with contemporary technical developments, the BSR Board has taken up the challenge to migrate the Journal to a new and appealing open-access electronic platform, opening unprecedented opportunities for easy manuscript submission, editing, peer-review, publishing and archiving. All published articles will be freely available online in an attractive browsable format, with all necessary features for easy searching and retrieval. With this innovation, the BSR has chosen the path of a proficient and cost-efficient use of its resources.

A special tribute to our eminent Editor-in-Chief, Professor Jacques Pringot, is in order. For many years, Professor Pringot has been a highly devoted, trustworthy and persistent Editor-in-Chief, without whom the Journal would not have reached its current standards. The BSR is therefore immensely pleased that Professor Pringot accepted to continue his efforts as Editor-in-Chief of our refurbished Journal. A special word of thanks also goes to Dr. Patrick Seynaeve who as Managing Editor kept the financial status of our Journal in good health, and to Dr. Piet Vanhoenacker who facilitated the smooth transition of our Journal to its digital platform.

Farewell JBR-BTR after many years of high-quality publishing!

And welcome back JBR-BTR (http://www.bsr-web.be/Journals), our anchor in the digital era!

Geert VilleirsPresident of the Belgian Society of Radiology (BSR)

00-editorial.indd 1 19/02/15 15:37

00-editorial.indd 2 19/02/15 15:37

Connective tissue diseases (CTDs) are a heterogeneous group of in-flammatory diseases derived from an immunologic deregulation affect-ing various organs. A thoracic in-volvement (pulmonary, pleural or mediastinal) can be frequently found; its frequency and expression depends on the type of disease, and may induce an extremely wide range of abnormalities (1-3).

All CTDs can present a pulmonary involvement: rheumatoid arthritis (RA), progressive systemic sclerosis (PSS), systemic lupus erythemato-sus (SLE), polymyositis and derma-tomyositis (PM/DM), mixed connec-tive tissue disease (MCTD), Sjögren syndrome (SJOS), and relapsing polychondritis (RP). The identifica-tion of a pulmonary involvement at its initial stage is crucial, as an early treatment can often improve pa-tients’ prognosis. Interstitial lung disease and pulmonary arterial hy-pertension, both significantly affect-ing morbidity and mortality in these patients, represent the two most clinically relevant thoracic manifes-tations (1, 4).

Interstitial lung disease (ILD)

The most common CTDs associ-ated with ILD are RA, PSS, SLE, PM/DM, MCTD and SJOS. ILD may precede the clinical and laboratory manifestations of CTDs for several months or years, and be present to-gether with systemic manifestations

chronic inflammation in the alveolar walls. The patients usually response well to corticosteroid therapy and have a good prognosis. However pa-tients with OP associated with CTD seem to have a greater tendency to develop fibrosis and a higher mortal-ity than patients with cryptogenic OP (5, 6). The CTD, typically associ-ated with LIP, is SJOS; however LIP may also occasionally be seen in SLE and RA (Table I).

Patients with CTD and chronic ILD similar to those with idiopathic ILD, may develop acute exacerbation. Such acute exacerbation is most common in chronic ILD associated with RA but may be seen in PSS, PM/DM and SJOS. Despite intensive an-ti-inflammatory immunosuppressive therapy, the prognosis of acute exac-erbation of CTD associated with ILD is poor, with high mortality rate (5). Occasionally, patients with CTD without evidence of prior ILD may present with acute respiratory dis-tress syndrome due to DAD (8). This pattern may be the initial manifesta-tion of lung involvement in these pa-tients with rapid progression to re-spiratory failure. This presentation has been described most commonly in patients with SLE and PM/DM.

CT is commonly used in the initial evaluation and follow-up of patients with CTD and clinically suspected or proven ILD. The CT findings of chronic ILD seen in patients with CTD are similar to those seen in idio-pathic interstitial pneumonia (9). In the context of ILD occurring in a pa-tient having a CTD, CT is particularly helpful at it often shows more than one pulmonary disease. Diagnostic precision in CTD-ILD is challenging because of a wide range of potential simultaneous pathologies and the possibility of concurrent CTD (i.e.

at the time of diagnosis of CTD, or more commonly manifest later in the course of the disease (5, 6).

The most common histopatholog-ic patterns of ILD seen in the setting of CTDs are non specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneu-monia (OP), diffuse alveolar damage (DAD), and lymphocytic interstitial pneumonia (LIP).

NSIP is the most common histo-pathologic and high resolution CT pattern of ILD seen in patients with CTDs, particularly PSS, PM/DM and MCTD. It is characterized histologi-cally by relatively homogeneous ex-pansion of the alveolar walls by in-flammation, fibrosis or both. UIP is the second most commonly pattern of chronic ILD and seen most fre-quently in patients with PSS and RA. The pathologic findings of UIP con-sist of patchy heterogeneous pattern with foci of normal lung, interstitial inflammation, fibrosis and honey-combing. Fibroblastic foci are pres-ent but less extensive in CTD than in idiopathic pulmonary fibrosis, and this feature probably accounts for the better prognosis in these pa-tients (5, 7).

The most common CTD associat-ed with OP is PM/DM. OP also occurs in increased frequency in RA and has been described in SLE and SJOS. The histologic pattern of OP is made of intraluminal plugs of granulation tissue within alveolar ducts and sur-rounding alveoli associated with

JBR–BTR, 2015, 98: 3-19.

PICTORIAL REVIEW

ThORACIC InVOLVEmEnT In COnnECTIVE TIssuE DIsEAsEs: RADIO-LOgICAL PATTERns AnD fOLLOW-uPG. Serra1, A.-L. Brun1, P. Ialongo2, M.-L. Chabi1, P.A. Grenier1

Connective tissue diseases (CTDs) are a heterogeneous group of idiopathic inflammatory diseases involving various organs. A thoracic involvement is frequent, and chest-CT represents the imaging technique of reference in its assess-ment. Pulmonary abnormalities related to CTDs are various; although several disease-specific aspects have been described, the two most clinically relevant complications are represented by interstitial lung disease and pulmonary arterial hypertension. The early identification of a thoracic involvement, with the adoption of specific therapies, can significantly change patient’s prognosis. The aim of this article is to review the most common typical and atypical CT features of thoracic involvement occurring in CT, especially focusing on interstitial lung disease.

Key-word: Connective tissue, diseases – Lung, interstitial disease – hypertension, pulmonary.

From: 1. Service de Radiologie Polyvalente et Oncologique – Groupe Hospitalier Pitié-Salpêtrière, Charles Foix, APHP Université Pierre et Marie Curie, Paris, France et 2. Service de Radiologie, Hôpital San Camillo-Forlanini, Rome, Italy.Address for correspondence: Pr Ph. A. Grenier, Service de Radiologie Polyvalente et Oncologique - Groupe Hospitalier Pitié-Salpêtrière, Charles Foix, APHP, 83, Bld de l’Hôpital, 75651 Paris, France. E-mail: philippe.grenier@psl.aphp.fr

serra-.indd 3 19/02/15 15:50

4 JBR–BTR, 2015, 98 (1)

sia of bronchus-associated lymphoid tissue (BALT) with the follicles dis-tributed along the bronchioles. Fol-licular bronchiolitis is part of the spectrum of lymphoproliferative dis-ease and may be sometimes overlap with LIP. Most cases of follicular bronchiolitis or lymphocytic bronchi-olitis are associated with CTD, espe-cially RA, SJOS and PSS.

Bronchial inflammation (follicular bronchitis) may result in bronchiec-tasis unrelated to interstitial lung fi-brosis. Bronchiectasis and/or associ-ated obliterative bronchiolitis occur relatively frequently in patients with RA, SJOS and SLE.

Airway wall thickening and lumi-nal narrowing due to inflammatory and fibrotic changes in cartilage are characteristic of RP.

Alveolar hemorrhage due to capil-laritis is a well-known complication of SLE, and may also occur in pa-tients with RA, PSS, PM/DM or MCTD.

Other extrapulmonary thoracic abnormalities, such as esophageal dilatation, pleuro-pericardial effu-sion/thickening or an osteoarticular involvement can suggest an underly-ing CTD. Mediastinal lymphadenop-athies are another frequent finding in this group of patients, with or without the association with ILD.

Rheumatoid Arthritis (RA)

RA is a relatively frequent disease (1-2% of the adult population world-

Pulmonary arterial hypertension (PAh)

PAH is defined as mean resting pulmonary artery pressure higher or equal to 25 mmHg and a pulmonary capillary wedge pressure lower than 15 mmHg. Patients with CTD present a higher risk to develop PAH, with or without the association with pulmo-nary interstitial involvement (1, 4). Clinical presentation, symptoms and therapeutic approach are the same of those of a primary pulmonary hy-pertension. PSS and MCTD are the two entities more frequently associ-ated with PAH (12); it is less frequent in case of SLE and unusual in pa-tients with RA and PM/DM (Table I). The physiopathologic process ex-plaining the development of pulmo-nary hypertension in patients with CTDs has not been completely clari-fied yet (13). The histopathologic features resemble those of a primary pulmonary hypertension. A throm-boembolic origin could be suggest-ed in patients with SLE with an-tiphospholipid syndrome. Screening echocardiography is indicated in all patients affected by PSS or MCTD. In selected cases, right catheterism (gold standard) is necessary for a de-finitive diagnosis.

Other thoracic manifestations (3, 4)

Follicular bronchiolitis is charac-terized histologically by a peribron-chial lymphocytic follicular hyperpla-

rheumatoid arthritis and Sjögren syndrome). In addition further pos-sible confounders include the pres-ence of smoking or drug-related ab-normalities and immunosuppression related infection (1). Actually ILD, particularly NSIP, OP and DAD may also be a reaction pattern to many drugs. Because most patients with CTD are treated with anti-inflam-matory or immunosuppressive med-ication, drug-induced lung disease should always be considered in the differential diagnosis, or a potential cause of the lung abnormalities (10). Patients with drug-induced pulmo-nary toxicity usually present sub-acute clinical symptoms (fever, dys-pnea, cough) progressing over many weeks. The identification of a drug-induced pulmonary involvement is based on an exclusion diagnosis: pulmonary infection or acute exacer-bation of interstitial pneumonia should always be taken into first con-sideration (5).

Because patients with CTD are treated with steroids or other immu-nosuppressive drugs, they are at risk of bacterial pneumonia and opportu-nistic infections. The prevalence of pneumocystis Jiroveci pneumonia seems to be particularly increased in patients with CTD and ILD who are being treated with corticoste-roids (11). As a result, pneumocystis Jiroveci pneumonia should be the differential diagnosis of new exten-sive ground glass opacity on CT in these patients.

Table I. — Thoracic manifestations of connective tissue diseases.

RA PSS SLE PM/DM MCTD SJOS RPUIP +++ + ++ + ++ +NSIP ++ +++ ++ ++ +++ ++OP + + + +++ +DAD + + ++ ++ +Follicular bronchiolitis/LIP + + + +++Alveolar hemorrhage + + +++ +Aspiration pneumonia ++ ++ +Tracheobronchial WT/Stenosis +++Bronchiectasis ++ + ++ +Obliterative bronchiolitis ++ + ++Nodules ++PAH + +++ + + ++Pleural effusion ++ + ++Diaphragm dysfunction + ++ +UIP Usual Interstitial Pneumonia RA Rhumatoid ArthritisNSIP Non Specific Interstitial Pneumonia PSS Progressive Systemic SclerosisOP Organizing pneumonia SLE Systemic Lupus ErythomatosusDAD Diffuse Alveolar Damage PM/DM Polymyositis/DermatomyositisLIP Lymphocytic Interstitial Pneumonia MCTD Mixed Connective Tissue DiseaseWT Wall thickening SJOS Sjögren’s syndromePAH Pulmonary Arterial Hypertension RP Relapsing Polychondritis

serra-.indd 4 19/02/15 15:50

THORACIC INVOLVEMENT IN CONNECTIVE TISSUE DISEASES — SERRA et al 5

wide). Its incidence is higher in wom-en (age: 25-50 years). It is character-ized by bilateral and symmetric arthritis, morning stiffness, and the presence of rheumatoid factor. Extra-articular manifestations are observed in half of the patients and occur more frequently in men. Pul-mo nary involvement is the second cause of death after infections. By contrast, in individuals with no symptoms of respiratory disease, up to half have lung parenchymal ab-normalities on CT. Cigarette smok-ing has a synergistic effect on the pulmonary manifestations of RA, and smoking is the most consistent independent predictor of ILD in RA.

Radiological manifestations of dis-ease (1, 4, 14)

Pleural thickening (probably secondary to previous effusions) represents the most frequent thoracic abnormality. Effusions are less frequent, usually unilateral, containing small amounts of liquid and often self-resolving over a period of weeks to months; in some patients they can persist over years.

Rheumatoid pulmonary nodules (necrobiotic nodules) are more fre-quently observed in smokers (men > women), in association with sub-cutaneous nodules and significant blood elevation of the rheumatoid factor. Rheumatoid nodules can also appear before the clinical manifesta-tions of disease and their histologi-cal characteristics (fibrinoid necro-sis) resemble those of the sub- cutaneous nodules. Their diameter

Fig. 2. — Typical CT pattern of UIP in a patient with RA.Axial and coronal reformat show bilateral subpleural honeycombing in the lower lobes. Associated findings include ground-glass

opacities with superimposed intralobular reticulations and traction bronchiectasis.

Fig. 1. — Rheumatoid nodules in a 50 y.o. female smoker patient who have suffered from RA for many years.

Axial CT images show the presence of bilateral pulmonary nodules with irregular and spiculated contours predominantly distributed in the upper lung zones. Some of these nodules are cavitated.

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ranges between 0.5 and 5 cm and they usually present a peripheral dis-tribution, with middle and upper pre-dominance (Fig. 1). These nodules, usually asymptomatic, can cavitate (50-100% of cases), grow in dimen-sions or spontaneously disappear over time. Rarely, a communication with the pleural surface and cavitat-ed nodules can occur, causing pneu-mothorax, pleural effusions or em pyemas. Because of drug-related immunosuppression, cavitated nod-ules may become infected. Inner cal-cifications are uncommon; the pres-ence of calcifications has been associated with the Caplan-Colinet syndrome (association between rheumatoid polyarthritis and pneu-moconiosis).

The most common fibrotic ILD in RA are UIP and NSIP. Unlike other CTDs, UIP is more frequent than NSIP in patients with RA.

Fig. 3. — CT pattern of fibrotic NSIP in a 48 y.o. female patient RA.Axial CT images and coronal reformatted slab on which minimal intensity projection was applied, show bilateral patchy areas of

ground-glass opacities with superimposed intralobular reticulations and traction bronchiectasis. The abnormal areas have a pre-dominant peribronchovascular distribution.

Fig. 4. — Follicular bronchiolitis in a patient with RA.Axial CT image showing multiple small ill-defined centrilobu-

lar nodular opacities with diffuse and homogeneous distribu-tion.

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ground-glass opacities, poorly de-fined centrilobular nodules, inter-lobular septal thickening and lung cysts. However most of the cases of LIP are associated with SJOS rather than RA.

There is a strong association between the duration of RA and air-way disease, with obliterative bron-chiolitis and bronchiectasis frequent-ly coexisting (15) (Fig. 5).

Bronchiectasis may be related to autoimmune bronchial destruction, smoking or infection (16). Oblitera-tive bronchiolitis is subclinical in many patients with RA. The charac-teristics HRCT findings consist of ar-eas of decreased lung attenuation and vascularity with redistribution of blood flow to more normal lung resulting in a mosaic perfusion attenuation pattern with expiratory air trapping.

About 20% of patients with RA have mild PAH because of pulmo-nary vasculopathy affecting the smaller vessels. In patients with RA, PAH may also occur secondary to ILD or cardiac disease.

Radiological manifestations of complications

Amyloidosis can be difficult to identify on CT because the appear-ances are not specific. Irregular or amorphous calcifications are occa-sionally identifiable within large mediastinal or pulmonary amyloid deposits.

Several drugs employed in the medical treatment of RA can cause pulmonary toxicity: gold salts and

distortion may be present in case of advanced fibrosis (Fig. 3).

Other lung diseases that may occur in patients with RA include OP and follicular bronchiolitis. OP is usually seen in the middle or lower zones, frequently in a peribroncho-vascular or peripheral distribution. The CT pattern of OP is made of bilateral areas of airspace consolida-tion more or less associated with air bronchogram and some areas of ground-glass opacities.

CT signs of follicular bronchiolitis include centrilobular and peribron-chial nodules measuring 1 to 12 mm in diameter more or less associated with patchy areas of ground-glass opacities that are generally bilateral and diffuse in distribution (Fig. 4). The CT characteristics of LIP include

The characteristic CT features of UIP are intralobular reticulations associated with honeycombing in a peripheral and basal distribution (Fig. 2). The disease tends to creep anteriorly and subpleuraly in the upper lobes. Traction bronchiectasis and bronchiolectasis are frequently associated. Ground-glass opacities may be observed, but with less ex-tent than NSIP. In advanced disease, architectural distortion and volume loss are present.

The CT pattern of NSIP is made of ground-glass opacities with underly-ing distortion and fine reticulation which may or may not be subpleural and basal. Traction bronchiectasis and traction bronchiolectasis may appear in case of fibrotic NSIP, and, a loss of volume and architectural

Fig. 5. — 47 y.o. male patient with RA who has presented with cough and chronic sputum for several years.

Axial CT image showing bilateral cylindrical bronchiectasis in the lower lobes. Some foci of small centrilobular nodular and linear branching opacities (tree-in-bud sign) are also seen in the lower lobes, reflecting chronic infectious bronchiolitis.

Fig. 6. — NSIP pattern in a 33 y.o. female patient with PSS.Axial CT image showing diffuse bilateral ground-glass opaci-

ties. Note the minimal bilateral peripheral reticulation and dila-tion of the esophagus.

Fig. 7. — Typical appearance of fibrotic NSIP in a patient with PSS.

Bilateral traction bronchiectasis superimposed to ground-glass opacities predominant in the lower lung zones. Note the sparing of the subpleural areas of lung parenchyma, and the dilatation of the esophagus due to esophageal dysmotility.

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main pulmonary manifestations are interstitial fibrosis and PAH (1).

Interstitial pulmonary fibrosis re-presents the most frequent thoracic manifestation (50% of patients) and it is associated to the presence of anti-topoisomerase antibodies (anti-Scl-70) (18). The majority of patients with pulmonary lung fibrosis and PSS have a histological pattern of NSIP rather than UIP (19).

PAH represents the most frequent cause of death. It may occur in pa-tients with very restrictive lung dis-ease at pulmonary function tests and is probably secondary to the pres-ence of ILD or heart disease. Isolated PAH may occur in patients with PSS, particularly those with limited form (PAH occurs in 50% of patients with CREST) (1).

Radiological manifestations of dis-ease

The main CT features are those of fibrotic or non fibrotic NSIP includ-ing intralobular reticulations super-imposed on ground-glass opacities (Fig. 6); this is potentially associated with traction bronchiectasis and bronchiolectasis (Fig. 7). In case of advanced fibrosis a volume loss and architectural distortion can also be observed. A subpleural sparing of the parenchyma is often present (5, 20, 21) (Fig. 7). Less frequently, foci of airspace consolidation, pulmo-nary cysts or rare honeycombing foci can be found (Fig. 8).

CT signs of PAH are primarily given by an enlarged caliber of the pulmonary trunk (> 29 mm), a dilata-tion of the right and left pulmonary arteries and their segmental branch-es. A diameter of the pulmonary

ogy, characterized by three patho-logical features: inflammation, vascular damage and fibrosis. It is a rare disease more frequent in wom-en with a peak incidence between 45 and 64 years. Only 1% of the patients with PSS present respiratory symp-toms at time of diagnosis, but about 60% of them will develop a pulmo-nary involvement.

CREST syndrome, a limited form of systemic sclerosis, is character-ized by five main clinical features: calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodacty-ly and telangiectasia. Lung involve-ment occurs more frequently in the diffuse forms of PSS than in limited forms (CREST syndrome). The two

penicillamine can possibly cause DAD or obliterative bronchiolitis; also methotrexate can frequently deter-mine pulmonary damage with pneu-monia (17). The most frequent CT features of a drug-induced pulmo-nary toxicity are multiple ground-glass opacities, centrilobular nodules and mediastinal lympha de no pathies (10, 17). A complete resolution can often be achieved by the interruption of treatment and administration of high doses of corticosteroids.

Progressive systemic sclerosis (Pss)

PSS is a multisystemic chronic au-toimmune disease of unknown etiol-

Fig. 8. — Typical CT pattern of UIP occurring in a patient with PSS.Bibasal and subpleural honeycombing (left). Note the dilatation of pulmonary arteries reflecting pulmonary arterial hypertension

(PAH) (pulmonary artery/ascending aorta diameters ratio is > to 1) (right). Dilatation of the esophagus due to esophageal dysmotility is also present.

Fig. 9. — Pulmonary infection in a patient with SLE presenting with fever, dyspnea and chest pain.

Post-contrast CT scan showing bilateral pleural effusion and airspace consolidation with air bronchogram in the right middle, lingula, and lower lobes. Note a round area of hypoattenuation seen within the right lower lobe (arrow) corresponding to a lung abscess.

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THORACIC INVOLVEMENT IN CONNECTIVE TISSUE DISEASES — SERRA et al 9

Fig. 10. — A 38 y.o. male patient with SLE.Axial CT images showing bilateral patchy areas of ground-glass opacity with superimposed reticulation and traction bronchiecta-

sis suggesting fibrotic NSIP.

Fig. 11. — A 27 y.o female patient with SLE presenting with dyspnea. Absence of infection.A,B. Axial CT images (left) showing patchy areas of airspace consolidations associated with some areas of ground-glass opacity

suggestive of OP pattern.C,D. CT scan performed 2 years later (right). In spite of corticosteroid and immuno-suppressive treatment there is persistence of

ILD. Airspace consolidation was replaced by ground-glass opacities, linear opacities and small cysts suggestive of lung fibrosis.

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nary embolism is a consideration, particularly in patients who have an-tiphospholipid syndrome (1).

Lower respiratory tract infections are caused by both common and opportunistic pathogens because patients are frequently immuno-suppressed and have altered cellular immunity.

The shrinking lung syndrome is characterized by pulmonary volume loss causing dyspnea and pleural pain, associated with a restrictive syndrome at pulmonary function tests (24). Respiratory muscle weak-nesses, diaphragmatic neuropathy and pleural inflammation might play a role in the genesis of this syndrome which usually shows a good re-sponse to medical treatment with good prognosis.

Acute Lupus pneumonitis is a rare and severe complication (mortality 50%) caused by a DAD with necrosis, cellular infiltrations, hyaline mem-branes deposition, capillary inflam-mation and hemorrhage (not always present). It is clinically characterized by fever, dyspnea, cough, hypox-emia and pleural pain (22).

Interstitial pneumonia is an un-usual complication, most frequently presenting as NSIP (22); SLE-associ-ated NSIP shows no response to medical treatment.

More frequent in patients with antiphospholipid antibodies, pulmo-

with PSS have been reported. On CT scans only non specific appearances are present (widespread consolida-tion and ground-glass opacities).

Patients with PSS show a higher risk of developing lung cancer (usu-ally adenocarcinoma) including non smokers.

systemic Lupus Erythematosus (sLE)

SLE is a systemic autoimmune disease more common in women in reproductive age principally affect-ing skin, articulations, kidneys, blood cells and the nervous system. SLE tissular lesions derive from an im-mune-complex deposition with com-plement activation. The presence of anti-DNA and anti-smooth muscle antibodies is highly specific for SLE. A thoracic involvement is observed in a high percentage of patients (50-100%), most frequently represented by pleural disease and infectious pneumonia (22, 23). Most of the patients with SLE usually present minor respiratory symptoms or no symptoms at all.

Pulmonary involvement is not necessary associated with signifi-cant morbidity and may be asymp-tomatic. Pleural effusions are usually small, bilateral, protein-rich exu-dates. Although pleuritic symptoms may express active lupus, pulmo-

trunk being greater than that of the adjacent ascending aorta has proven to be a useful CT sign of PAH (Fig. 8). In advanced cases, a dilatation of the right cardiac cavities and the azygos and hemiazygos venous systems can be associated. Presence of con-trast media reflux in the inferior vena cava and the hepatic veins repre-sents another relevant radiological sign of right heart dysfunction. Peri-cardial effusion, an indirect sign of insufficient lymphatic and venous drainage (secondary to right heart high blood pressures), can be ob-served in severe cases, as indicator of poor prognosis. However a non dilated pulmonary artery does not necessary exclude PAH. Echocar-diography is useful to screen for pul-monary hypertension (12).

An esophageal involvement is very common in patients with PSS (97% of cases), presenting with a lumen dilatation (Figs. 7, 8) and/or motility disorders, which can be the cause of inhalation pneumonia or bronchiolitis. CT scan will show mucus plugs, “tree-in-bud” patterns, centrilobular nodules, lobular or perilobular airspace consolidations and bronchiectasis (4).

A pleural involvement is rare, seen as pleural pseudoplaques or diffuse pleural thickening.

Few isolated cases of DAD and dif-fuse alveolar hemorrhage in patients

Fig. 12. — Airway disease occurring in a 37 y.o. female patient with SLE.

Axial CT image showing bilateral cylindrical bronchiectasis in the lower lobes, associated with obliterative bronchiolitis (de-creased lung attenuation and vascularity).

Fig. 13. — Diffuse pulmonary hemorrhage occurring in a 42 y.o. male patient with SLE and antiphospholipid syndrome.

Axial CT image showing bilateral patchy areas of ground-glass opacities and airspace consolidation.

→Fig. 14. — Severe PAH in a 28 y.o. female patient with SLE.A. Post-contrast CT scan (axial and sagittal MIP reformatted images) showing dilatation of the trunk and main branches of the

pulmonary artery without any arterial thrombosis.B. Coronal reformatted CT images, targeted on the right lung, showing the presence of small ill-defined centrilobular nodular

opacities (arrows).

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A

B

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frequent in women (40-50 y.o.). Typi-cal features are subacute onset, proximal symmetric muscle weak-ness, elevated serum creatine kinase activity, and mononuclear cell infil-trates in the muscle biopsy. In addi-tion patients with DM have charac-teristics skin abnormalities. PM/DM occur isolated or in connection with another CTD.

Since the results of auto-antibod-ies were available, patients with idio-pathic inflammatory myopathies have been classified according to the clinico-serologic classification. This includes pure polymyositis, pure dermatomyositis, overlap myositis, and cancer associated myositis (26). Overlap myositis is defined as myo-sitis with at least one clinical overlap feature and/or an overlap auto-anti-body. Cancer associated myositis is defined by the presence of clinical paraneoplastic features and without an overlap auto-antibody or anti-MI-2.

ILD is frequent in overlap myositis and seems to be associated with the presence of autoantibodies tRNA synthetase. The antisynthetase syn-drome features include arthritis, ILD, fever, Raynaud’s phenomenon, and mechanic hands. ILD is particularly common in the antisynthetase syn-drome occurring up to 80%. Anti-JO-1 is the most common overlap autoantibody. Antibodies to other synthetases also exist (anti-PL-7, anti-PL-12, anti-OJ, anti-KU) which seem to preferentially identified indi-viduals with amyopathic lung dis-ease. Antisynthetase predicts more severe ILD, and ILD progression is associated with acute onset (27). In addition, pulmonary involvement

adenopathies, d) pleuropericardial abnormalities and pleural irregulari-ties. In case of diffuse alveolar hem-orrhage, the CT appearances are not specific, made of widespread ground-glass opacities and airspace consolidation (Fig. 13). The CT fea-tures of diffuse alveolar hemorrhage may be indistinguishable from those of infectious pneumonia or DAD (5). In acute lupus pneumonitis, exten-sive ground-glass opacities and con-fluent airspace consolidations are associated with air bronchogram and pleural effusion. These radiolog-ical features may be indistinguish-able from other causes of bilateral airspace consolidation that may complicate SLE (infection, alveolar hemorrhage, or OP).

In case of shrinking lung syn-drome CT scans or chest radiographs can show unilateral or bilateral dia-phragmatic elevation with or without parenchymal involvement, made of passive atelectasis in the subpleural area along the diaphragm and asso-ciated with some parenchymal bands in the lung bases (24).

When PAH is present CT scan may show small ill-defined centrilobular nodules varied in numbers and con-spicuity and having no lung zone predominance (Fig. 14). These small nodular opacities may reflect the presence of cholesterol granulomas resulting from pulmonary hemor-rhage (25).

Polymyositis and dermatomyositis (Pm/Dm)

PM and DM are idiopathic inflam-matory myopathies with an autoim-mune pathogenesis. They are more

nary embolism is a complication possibly leading to chronic pulmo-nary thromboembolic disease. Pul-monary arterial hypertension (PAH) can be secondary to thrombosis, ILD, valvular disease, or may occasional-ly be primary and results from unex-plained plexiform lesions in the pre-capillary vasculature (22).

Diffuse alveolar hemorrhage is a rare and severe complication (2-5% of cases), not always presenting with hemoptysis, associated with a high rate of mortality.

Radiological manifestations of dis-ease

The most common CT abnormali-ties are bilateral pleural effusion, pleural thickening, pericardial effu-sion and infectious pneumonia (Fig. 9). Infectious pneumonia can be community-acquired pneumonias or be caused by atypical germs, such as mycobacteria, pneumocystis j., CMV, aspergillus or nocardia. Pulmonary tuberculosis should always be screened during high-dose cortico-steroids and immunosuppressive drugs administration.

Several CT abnormalities may be observed in patients with SLE who did not have respiratory symp-toms (22, 23). They include a) CT findings of ILD, more often taking the appearance of fibrotic or non-fibrotic NSIP, and less frequently those of UIP or OP (Fig. 10, 11); b) bronchiec-tasis made mainly of mild dilatation, more or less associated with obliter-ative bronchiolitis (decreased lung attenuation, decreased vascularity and expiratory air trapping) (Fig. 12); c) axillary and mediastinal lymph-

Fig. 15. — Association of OP and NSIP in a 40 y.o. female patient with dermatomyositis.A. Bilateral subpleural and perilobar areas of airspace consolidation and ground-glass opacities.B. Follow-up CT scan performed 18 months later showing the disappearance of airspace consolidation, replaced by subtle ground-

glass opacities in the periphery of the lungs.

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THORACIC INVOLVEMENT IN CONNECTIVE TISSUE DISEASES — SERRA et al 13

in patients with antisynthetase syn-drome (anti JO1) predicts disease-modifying antirheumatic drug use (28).

The main clinical manifestations of pulmonary involvement in PM/DM are represented by hypoventilation with secondary respiratory insuffi-ciency, which can be determined by respiratory muscles functional de-fects, ILD or aspiration pneumonia or diffuse bronchiolitis (caused by pha-ryngeal muscles dysfunction) (29).

The presence of an interstitial in-volvement significantly affects the morbidity and mortality of patients with PM/DM. Its onset can precede the appearance of cutaneous and muscular abnormalities in 20-50% of cases; otherwise, it can be identified at time of diagnosis or during follow-up (30). The most common patho-logic findings include NSIP and OP often occurring in combination (31). A rapidly progressive form of lung disease may occur, reflecting DAD.

Radiological manifestations of the disease

The characteristic CT appearance consists of patchy bilateral airspace consolidation which has a subpleu-ral and/or peribronchial distribution in the majority of cases (Fig. 15). It most commonly involves the lower lung zones to a greater degree than the upper lung zones. This pattern reflects the presence of OP (5). Poor-ly defined band-like opacities that have an arcade-like or polygonal ap-pearance (perilobular pattern) are also common. Other findings include

Fig. 17. — UIP pattern in a 57 y.o. female patient with anti-synthetase syndrome (anti-JO1).Axial and coronal CT images showing bi-basal and peripheral honeycombing associated with reticulations superimposed to

ground-glass opacities, and some traction bronchiectasis.

Fig. 16. — Association of OP and fibrotic NSIP in a 53-yo- female patient with polymyositis and antisynthetase syndrome (anti-JO1).

A. Initial CT scan showing airspace consolidation with air bronchogram made of traction bronchiectasis.

B. The CT scan performed after 3 years of treatment shows the disappearance of airspace consolidation replaced by ground-glass opacities with superimposed traction bronchiectasis and some small lung cysts.

B

A

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the most serious complication of MCTD, and infection secondary to immunosuppression in the other fre-quent cause of death.

Most patients with MCTD present with one of the following clinical fea-tures: Raynaud’s phenomenon, ar-thralgia, arthritis, swollen hands, sclerodactyly or acrosclerosis and myositis. Esophageal dysmotility and reflex are frequently reported. Generalized lymphadenopathy is also a frequent manifestation and secondary Sjögren syndrome is rela-tively common.

Thoracic involvement is quite common. Cardiac abnormalities in-clude pericarditis, myocarditis, and complete heart block. Pleural effu-sion is one of the most common clin-ical manifestations usually small and resolving spontaneously.

Pulmonary hemorrhage, diffuse alveolar damage, organizing pneu-monia, NSIP, UIP, airway disease may be seen in these patients (35). This is consistent with the other manifestations of MCTD which have features of PSS, SLE or PM/DM. A

ways associated with ILD. PAH is usually severe, associated with a lower survival rate, suggesting the specific pulmonary vascular involve-ment (30-34).

mixed Connective Tissue Disease (mCTD)

Patients having characteristic fea-tures of more than one CTD, are said to have an overlap syndrome or undifferentiated CTD. MCTD is a distinct condition characterized by antiribonucleoprotein antibody, dis-ease-specific HLA profiles, and mod-erately specific clinical features. The majority of patients with MCTD do not evolve into other CTD. The ma-jority of patients are women with an average age of diagnosis of 37 years. The main characteristics are the presence of features of SLE, PSS, PM/DM occurring together or evolv-ing sequentially during observation. The prognosis for patients with MCTD is highly variable, but a severe progressive disease course in un-usual. Pulmonary hypertension is

ground glass opacities, ill-defined peribronchiolar or centrilobular nod-ular opacities, large nodular or mass-like area of consolidation, ring-like or crescent shaped opacities and occa-sionally irregular linear opacities. On serial evaluation under treatment, airspace consolidation may be partly or fully reversible (Fig. 15) or re-placed by ground glass opacities more or less associated with super-imposed intralobular reticulations, linear pattern and traction bronchi-ectasis corresponding to NSIP that becomes apparent after clarification of consolidation (32, 33) (Fig. 16). Honeycombing is relatively uncom-mon and reflects UIP (Fig. 17).

In patients with acute rapidly pro-gressive lung disease due to DAD, extensive consolidation and ground glass opacities are present.

When the diaphragm becomes in-volved, there is a bilateral hemidia-phragm elevation, reduced lung vol-ume and linear basal atelectasis (1, 4).

The occurrence of PAH in antisyn-thetase syndrome is significant, al-

Fig. 18. — Fibrotic NSIP in a 58 y.o. female patient with SJOS.Axial and sagittal reformatted CT images showing reticulations with traction bronchiectasis and bronchiolectasis, distortion of

thickened interlobular septa and fissures.

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THORACIC INVOLVEMENT IN CONNECTIVE TISSUE DISEASES — SERRA et al 15

Patients with SJOS have a higher risk of developing a lymphomatous disease (most commonly B type, non Hodgkin), arising from the salivary glands with possible pulmonary or gastric involvement.

Global prognosis for patients af-fected by SJOS is relatively good; it get worse in case of secondary forms or in patients developing a lympho-matous disease.

Radiological manifestations of dis-ease (38-41)

CT provides substantial informa-tion regarding the panel of pulmo-nary involvement in SJOS. The pat-terns include ILD (Fig. 18), airway abnormality, PAH and patterns sug-gestive of LIP.

Airway related abnormalities are frequent and consist of bronchial wall thickening, bronchiectasis, tree-in-bud sign and mosaic attenuation pattern with expiratory air trapping reflecting obliterative bronchiolitis (Fig. 19).

The main CT abnormalities of LIP consist of patchy or confluent bilat-eral ground-glass opacity and poorly defined centrilobular nodules. Thin-walled cysts, usually few in number are seen in 60-80% of patients usu-ally associated with ground-glass opacities, but occasionally as an iso-lated finding (42) (Fig. 20).

The association of nodules and lung cysts should raise the diagnosis of amyloidosis associated with LIP.

SJOS is histologically character-ized by a polyclonal lymphocytic-B infiltration of several organs, most frequently the salivary and lacrimal glands and the tracheobronchial tree. Most of the patients present a typical glandular involvement with xerophthalmia and xerostomy; one third of the patients can present ex-tra-glandular manifestations. The in-volvement of the upper respiratory tract can cause dryness and a crusty appearance of the nasal mucosa, sometimes complicating with epi-staxis, nasal septum perforation or otitis media. An involvement of the lower respiratory tract can cause chronic dry cough, recurrent bron-chitis and exercise-induced dyspnea.

Interstitial pulmonary involve-ment is common in patients with a primitive form of disease, presenting as LIP, NSIP, UIP or OP (37).

Both types of SJOS may develop a lymphoproliferative disorder sometimes associated with amyloï-dosis. The spectrum of lymphop-roliferative diseases in Sjögren syndrome includes follicular bron-chiolitis, LIP, nodular lymphoid hy-perplasia, mucosa-associated lym-phoid tissue (MALT) lymphoma, and lymphoma (38). Follicular bronchiol-itis is the most common form of bronchiolitis occurring in patients with SJOS; lymphocytic bronchiol-itis (lymphocytic infiltration without follicles) and infectious bronchiolitis represent the main differential diag-nosis.

large proportion of patients with ear-ly MCTD appear to have steroid-re-sponsive and reversible interstitial lung disease. Approximately a third of patients have fibrotic lung disease on CT (36).

Radiological manifestations of dis-ease (1, 4, 36)

The abnormalities that could be described in MCTD include small nodules (presumably reflecting lym-phoid hyperplasia), ground-glass opacities, intralobular reticulations predominant in the lower lung zones. Evidence of fibrosis is seen as intra-lobular reticulations, architectural distortion and traction bronchiecta-sis. A small proportion of patients have airspace consolidation, honey-combing, cysts and bronchiectasis. PAH may be associated with ILD or be the only intrathoracic manifesta-tion, manifested as pulmonary arte-rial enlargement. Small pericardial effusion and small ill-defined centri-lobular nodules may be present.

sjögren’s syndrome (sJOs)

SJOS is an autoimmune disorder characterized by dry eyes, (kerato-conjunctivitis sicca) and dry mouth (xerostomia) which particularly af-fects middle age women. A primitive and a secondary form of disease have been described: the first is an isolated form; the second is associ-ated to other CTDs.

Fig. 19. — Diffuse obliterative bronchiolitis in a patient with SJOS suffering from airflow limitation.A. Inspiratory CT scan (left) showing diffuse hypoattenuation of lung parenchyma due to reduced vascularity. Presence of bron-

chial wall thickening.B. Expiratory CT scan (right): absence of increased in lung attenuation at expiration compared to inspiration as normally expected.

This reflects the presence of diffuse expiratory air trapping.

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Fig. 20. — A 64 y.o. female patient with SJOS.CT images showing the presence of several thin-walled lung

cysts having a predominant subpleural and peribronchiolar distribution.

Fig. 21. — SJOS in a 52 y.o. male patient.Axial CT images showing a focal area of airspace consolida-

tion within the left lower lobe surrounded by ground-glass opacity revealing a low grade B-lymphoma. Presence of several thin-walled cysts with peribronchovascular and subpleural distribution very suggestive of LIP.

Fig. 22. — Airway disease in a 69 y.o. male patient who has suffered from RP from many years.A., B. Axial CT images targeted on the trachea and main stem bronchi showing thickened and partly calcified anterior and lateral

walls of the trachea and main stem bronchi (arrow heads).B. There is also a large thickening with calcifications of costal cartilages (arrows).

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including the ears, nose, joints and tracheobronchial tree. Histologically, the acute inflammatory infiltrates present in the cartilages and peri-chondrial tissue induces progressive dissolution and fragmentation of the cartilage followed by fibrosis.

The mean age at diagnosis is the fifth decade with an almost equal sex ratio and more severe airway dis-ease in female patients. About 50% of patients will develop laryngeal, tracheal or bronchial involvement. One third of patients have an associ-ated autoimmune disease, most

Relapsing polychondritis (RP)

RP is a rare autoimmune disease of unknown aetiology characterized by recurrent inflammatory episodes that affects cartilage at various sites,

In such cases, nodules represent amyloid deposits that may calcify.

Pulmonary lymphoma should be suspected in presence of pulmonary consolidations (Fig. 21), large nod-ules or pleural effusions.

Fig. 23. — Narrowing of the trachea and bronchi lumens in a 32 y.o. female patient with RP.

A. Axial CT images targeted on the tracheal cross-section at the level of the aortic arch (left image). The thickening of the anterior and lateral walls of the trachea (arrow heads) increased significantly (from 23 to 41 mm) after 5 months of follow-up (right image).

B. Axial CT images with lung window settings showing diffuse narrowing of the trachea and main stem bronchi lumens (arrows).

C. Dynamic expiratory CT scan showing air trapping in the left lung due to complete collapse of the left main bronchus at expiration (arrow).

A

B

C

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vascular disease. Rheumatol Int, 2009, 29: 1327-1330.

12. Zompatori M., Leone M.B., Giannotta M., et al.: Pulmonary hypertension and systemic sclerosis: the role of high-resolution computed tomogra-phy. Radiol Med, 2013, 118: 1360-1372.

13. Hansell D.M. Small-vessel disease of the lung: CT pathologic correlates. Radiology, 2002, 225: 639-653.

14. Tanaka N., Kim J.S., Newell J.D. Rheumatoid arthritis-related lung diseases: CT findings. Radiology, 2004, 232: 81-91.

15. Mori S., Cho I., Koga Y., et al.: Com-parison of pulmonary abnormalities on high- resolution computed tomo-graphy in patients with early versus longstanding rheumatoid arthritis. J Rheumatol, 2008, 5: 1513-1521.

16. Kaushik V.V., Hutchinson D., Desmond J., et al.: Association between bronchiectasis and smoking in patients with rheumatoid arthritis. Ann Rheum Dis, 2004, 63: 1001- 1002.

17. Nakajima R., Sakai F., Mimura T., et al.: Acute- or subacute-onset lung complications in treating patients with rheumatoid arthritis. Can Assoc Radiol J , 2013, 64: 200-2007.

18. Fujita J., Yoshinouchi T., Ohtsuki Y., et al.: Non-specific interstitial pneu-monia as pulmonary involvement of systemic sclerosis. Ann Rheum Dis, 2001, 60: 281-283.

19. Kim D.S., Yoo B., Lee J.S., et al.: The major histopathologic pattern of pulmonary fibrosis in sleroderma is nonspecific interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis, 2002, 19: 121-127.

20. Strollo D., Goldin J. Imaging lung disease in systemic sclerosis. Curr Rheumatol Rep, 2010, 12: 156-161.

21. Desai S.R., Veeraraghavan S., Hansell D.M., et al.: CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and non specific interstitial pneumonia. Radiology, 2004, 232: 560-567.

22. Kim J.S., Lee K.S., Koh E.M., et al.: Thoracic involvement of systemic lupus erythematosus: clinical, pathologic, and radiologic findings. Comput Assist Tomogr, 2000, 24: 9-18.

23. Lalani T.A., Kanne J.P., Hatfield G.A., et al.: Imaging findings in systemic lupus erythematosus. Radiographics, 2004, 24: 1069-1086.

24. Karim M.Y., Miranda L.C., Tench C.M., et al.: Presentation and prognosis of the shrinking lung syndrome in sys-temic lupus erythematosus. Semin Arthritis Rheum, 2002, 31: 289-298.

25. Nolan R.L., McAdams H.P., Sporn T.A., et al.: Pulmonary cholesterol granulomas in patients with pulmonary artery hypertension: chest radiographic and CT findings. AJR Am J Roentgenol, 1999, 172: 1317-1319.

26. Troyanov Y., Targoff I., Tremblay J.L., et al.: Novel classification of idiopathic

abnormalities, all thoracic structures can be implied, and should always be attentively assessed. Moreover, compared to idiopathic forms, ILD occurring in CTD patients may pres-ent with some particular characteris-tics of patterns, evolution over time and prognosis. The role of the radi-ologist, with a complete knowledge of the main CT findings related to each type of CTD and the familiarity with the most frequent complica-tions, is crucial for an adequate clini-cal management.

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commonly systemic vasculitis or RA. The prognosis of patients who have both systemic vasculitis and RP is worse than of patients with only RP (43).

The respiratory tract symptoms are usually the results of laryngotra-cheal chrondritis and include hoarse-ness, breathlessness, cough, stridor, wheezing and tenderness over the laryngotracheal cartilage. At disease onset, approximately a quarter of patients have respiratory symptoms. Airway involvement is the most cause of death in patients with RP.

Radiological manifestations of dis-ease (44, 45)

The larynx and trachea are most commonly affected. The disease may also involve the airways more distally to the level of subsegmental bronchi.

At the early stage of disease, CT shows smooth anterior and lateral airway wall thickening with sparing of the posterior membranous wall. There is also often an increased attenuation of the cartilages (rang-ing from subtle to frankly calcify) (Fig. 22). In more advanced disease, thickening of airway walls become circumferential because the poste-rior (non cartilaginous) wall of the trachea and main bronchi appears thickened. At this stage, luminal narrowing of the trachea and bron-chi may occur resulting from fibrosis following cartilaginous destruction (Fig. 23A,B). Focal narrowing of the trachea and bronchi may be present .

Loss of cartilaginous support due to cartilaginous inflammation and destruction may result in exces-sive dynamic expiratory collapsi-bility (tracheomalacia and broncho-malacia). Expiratory air trapping is frequently observed involving one lung, lobes, segments or lobules (46) (Fig. 23C).

Conclusion

A thoracic involvement can be fre-quently found in patients affected by CTDs; it is usually identified after or at the time of diagnosis, but even precedes other systemic signs. A wide spectrum of pulmonary abnor-malities has been described, and each CTD may present characteristic CT features; nevertheless, possible complications due to superinfections or drug toxicity should be taken in account in suggestive clinical con-texts. Although ILD and airways dis-ease represent the most common

serra-.indd 18 19/02/15 15:50

THORACIC INVOLVEMENT IN CONNECTIVE TISSUE DISEASES — SERRA et al 19

pulmonary abnormalities and com-puted tomography findings in 60 pa-tients. J Thorac Imaging, 2001, 16: 290-296.

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41. Taouli B., Brauner M.W., Mourey I., et al.: Thin-section chest CT findings of primary Sjögren’s syndrome: correlation with pulmonary function. Eur Radiol, 2002, 12: 1504-1511.

42. Silva C.I., Flint D.J., Levy R.D., et al.: Diffuse lung cysts in lymphoid inter-stitial pneumonia: high-resolution CT and pathologic findings. J Thorac Imaging , 2006, 21: 241-244.

43. Tsunezuka Y., Sato H., Shimizu H. Tracheobronchial involvement in relapsing polychondritis. Respiration, 2000, 67: 320-322.

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45. Lin Z.Q., Xu J.R., Chen J.J., et al.: Pulmonary CT findings in relapsing polychondritis. Acta Radiol, 2010, 51: 522-526.

46. Lee K.S., Ernst A., Trentham D.E., et al.: Relapsing polychondritis: preva-lence of expiratory CT airway abnor-malities. Radiology, 2006, 240: 565-573.

olution CT findings and functional correlation. Chest, 2003, 123: 1096-1103.

33. Tanizawa K., Handa T., Nakashima R., et al.: The prognostic value of HRCT in myositis-associated interstitial lung disease. Respir Med, 2013, 107: 745-752.

34. Hervier B., Meyer A., Dieval C., et al.: Pulmonary hypertension in anti-synthetase syndrome: prevalence, aetiology and survival. Eur Respir J, 2013, 42: 1271-1282.

35. Bodolay E., Szekanecz Z., Devenyi K., et al.: Evaluation of interstitial lung disease in mixed connective tissue-disease (MCTD). Rheumatology (Oxford), 2005, 44: 656- 661.

36. Kosuka T., Johkoh T., Honda O., et al.: Pulmonary involvement in mixed connective tissue disease: high-resolution CT findings in 41 patients. J Thorac Imaging , 2001, 16: 94-98.

37. Parambil J.G., Myers J.L., Lindelle R.M., et al.: Interstitial lung disease in primary Sjögren syndrome. Chest, 2006, 130: 1489-1495.

38. Lee G., Lee H.Y., Lee K.S., et al.: Imaging manifestations of auto-immune disease-associated lympho-proliferative disorders of the lung. Clin Rheumatol, 2013, 32: 1459- 1465.

39. Koyama M., Johkoh T., Honda O., et al.: Pulmonary involvement in prima-ry Sjögren’s syndrome: spectrum of

inflammatory myopathies based on overlap syndrome features and autoantibodies. Medicine, 2005, 84: 231-249.

27. Jordan Greco A.S., Métrailler J.C., Dayer E. The antisynthetase syn-drome: a cause of rapidly progressive interstitial lung disease. Rev Med Suisse, 2007, 3: 2675-2676, 2679-2681.

28. Stanciu R., Guiguet M., Musset L., et al.: Antisynthetase syndrome with anti-Jo1 antibodies in 48 patients: pulmonary involvement predicts dis-ease-modifying antirheumatic drug use. J Rheumatol, 2012, 39: 1835-1839.

29. Fathi M., Dastmalchi M., Rasmussen E., et al.: Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. Ann Rheum Dis, 2004, 63: 297-301.

30. Selva-O’Callaghan A., Labrador–Horrillo M., Munoz-Gall X., et al.: Poly-myositis/dermatomyositis-associated lung disease: analysis of a series of 81 patients. Lupus, 2005, 14: 534-542.

31. Douglas W.W., Tazelaar H.D., Hart-man TE., et al.: Polymyositis-derma-tomyositis-associated interstitial lung disease. Am J Respir Crit Care Med, 2001, 164: 1182- 1185.

32. Arakawa H., Yamada H., Kurihara Y., et al.: Nonspecific interstitial pneu-monia associated with polymyositis and dermatomyositis: serial high-res-

serra-.indd 19 19/02/15 15:50

Nodular disease is the most fre-quent pathology of the thyroid gland and its incidence is very high in pop-ulation. Among adults it is 4-8% in palpation, 10-41% in ultrasonograph-ic evaluation and 40-60% in autopsy series (1-7). Especially in the recent years, by the extensive use of ultra-sonography and high resolution de-vices, the frequency of the determi-nation of thyroid nodules has increased in patients without a com-plaint or a palpable nodule. The ma-jority of these nodules are benign and 7-15% are malignant (8, 9).

Different results had been report-ed in the studies about the efficiency of ultrasonography in the differential diagnosis of thyroid nodules. Al-though there are some helpful sono-graphic findings, none of them is able to provide the diagnosis of ma-lignancy alone (5, 10-12). Following ultrasonography, fine needle aspira-tion biopsy (FNAB) is the second fre-quently used method in the differen-tial diagnosis of thyroid nodules. The most of the nodules suspected as malignant in conventional ultraso-nography are diagnosed as benign after FNAB or operation and this sit-uation has created a need for a new method which can contribute to dif-ferentiate malignant nodules from benign ones and help to decrease the number of nodules undergoing these invasive procedures (13-15).

It is well known that malignant tis-sue is harder than normal tissues. When a malignant lesion is com-

position. First, demographic data of the patient was recorded. Then, the nodule was examined by real-time B-mode and color Doppler ultraso-nography. The number of nodules (multinodular/solitary), size, margin, echogenicity, inner structure (solid/mixed solid-cystic), presence of pe-ripheral halo, presence of microcalci-fications and vascularity of the nod-ule were evaluated. During this evaluation, spot B-mode images and static and dynamic color Doppler ul-trasonography images were record-ed digitally. After conventional ultra-sonography, real-time elastography examination was performed. Probe was placed on the thyroid gland in vertical position, the slice was found in which the largest size of the nod-ule was seen and slight freehand pressures were applied on the gland with short intervals. The sufficiency of the compression was decided by an indicator numbered from 1 to 4 on the screen. The images with the value 3 or 4 were accepted to be suf-ficient for evaluation (Figs. 1 and 2). Dynamic elastography images were recorded digitally to be evaluated later on. Afterwards, strain ratio (the ratio of the strain of normal paren-chyma to the strain of the nodule) was measured twice for each nodule and mean strain ratio values were calculated. During this process, both the strain of nodule and the strain of parenchyma were measured on the same image seperately and propor-tioned automatically (Figs. 1 and 2). Strains of parenchyme and nodule were measured by the help of ROI (region of interest) and ROI of paren-chyme was tried to place on the same depth with nodule. Strain ratio measurements were done mostly on transverse images, although sagittal

pressed with the ultrasound probe, it displaces more than a benign lesion. Ultrasound elastography (USE), also called as digital finger, is a relatively new method which measures the de-gree of strain in different tissues and demonstrates them in different col-ors. It also provides quantitative in-formation for more objective evalua-tion.

The aim of this study is to evalu-ate the efficiency of USE in the dif-ferential diagnosis of thyroid nod-ules.

Materials and methods

In this prospective study, 100 pa-tients with 100 thyroid nodules (79 females, 21 males, age range 18-78; mean age = 45.6 years) sent to Ra-diology department for ultrasound guided FNAB were evaluated with B-mode, color Doppler ultrasonography and USE, between February 2010 and April 2011. Pure cystic nodules and those with coarse calcifications were not included in the study, due to futility of USE on them. FNAB was applied to all solitary nodules, to the largest ones among multiple similar nodules and to the most suspicious ones among multiple nodules with different gray scale sonographic fea-tures.

All patients were evaluated by the same radiologist using a real-time instrument (EUB-7500 HV, Hitachi Medical Systems, Tokyo, Japan) and a 13-8 MHz linear probe in supine

JBR–BTR, 2015, 98: 20-26.

The efficiency of UlTrasoUnd elasTography in The differenTial diagnosis of Thyroid nodUlesN. Çetin1, C. Yücel1, P. Uyar Göçün2, S. Aladag Kurt1, F. Taneri3, S. Oktar1, H. Özdemir1

Aim: To evaluate the efficiency of ultrasound elastography (Use) in the differential diagnosis of thyroid nodules.Methods: one hundred thyroid nodules in 100 patients (79 females, 21 males, age range 18-78; mean age = 45.6 years) were evaluated with real-time freehand Use, using hitachi eUB 7500 equipment and elasticity scores were obtained. The elasticity was scored as follows: score 1, elasticity in the entire nodule; score 2, mainly elastic nodule with the presence of inelastic areas not constant during real time examination; score 3, constant inelastic areas prevalently arranged at the periphery of the nodule; score 4, constant inelastic areas prevalently arranged at the center of the nodule; score 5, no elasticity in the nodule. also mean strain ratio values were calculated for all nodules. Results: eighty-four (86%) of cases were benign and sixteen (16%) were malignant. elasticity score 3 and higher and strain ratio higher than 2.485 had statistically significant relation with malignancy (p < 0.05). Conclusions: Use including strain ratio calculations besides subjective evaluation of elasticity scores is an efficient imaging method which may contribute to the differential diagnosis of thyroid nodules.

Key-words: Thyroid, Us.

From: Department of 1. Radiology, 2. Medical Pathology, 3. General Surgery, Gazi University School of Medicine, Ankara, Turkey.Address for correspondence: Dr N. Çetin, M.D., Department of Radiology, Gazi Univer-sity School of Medicine, 06510 Besevler, Ankara, Turkey.E-mail: mdnurcan@hotmail.com

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US ELASTOGRAPHY IN THE DIAGNOSIS OF THYROID NODULES — ÇETIN et al 21

sels are irregular (anarchic or suddenly branching vascular struc-tures); Score 4A, peripheral and abundant intranodular vascularity, courses of vessels are regular; Score 4B, peripheral and abundant intrano-dular vascularity, courses of vessels are irregular (anarchic or suddenly branching vascular structures).

Elasticity was scored as follows: Score 1, elasticity in the entire nod-ule; Score 2, mainly elastic nodule with the presence of inelastic areas not constant during real time exami-nation; Score 3, constant inelastic areas prevalently arranged at the pe-riphery of the nodule; Score 4, con-stant inelastic areas prevalently ar-ranged at the center of the nodule; Score 5, no elasticity in the nodule (Fig. 3). In addition, modified TIRADS classification proposed by Russ et al. was used to classify the nodules in terms of B-mode features (Fig. 4) (16).

TIRADS categories, color Doppler ultrasonography and elastography scores of the nodules were decided by two experienced radiologists, who were blind to the histopatho-logic diagnosis, in consensus. Mean strain ratio of each nodule was also calculated from recorded values.

The statistical analysis of all find-ings was made by the programme “SPSS for WINDOWS 11.5”. Chi-square, Fisher’s exact and indepen-dent t tests were used for the statisti-cal analysis and p values below 0,05 were accepted to be statistically sig-nificant.

results

The largest sizes of total 100 nod-ules were between 5-65 mm (mean 18.71 mm). Pathologic results of 56 were obtained by cytologic examina-tion, while 44 were obtained by his-topathologic examination. Eighty-four were benign and 16 were malignant.

Seventy-seven of benign nodules were colloid nodules and 7 were follicular adenomas. All follicular adenomas were diagnosed by histo-pathologic evaluation, demon-strating that there was no capsular or vascular invasion. While 15 of malignant nodules were papillary carcinoma, one of them was undif-ferentiated thyroid carcinoma.

Mean age of patients with benign and malignant nodules were 45.84 and 44.31 respectively and there was no statistically significant difference between the two groups (p = 0.629) (Table I). Besides, there was no sta-tistically significant difference be-tween males and female patients in

tranodular vascularity; Score 2, there is peripheral vascularity but no intra-nodular vascularity; Score 3A, pe-ripheral and slight-moderate intra-nodular vascularity, courses of vessels are regular; Score 3B, pe-ripheral and slight-moderate intra-nodular vascularity, courses of ves-

images were used in some instances due to size and placement of the nodule.

Scoring systems were constituted and used for vascularity and elastici-ty of nodules by the reference of lit-erature. Vascularity was scored as follows: Score 1, no peripheral or in-

Fig. 1. — Elastogram of a completely elastic nodule with elasticity score 1 in the right thyroid lobe of a 34-year-old female patient (A). Strain ratio measurement of the nodule (B). Mean strain ratio was 1.01 and it was diagnosed as colloid nodule after surgery.

Fig. 2. — Elastogram of a mainly inelastic nodule with elasticity score 4 in the left thyroid lobe of a 20-year-old female patient (A). Strain ratio measurement of the nodule (B). Mean strain ratio was 5.43 and it was diagnosed as papillary carcinoma after surgery.

A

A

B

B

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22 JBR–BTR, 2015, 98 (1)

Score 5 elasticity among malignant ones. Therefore, the nodules with Score 4 and 5 elasticity were includ-ed in the same group to make a meaningful statistical analysis. As a result of this, a statistically signifi-cant difference occurred between the groups in terms of malignancy (p < 0.001) (Table VI).

The mean strain ratio values of benign and malignant nodules were 1.889 and 3.334, respectively. Cut-off point for strain ratio was found 2.485 by the assistance of SPSS pro-gramme and it was seen that there was a statistically significant relation between the strain ratios higher than

Presence of microcalcifications was the finding with the highest sen-sitivity (93.7%), positive predictive value (68.2%), negative predictive value (98.7%) and accuracy (92%) among all B-mode, color Doppler ul-trasonography and elastography findings (Table IV). It was also one of findings with the highest specificity together with ‘taller than wide’ sign (91.6%) (Table V).

Ultrasound Elastography

There was no nodule with Score 5 elasticity among benign nodules and there was only one nodule with

terms of the number of benign and malignant nodules (p = 0.789) (Ta-ble I).

The means of the largest sizes of benign and malignant nodules were 18.71 and 18.69 respectively and there was also no statistically signifi-cant difference between them (p = 0.992) (Table I).

B-Mode and Color Doppler Ultra-sonography

There was no statistically signifi-cant relation of solitary nodule, ‘tall-er than wide’ sign and solid inner structure with malignancy whereas, hypoechogenicity, blurred margin, presence of microcalcifications and absence of peripheral halo were in statistically significant relation with malignancy (Table II). There was a statistically significant relation of high TIRADS categories with malig-nancy (p < 0.001) (Table III). While ir-regular course of vessels was in a statistically significant relation with malignancy (p = 0.001), intranodular vascularity and abundant intranodu-lar vascularity were not (Table IV).

Fig. 3. — Schematic representation of the elasticity scores used. Score 1, elasticity in the entire nodule; Score 2, mainly elastic nodule with the presence of inelastic areas not constant during real time examination; Score 3, constant inelastic areas prevalently arranged at the periphery of the nodule; Score 4, constant inelastic areas prevalently arranged at the center of the nodule; Score 5, no elasticity in the nodule.

Fig. 4. — TIRADS classification algorithm (16)

Table I. — Distribution of nodules according to the age, sex and the largest size.

Benign Malignant Total P

Age (mean) 45.84 ± 11.13 44.31 ± 12.85 45.60 ± 11.38 0.629Sex Female

Male67 (79,8%)17 (20,2%)

12 (75%)4 (25%)

79 (79%)21 (21%) 0.789

Largest size (mean)

18.71 ± 9.059 mm 18.69 ± 10.836 mm 18.71 ± 9.305 mm 0.992

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US ELASTOGRAPHY IN THE DIAGNOSIS OF THYROID NODULES — ÇETIN et al 23

ered to be enough for the diagnosis of malignancy alone. B-mode and color Doppler ultrasonography find-ings of this study were also compat-ible with the literature. While all of the nodules categorized in TIRADS 3 and 4A were benign, six of eight TIRADS 5 nodules were malignant. According to this result, by using modified TIRADS classification pro-posed by Russ et al., it could be stat-ed that the nodules categorized in TIRADS 3 and 4A are probably be-nign and malignancy risk is consid-erably low, and the nodules catego-rized in TIRADS 5 are probably malignant. Therefore, FNAB or sur-gery should definitely be recom-mended for TIRADS 5 nodules, while there’s no need for TIRADS 3 and 4A. The nodules categorized in TIRADS 4B could be thought as moderately suspicious and FNAB or follow-up could be recommended. Unfortu-nately, there hasn’t been any TIRADS classification established yet like BI-RADS and all TIRADS classifications are in study phase (16, 25-30). In this study, due to its incomplexity and practicality in usage of all sonogra-phers, modified TIRADS classifica-tion proposed by Russ et al. was prefered.

In a similar research on USE, B-mode and color Doppler ultrasono-graphy, Rago et al. reported that high elasticity score, being the find-ing with the highest sensitivity and spesificity, was in statistically signifi-cant relation with malignancy (31). In another study, Rago et al. reported a statistically significant relation be-

has been reported that USE could be used in differential diagnosis of breast and prostate gland disor-ders (21-24). Like other US tech-niques, it is cheap, real-time, nonin-vasive, easy to access and applicate. Also, it does not include ionizing ra-diation. As a result of all these ad-vantages, USE is gaining popularity in imaging and differential diagnosis of thyroid nodules.

In this study, while hypoecho-genicity, blurred margin, absence of halo, microcalcifications and irregu-lar course of vessels were in statisti-cally significant relation with malig-nancy; sex, number of nodules, ‘taller than wide’ sign, inner struc-ture, intranodular vascularity and abundant vascularity were not. Pres-ence of microcalcifications was the finding with the highest sensitivity, specificity (together with ‘taller than wide’ sign), positive predictive value, negative predictive value and accuracy among all B-mode, color Doppler ultrasonography and elas-tography findings, so it was consid-

2.485 and malignancy (p = 0.001) (Table VII).

discussion

Palpation is the oldest method in the evaluation of tissue stiffness and it has been used in medicine since Ancient Egypt. It is currently used in physical examination of breast, thy-roid and liver. However, evaluation of stiffness by palpation is subjective and insufficient in detection of all mass lesions (17, 18). This fact has lead researchers to look for new techniques to evaluate the stiffness of pathologic tissues.

Elastography is an imaging tech-nique that measures strain respons-es of tissues against compressive forces applied on them (19, 20). USE was first used in experimental media in the 80s by Ophir et al. It has been commonly used in imaging field for last 10 years, as a result of develop-ments in computer and ultrasound technologies. In previous studies, it

Table II. — Distributions of the benign and malignant nodules according to B-mode ultrasonography findings and p values of the suspicious findings.

Benign(n = 84)

Malignant(n = 16) P

Number MultipleSolitary

6519

115 0.525

‘Taller than wide’ sign AbsentPresent

777

151 1.00

Echogenicity Iso-hyperechoicHypoechoic

5529

412 0.003

Margin RegularBlurred

6123

79 0.023

Peripheral halo PresentAbsent

4638

412 0.029

Microcalcifications AbsentPresent

777

115 < 0.001

Inner structure Mixed solid-cysticSolid

3450

511 0.488

Table III. — Distribution of the nodules according to TIRADS classification.

TIRADS Benign (n = 84) Malignant (n = 16) Total

3 34 (100%) 0 (0%) 34 (100%)

4A 18 (100%) 0 (0%) 18 (100%)

4B 30 (75%) 10 (25%) 40 (100%)

5 2 (25%) 6 (75%) 8 (100%)

Total 84 (84%) 16 (216%) 100 (100%)

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24 JBR–BTR, 2015, 98 (1)

statistically significant relation with malignancy. But, it’s obvious that they are not sufficient alone in the di-agnosis of malignancy due to their low sensitivity and specificity values. However, real-time elasto graphy seems as a valuable method in the differential diagnosis of thyroid nod-ules, not alone but together with con-ventional ultrasonography. Accord-ing to this study, it could be reasonable to categorize nodules with elasticity score 1 or 2 as proba-bly benign, 3 as moderately suspi-cious and 4 or 5 as probably malig-nant and this categorization may be a useful guide for FNAB. As an exam-ple, recommending follow-up may be appropriate for nodules with score 1 or 2, and FNAB for nodules with score 3, 4 or 5. In this study, 68 of 72 nodules with elasticity score 1 and 2 were benign, and it was possible to avoid from biopsies for 68% of all pa-tients by using only this scoring.

2.485 was determined as cut-off point of the strain ratio. Although, the strain ratio higher than 2.485 is in

tween the strain ratio higher than 4 and malignancy.

In this study, it was found that there were significant contributions of both real-time examination and strain ratio measurements to the dif-ferential diagnosis of thyroid nod-ules (37). Following the presence of microcalcifications, the other accept-able values of sensitivity, specificity and accuracy were belonged to the elasticity score 3 and higher. Irregu-lar course of vessels and some of other gray scale findings were also in

tween low elasticity score and benig-nity (32). Similarly, in some other studies related to USE, high sensitiv-ity and specificity values were re-ported related to high elasticity scores and malignancy (33-36).

In addition to these, Ning et al. re-ported that strain ratio values higher than 4.2 was in statistically signifi-cant relation with malignancy, in the study including both strain ratio and elasticity score (36). Moreover, in an-other study Lyshchic et al. reported a statistically significant relation be-

Table IV. — Distributions of nodules according to vascularity patterns and p values of the suspicious findings.

Benign Malignant P

Intranodular vascularity Score 2Score 3A + 3B +4A + 4B

479

212 0.174

Abundant intranodular vascularity Score 3A + 3BScore 4A + 4B

5029

111 0.051

Irregular course of vessels Score 3A + 4AScore 3B + 4B

6118

57 0.01

Table V. — Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of all findings.

Sensitivity SpecificityPositive

Predictive Value

Negative Predictive

ValueAccuracy

Solitary nodule 31.2% 77.4% 20.8% 85.5% 70%

‘Taller than wide’ sign 6.2% 91.6% 12.5% 83.7% 88%

Hypoechogenicity 75% 65.5% 29.3% 93.2% 67%

Blurred margin 56.2% 72.6% 28.1% 89.7% 70%

Absence of halo 75% 54.7% 24% 92% 58%

Microcalcifications 93.7% 91.6% 68.2% 98.7% 92%

Solid iner structure 68.7% 40.5% 18% 87.2% 45%

Intranodular vascularity 75% 6% 13.2% 55.5% 17%

Abundant vascularity 6.2% 65.5% 3.3% 78% 56%

Irregular course of vessels 43.7% 78.6% 28% 88% 73%

TIRADS ≥ 4B 100% 62% 33% 100% 68%

Strain ratio > 2,485 56.2% 85.7% 42.8% 91.1% 81%

Elasticity score ≥ 3 75% 81% 42.8% 94.4% 80%

Table VI. — Distribution of the nodules according to elasticity scores.

Elasticity score Benign(n = 84)

Malignant(n = 16)

Total

1 21 (100%) 0 (0%) 21 (100%)

2 47 (92.2%) 4 (7.8%) 51 (100%)

3 12 (75%) 4 (25%) 16 (100%)

4 + 5 4 (33.3%) 8 (66.7%) 12 (100%)

Total 84 (84%) 16 (216%) 100 (100%)

cetin-.indd 24 19/02/15 15:52

US ELASTOGRAPHY IN THE DIAGNOSIS OF THYROID NODULES — ÇETIN et al 25

sum up, USE when combined with strain ratio calculations is a promis-ing ultrasound technique that may contribute to the differential diagno-sis of thyroid nodules.

ethical consideration

All procedures followed were in ac-cordance with the ethical standards of the responsible committee on hu-man experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008. Informed consent was obtained from all patients for being included in the study. Additional informed consent was obtained from all pa-tients for which identifying informa-tion is included in this article.

references

1. Howlett D.C., Speirs A.: The thyroid incidentaloma: ignore or investigate? J Ultrasound Med, 2007, 26: 1367-1371.

2. Gharib H., Papini E., Paschke R.: Thyroid nodules: a review of current guidelines, practices, and prospects. Eur J Endocrinol, 2008, 159: 493-505.

3. Screaton N.J., Berman L.H., Grant J.W., US-guided core-needle biopsy of the thyroid gland. Radiolo-gy, 2003, 226: 827-832.

4. Rausch P., Nowels K., Jeffrey R.B. Jr.: Ultrasonographically guided thyroid biopsy: a review with emphasis on technique. J Ultrasound Med, 2001, 20: 79-85.

5. Frates M.C., Benson C.B., Charboneau J.W., et al.: Management of thyroid nodules detected at US: Society of Radiologists in Ultrasound consensus conference statement. Radiology, 2005, 237: 794-800.

6. Wiest P.W., Hartshorne M.F., Inskip P.D., et al.: Thyroid palpation versus high-resolution thyroid ultrasono graphy in the detection of nodules. J Ultrasound Med, 1998, 17: 487-496.

7. Mikosch P., Gallowitsch H.J., Kresnik E., et al.: Value of ultrasound-guided fine-needle aspiration biopsy of thy-roid nodules in an endemic goitre area. Eur J Nucl Med, 2000, 27: 62-69.

8. Baier N.D., Hahn P.F., Gervais D.A., et al.: Fine-needle aspiration biopsy of thyroid nodules: experience in a co-hort of 944 patients. Am J Roentgen-ol, 2009, 193: 1175-1179.

9. Izquierdo R., Arekat M.R., Knudson P.E., et al.: Comparison of palpation-guided versus ultrasound-guided fine-needle aspiration biop-sies of thyroid nodules in an outpa-tient endocrinology practice. Endocr Pract, 2006, 12: 609-614.

10. Solbiati L., Charboneau J.W., Osti V., et al.: The thyroid gland. In: Diagnostic Ultrasound. Edited by Rumack C.M., Wilson S.R., Charboneau J.W. Printed

ments, comparing them with each other and having coherent results are focus points of a correct strain ratio measurement.

Including both real-time examina-tion and strain ratio measurement is the superiority of this study when compared to others. Also, B-mode and color Doppler ultrasonography examinations made it possible to compare efficiencies of USE and conventional ultrasonography.

There were 86 benign (86%) and 16 malignant (16%) thyroid nodules in this study. It seems that the num-ber of the malignant nodules is low compared to whole. In fact, the ratio of malignancies in this study is simi-lar to the ratio in the population (7-15%) (8, 9). All of the malignant nod-ules except one were papillary carcinoma and this can be consid-ered as a handicap for this study. Due to the fact that the papiller carci-noma is the most common type of thyroid cancers (75-85%), this situa-tion is not suprising. We hope that it will be possible to eliminate this limitation by the help of wide-range studies with wider histopathologic profile.

conclusion

USE should be considered as an imaging technique to be used in combination with conventional ultra-sonography, rather than a magic wand diagnosing malignancy alone. B-mode ultrasonography is unques-tionably the most important imaging modality in the differential diagnosis of thyroid nodules. The presence of microcalcifications with the highest sensitivity, specificity and accuracy in this study shows the efficiency of B-mode ultrasonography once again. However radiologists sometimes en-counter with confusing cases and this situation is not rare. At this point, USE may play a significant role in the evaluation of the nodules that could not be differentiated by con-ventional ultrasonography alone. To

statistically significant relation with malignancy (p = 0.001), it stays be-hind the real-time examination due to its low sensitivity (56.2%). It is also lower than the values found in previ-ous studies. It is thought that this is a result of our insufficiency in measur-ing the strain ratio from correct elas-tograms reflecting the real strain of the nodules. Hopefully this problem will disappear with the increase of USE experience. This will be possi-ble with more and wide-ranging studies and with the addition of elas-tography in routine thyroid ultraso-nography examinations.

Different scoring systems have been used in the studies related to USE (31-36). In some of these, the scoring system of Ueno and Ito was used for thyroid nodules (31, 32). In this system, inelasticity of tissue around the nodule was also added as criteria. But this criterion repre-sents the desmoplastic reaction seen in breast cancer and it’s not neces-sary and appropriate for the evalua-tion of thyroid nodules. Moreover, in our experience on real-time exami-nation, we also observed alterations in color codes as a result of serial compression and decompression movements. Because of this, the consistency of inelastic areas throughout the examination was added as criteria in our scorring sys-tem. This observation also showed that the strain ratio which could only be measured from spot images, is not sufficient alone due to its higher error margin. Therefore it was seen that the real-time examination is es-sential in both studies and clinical practice.

Real-time examination is operator dependent and this is its most im-portant limitation. This situation cre-ates a need for a new method pre-senting quantitative data. The most commonly used value for this aim is the strain ratio, as used in this study. Measuring the strain ratio from im-ages reflecting the real-time exami-nation, making multiple measure-

Table VII. — Mean strain ratio values of nodules and distribution of the nodules according to the cut-off point.

Benign(n = 84)

Malignant(n = 16) Total

Mean strain ratio 1.889 ± 1.338 3.334 ± 2.703 2.120 ± 1.7

< 2.485 72 (91.1%) 7 (8.9%) 79 (100%)

> 2.485 12 (57.1%) 9 (42.9%) 21 (100%)

Total 84 (84%) 16 (16%) 100 (100%)

cetin-.indd 25 19/02/15 15:52

26 JBR–BTR, 2015, 98 (1)

thyroid imaging reporting and data system (TIRADS) classification in dif-ferentiating benign from malignant thyroid nodules. Open Journal of Radiology, 2013, 3: 103-107.

30. Wei X., Li Y., Zhang S., Gao M.: Thy-roid imaging reporting and data sys-tem (TIRADS) in the diagnostic value of thyroid nodules: a systematic re-view. Tumour Biol, 2014, 35: 6769-6776.

31. Rago T., Santini F., Scutari M., et al.: Elastography: new developments in ultrasound for predicting malignancy in thyroid nodules. J Clin Endocrinol Metab, 2007, 92: 2917-2922.

32. Rago T., Scutari M., Santini F., et al.: Real-time elastosonography: useful tool for refining the presurgical diag-nosis in thyroid nodules with indeter-minate or nondiagnostic cytology. J Clin Endocrinol Metab, 2010, 95: 5274-5280.

33. Wang Y., Dan H.J., Dan H.Y., et al.: Differential diagnosis of small single solid thyroid nodules using real-time ultrasound elastography. J Int Med Res, 2010, 38: 466-472.

34. Asteria C., Giovanardi A., Pizzocaro A., et al.: US-elastography in the dif-ferential diagnosis of benign and ma-lignant thyroid nodules. Thyroid, 2008, 18: 523-531.

35. Rubaltelli L., Corradin S., Dorigo A., et al.: Differential diagnosis of benign and malignant thyroid nodules at elastosonography. Ultraschall Med, 2009, 30: 175-179.

36. Ning C.P., Jiang S.Q., Zhang T., et al.: The value of strain ratio in differential diagnosis of thyroid solid nodules. Eur J Radiol, 2012, 81: 286-291.

37. Lyshchik A., Higashi T., Asato R., et al.: Thyroid gland tumor diagnosis at US elastography. Radiology, 2005, 237: 202-211.

method for imaging the elasticity of biological tissues. Ultrasound Imag-ing, 1991, 13: 111-134.

21. Lerner R.M., Huang S.R., Parker K.J.: “Sonoelasticity” images derived from ultrasound signals in mechanically vi-brated tissues. Ultrasound Med Biol, 1990, 16: 231-239.

22. Ophir J., Alam S.K., Garra B., et al.: Elastography: ultrasonic estimation and imaging of the elastic properties of tissues. Proc Inst Mech Eng H, 1999, 213: 203-233.

23. Garra B.S., Cespedes E.I., Ophir J., et al.: Elastography of breast lesions: initial clinical results. Radiology, 1997, 202: 79-86.

24. Cochlin D.L., Ganatra R.H., Griffiths D.F.: Elastography in the detection of prostatic cancer. Clin Radiol, 2002, 57: 1014-1020.

25. Horwath E., Majlis S., Rossi R, et al.: An ultrasonogram reporting system for thyroid nodules stratifying cancer risk for clinical management. J Clin Endocrinol Metab, 2009, 94: 1748-1751.

26. Park J.Y., Lee H.J., Jang H.W., et al.: A proposal for a thyroid imaging report-ing and data system for ultrasound features of thyroid carcinoma. Thyroid, 2009, 19: 1257-1264.

27. Kwak J.Y., Han K.H., Yoon J.H., et al.: Thyroid imaging reporting and data system for US features of nodules: a step in establishing better stratifica-tion of cancer risk. Radiology, 2011, 260: 892-899.

28. Russ G., Bigorgne C., Royer B., Rouxel A., Bienvenu-Perrard M.: The thyroid imaging reporting and data system (TIRADS) for ultrasound of the thy-roid. J Radiol, 2011, 92: 701-713.

29. Moife B., Takoeta E.O., Tambe J., Blanc F., Fotsin J.G.: Reliability of

by Mosby, 3rd ed, St. Louis (Missouri), 2005, pp. 735-770.

11. Moon W.J., Jung S.L., Lee J.H., et al.: Benign and malignant thyroid nod-ules: US differentiation – multicenter retrospective study. Radiology, 2008, 247: 762-770.

12. Brander A., Viikinkoski P., Nickels J., et al.: Thyroid gland: US screening in a random adult population. Radiolo-gy, 1991, 181: 683-687.

13. Lou S., Kim E., Dighe M., et al.: Thy-roid nodule classification using ultra-sound elastography via linear dis-criminant analysis. Ultrasonics, 2011, 51: 425-431.

14. Gharib H., Goellner J.R.: Fine-needle aspiration biopsy of the thyroid: an appraisal. Ann Intern Med, 1993, 118: 282-289.

15. Raab S.S., Grzybicki D.M., Sudilovsky D., et al.: Effectiveness of Toyota process rediesign in reducing thyroid gland fine-needle aspiration error. Am J Clin Pathol, 2006, 126: 585-592.

16. Russ G.: Thyroid Imaging and Report-ing Database System. www.tirads.com, 2013.

17. Khaled W., Reichling S., Bruhns O.T., et al.: Ultrasonic strain imaging and reconstructive elastography for bio-logical tissue. Ultrasonics, 2006, 44: 199-202.

18. Luo J., Ying K., Bai J.; Elasticity recon-struction for ultrasound elastography using a radial compression: an in-verse approach. Ultrasonics, 2006, 44: 195-198.

19. Ophir J., Kallel F., Varghese T., et al.: Elastography. Optical and acoustical imaging of biological media. Acad Sci 2001, 4: 1193-1212.

20. Ophir J., Céspedes I., Ponnekanti H., et al.: Elastography: a quantitative

cetin-.indd 26 19/02/15 15:52

The ingestion of foreign objects is a common problem in clinical prac-tice. However most of the ingested objects pass through the gastroin-testinal tract without any problems within a week (1). Perforation in the gastrointestinal tract is very rare and is only seen in about 1% of the pa-tients ingesting a foreign object (2, 3). Chicken bones and fish bones are amongst the most common acciden-tally ingested foreign objects and are also the most common cause of bowel perforation. Fish bones are rarely detected radiographically as they aren’t always radiopaque, chick-en bones however can be detected with computed tomography, when large enough.

Case reports

Case 1

A 67-year-old woman presented at the emergency ward with right abdominal pain of one day duration. On clinical examination she had a fe-ver of 38,4° Celsius and abdominal distress with peritoneal signs. She had a known muscle dystrophia and lupus for which she took several drugs, corticoids being the most important. The laboratory results showed no other than an elevated white blood count and a CRP of 9,51 mg/dl. The decision was made

eign object, perforating the antral wall of the stomach, but no free air in the peritoneal cavity. The surgeons performed a medial laparotomy and dissected a pheasant bone of ap-proximately 4 cm in an inflamed area of the stomach. Thereafter an omentoplasty was performed and the patient left the hospital ten days later after treatment with intra-venous antibiotics.

to perform an abdominal CT scan after admission of intravenous con-trast, in the portovenous phase (Fig. 1, 2). This clearly shows a for-

JBR–BTR, 2015, 98: 27-31.

Gastrointestinal CompliCations of aCCidental inGestion of foreiGn objeCtsJ. Huyskens1, E. Van Hedent2, L. Trappeniers2, W. Simoens2, T. Jager2

ingestion of foreign objects is common and most of the time, they pass without major problems. However some-times they could cause significant morbidity or even mortality. most of the time they cause pain in the pharyngeal or oesophageal area. in these instants, diagnosing the problem is straightforward, limiting the use of radiographic diagnosis.However the intraperitoneal complications include stomach or bowel perforation, obstruction, abscedation, septice-mia or even hemorrhage or thrombosis of the abdominal veins.because of the considerable risks, accompanied by the accidental ingestion of a metallic object, the preferred tech-nique for screening is still Computed tomography. However not all of these objects are radio-opaque and therefore could not always be diagnosed radiographically.in this article we will describe several cases of complications, due to the accidental ingestion of foreign objects. also we will describe certain patient related risk factors significantly increasing, not only the amount, but also the sever-ity of those complications. diverticulosis seems the most common risk factor amongst the patients described and so it could be one of the more common triggers causing (recidivating) diverticulitis attacks. but because not all of the ingested foreign objects are radiopaque or still in the gastrointestinal tract, such a theory is difficult to prove.

Key-word: foreign bodies, in air and food passages.

From: 1. Department of Radiology, University Hospital Antwerp, Edegem, 2. Department of Radiology, Algemeen Stedelijk Ziekenhuis, Aalst, Belgium.Address for correspondence: Dr J. Huyskens, Department of Radiology, University Hospital Antwerp, Wilrijkstraat 10, 2650 Edegem, Belgium. E-mail: jef_huyskens@hotmail.com

Fig. 1. — CT scan with intravenous contrast in the portove-nous phase: coronal reconstruction shows a dense foreign ob-ject (arrow) perforating the antral gastric wall. The perforation is covered and gastric wall is thickened and hypodense, suggest-ing inflammation.

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28 JBR–BTR, 2015, 98 (1)

perilesional inflammation and thrombosis of main portal vein. Some free air in the hilar region, sug-gesting some degree of cholangitis. This patient was treated conserva-tively with antibiotics and therapeu-tic dosed low molecular weight hep-arines. After two months, this septic trombophlebitis of the main portal vein resolved. After conservative management there was a spontane-ous evacuation of the foreign object without any residual inflammation.

Case 4

A 53-year old man presented at the emergency room with malaise, fatigue, night sweats, diffuse pain, arthralgia. the patient had a fever of unknown cause. Clinical examina-tion was negative. Laboratory results showed an elevated C-reactive pro-tein We performed a CT scan with intravenous contrast in the portove-nous phase (Fig. 7, 8). This showed a foreign object that perforated through the stomach with important

Case 2

A 79-year old woman was referred by gastroenterologist for a virtual colonoscopy. She already com-plained of abdominal pain and anal blood loss for several days. She had a known history of recidivating di-verticulitis for several years. The vir-tual colonoscopy was performed af-ter the admission of intravenous contrast and rectal air insufflation in the portovenous phase (Fig. 3, 4). The foreign object lied in the centre of and is the base of the accompany-ing diverticulitis. This foreign object was simply removed by colonosco-py and identified as a small fish bone. Afterwards the patient was further treated for diverticulitis with antibiotics.

Case 3

A 77-year old man presented at the emergency ward with severe left abdominal pain of two days dura-tion. He is a known COPD patient and already underwent a gastrectomy af-ter several gastric ulcerations. He had no fever, but he did have clear peritoneal signs. Laboratory results showed and elevated white blood count (16800/mm³) and C-reactive protein (11,53 mg/dl). We performed a CT scan with intravenous contrast in the portovenous phase (Fig. 5, 6).

He was immediately operated on, a laparotomy for partial colectomy was performed. In the dissected colon they retrieved a sharp wooden meat stick. After antibiotic therapy of several days, the patient could return home.

Fig. 2. — CT scan with intravenous contrast in the portove-nous phase: the axial MIP (maximum intensity projection) recon-struction shows the foreign object (arrow) in close proximity of the liver and biliary ducts. Fig. 3. — CT scan with intravenous contrast in the portove-

nous phase: coronal reconstruction shows a foreign object (arrow) in a thickened sigmoid wall.

Fig. 4. — CT scan with intravenous contrast in the portove-nous phase: axial image shows the foreign object (arrow) in the thickened sigmoid wall with accompanying fatty infiltration as a sign of inflammation. Clearly several sigmoid diverticula are seen on this slide.

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GI COMPLICATIONS OF INGESTION OF FOREIGN OBJECTS — HUYSKENS et al 29

vanilla stick. After a consecutive treatment of 10 days with antibiotics, the woman remained asymptomatic.

discussion

A wide variety of offending for-eign objects have been reported to result in perforation, including chick-en bones, fish bones, metallic ob-jects and toothpicks. Most perfora-tions occur at narrowings or angulations such as ileocecal and rectosigmoid junctions, with approx-imately 83% occur at the ileum (4-6).

To make the diagnosis, clinical suspicion must be high, because

moidal wall with associated focal di-verticultitis of the sigmoid. In first instance, the object could not be re-moved endoscopically because of the inflammation. After a period of conservative treatment with antibiot-ics, the object was removed endo-scopically. On macroscopical exami-nation the object was identified as a

Case 5

A 81-year-old woman was re-ferred to a gastroenterologist by her clinician because she could not sit comfortable for several months. We performed a CT scan with intrave-nous contrast in the portovenous phase (Fig. 9, 10, 11). This shows a foreign object, perforating the sig-

Fig. 5. — CT scan with intravenous contrast in the portove-nous phase: axial reconstructions shows a low density foreign object (arrow), perforation the bowel wall of the descending colon. Note the extensive diverticulosis of the sigmoid and descending colon.

Fig. 7. — CT scan with intravenous contrast in the portove-nous phase: coronal reconstruction shows the foreign object (arrow), perforating through the antral wall. Hypodense main portal vein without contrast, suggesting thrombosis.

Fig. 8. — CT scan with intravenous contrast in the portove-nous phase: coronal reconstruction shows the foreign object, perforating through the antral wall with perilesional hypodense tissue, ie inflammation. Free air in the hilar region suggesting cholangitis.

Fig. 6. — CT scan with intravenous contrast in the portove-nous phase: coronal reconstruction shows the low density foreign object (arrow) in the wall of the inflamed wall of the descending colon. Again note the diverticulosis.

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30 JBR–BTR, 2015, 98 (1)

Ultraound can be very useful, es-pecially in young children, where an ingested object can obstruct the gastric outlet, causing persisting vomiting and dehydration (11). Sometimes a foreign object can be visualized sonographically more dis-tal, for example at the ileocaecal valve. Children tend to ingest plastic or wooden toys, making convention-al radiography or CT less useful. Ultra sound is not dependent on the radiographic density and it does not involve ionizing radiation.

As illustrated in three of our cases, perforation of a foreign object was accompanied by the presence of di-verticulitis around the site of perfora-tion. Therefore it could be a possible cause of diverticulitis. As diverticulo-sis is very common, with a preva-lence of more than 50% above the age of 60 (10), it is very likely that the ingestion of foreign objects is a trig-ger for the occurrence of diverticuli-tis. Before this report, this was not yet described, to our knowledge. Maybe in the future it will be de-scribed more, as CT is more widely used for the diagnosis of diverticuli-tis, as long as we look for it, and also keeping in mind that the foreign ob-ject causing the perforation of diver-ticulum may already passed with the stool.

references

1. McCanse D.E., et al.: Gastro-intestinal Foreign Bodies. Am J Surg, 1981, 142: 335-337.

Detection of foreign objects can be challenging. It is easily detected with gross perforation, showing free intra-peritoneal gas on radiographs, al-though this is not seen in the majori-ty of cases. Computed Tomography (CT) is the imaging modality of choice for identifying and localizing the for-eign object because of the high spa-tial resolution, but is still largely de-pendent on the size and radiodensity of the foreign objects (9). In normal circumstances, metal and wood ob-jects are readily identified on CT but it is slightly more difficult for chicken bones. Fish bones are not always ra-diopaque and thus are less frequent-ly identified radiographically.

many medical conditions can simu-late this abnormality. As previously described, colon diverticulitis or un-suspected colon carcinoma can be a secondary finding (7, 8).

Ingestion of foreign objects is more common amongst children, psychiatric patients, elderly patients with dentures, patients with alcohol abuse and the mentally or physically disabled.

As described in the cases above, clinical presentation is variable. Al-though the gold standard for the treatment of bowel perforation re-mains surgery or endoscopy, con-servative treatment and long-term follow-up can be indicated.

Fig. 9. — CT scan with intravenous contrast in the portove-nous phase: axial image shows a hyperdense foreign object (arrow) in the sigmoid wall. Note multiple diverticula with peri-lesional focal inflammation, ie diverticulitis.

Fig. 10. — CT scan with intravenous contrast in the portove-nous phase: coronal reconstruction shows a long hyperdense foreign object (arrow) perforating the sigmoid wall with perile-sional inflammation.

Fig. 11. — CT scan with intravenous contrast in the portove-nous phase: sagittal reconstruction clearly shows an area of diverticulitis surrounding a focal hyperdensity, ie the foreign object (arrow).

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GI COMPLICATIONS OF INGESTION OF FOREIGN OBJECTS — HUYSKENS et al 31

9. Mcmanus J.E.: Perforation of the in-testine by ingested foreign bodies: report of two case and review of the literature. Am J Surg, 1941, 53: 393-401.

10. Martel J., et al.: History, incidence and epidemiology of diverticulosis. J Clin Gastroenterol, 2008, 42 (10): 1125-1127.

11. Moammar H., et al.: Sonographic diagnosis of gastric-outlet foreign body: case report and review of the literature. J Family Community Med, 2009, 16: 33-36.

literature. Int Surg, 1981, 66: 181-183.6. Noh H.M., et al.: Small bowel perfora-

tion by a foreign object. AJR, 1998, 171: 1002.

7. Gomez N., et al.: Intestinal perforation caused by chicken bone mimicking perforated colonic diverticulitis. Acta Gastroeneterol Latinoam, 1997, 27: 329-330.

8. Osler T., et al.: Perforation of the co-lon by the ingested chicken bone, leading to the diagnosis of carcinoma of the sigmoid. Dis Colon Rectum, 1985, 28: 177-179.

2. Maleki M., et al.: Foreign-body perfo-ration of the intestinal tract: report of 12 cases and review of the literature. Arch Surg, 1970, 101: 474-477.

3. McPherson R.C., et al.: Foreign body perforation of the intestinal tract. Am J Surg, 1957, 94: 564-566.

4. Goh H.M., et al.: Preoperative diagno-sis of fish bone perforation of the gastrointestinal tract. AJR, 187: 710-714.

5. Singh R.P., et al.: Perforation of the sigmoid colon by swallowed chicken bone: case reports and review of the

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Sciatic hernia is a rare pelvic floor hernia that occurs through the great-er or lesser sciatic foramen. It is gen-erally determined by the symptoms related to herniated content such as bowel obstruction, pelvic pain, and ureteric obstruction. Sciatic hernias may present as a rare cause of sciat-ica (1-4). Retroperitoneal neoplasm with sciatic hernia is considered the rarest, with a very limited number of published reports in the literature. The lesions are deeply located and clinical diagnosis is uneasy. In this respect, imaging modalities are needed for both identification of the lesion content and assessment of the sciatic nerve. We present a case of retroperitoneal lipoma which was di-agnosed through the lower leg symptoms, along with the magnetic resonance imaging (MRI) findings.

Case report

A 39-year-old woman was admit-ted to our hospital with a five-month long pain in the left leg and difficulty in walking. Clinical history revealed that she has been suffering from pain spreading from the left hip/sci-atic to the leg/limb for one year. She had no history of trauma, systemic disease or drug use. Physical exami-nation did not reveal dorsiflexion in the ankle or toe of the left foot. Labo-ratory findings were normal. On lumbar MRI, disc herniation pressing on the nerve root was not observed. However, as a presacral hyperin-tense mass was observed on T2 weighted sagittal sections, lower ab-dominal MRI was performed. MRI demonstrated a large intra-and ex-tra-pelvic fatty mass traversing the greater sciatic foramen. The diame-ter of the lesion was 6 x 13 x 15 cm.

painful when they compress periph-eral nerves (5). Clinical diagnosis is uneasy due to the gluteal muscle covering any mass protruding through the sciatic foramen (4). Di-agnosis is made possible by imaging modalities. Fat has a characteristic appearance on cross-sectional imag-ing methods. Magnetic resonance signal characteristics of a fat contain-ing neoplasm allows a reliable diag-nosis by detecting signal intensity areas within the mass equal to that of subcutaneous fat on all the pulse sequences, with loss of signal on fat-suppression techniques. On T1- and T2 weighted images, there may be thin fibrous septae with low signal intensity whereas the lesions that do not typically show contrast enhance-ment (8, 9).

Pathology of lipomatous lesions with sciatic herniation from the ret-roperitoneal region has been report-ed as well-differentiated liposarcoma (atypical lipoma) which is different from our case (3, 4, 7). While liposar-coma is the common primary neo-plasm in the retroperitoneal region, lipoma is rare in this region (7). MRI is helpful in distinguishing these two entities. Kransdorf et al defined sig-nificant features to help distinguish lipoma from liposarcoma in their study, the most important one being the presence of thickened septa, nodular and/or globular areas of non-adipose tissue within the lesion, associated non-adipose masses, and a total amount of non-adipose tissue composing more than 25% of the le-sion (8). Ohguri et al. reported that thick septa, nodular or lobular/patchy non-adipose components in retro-peritoneal and deeply located well-differentiated liporsarcomas were more common than the subcutane-ous lesions (10).

Preoperative diagnosis of these deep lipomatous lesions is impor-tant for treatment planning (5). MRI is the modality of choice to evaluate the sciatic nerve in cases of sciatic hernia (2). Surgical treatment of the

This mass was well-circumscribed, isointense with the subcutaneous fatty tissue (Fig. 1A, B) and did not show contrast enhancement after in-travenous contrast agent administra-tion (Fig. 1C). The mass displaced adjacent anatomic structures , for example the rectum was displaced to the right. No atrophy was ob-served in gluteal muscles. Pathology following the surgery established the diagnosis of osteolipoma with mature osteoid tissue regions and mature lipocytes (Fig. 2).

Discussion

Sciatic hernia is a rare pelvic floor herniation . It was first reported by Papen in 1750 (1). It is more common in adults and women (2, 3). Sciatic hernia may present as a gluteal mass or with complications of the pelvis content (2-4). This content may be small intestine (obstruction), ureter and bladder (infection and obstruc-tion), ovaries and fallopian tubes (pelvic pain), colon, omentum and Meckel’s diverticulum (1). Sciatica occurs as a result of compression of the sciatic nerve by the herniated sac (1-5). To our knowledge, four well-documented descriptions of a lipoma herniating through the sciatic foramen have been reported, and it was first defined in 1964 (3, 4, 6, 7). In our case, retroperitoneal lipoma which showed sciatic herniation and made nerve compression was deter-mined during the examination for the lower leg.

Lipomas are mesenchymal soft tissue neoplasms which consist of mature fatty cells. They are com-monly observed in adults. They usu-ally present as painless soft-tissue masses, although larger ones can be

JBR–BTR, 2015, 98: 32-33.

A GiAnt RetRopeRitoneAl lipomA pResentinG As A sCiAtiC HeRniA: mRi FinDinGs S. Duran1, M. Cavusoglu1, E. Elverici1, T.D. Unal2

sciatic hernia is a rare condition and its clinical diagnosis is uneasy. Herniation of pelvic organs as well as of ret-roperitoneal neoplasm has been reported in the literature. sciatica occurs as a result of compression of the sciatic nerve by the herniated sac. We present a case of retroperitoneal lipoma in a patient who had lower leg complaint and describe the imaging findings.

Key-word: nerves, sciatic.

From: 1. Clinic of Radiology, 2. Clinic of Pathology, Ankara Numune Education and Research Hospital, Ankara, Turkey.Address for correspondence: Dr Semra Duran, M.D., Clinic of Radiology, Ankara Numune Education and Research Hospital, Ankara Numune Egitim ve Arastırma Hastanesi, Talatpasa Bulvarı No: 5, Altındag-Ankara 06100, Turkey. E-mail: Semraduran91@gmail.com

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A GIANT RETROPERITONEAL LIPOMA — DURAN et al 33

and well-differentiated liposarcoma. Radiology, 2002, 224: 99-104.

9. Galant J., Marti-Bonmati L., Saez F., et al.: The value of fat-suppresed T2 or STIR sequences in distinguishing lipoma from well-differentiated lipo-sarcoma. Eur Radiol, 2003, 13: 337-343.

10. Ohguri T., Aoki T., Hisaoka M., et al.: Differential diagnosis of benign pe-ripheral lipoma from well-differentiat-ed liposarcoma on MR imaging: Is comparison of margings and internal characteristic useful? AJR, 2003, 180: 1689-1694.

pressing the sciatic nerve: An inverted sciatic hernia. J Neurosurg, 2012, 117: 795-799.

6. Kerry R.L., Tygart R.L., Glas W.W.: Lipoma: a ‘’reversed’’perineal sciatic hernia. Am J Surg, 1964, 107: 883-884.

7. Hansch A., Gajda M., Boettcher J., Pfeil A., Kaiser W.A.: Incomplete pare-sis of the sciatic nerve due to massive atypical lipoma of the pelvis: A case report. Cases Journal, 2008, 1: 296.

8. Kransdorf M.J., bancroft L.W., Peterson J.J., et al.: Imaging of the fatty tumors: Distinction of lipoma

symptomatic sciatic hernia is possi-ble with transabdominal or trans-gluteal approach (3).

In conclusion, sciatic hernia is a rare entity and MRI enables a precise diagnosis of the tumor and the adja-cent anatomical structures.

References

1. Losanoff J.E., Basson M.D., Gruber S.A., Weaver D.W. Sciatic hernia: A comprehensive review of the world literature (1900-2008). Am J Surg, 2010, 199: 52-59.

2. Chitranjan S.R., Kandpal H., Madhu-sudhan K.S.: Sciatic hernia causing sciatica: MRI and MR neurography showing entrapment of sciatic nerve. Br J Radiol, 2010, 83 (987): e65-6.

3. Skipworth R.J., Smith G.H., Stewart K.J., Anderson D.N. The tip of the ice-berg: A giant pelvic atypical lipoma presenting as a sciatic hernia. World J Surg Oncol, 2006, 4: 33

4. Cappellani A., Zanghi A., Di Vita M., et al.: Very atypical presentation of a retroperiteonal “atypical lipoma”. Ann Ital Chir., 2007, 78: 69-72.

5. López-Tomassetti Fernández E.M., Hernández J.R., Esparragon J.C., García A.T., Jorge V.N.: Intermuscular lipoma of the gluteus muscles com-

Fig. 1. — T1 weighted axial (A) and T2 weighted axial (B) images showing retroperiteonal lipoma isointense to fat tissue. At the level of the hip joint herniation of the lesion to sciatic foramen and compression of the left sciatic nerve is observed (arrow: right sciatic nerve). C. Fat-suppressed postcontrast T1 weighted coronal image shows signal loss of the retroperiteonal lipoma. No postcontrast enhancement is observed in the lesion.

A

B

C

Fig. 2. — HE ×16 mature lamelles, bone tissue and mature lipocytes.

duran-.indd 33 19/02/15 15:54

Case report

A 43-year-old textile worker con-sulted our hospital with a several-year history of a painless, slow-growing ankle mass. He had noticed the mass for the first time approxi-

Conventional lateral radiograph of the left ankle showed a non-specific but well-defined soft-tissue mass in the posterior ankle region without obvious calcifications (Fig. 1A). The mass was located along the expect-ed course of the Achilles tendon. Ultrasound examination clarified a well-defined lesion which was situ-ated adjacent to the Achilles tendon and characteristics of intralesional reflections and retro-acoustic shad-owing (Fig. 1B). The mass was non-compressible and no internal calcifi-cations were seen.

On MR the mass had a hetero-geneous low to intermediate signal intensity pattern on theT2 weighted imaged images (Fig. 2A), the T1-weighted appearance was consis-tent, with a homogeneously low to

mately 5 years previously as a small lump, painlessly increasing in size over time.

The medical history of the patient revealed that he had visited an or-thopaedic surgeon 12 months earlier for anterior knee pain which was conservatively treated. Physical ex-amination noted a normal gait. In-spection and palpation showed an obvious, firm and mobile mass situ-ated in the posterior-medial aspect of the ankle and adjacent to the Achilles tendon. It was tender, mea-suring approximately 2,5 cm. His muscle bulk, strength, reflexes, and sensation were enterily normal. No obvious vascular findings were found. The patient had a normal neu-rologic status with no Tinel’s sign of the sural nerve.

JBR–BTR, 2015, 98: 34-36.

Pseudotumoral toPhaCeous involvement of the aChilles Paratenon T. Ryckaert, I. Crevits, S. Brijs, G. Debakker, F. Rosseel, A. Tieleman, R. De Man1

Gout is the most common form of microcrystalline arthropathy which usually does not pose a diagnostic challenge when patients have typical presentation, appropriate biochemical picture and classical radiographic appearance.however, formation of gouty tophi in unusual locations and with atypical presentations may mislead clinicians and radiologists, thereby justifying gout nickname as the “great mimicker”. When interpreting images of tendon related masses, radiologists should be aware of gouty tophi as a possible differential given its variable and nonspecific imaging appearance.in this article, we present a case of a patient with a painless tophaceous gout nodule, adjacent to the achilles tendon.

Key-word: Gout.

From: Department of Radiology, AZ Delta, Roeselare, Belgium.Address for correspondence: Dr T. Ryckaert, M.D., Department of Radiology, AZ Delta, Roeselare, Rode-Kruisstraat 20, B-8800 Roeselare, Belgium. E-mail: thomasryckaert@me.com

Fig. 1. — Soft-tissue mass in the posterior ankle region, along the expected course of the Achilles tendon (A) without obvious calcifications on the conventional lateral radio-graph, although on a longitudinal ultrasound image along the Achilles tendon the mass shows a significant hypoechoic shadowing (B).

B

A

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TOPHACEOUS INVOLVEMENT OF ACHILLES PARATENON — RYCKAERT et al 35

uric acid as an inborn error of metabolism. Secondary gout is an acquired form of hyperuricemia, which is most commonly related to an underexcretion of uric acid. The presentation of gout is generally de-scribed in the joints of the extremities, but gout may also present in soft-tissue structures.

Tophaceous gout causes chronic inflammation in an area of soft tissue leading to fibrinoid necrosis of adja-cent structures.

Tophaceous gout infiltration of tendon structures has rarely been re-ported in the literature, the majority involve the main body of the Achilles and quadriceps tendon itself. Despite the the insertional tendinopathy and enthesophyte formation, this case is particularly interesting because we have a pseudotumoral involvement of paratenon, without direct involve-ment of the tendon. Maybe the paratenon acts as a protective barri-er against the crystal deposition be-sides. The slow rate of progression of the disease is another particularity of the case.

A tophus histologically consists of a central acellular core of crystalline or amorphous urates surrounded by a peripheral “fibrovascular zone” of macrophages, lymphocytes, and fi-broblasts. Large foreign-body-type giant cells, which are often wrapped around masses of precipitated salts, are also very prominent (2).

Advanced imaging methods such as ultrasonography, CT, and MRI provide detailed images of the tophus. Ultrasonography studies have described two types of tophi on ultrasonographic examination: soft tophi, that are typically of varying echogenicity and soft also to palpa-tion, long standing hard tophi that contain monosodium urate deposits generate a hyperechoic band with an acoustic shadow and are harder in consistency (3). Conventional CT can be used to detect and measure tophi in patients with gout. The tophus is typically visualized as a soft tissue mass, with a density of 170 HU (4). The MR imaging appearance of gouty tophi varies (5). Their appear-ances on T2- weighted sequences range from homogenously low to homogenously high signal, depend-ing on the degree of hydratation and calcification. The most common ap-pearance on a T2 weighted sequence is a heterogeneous low to intermedi-ate signal intensity pattern. T2-weighted hypointensity has been at-tributed to the presence of calcium, crystal and fibrous tissu, whereas tophi with high water content hyper-

hyperuricemia, which develops in response to an excessive rate of pro-duction of uric acid, a decrease in re-nal excretion of uric acid, or a combi-nation of these two (1).

The disease is manifested by a combination of characteristics, these include : hyperuricemia, recurrent at-tacks of acute arthritis triggered by crystallization of urates within joints, intercritical periods of varying lengths, and development of chronic tophaceous gout.

This metabolic disorder can be classified as either primary gout or secondary gout. A primary gout con-dition is due to an overproduction of

intermediate signal intensity (Fig. 2B) and after administration of contrast the mass showed peripheral en-hancement pattern (Fig. 2C).

Based on the clinical and radio-logical findings the decision was made to remove the lesion surgically and a local resection was performed. Histologically, the diagnosis of to-phaceous gout was made.

discussion

Gout is defined as a metabolic dis-ease characterized by deposition of monosodium urate crystals. The bio-chemical hallmark of the disease is

AB

C

Fig. 2. — Sagittal PD (A) and sagittal fat-suppressed T2-weighted image (B) of the left ankle reveals a rounded soft tissue deposit adjacent to the Achilles tendon with hetero-geneous low to intermediate signal. On the axial T1-weighted image (C) the mass is homogeneously low in signal with an peripheral enhancement after contrast adminis-tration (D).

D

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36 JBR–BTR, 2015, 98 (1)

Cornish J.: Cellular characterization of the gouty tophus: A quantitative analysis. Arthritis & Rheumatism, 2010, 62 (5) : 1549-1556.

3. de Avila Fernandes E., Sandim G.B., Mitraud S.A., et al.: Sonographic description and classification of tendinous involvement in relation to tophi in chronic tophaceous gout. Insights Imaging, 2010; 1: 143-148.

4. Dalbeth N., Kalluru R., Aati O., Horne A., Doyle A., M McQueen F.: Tendon involvement in the feet of pa-tients with gout: a dual-energy CT study. Ann Rheum Dis doi:10.1136.

5. Yu J.S., Chung C., Recht M., Dailiana T., Jurdi R.: MR Imaging of Tophaceous Gout. AJR, 1997, 168: 523-527.

tophi as the first manifestation of the disease could be a challenge for the clinician. Generally these cases differ from those with typical gout (older patients, frequently women, usually with predominant or exclusive finger involvement, most of them with re-nal insufficiency and with anti- in-flammatory or diuretic therapy).

references

1. Dhanda S., Jagmohan P., Quek S.T.: A re-look at an old disease: A multi-modality review on gout. Clin Radiol, 2011, 66: 984-992.

2. Dalbeth N., Pool B., Gamble G.D., Smith T., Callon K.E., McQueen F.,

intense. Its T1-weighted appearance is more consistent, with usually ho-mogeneously low to intermediate signal intensity. Enhancement pat-tern is also variable, with homoge-nous enhancement being the most common appearance, reflecting hy-pervascularity of the tophus. Periph-eral enhancement has also been re-ported and was also seen in this case (Fig. 2D), this would be proportional to the vascularity predominantly in the outer “fibrovascular zone” seen on histology.

While gout is a common disease, this case was a diagnostic challenge, because there was no clear history of gouty attacks. Cases presenting with

ryckaert-.indd 36 19/02/15 15:55

Case report

A 61-year-old man presented to the emergency department with a 5-week history of dyspnea, cough and transient episodes of fever. These symptoms did not respond to antibiotic treatment. He was known with COPD GOLD III and α1-antitrypsin deficiency. Initially he had an arterial oxygen saturation of 87%, which could not be increased with O2-administration.

Clinical findings

Lung auscultation yields bilateral rhonchi and discrete expiratory wheezing. Laboratory findings in-clude elevated C-reactive protein of 225 mg/l, leukocytosis of 10.200/mm³ and increased D-dimer of 1074 ng/ml. Plain chest film showed a Chron-ic Obstructive Pulmonary Disease (COPD) configuration of the chest, with an opacification in the lower left lobe. To rule out pulmonary embo-lism a chest CT angiography was performed, using 60cc Iobitridol 300mg (Xenetix 300, Guerbet, Bel-gium). This showed (Fig. 1) predom-inantly basal centrilobular emphyse-ma with a consolidation in the lower left lobe. There were no signs of pul-monary embolism. By coincidence, we depicted an anomalous drainage of the left upper lobe pulmonary vein via a large (anomalous) vertical vein, into the innominate vein, causing a left-to-right shunt (Fig. 2).

Discussion

Partial anomalous pulmonary ve-nous return (PAPVR) is a rare condi-tion with a prevalence of 0.4-0.7% (1). In PAPVR, up to three pulmonary

ventricular septal defect or a patent ductus arteriosus (1).

The clinical presentation varies widely from asymptomatic patients to congestive heart failure. Long-standing PAPVR predisposes the pa-tient to right-sided volume overload, tricuspid regurgitation (TR), arrhyth-mias, pulmonary arterial hyperten-sion, irreversible pulmonary vascu-lar disease, right ventricular dysfunction and right ventricular fail-ure (2). Several pulmonary anoma-lies may be associated with PAPVR. These malformations include bron-chopulmonary sequestration, pul-monary arteriovenous malforma-tion, congenital diaphragmatic hernia and cystic adenomatoid mal-formation (3). Another rare form of PAPVR, called the Scimitar syn-drome or hypogenetic lung syn-drome, is characterised by pulmo-nary venous drainage of a portion or all of the right lung to the inferior vena cava. The anomalous vessel

veins connect to the right atrium or its tributaries, rather than to the left atrium. The most common presenta-tion of PAPVR is a right upper lobe vein draining into either the right atrium or superior vena cava. The right-sided PAPVR is typically associ-ated with an atrial septal defect (ASD) of the sinus venosus type.

Only 10% of the PAPVR are left-sided, and 3% of the reported cases show drainage from the left lung into the innominate vein, as seen in our case. In contrast to the life-threaten-ing total anomalous pulmonary venous return, the PAPVR is usually detected at a later age or during autopsy. The PAPVR results in a left-to-right shunt, similar to an ASD,

JBR–BTR, 2015, 98: 37-38.

REPORT OF A RARE ANATOMIC VARIANT: LEFT UPPER LOBE PARTIAL ANOMALOUS PULMONARY VENOUS RETURNY. De Brucker1, B. Ilsen1, C. Muylaert1, L. Goethals1, K. Nieboer1, A. Fares1, T. Jager2, J. de Mey1

We report the CT findings in a case of partial anomalous pulmonary venous return (PAPVR) from the left upper lobe in an adult. PAPVR is an anatomic variant in which one to three pulmonary veins drain into the right atrium or its tributaries, rather than into the left atrium. This results in a left-to-right shunt with varying clinical presentation. These can range from asymptomatic patients to advanced cardiac failure.

Key-word: Pulmonary veins, abnormalities.

From: 1. Department of Radiology, UZ Brussel, Jette, Belgium, 2. Department of Radiology, ASZ Aalst, Aalst, Belgium.Address for correspondence: Dr Y. De Brucker, Department of Radiology, UZ Brussel, Laarbeeklaan 101, 1090 Jette, Belgium. E-mail: ydebruck@vub.ac.be

Fig. 1. – Curved coronal CT image of the chest demonstrates a 15 mm-large vertical vein (VV) draining blood from the left upper lobe pulmonary vein into the innominate vein (IV).

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38 JBR–BTR, 2015, 98 (1)

administered via the left arm or left jugular vein.

Echocardiography may also directly show the PAPVR, by identify-ing the vertical vein draining into the innominate vein. Since some au-thors believe that PAPVR becomes clinically significant when 50% or more of the pulmonary blood flow returns anomalously, velocity- encoded MRI can play an important role in measuring the ratio of pulmonary to systemic blood flow (Qp:Qs) (5).

Surgical or transcatheter endo-vascular treatment is indicated in symptomatic PAPVR and asymptom-atic patients with Qp:Qs > 1.5, right ventricular dilation, mild-to-moder-ate tricuspid regurgitation (TR), or early stages of hypertensive pulmo-nary vascular disease. Treatment can prevent the development and progression of right ventricular fail-ure and irreversible pulmonary vas-cular disease. Watchful waiting is recommended in asymptomatic patients without evidence of right ventricular dilation or TR (6).

References

1. Dillman J.R., Yarram S.G., Hernandez R.J.: Imaging of pulmo-nary venous developmental anoma-lies. AJR Am J Roentgenology, 2009, 192: 1272-1285.

2. Edwin F.: Left-sided partial anoma-lous pulmonary venous connection – should diagnosis lead to surgery? Interact CardioVasc Thorac Surg, 2010, 11: 847-848.

3. Felker R.E., Tonkin I.L.D.: Imaging of pulmonary sequestration. AJR, 1990, 154: 241-249.

4. Latson L.A., Prieto L.R.: Congenital and acquired pulmonary vein steno-sis. Circulation, 2007, 115: 103-108.

5. Haramati L.B., Moche I.E., Rivera V.T., et al.: Computed tomography of par-tial anomalous pulmonary venous connection in adults. J Comput Assist Tomogr, 2003, 27: 743-749.

6. Elbardissi A.W., Dearani J., Suri R., Danielson G.: Left-sided partial anomalous pulmonary venous con-nections. Ann Thorac Surg, 2008, 85: 1007-1014.

sulting in a persistent vertical vein, which drains into the innominate vein (4).

Chest radiograph is often normal, however secondary signs of left-to-right shunt can be seen. These signs include cardiomegaly and pulmo-nary vascular prominence. Anoma-lous tip positioning of a left jugular/subclavian central venous catheter at the left mediastinum, should raise suspicion of PAPVR or persistent left-sided VCS. If the vertical vein is large, it can be depicted on X-ray as a small knob at the left side of the aortic arch (“aortic nipple”).

CT angiography and 3D CT clearly shows the common upper pulmo-nary vein connecting to the anoma-lous vertical vein, which in turn drains into the innominate vein. In a well-performed CT angiography of the pulmonary arteries, the pulmo-nary veins of the upper lobe may show early and intense opacifica-tion, due to retrograde flow of the contrast. This will be more obvious when the contrast media is being

has a characteristic scimitar appear-ance on chest radiographs as it runs from the middle of the right lung to the cardiophrenic angle (1).

The embryonic pathway of devel-opment of the pulmonary veins helps to understand the cause of the anomalous character of the pulmo-nary venous return. Initially, blood from both embryonic lungs drains into the splanchnic plexus, in turn communicating with a systemic car-dinal vein (CV) on each side. Later the right CV becomes the VCS and the left CV involutes or persists as a left VCS or vertical vein. Primitive common pulmonary veins (PCPV) arise as outpouching structures at the dorsal wall of the left atrium, and with time communicate to the splanchnic plexus and CV. In normal situation, the venous connection be-tween the PCPV and the CV invo-lutes, giving rise to four separate pulmonary veins draining to the left atrium. In our case the venous con-nection between the PCPV and the CV of the left upper lobe failed, re-

Fig. 2. – A. 3D CT of the heart and shows the early opacification of the left upper lobe pulmonary vein (LULPV) draining into the vena cava superior (VCS) via the anomalous vertical vein (VV) and the innominate vein (IV). B. Illustration of PAPVR of the left upper lobe.

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Ischiofemoral impingement (IFI) was first described in 1977 by John-son in three patients with persistent hip pain after hip surgery, two after total hip arthroplasty and one after proximal femoral osteotomy (1). More recently, other cases of IFI were reported in patients without history of surgery or trauma (2, 3, 4). The aim of this paper is to report the imaging features of a case of IFI, secondary to a solitary cartilaginous exostosis at the lesser trochanter in a young patient.

Case report

A 22-year old male was referred to the radiology department because of right groin pain, aggravating by external rotation of the hip.

Standard radiographs (Fig. 1) of the right hip revealed a large sessile exostosis at the medial aspect of the lesser trochanter, resulting in narrowing of the distance between the lesser trochanter and the ischial tuberosity.

On magnetic resonance imaging (MRI) (Fig. 2), a marked narrowing of the ischiofemoral space with accom-panying edema of the quadratus femoris muscle (QFM) was seen. There also was a thin overlying layer of cartilage at the surface of the exostosis, as well as some foci of hyaline cartilage protruding into the exostosis. After administration of intravenous gadolinium contrast, a faint enhancement of the surface cartilage and the cartilaginous changes was observed. There were no signs of malignancy.

Based on the imaging findings, the diagnosis of ischiofemoral impingement due to a benign

JBR–BTR, 2015, 98: 39-42.

ISCHIOFEMORAL IMPINGEMENT DUE TO A SOLITARY EXOSTOSISJ. Schatteman1, F.M. Vanhoenacker2,3,4, J. Somville5, K.L. Verstraete1,4

Ischiofemoral impingement is a rare cause of hip pain related to narrowing of the space between the ischial tuberos-ity and the lesser trochanter. It is usually seen in middle-aged women. We report a rare case of a young male patient presenting with ischiofemoral impingement due to a solitary exostosis at the lesser trochanter. Imaging, especially Magnetic Resonance Imaging (MRI), is an excellent tool to confirm the diagnosis by demonstrating narrowing of the ischiofemoral space and soft tissue edema in the muscle belly of the quadratus femoris muscle.

Key-word: Bones, osteochondrodysplasias.

From: 1. Department of Radiology, Ghent University Hospital, University of Ghent, 2. Department of Radiology, AZ Sint-Maarten, Duffel-Mechelen, 3. Department of Radiology, Antwerp University Hospital, University of Antwerp, Edegem, 4. Faculty of Medicine and Health Sciences, University of Ghent, 5. Department of Orthopaedic Surgery, Antwerp University Hospital, University of Antwerp, Edegem.Address for correspondence: Prof Dr F.M. Vanhoenacker, Dept. of Radiology, AZ Sint-Maarten, Duffel-Mechelen, Rooienberg 25, 2570 Duffel, Belgium.E-mail: filip.vanhoenacker@telenet.be

Fig. 1. — Initial anteroposterior radiography of both hips. A large exostosis is seen on the medial aspect of the right femoral neck (arrow), resulting in narrowing of the distance between the ischial tuberosity (star) and the lesser trochanter.

Fig. 2. — MRI of the pelvis. Axial T1-weighted images (WI) (A) show a signifi-cant narrowing of the ischiofemoral space (straight arrow) due to the exosto-sis (star), in comparison with the normal left side. On axial fat-suppressed T2-WI (B) focal edema of the muscle belly of the QFM (curved arrow) is seen, caused by impingement between the surface of the exostosis and the ischial tuberosity. Note the presence of a thin layer of cartilage at the surface of the exostosis (arrowhead).

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40 JBR–BTR, 2015, 98 (1)

The etiology of narrowing of the IFS can be divided into three main categories: congenital, acquired and positional (Table I). Most often, IFI is acquired and is seen in middle-aged or older women after valgus

surface of the hamstring tendons and the posteromedial surface of the distal iliopsoas tendon (6, 7). A dis-tance of less than 7 mm is abnormal, with 12 mm being a normal value (Fig. 4).

exostosis at the femoral neck was made. Because of failure of the initial conservative treatment, a resection of the exostosis was planned. In order to evaluate the exact narrow-ing of the bony ischiofemoral space, computed tomography (CT) was performed (Fig. 3), which confirmed a marked narrowing of the right IFS compared to the contralateral left side.

Histological examination of the resection specimen confirmed the diagnosis of a benign cartilaginous exostosis. The immediate postoper-ative recovery was uneventful.

Discussion

Ischiofemoral impingement con-sists of a rare cause of hip pain re-lated to narrowing of the space be-tween the ischial tuberosity and the lesser trochanter. This results in me-chanical impingement of the inter-vening soft tissues, most frequently the quadratus femoris muscle (2, 5). Both bony and soft tissue landmarks have been described to evaluate IFI. The ischiofemoral space (IFS) is the narrowest osseous distance between the lateral cortex of the ischial tuber-osity and the medial cortex of the lesser trochanter. In normal circum-stances, this distance should be larg-er than 23 mm, whereas a distance of less than 13 mm is abnormal. The quadratus femoris space (QFS) mea-sures the distance between the soft tissue landmarks of the superolateral

Fig. 3. — Three-dimensional (3D) CT provides a more accurate evaluation of the narrowing of the bony ischiofemoral tunnel

Fig. 4. — Normal axial T1-WI of the left hip. A normal QFM (thick arrow) is seen. The ischiofemoral space (line A) is defined by the narrowest distance between the lateral cortex of the ischial tuberosity (sphere) and the medial cortex of the lesser trochanter (star). The quadratus femoris space (line B) is delineated by the superolateral surface of the hamstring tendons (thin arrow) and the posteromedial surface of the distal iliopsoas tendon (curved arrow).

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ISCHIOFEMORAL IMPINGEMENT DUE TO SOLITARY EXOSTOSIS — SCHATTEMAN et al 41

cific, imaging plays a pivotal role in the diagnosis. Plain radiographs of the hip are usually normal in IFI. Nonetheless, for evaluation of an ex-pansile bone lesion as underlying cause, plain radiography can be helpful. In longstanding IFI, second-ary osseous changes such as sclero-sis and cystic changes of the lesser trochanter and ischium can occur as a consequence of chronic bony im-pingement (5). However, the pre-ferred imaging technique to confirm IFI is MRI, allowing direct measure-ment of the IFS and QFS and assess-ing edema of the QFM. Whereas acute posttraumatic edema caused by tear or strain is typically seen at the myotendinous junction, edema due to IFI is more likely to be located at the site of maximal impingement,

provoked by exorotation of the hip, extension and adduction and stretch-ing with the hip held in endorotation, flexion and abduction. Focal pres-sure at the ischial tuberosity can be painful (2, 5).

The differential diagnosis of IFI includes a wide variety of intra- and extra-articular causes of hip or groin pain, such as degenerative hip disease, labral tear or adductor tendinopathy. When pain irradiates to the lower leg, IFI may even mimic lumbar discopathy, spinal stenosis and hamstring tendinopathy. Further potential differential diagnoses are summarized in Table II and are beyond the scope of this case re-port (2, 5).

Because clinical findings and symptoms of IFI are rather nonspe-

osteotomy or hip arthroplasty (1). Secondary IFI due to a solitary of bi-lateral exostosis (in patients with He-reditary Multiple Exostosis Syn-drome) is a rare cause (3). In 20% to 45% of the patients, IFI is bilateral or occurs in young people, supporting the hypothesis of predisposing con-genital narrowing.

Clinically, IFI usually presents as chronic, non-traumatic groin or but-tock pain in middle-aged women. This pain may radiate from the pos-terior side of the upper leg to the knee (ischialgia), caused by the pres-sure effect of an edematous QFM on the sciatic nerve. Other symptoms described in IFI are snapping, crepi-tation and locking (2, 5).

There is no specific clinical test to diagnose IFI. However, pain can be

Table I. — Pathogenetic factors causing ischiofemoral impingement.

Congenital Acquired Positional

Bone lesions Soft tissue lesionsPosteromedial position of the femur1

Expansile bone lesions Chronic bursitis Extension, adduction and exorotation

Larger cross-section of the proximal femur1

Degenerative hip disease3 Expansile soft tissue lesions

Hip stretching4

Prominence of the lesser trochanter

Valgus-producing osteotomy

Low position of the ischiopubic ramus

Posttraumatic

Female pelvic configuration2

1 causing narrowing of the distance between the lesser trochanter and the ischial tuberosity.2 wider pelvis, with greater distance between the ischial tuberosities in comparison to the male pelvis.3 causing cranial and medial migration of the femur.4 with hip held in flexion, abduction and endorotation.

Table II. — Main differential diagnosis of ischiofemoral impingement.

Intra-articular Extra-articular

Degenerative hip disease Ischiofemoral impingement

Labral tear Strain/tear QFM without narrowing of the IFS

Femoroacetabular impingement Adductor-, hamstring- or iliopsoas tendinopathy/bursitis

Spinal stenosis

Lumbar discopathy

Pathology of the sacro-iliacal joints

Piriformis syndrome

Inguinal hernias/mass lesions

Urinary tract problemsAbbreviations: QFS: Quadratus femoris muscle; IFS: Ischiofemoral space.

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42 JBR–BTR, 2015, 98 (1)

2. Craenen K., Vanheste R., Peers K., Defrancq D., Vermeersch G.: Ischio-femoraal impingement: casus en literatuuroverzicht. Tijdschr voor Genees kunde, 2013, 69: 616-620.

3. Viala P., Vanel D., Larbi A., Cyteval C., Laredo J.D.: Bilateral ischiofemoral impingement in a patient with here-ditary multiple exostoses. Skeletal Radiol, 2012, 41: 1637-1640.

4. Tosun Ö., Çay N., Bozkurt M., Arslan H.: Ischiofemoral impinge-ment in an 11-year-old girl. Diagn Interv Radiol, 2012, 18: 571-573.

5. Taneja A.K., Bredella M.A., Torriani M.: Ischiofemoral impinge-ment. Magn Reson Imaging Clin N Am, 2013, 21: 65-73.

6. Torriani M., Souto S.C., Thomas B.J., Ouellette H., Bredella M.A.: Ischio-femoral impingement syndrome: an entity with hip pain and abnormalities of the quadratus femoris muscle. AJR, 2009, 193: 186-190.

7. Renoux J., Mercy G., Massein A., Brasseur J.-L.: Conflits proximaux et distaux des ischio-jambiers. In: Le tendon et son environnement. Edited by Bard H., Bianchi S., Brasseur J.-L, et al. Printed by Sauramps Médical, Montpellier, 2013, 321-328.

Conclusion

Ischiofemoral impingement should be considered in the differen-tial diagnosis of hip pain. As symp-toms and clinical findings are often non-specific, imaging plays a crucial role in the diagnosis, evaluation of the predisposing anatomy and treat-ment planning. Although plain radi-ography may be useful to demon-strate predisposing bony abnormal- ities (e.g. exostosis), causing nar-rowing of the ischiofemoral space, evaluation of secondary effect on the intervening soft tissue is not possi-ble. MRI is the preferential tool for direct assessment of both narrowing of the ischiofemoral space and asso-ciated soft tissue edema in the qua-dratus femoris muscle.

References

1. Johnson K.A.: Impingement of the lesser trochanter on the ischial ramus after total hip arthroplasty. Report of three cases. J Bone Joint Surg Am, 1977, 59: 268-269.

i.e. the muscle belly. Additional imaging findings are edema adjacent to the hamstring- and iliopsoas tendons and formation of an inter-vening bursa along the medial as-pect of the lesser trochanter. Long-standing IFI may lead to muscle atrophy and fatty infiltration (2, 5). CT imaging may be useful to docu-ment narrowing of the bony ischiofe-moral tunnel in the preoperative set-ting, but is usually not recommended due to radiation restraints. In pa-tients with large bony protuberanc-es, such as an exostosis in our pa-tient, it may allow a precise preoperative mapping for the sur-geon, in cases where surgery is con-sidered.

In most cases of IFI treatment is initially conservative, consisting of rest, nonsteroidal anti-inflammatory drugs, steroid injections and physio-therapy. Surgical treatment is re-stricted for patients in whom conser-vative treatment fails or with a marked narrowing of the IFS, such as in our patient.

schatteman-.indd 42 19/02/15 15:56

Macrodystrophia lipomatosa (MDL) is a congenital progressive gigan-tism of the hand and foot, which may be local or may involve the entire limb. Macrodystrophia lipomatosa comes to clinical attention mostly because of cosmetic reasons, and rarely due to development of neuro-vascular compression or mechanical problems like secondary degenera-tive joint disease. Magnetic reso-nance imaging (MRI) is considered pathognomonic of MDL and helps to differentiate it from other causes of localized gigantism (1). Although MDL of hand is more common along median nerve distribution; we report a rare case of MDL in a 20 years old female involving ring and little fin-gers of right hand with predominant ulnar nerve distribution.

Case report

A twenty-year-old girl presented to outpatient clinic of orthopaedics department with long standing swelling of the medial aspect of right hand. According to the history, she was born with disproportionately large medial two fingers with pro-gressive growth in relation to the re-maining digits, more marked in the ring finger. She had no pain or neu-rovascular symptoms. On examina-tion, the patient had hypertrophy of the ring and the little finger (Fig. 1A). The other hand was normal. Swell-ing was firm on palpation without any indurations or tenderness. As-sessment of the other systems showed no abnormality. Patient was subjected to routine lab examination along with radiological investiga-

proliferation of normal-appearing adipose tissue. Patient was diag-nosed as a case of macrodystrophia lipomatosa. Patient was advised cosmetic and reconstructive surgery.

Discussion

Macrodystrophia lipomatosa is a congenital progressive gigantism of the hand and foot that may be local-ized or may involve the entire limb. It is characterized by proliferation of all mesenchymal components, particu-larly fibroadipose tissue, most fre-quently in the distribution of the median nerve in the upper extremity and in the distribution of the plantar nerves in the lower extremity. Radio-graphs reveal soft-tissue and osse-ous overgrowth often with elongated, broadened, and splayed phalanges

tions, namely Radiograph & MRI. Ra-diograph of the right hand revealed enlarged phalanges of the ring and the little finger with prominent over-lying soft tissue (Fig. 1B). MRI showed gross thickening and hyper-trophy of osseous and soft tissue component. The soft tissue appeared hyperintense on both T1 and T2 weighted sequences (Fig. 2 A, B) with evidence of suppression of bright signal on fat-suppressed STIR sequence (Fig. 3 A, B) consistent with fatty proliferation of the soft tis-sues. Small tissue was biopsied for histological analysis, which revealed

JBR–BTR, 2015, 98: 43-44.

MACRODYSTROPHIA LIPOMATOSA WITH ULNAR DISTRIBUTION IN HAND: MR EVALUATION OF A RARE DISORDERM. Azfar Siddiqui1, M. Ahmad1, N. Redhu1, I. Ahmad1, E. Ullah1

Macrodystrophia lipomatosa is a rare, non-hereditary congenital anomaly characterized by localised gigantism in the form of macrodactyly affecting a part of limb and rarely entire limb due to overgrowth of all mesenchymal elements. Radiological and pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tis-sue, nerve sheaths, and periosteum that lead to soft tissue and bony enlargement. We present the case of a twenty years old female who presented with history of gradual enlargement of the medial aspect of right hand along ulnar nerve distribution since birth. MRI showed hypertrophy of bones and soft tissue with fatty proliferation, leading to the diagnosis of macrodystrophia lipomatosa.

Key-word: Fingers and toes, abnormalities.

From: 1. Department of Radiodiagnosis, Jawaharlal Nehru Medical College, A.M.U. Aligarh, India.Address for correspondence: Dr M. Azfar Siddiqui, M.D., Department of Radiodiagno-sis, Jawaharlal Nehru Medical College, Aligarh, India-202002.E-mail: drazfarsiddiqui@gmail.com

A BFig. 1. — A. Clinical photograph of the right hand of the patient showing diffuse en-

largement of the ring and the little fingers. B. Antero-posterior radiograph of the hand shows enlargement of phalanges of right 4th and 5th digit with prominent overlying soft tissue.

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44 JBR–BTR, 2015, 98 (1)

References

1. Blacksin M., Barnes F.J., Lyons M.M.: MR diagnosis of macrodystrophia li-pomatosa. AJR Am J Roentgenol, 1992, 158: 1295-1297.

2. Singla V., Virmani V., Tuli P., Singh P., Khandelwal N.: Macrodystrophia li-pomatosa – Illustration of two cases. Indian J Radiol Imag, 2008, 18: 298-301.

3. Gupta S.K., Sharma O.P., Sharma S.V., Sood B., Gupta S. Macrodystro-phia lipomatosa: radiographic obser-vations. Br J Radiol, 1992, 65: 769-773.

4. Stuart R.M., Koh E.S., Breidahl W.H.: Sonography of peripheral nerve pa-thology. AJR Am J Roentgenol, 2004, 182: 123-129.

5. Soler R., Rodríguez E., Bargiela A., Martınez C.: MR findings of macro-dystrophia lipomatosa. Clin Imaging, 1997, 21: 135-137.

6. Jamis-Dow C.A., Turner J., Biesecker L.G., Choyke P.L.: Radiologic manifes-tations of Proteus syndrome. Radio-graphics, 2004, 24: 1051-1068.

7. Ho C.A., Herring J.A., Ezaki M.: Long term follow-up of progressive macro-dystrophia lipomatosa. J Bone Joint Surg Am, 2007, 89: 1097-1102.

syndrome, also known as Wiede-mann’s syndrome is characterized by mosaic overgrowth of skin, bones, muscles, fatty tissues, blood and lymphatic vessels, as well as by vis-ceromegaly, lung cysts, and predis-position to benign and malignant tu-mors (6). Finally, in neurofibromatosis cutaneous manifestations of the dis-ease (cafe au lait macules, neurofi-bromas, freckling in the axillary and inguinal regions) and positive family history are typically present.

As the patient seeks medical ad-vice usually for cosmetic reasons, surgical correction is the manage-ment of choice. The surgery is usu-ally planned after puberty when the growth of affected parts stop. Multi-ple debulking procedures, osteoto-mies and epiphysiodesis may be re-quired for satisfactory outcome (7).

(2). The imaging appearance, partic-ularly with sonography and MRI is usually distinctively characteristic and reflects the underlying disease. Sonography reveals alternating hy-perechoic (fat) and hypoechoic (nerve fascicles) bands in a diffusely enlarged nerve, thus creating a ca-ble-like appearance (3, 4). MR imag-es are similar, with longitudinally oriented cylindrical areas of low to intermediate signal intensity (nerve fascicles) surrounded by high signal intensity thickened adipose tissue (5). MDL should be differentiated from other forms of congenital local-ized gigantism such as Klippel-Tre-naunay syndrome (KTS), Proteus syndrome and neurofibromatosis. KTS is differentiated from MDL by presence of varicose veins and anomalous lymph system. Proteus

Fig. 2. — Coronal (A) and sagittal (B) T1-weighted MR image of the right hand reveals enlargement of phalanges of 4th and 5th digit with gross thickening and hypertrophy of overlying soft tis-sue component that shows hyperintense signal intensity.

Fig. 3. — Coronal (A) and axial (B) STIR sequence showed sup-pression of bright signal intensity of soft tissue suggesting fatty proliferation consistent with the diagnosis of macrodystrophia lipomatosa.

azfar siddiqui-.indd 44 19/02/15 15:57

Case report

A 33-year-old man was referred by his general practitioner to the de-partment of orthopaedic surgery with persisting pain and impression of a growing mass of the left shoul-der. The patient had a medical histo-ry of osteochondromas of the knee and pelvis in the context of Multiple Hereditary Exostosis (MHE). One le-sion has previously been resected because of high risk for malignant transformation. There was no recent history of trauma.

Clinical inspection of the left shoulder girdle illustrated a soft tis-sue swelling medial from the proxi-mal humeral diaphysis. Clinical ex-amination showed a general painful shoulder mobility and minimal loss of range of motion with active endo- and exorotation.

Conventional imaging of the left shoulder showed a well defined bony lesion extending from the proximal humeral metaphysis to di-aphysis with a pattern of chondroid matrix mineralisation suggesting an osteochondroma. Accessory bone fragments were located in the sur-rounding axillary soft tissues (Fig. 1). Further assessment of the lesion was done by MRI (fig. 2) Illustrating an osteochondroma surrounded by a thin walled fluid collection postero-medial. Cystic wall and surrounding soft tissue enhancement post con-trast injection suggested the diagno-sis of frictional bursitis. The bone fragments illustrated on radiography were situated in this bursa and showed a similar pattern of chon-droid mineralisation suggesting these are primary originating from the osteochondroma.

The patient was successfully treat-ed with bursectomy and resection of the osteochondroma with relief of his discomforts.

Discussion

An osteochondroma is defined as a hyaline cartilage capped lesion of cortical and medullary bone arising on the external surface of a primary or parental bone. Continuity of le-sional cortex and medullary canal with the underlying bone is pathog-nomonic for osteochondromas (1).

The diagnosis was made of ‘exos-tosis bursata’: frictional bursitis secondary to an osteochondroma. Osteochondral fragments of differ-ent shape and size became separat-ed from the primary osteochondral lesion and migrated into the bursa resulting in a secondary form of synovial osteochondro matosis.

JBR–BTR, 2015, 98: 45-47.

OsteOChOnDrOma Of the prOximal humerus with friCtiOnal bursitis anD seCOnDary synOvial OsteOChOnDrOmatOsisJ. De Groote1,2, B. Geerts1,2, K. Mermuys1, K. Verstraete1,2

we report a case of multiple hereditary exostosis in a 33-year old patient with clinical symptoms of pain and impression of a growing mass of the left shoulder alerting potential risk of malignant transformation of an osteo-chondroma. imaging studies illustrated perilesional bursitis surrounding an osteochondroma of the proximal humerus. malignant transformation was excluded with mri. fragments of the osteochondroma were dislocated in the inflammatory synovial bursa illustrating a case of secondary synovial osteochondromatosis.

Key-word: Osteochondroma.

From: 1. Department of Radiology, AZ Sint Jan, Brugge, 2. Department of Radiology, UZ Gent, Ghent, Belgium.Address for correspondence: Dr J. De Groote, Department of Radiology, UZ Gent, De Pintelaan 185, 9000 Gent, Belgium. E-mail: jeroen.degroote@icloud.com

Fig. 1. — Conventional radiography of the left shoulder. A: AP view in neutral posi-tion: osteochondroma of the medial side of the proximal humerus. The lesion shows a pattern of rings and arcs to confluent calcifications. An accessory bone fragment (ar-row) projecting over the axillary soft tissues associated with a surrounding soft tissue component. A smaller isolated loose fragment is located close to the inferomedial bor-der of the primary lesion (arrowhead). B: AP view in exorotation: superposition of the isolated bone fragment and the superomedial border of the osteochondroma (arrow). Small focal hyperdensities are located inferior to the lesion associated by a surround-ing soft tissue mass (arrowhead).

A B

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46 JBR–BTR, 2015, 98 (1)

The lesion is usually located at the metaphysis of a long bone, most frequently in the distal femur but any bone developing from preformed cartilage may be involved (1).

The hyaline cartilage is difficult to assess on conventional radiography but may be suggested by the identifi-cation of rings and arcs or flocculent calcifications as the result of chon-droid mineralisation (1, 3).

MRI may also demonstrate corti-cal and medullary continuity and is considered the best imaging modal-ity to evaluate cartilage cap thick-ness and its surrounding soft tissues.

The high water content of the hya-line cartilage cap creates an interme-diate to low signal on T1-weighted sequences and a high signal on T2-weighted sequences. Mineralised portions in the cartilage cap remain low in signal on all MR pulse se-quences (2).

Thickness of the hyaline cartilage cap is the most important imaging finding considering the risk of malig-nant transformation to a secondary chondrosarcoma. Cartilage cap thickness of more than 15 mm in a skeletal mature patient should be considered with great suspicion (1). Other signs of malignant transfor-mation include growth of a previ-ously unchanged osteochondroma in a skeletal mature patient, irregular lesion surface, focal interior radiolu-cencies, erosion or destruction of ad-jacent bone and surrounding soft tis-sue mass formation containing irregular calcifications (1, 2, 4-6).

Osteochondromas usually are as-ymptomatic but may show compli-cations varying from cosmetic defor-mity, fracture, vascular or neurogenic compression, bursa formation and malignant transformation (1).

Anatomically an inconsistent bur-sa is located in the axilla in between the inferior angle of the scapula and the superior fibers of the latissimus dorsi muscle, more posterosuperior-ly a bursa is located between the ser-ratus anterior and the subscapularis muscle (5). Bursa neoformation be-tween an osteochondroma and the perilesional soft tissues has been de-scribed as “exostosis bursata”, a re-sult of mechanical impingement upon the adjacent muscles and ten-dons (1, 4). Bursae are lined by synovium and may become symp-tomatic due to inflammation, infec-tion or haemorrhage. Clinically, bursa formation may present as a painful growing perilesional mass, simulat-ing malignant transformation (1).

In rare cases chondral or fibrin fragments may be dislocated from

secondary enchondral ossification (maturation). Osteochondromas usually show a growth pattern similar to a normal physeal plate un-til skeletal maturity (2). Imaging characteristics of an individual os-teochondroma in HME is identical to solitary lesions.

An osteochondroma has the typi-cal radiographic appearance of a le-sion consisting of cortical and med-ullary bone continuous with the underlying parental bone.

Osteochondromas are the most common benign bone tumors (20-50%) or bone tumors in general (10-15%) (1, 2). The majority are solitary, non hereditary (85%) but lesions can also be multifocal in the context of HME, a disorder inherited in an auto-somal dominant manner (1, 2).

Development of an osteochondro-ma results from separation and dislocation of an epiphyseal growth plate fragment with persistent growth of the cartilage fragment and

Fig. 2. — MRI of the left shoulder. A: Axial T1 shows low intensity soft tis-sue component surrounding the osteo-chondroma with an isolated bone frag-ment (arrow) located in this soft tissue component. B,C: coronal STIR demon-strates hyper-intense synovial fluid col-lection (horizontal arrow) surrounding the osteochondroma and extending pos-terior of the proximal humerus, contain-ing an isolated bone fragment (arrow-head). D: on axial STIR, the posterobasal facet of the lesion is peripherally sur-rounded by a hyper-intense rim (arrow-head) representing the residual hyaline cartilage rim surrounded by a hypo-in-tense perichondrium. The cartilage rim has a maximal thickness of 4 mm. Central hypo-intensities represent calcific chon-droid mineralisation of hyaline cartilage, low in intensity on all MR pulse sequenc-es (arrow). E: axial T1 TSE + Gd shows contrast enhancement of the thin lined wall of the fluid collection representing a synovial bursa and surrounding soft tis-sues anterolateral of the cranial segment of the latissimus dorsi muscle (arrow).

C

▲

D

E

▲A

B

de groote-.indd 46 19/02/15 15:57

OSTEOCHONDROMA OF THE PROXIMAL HUMERUS — DE GROOTE et al 47

and Pathological Features. In Vivo, 2008, 22: 633-646.

3. Murphey M.D., et al.: From the Archives of the AFIP: Imaging of Synovial Chondromatosis with Radiologic-Pathologic Correlation. RadioGraphics, 2007, 27: 1465-1488.

4. Peh W.C.G., et al.: Osteochondroma and secondary synovial osteochon-dromatosis. Skeletal Radiology, 1999, 28: 169-174.

5. Shackcloth M.J., Page R.D.: Scapular osteochondroma with reactive bursi-tis presenting as a chest wall tumour. Eur J Cardiothorac Surg, 2000, 18: 495-496.

6. Wright J.M., Matayoshi E., Goldstein A.P.: Bursal osteochondromatosis overlying an osteochondroma of a rib. A case report. J Bone Joint Surg Am, 1997, 79: 1085-1088.

frictional bursitis, clinically present-ing with symptoms of pain and a growing mass, mimicking malignant transformation. We illustrated a case of exostosis brusata with fragments of the primary osteochondroma dis-located in the surrounding bursa as the result of friction with secondary synovial osteochondromatosis.

references

1. Murphey M.D., et al.: From the Archives of the AFIP: Imaging of Osteochondroma: Variants and Complications with Radiologic-Patho-logic Correlation. RadioGraphics, 2000, 20: 1407-1434.

2. Kitsoulis P., et al.: Osteochondromas: Review of the Clinical, Radiological

the osteochondroma into the bursa resulting in chondro-metaplasia in the synovial lining and secondary formation of multiple chondroid bodies as in primary synovial osteo-chondromatosis (1, 4).

Secondary synovial osteochondro-matosis is a more common finding in which several osteochondral bod-ies of different shapes and sizes are seen in a synovial fluid collection (3).

Conclusion

Osteochondromas are primary benign bone tumors with low risk of malignant transformation. Impinge-ment of surrounding soft tissues by an osteochondroma can result in

de groote-.indd 47 19/02/15 15:57

Case report

We report the case of a 73-year-old man with history of hypertension and paroxysmal atrial fibrillation, who presented with recent dyspnea. The patient was admitted to the hos-pital for evaluation and treatment. Echocardiography showed a hyper-trophied left ventricle with a systolic and diastolic dysfunction, no sig-nificant valve disease was found. Coronarography did not show vas-cular impairment.

CMR was performed for assess-ment of the heart. It revealed a bi-ventricular increase of wall thickness and dilated atria. The functional studies showed a decrease of sys-tolic function with an ejection frac-tion of 37%. Late gadolinium en-hancement images showed global subendocardial hypersignal of both ventricles and atria. Phase-contrast sequences acquired on the mitral valve and the pulmonary vein showed a type II diastolic dysfunc-tion (pseudonormalisation). Based on MRI findings, the diagnosis of cardiac amyloidosis was highly sug-gested. A subsequent rectal biopsy confirmed the amyloidosis.

Discussion

Amyloidosis is defined by an ex-tracellular deposit of fibrils which are composed of low molecular weight subunits. These deposits of fibrils lead to a histologic change of the af-fected tissues. The most common type is systemic light-chain amyloi-dosis (AL) and is due to deposition of protein derived from immunoglobu-lin light chain fragments, often asso-ciated with multiple myeloma or other monoclonal gammopathies. Cardiac involvement occurs in up to 50% or more of cases in AL.

the endocardial biopsy, but this is an invasive procedure and is related to severe complications. A strong diag-nostic suspicion can be made by identifying several specific bio-clini-cal and imaging findings (1, 2).

The differential diagnosis of amy-loid heart disease includes hyper-trophic cardiomyopathy, various causes of restrictive cardiomyopa-thies and hypertensive heart disease.

Echocardiography is a simple non invasive technique for the morpho-logical study and evaluation of myo-cardial function. However these indi-cators aren’t specific of cardiac amyloidosis.

MRI is a non-invasive and non-operator dependent technique. It al-lows a three-dimensional assess-ment of the heart and evaluates more accurately changes in cardiac function.

CMR imaging can provide evi-dence strongly suggestive of amy-loid cardiomyopathy. One of the characteristic features of cardiac am-

Cardiac involvement is also found in almost all cases of senile type amyloidosis (Wild-Type ATTR) and is frequent in some types of hereditary amyloidosis (variant ATTR) while it is rather uncommon in secondary amyloidosis (AA) complicating chronic inflammatory diseases (1).

The cardiac involvement in the amyloidosis is, in most cases, sec-ondary to a systemic disease. How-ever an isolated cardiac involvement can occur. Cardiac amyloidosis pres-ents as a restrictive cardiomyopathy with progressive diastolic and systolic dysfunction. Dyspnea and oedema are the most common clini-cal manifestations of heart failure due to cardiac amyloidosis.

The gold standard for the diagno-sis of a cardiac amyloidosis remains

JBR–BTR, 2015, 98: 48-49.

CARDIAC AMYLOIDOSIS DIAGNOSED ON MRIP. Lu1, H. Van Acker2, P. Waer1

The frequency of cardiac involvement varies among other types of amyloidosis. Cardiac amyloidosis leads to systolic and diastolic dysfunction with symptoms of heart failure. Cardiac magnetic resonance (CMR) findings are helpful in supporting the diagnosis of amyloid cardiomyopathy. We report a case of a 73-year-old man who presented with shortness of breath. Echocardiography showed a hypertrophic, diffusely hypocontractile left ventricle with a restrictive filling pattern. The diagnosis of an isolated amyloidosis was made on CMR.

Key-words: Amyloidosis – Heart, MR.

From: Department of 1. Radiology, 2. Cardiology, Europe Hospitals, Site Ste Elisabeth, Brussels, Belgium.Address for correspondence: Dr P. Lu, M.D., Radiology Department, Europe Hospitals, Site Ste Elisabeth, Av. De Fré 206, B-1180, Brussels, Belgium. E-mail: luanhtai2@gmail.com

Fig. 1. — bSSFP image in four chamber orientation show a biventricular increase of wall thickness and dilated atria.

lu-waer-.indd 48 19/02/15 15:58

CARDIAC AMYLOIDOSIS DIAGNOSED ON MRI — LU et al 49

References

1. Dubrey S.W., Hawkins P.N., Falk R.H.: Amyloid diseases of the heart: as-sessment, diagnosis, and referral. Heart, 2011, 97: 75-84.

2. Banypersad S.M., Moon J.C., Whelan C., Hawkins P.N., Wechalekar A.D.: Updates in Cardiac Amyloidosis: A Review. J AM Heart Assoc, 2012, 1.

3. Seeger A., Klumpp B., Kramer U., Stauder N.I., Fenchel M., Claussen C.D ., Miller S.: MRI assessment of cardiac amyloidosis : experience of six cases with review of the current literature. Br J Radiol, 2009, 82: 337-442.

4. Austin B.A., Wilson Tang W.H., et al.: Delayed Hyper-Enhancement Mag-netic Resonance Imaging Provides Incremental Diagnostic and Prognos-tic Utility in Suspected Cardiac Amy-loidosis. JACC Cardiovascular Imag-ing, 2009, 2: 1369-1377.

5. Imran S.: Syed Role of Cardiac Magnetic Resonance Imaging in the Detection of Cardiac Amyloidosis. JACC Cardiovascular Imaging, 2010, 3: 1369-1377.

6. Selvanayagam J.B., Leong D.P.: MR Imaging and Cardiac Amyloidosis. J Am Coll Cardiol Img, 2010, 3: 165-167.

shows particularly a distinctive pattern of global late gadolinium enhancement (LGE) rarely seen in other cardiomyopathies. A global subendocardial LGE is the dominant pattern that corresponds to the myocardial distribution of amyloid protein in the extracellular space that is expanded. A patchy focal LGE pattern can also occur, but is rare (5).

Among other there is a positive correlation between degree of LGE and markers of disease severity (NYHA, cardiac biomarkers) and prognosis (6).

Treatment of the different types of amyloidosis varies with the cause of fibril production. Cardiac amyloido-sis is generally diagnosed when there are already morphologic changes apparent in echocardiogra-phy. LGE-CMR represents a good way for screening with an adequate sensitivity and specificity of a sub-clinical amyloid infiltration. It allows to detect a cardiac involvement ear-lier than could otherwise be possible by morphologic assessment and therefore could change the clinical course and prognosis.

yloidosis by CMR imaging is an in-creased ventricular wall thickness. A thickening of the interatrial septum and right atrial free wall is highly specific (3). Pericardial effusion is of-ten present (4). CMR can also con-firm the presence of myocardial dys-function.

Finally, the “late gadolinium enhancement” (LGE) technique has an established role in diagnosis. It

Fig. 3. — Phase contrast sequences acquired on pulmonary vein S < D (A) and mitral valve 0.75 < E/A < 1.5 (B) show a moderate diastolic dysfunction (type II).

Fig. 2. — Late gadolinium enhance-ment images with inversion recovery saturation of the myocardium in short axis (A, B) and four chamber orientation (C) show classic amyloid global, suben-docardial LGE pattern in both ventricle and atria.

A

C

B

A

B

lu-waer-.indd 49 19/02/15 15:58

Gaucher disease presenting with vertebral compres-sion fractures and vertebral osteonecrosis

G. Clinckemaillie1, A. Larbi2, P. Omoumi2, J. Manelfe3, B. Dallaudière2,4

A 42-year-old man with no relevant medical history complaining of spontaneous thoracolumbar back pain was referred for an MRI of the spine after initial radio-graphic evaluation had demonstrated several compression fractures.

In addition to confirming the dorsal and lumbar compression fractures and some degenerative changes, the MRI demonstrated multifocal bone marrow anomalies with well-defined geographical zones of low signal on T1-weighted images (Fig. A) and intermediate to high signal on STIR images (Fig. B) in dorsal and lumbar verte-bral bodies as well as in the sacrum (closed arrows in both figures). In some verte-bral bodies, and in particular in the sacrum, a subtle double-line sign was present (arrow head in Figure B), suggesting the diagnosis of osteonecrosis.

After ruling out common causes of multiple vertebral osteonecrosis and taking the Ashkenazic Jewish descent of the patient into account, the diagnosis of Gaucher disease was proposed. Genetic testing confirmed the diagnosis. Supportive therapy was quickly started after the diagnosis was confirmed in this patient. Enzyme replacement therapy (ERT) might be offered in nonneuropathic GD but its high cost and the variable response make it necessary to define appropriate clinical indica-tions. ERT was not started in this patient.

Comment

Gaucher disease (GD) is an autosomal recessive metabolic disorder caused by deficiency of the enzyme glucocerebrosidase and hence characterized by the abnor-mal deposition and accumulation of glucocerebroside in the lysosomes of reticulo-endothelial cells (“Gaucher cells”). These cells can accumulate in the spleen, liver, kidneys, lungs, brain and bone marrow. Symptomatology, organ involvement and clinical course vary greatly among affected patients. Despite this heterogeneity, three basic clinical forms have been identified, based on the degree of neurological involvement. Most patients have the nonneuropathic form, referred to as type 1 GD or adult GD. Types 2 and 3, respectively acute and subacute neuropathic forms, occur in the remainder of the patients. Systemic symptoms are more common than neurological involvement in patients with GD.

Accumulated Gaucher cells in the bone marrow replace the normal cellular popu-lation, causing ischaemia and as a result edema, which is clinically accompanied by pain. Bone adjacent to marrow infiltration may exhibit cortical thinning or scallop-ing, osteopenia and deformity. Further accumulation of Gaucher cells leads to frac-tures, necrosis, and less frequently osteomyelitis. In the axial skeleton, multiple compression fractures can be observed, leading to kyphosis and eventually to spinal cord compression. The involved vertebral bodies may become completely flattened. Less frequently, H-shaped, step-like defects in the vertebral bodies may be observed.

On MRI, the described infiltration of the bone marrow with accumulating Gaucher cells will be manifested as a decrease in T1- and T2-signal intensity, and may display a homogeneous or heterogeneous pattern. MRI is the most sensitive imaging mo-dality to demonstrate osteonecrosis. The findings will depend on the stage of necro-sis, as described in the Mitchell classification, based on the signal characteristics within the central lesion. Class A is early osteonecrosis, with signal intensity charac-teristics analogous to those of fat, being high signal intensity on T1-weighted im-ages and intermediate signal intensity on T2-weighted images. Class B is manifested as a high signal on T1- and T2-weighted images, comparable to signal intensity characteristics of blood. Class C demonstrates signal intensity characteristics similar to those of water, with low signal on T1- and high signal on T2-weighted images. Class D is the most advanced stage and demonstrates low signal intensity on both

T1- and T2-weighted images, resulting from fibrous tissue proliferation. In GD, areas of necrosis are typically multiple, with well-defined, serpiginous margins, as was the case in the presented patient.

Reference

1. Wenstrup R.J., Roca-Espiau M., Weinreb N.J., et al.: Skeletal aspects of Gaucher disease: a review. BJR, 2002, 75: A2-A12.

JBR–BTR, 2015, 98: 50.

IMAGES IN CLINICAL RADIOLOGY

1. Department of Radiology, Universitaire Ziekenhuizen Leuven, Leuven, Belgium, 2. Department of Radiology, Cliniques Universitaires St-Luc, Brussels, Belgium, 3. Department of Radiology, Hôpital Bichat – Claude-Bernard, Paris, France, 4. Université Paris Diderot, Paris, France.

A

B

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Combination of unusual lesions after blunt trauma

L. Médart1, P. Lamborelle2, L. Collignon1

A 59 year-old man fallen from a roof covering 3 meters high, was carried to the emergency department. A short loss of consciousness was noted with scalp wound. Patient major complaint at arrival was sternal pain. Body-CT was realised for this blunt trauma. There was no cerebral trauma except left parietal cutaneous abrasion. An uncom-mon association of left cartilage fractures from piece 1 to 6 (Fig. A, B) and right adrenal haemorrhage with small retroperitoneal hematoma was diagnosed (not shown). The patient was observed in the intensive care unit for 5 days and 2 days more in gastroenterology department. He came back to hospital 1,5 month later for dyspnea and left thoracic pain. Chest radiography and CT revealed a huge right pleural effusion (Fig. C) and some healing right anterior arch costal fractures revealed by callus formation. Second look to basal body-CT did not find those not displaced rib lesions. A possible post-traumatic late chylothorax was suggested. He was definitively discharged 5 days after surgical pleural treatment via thoracotomy.

Comment

Body-CT is a daily practice in emergency radiologic department. Ever increasing number of images and pressure related to urgent man-agement make body-CT interpretation not so easy and may become in some circumstances an uncomfortable task for the radiologist. Un-common injuries may be present or associated as in this case report. This presentation also illustrates the fact that some lesions may be missed and that delayed injuries may appear.

Costal cartilages are easily recognized at CT (1), their shapes are well-known and their density is higher compared to direct environ-ment. Fracture classically corresponds to focal interruption of the cartilage with or without displacement. No gas was isolated in the six cartilage fractures.

Late post-traumatic chylothorax is rare. Chylothorax develops in penetrating and less often blunt trauma by damage to the thoracic duct and collection of chyle within the chest. Management combines inter-costal drainage and total parenteral nutrition to reduce chyle flow. When this conservative treatment fails, surgery consists in thoracic duct revision (repair of focal wound or ligation) and pleurodesis.

Adrenal gland trauma is present on 1-2% of CT imaging for blunt trauma although the occurrence is thought to be much higher. The right adrenal gland is more commonly affected with a ratio of 3-4/1. The only way to exclude a pre-existing adrenal mass is to compare with prior or further imaging test. Isolated adrenal gland trauma is uncommon (< 5%).

Body-CT seems very sensitive and specific concerning the two acute diagnoses exemplified in this traumatic history. CT is also the key diagnostic tool to handle delayed traumatic events.

Reference

1. Malghem J., Vande Berg B.C., Lecouvet F.E., Maldague B.E.: Costal cartilage fractures as revealed on CT and sonography. AJR, 2001, 176: 429-432.

JBR–BTR, 2015, 98: 51.

IMAGES IN CLINICAL RADIOLOGY

1. Department of Radiology, CHR Citadelle, Liège, Belgium, 2. Radiology assistant, CHU Sart Tilman, Liège, Belgium.

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Umbilical artery pseudoaneurysm

S. Tribolet1, J. Khamis2, M. Lewin2, T. Khuc3

A 5-week-old baby boy was referred to ultrasonography because of a discharging umbilical granuloma (UG) not re-sponding to silver nitrate applications. He had no medical history, birth occurred without complications. Ultrasonogra-phy (US) found an anechoic 5 mm umbilical mass (Fig. A). Doppler color (Fig. B, C) revealed a turbulent high velocity flow within the anechoic component. Surgery confirmed the diagnosis of Umbilical Artery Pseudoaneurysm (UPA). The infant was treated with ligation and resection of the lesion. Resected fragments show a fibrous tissue containing an arterial vascular structure which media is thickened and calcified.

Comment

Pseudo-aneurysm is defined as an extravascular hemato-ma secondary to a traumatic lesion of the vascular wall. The surrounding tissues contain blood. The arterial wall is weakened and hence there is a risk of rupture and of major bleeding. UPA is a rare condition in infant. There is only one case reported in the literature, related to an umbilical artery catheterization (1).

Clinically UPA can be misdiagnosed as umbilical granu-loma (UG). UG is a pinkish discharging mass, which corre-sponds to a residual umbilical cord. Sometimes it contains urachal or intestinal remnants. UG is usually treated by ap-plication of silver nitrate stick. In some patients, however, the discharge may not disappear. These cases should be re-ferred to ultrasonography and color-Doppler. In case of an anomaly of the urachus, the omphalomesenteric duct or the vascular component, surgical exploration is mandatory for resection and anatomo-pathological analysis.

Reference

1. Katz M., Perlman J., et al.: Neonatal Umbilical Artery Pseudo-aneurysm: sonographic Evaluation – Case Report. AJR, 1986, 147: 322-324.

JBR–BTR, 2015, 98: 52.

IMAGES IN CLINICAL RADIOLOGY

1. Student, Université of Medicine, Liège, 2. Departement of Pediatric Radiology, 3. Departement of Pediatric Surgery, Clinique de l’Espérance, CHC de Liège, Liège, Belgium.

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Extrapulmonary manifestation of lymphan-gioleiomyomatosis

N. Favoreel, S.F. Van Meerbeeck, R. Gosselin1

A 31-year-old female patient presented at the department of hema-tology with painful nodal swellings in the neck region for 2 months. Four weeks before presentation, she had an airway infection during which the nodes were swollen. Since 10 days she suffered from dys-pnea, a dry cough and a burning ache in the chest. There was no fever, neither hemoptoe.

At clinical examination painful supraclavicular nodes on the left side and along the sternocleidomastoid muscle on the right side were found and interpreted as lymph nodes. A blood investigation showed known ferriprive anemia but no reactive lymphocytosis. Chest x-ray was nor-mal (not shown) despite a clear scoliosis.

Some weeks later the patient mentioned that the nodes appeared to be fluctuating in volume, especially to be enlarged in a supine position or when bending over. There was increasing discomfort when wearing her seatbelt. An ultrasound of the painful neck region was performed and showed multiple fluid collections (not shown), probably mutual communicating. The collections enlarged in a supine position com-pared to a prone position. There was no flow in the collections.

Subsequently, a CT of the chest and abdomen was done and showed multiple thin-walled cystic masses along the sternocleidomastoid and scalene muscles (Fig. Aa), descending in the mediastinum (Fig. Ba). These cystic masses continued along the thoracic aorta to the abdomi-nal retroperitoneum and further down along the iliac arteries, to stop at the inguinal canal (Fig. Ab). The luminal density of these masses mea-sured 40HU in the thorax and 10HU in the abdomen. In the abdomen late enhancement of the thin cystic wall could be noted.

Along these findings the patient had multiple thin walled cysts in the lung with a perihilar predominance (Fig. C), which were the key to make the diagnosis of lymphangioleiomyomatosis or LAM.

No renal angiomyolipoma was found, nor was there any chylous ascites. No symptoms of tuberous sclerous complex were found. This was confirmed by MRI. The patient had no history of pneumothorax.

Two months after the initial diagnosis, the patient presented at the emergency department with dyspnea, coughing, hemoptoe and burn-ing chest ache. CT showed a chylothorax which was treated with thora-centesis (Fig. Bb). Pleural fluid was positive for chylomicrons.

Comment

LAM is a disease that presents typical in women of reproductive age. It is a proliferation of disorderly smooth muscle growth in the lungs, kidneys and the lymphatics. The pathogenesis is unknown but the latest data suggest that there is loss of tumour suppression function of some proteins, or that there are abnormalities in the enzymes for the synthesis of cate-cholamines. It is also clear that estrogen plays a role in the disease as it is almost only observed between menarche and menopause and it is known that there is disease progression during pregnancy and disease cessation after ovarectomy.

The pulmonary manifestations are the typical formation of small lung cysts. The atypical findings are pulmonary hyper-tension, pneumothorax and chylothorax. The extrapulmonary manifestations include chyloperitoneum, lymphangio-leiomyomas, renal angiomyolipomas and meningiomas. Our patient showed lymphangioleiomyomas in the lower neck extending to the mediastinal and retroperitoneal lymphatic system, and lung cysts.

Patients with LAM can present with progressive dyspnea, spontaneous pneumothorax, hemoptysis or other more common pulmonary complaints as wheezing, coughing or chest pain.

The 10-year survival rate ranges from 49-79%. It is a progressive disease and leads to respiratory failure. LAM can occur with increased frequency in patients with tuberous sclerosis complex (TSC), an autosomal dominant disorder due to muta-tions in the TSC1 or TSC2gene.

LAM can be diagnosed using CT, which is perfect for detecting the typical lung cysts, lymphangioleiomyomas and renal angiomyolipomas. Other techniques as MRI and ultrasound can also aid to the diagnosis.

References

1. Pallisa E., et al.: Lymphangioleiomyomatosis: Pulmonary and Abdominal Findings with Pathologic Correlation. RadioGraphics, 2002, 22: S185-S198.

2. Abbott G.F., et al.: Lymphangioleiomyomatosis: Radiologic-Pathologic Correlation. RadioGraphics, 2005, 25: 803-882.

JBR–BTR, 2015, 98: 53.

IMAGES IN CLINICAL RADIOLOGY

1. Department of Radiology, University Hospital of Ghent, Belgium.

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Arachnoidal cyst arising from the oculo-motor cistern

M. Eyselbergs1, P. Cheecharoen1, A. Bali1, C. Venstermans1, F. De Belder1, Ö. Özsarlak1, J. Van Goethem1, T. Menovsky2, M. Lammens3, F. Vanhoenacker1,4, P.M. Parizel1

A 36-year-old man with proptosis of the left eye complained of frontal headache. His medical history was relevant for amblyopia and loss of visual acuity on the left side. Opthalmological examina-tion revealed an oculomotor nerve paralysis of the left eye. Sub-sequent Magnetic Resonance Imaging (MRI) showed a well delin-eated T2 hyper- (asterisk in Fig. A) and T1 hypointense (not shown) cystic intraorbital mass. The lesion further extended intracranially through the superior orbital fissure in close proximity with the left oculomotor cistern (Fig. B, oculomotor cistern right (arrow) and cystic lesion (arrowhead) at the level of the oculomotor cistern left). The left optic nerve was displaced superomedially and was clearly delineated along its further course. After intravenous gadolinium administration, faint peripheral enhancement was noted (arrow in Fig. C). The MRI findings are compatible with an arachnoidal cyst arising from the oculomotor nerve cistern.

Surgical fenestration of the lesion was performed by a left-sided pterional approach. Histopathological analysis of the cyst wall showed collagenous tissue lined with arachnoidal cells, in keeping with an arachnoidal cyst arising from the oculomotor nerve cistern.

Comment

The oculomotor nerve cistern (OMC) is an arachnoid-lined dural cuff containing the oculomotor nerve as it enters the cavernous sinus roof. This variably sized cistern is filled with cerebrospinal fluid (CSF) and is continuous with the basal cistern. The opening of the OMC on the roof of the cavernous sinus is called the porus. From there on, the OMC is then gradually tapered along its course in the cavernous sinus and terminates below the tip of the anterior clinoid process. The oculomotor nerve more distally passes through the superior orbital fissure to enter the orbital apex.

An arachnoid cyst is a fluid filled cavity within the arachnoid membrane. On MRI, the content resembles CSF on all sequences. Subtle peripheral enhancement may be seen. Oblique sagittal orientated T2-WI may be helpful to visualize the course of the ocu-lomotor nerve. Various other lesions can arise in the OMC such as schwannoma, cavernous hemangioma, lymphoma, lymphangio-ma, metastases, dermoid and epidermoid cysts. The presence of an arachnoid cyst arising from the OMC is rare. Because of the inti-mate relationship with the oculomotor nerve, OMC lesions usually cause oculomotor nerve palsy. Therapy includes fenestration of the cyst with accompanying decompression and relief of the mass effect on the oculomotor nerve.

Reference

1. Kim M.K., Choi H.S., Jeun S.S., Jung S.L., Ahn K.J., Kim B.S.: Arachnoid cyst in oculomotor cistern. Korean J Radiol, 2013, 14: 829-831.

JBR–BTR, 2015, 98: 54.

IMAGES IN CLINICAL RADIOLOGY

1. Department of Radiology, 2. Department of Neurosurgery, 3. Department of Histopathology, Antwerp University Hospital, Edegem, 4. Faculty of Medicine and Health sciences, Ghent University, Ghent.

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Contribution of color Doppler sonography to the characterization of an unusual thick-ening of the common bile duct

N. Michalakis1, D. Van Gansbeke1

A 36-year-old man living in Algeria developed abdominal pain, asthenia, jaundice, and vomiting. He had no medical or surgical history. Laboratory tests revealed anemia, leukopenia, cytolysis, cholestasis and hyperbilirubinemia. Gastroscopy showed esopha-geal varices and duodenal cap ulcerations suggestive of portal hypertension. Abdominal sonography displayed a thickening of the common bile duct, splenomegaly and absence of gallbladder stones.

Two months later, the patient was admitted in our institution for progressive asthenia. A contrast enhanced CT scan was performed, showing a tubular soft-tissue enhancing mass extending from the porta hepatitis to the head of the pancreas within the hepato-duo-denal ligament. Intra and extrahepatic bile ducts were not dilated. The main portal vein and the intrahepatic branches of the portal vein appeared normal on axial CT scans. An additional ultrasound was performed, showing a non-dilated common bile duct (arrow Fig. A) with a marked thickening of the common bile duct wall and small serpiginous vessels within the thickening either in power Doppler as in color Doppler (arrowheads Figs. B and C). Addition-ally, a short and thigh narrowing of the main portal vein proximally to the portal division was disclosed, and secondarily confirmed on the multiplanar reformations of the contrast enhanced CT scan. A medullary biopsy was performed in the context of bicytopenia and splenomegaly and showed myelofibrosis.

Comment

Cavernous transformation of the portal vein refers to multiple wormlike vessels at the porta hepatitis and hepato-duodenal, which represent periportal collateral circulation. This pattern is observed in long standing portal vein thrombosis or occlusion. Usually, the portal vein cavernoma appears as a sponge-like mass around the main portal vein, and is independent of the biliary tree. Rarely,

veins within the wall of the common bile duct may be involved by the cavernomatous transformation, leading to an important wall thickening of the common bile duct. On B-mode sonogram, this thickening of the bile duct wall may be undistinguishable from other pathologic conditions like AIDS, cholangitis, cholangiocarcinoma, hepatocellular carci-noma, non-Hodgkin lymphoma or metastases. Color Doppler sonography easily helps to make a rapid distinction between portal vein cavernoma involving the biliary tree and other causes of bowel wall thickening. Myelofibrosis is a type of myeloproliferative neoplasm who is characterized by a disorder of the bone marrow. The annual incidence of myeloproliferative neoplasm is 2,1-3,5 per 100.000 peoples. In a retrospective study of 102 patients with myelo-proliferative disorders, the rate of thromboembolic complications in patients with myelofibrosis was 13,8%.

Reference

1. Denys A., Hélénon O., Lafortune M., Corréas J.-M., Patriquin H., Moreau J.-F., et al.: Thickening of the Wall of the Bile Duct Due to Intramural Collaterals in Three Patients with Portal Vein Thrombosis. AJR, 1998, 171: 455-456.

JBR–BTR, 2015, 98: 55.

IMAGES IN CLINICAL RADIOLOGY

1. Department of Radiology, Erasme Hospital, Brussels, Belgium.

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Foix-Chavany-Marie or opercular syndrome

P. Gillardin1, R. Van Eetvelde1, T. Kostermans2, R. De Potter3 , D. Dewilde1, M. Lemmerling1

A 69-year-old male was admitted to the emergency department with suspicion of a recurrent cerebrovascular accident (CVA). Medical history included a prior CVA in the vascular supply area of the left middle cerebral artery in 2010, due to a patent foramen ovale, causing cardiogenic emboli (Fig. A). The patient was re-spectively treated with anticoagulation and percutaneous closure of the fora-men; a significant motoric aphasia persisted. At a neurologic clinical investiga-tion, besides the known aphasia, apraxia of the tongue, dysarthria and a limited left hemisyndrome were noted with predominant paresis of the left hand and impairment of the left visual field. Imaging was requested, with CT only showing age-related atrophic changes and sequelae of left middle cerebral artery infarc-tion. MRI revealed additional diffusion restriction in the right opercular region, suggestive of recent stroke (Fig. B).

Holter monitoring revealed paroxysmal atrial flutter, confirming the suspicion of cerebral infarction as the basis of cardiogenic emboli. Following the recent ischaemia, the speech-language pathologist noticed the patient was not able to eat, but when food was deposited in his mouth, no eating difficulties or swallow-ing impairment were observed. Patient was treated with anticoagulant regimen, percutaneous entero-gastrostomy, anti-epileptic drugs and neurologic revalida-tion.

Comment

Facio-labio-pharyngo-glosso-masticator paralysis, also known as Foix- Chavany-Marie or opercular syndrome, consists of dissociation between automatic and voluntary movements. Bilateral lesions in the operculum cause a loss of volitional control of lingual, pharyngeal, facial and masticatory musculature, with pre-served automatic movement. Typical manifestations include difficulties in mouth opening, tongue protrusion, chewing and swallowing food as well as speech impairments. Opercular syndrome may be congenital or acquired (as is the case

in our patient), and intermittent or persistent. Possible causes of opercular syndrome include cerebrovascular disease (acute setting), CNS infections (subacute form), neuronal migration disorders (developmental), neurodegenerative disorders or epilepsy (reversible). Most reported cases, like our case, can be attributed to thrombo-embolism of mid-dle cerebral artery branches, which irrigate the operculum.

The operculum represents the cortex surrounding the insula, and can be divided into three parts: the frontal oper-culum, the frontoparietal operculum and the temporal operculum; with variable involvement of the subcortical white matter. Bilateral lesions in these areas clinically mimic a pseudobulbar palsy in the distribution of the 5th, 7th, 9th, 10th and 12th cranial nerves. The so-called “automatic-voluntary dissociation” can be elucidated by analysis of functional neuro-anatomy. Volitional control of the facial, oral and pharyngeal musculature demands intact motor cortices and pyramidal pathways; pathology in these areas leads to a selective palsy of voluntary use of these muscle groups. Emotional or spontaneous use of these muscle groups however require intact extrapyramidal pathways as well as parts of the hypothalamus and thalamus, thus explaining the dissociation between automatic and voluntary move-ments.

Some reports have also been made of patients with bilateral opercular syndrome who presented with only a unilateral lesion; it is believed that an occult underlying lesion on the contralateral side would account for these manifestations.

Imaging thus plays an important role in detection of opercular lesions, most frequently by detection of recent stroke (by way of diffusion weighted imaging), allowing adequate management.

Reference

1. Lekhjung T., Paudel R., Rana P.V.S.: Opercular syndrome: case reports and review of literature. Neurology Asia, 2010, 15: 145-152.

JBR–BTR, 2015, 98: 56.

IMAGES IN CLINICAL RADIOLOGY

Department of 1. Radiology, 2. Physiotherapy and Rehabilitation, 3. Neurology, AZ Sint-Lucas, Gent, Belgium.

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Sclerosing hemangioma of the lung

S. Van Petegem, J. De Cock, B. Houthoofd, A. Annicq, K. Verstraete1

A 28-year-old woman was referred to the radiology department for further work-up of an incidental finding of a nodular lesion on a routine x-ray of the chest. At the time of presentation, the woman was in excellent condition and had an unremarkable personal med-ical history. The chest x-ray demonstrated a well-defined lesion measuring 25 mm, confined to the left pulmonary hilum. Based on the patient’s age and x-ray findings, a bronchogenic cyst was suspected. A CT scan of the chest was performed to confirm the presumptive diagnosis, revealing a well-defined lesion with homo-geneous soft tissue density (50 Hounsfield Units), without calcifica-tions or fatty components (Fig. A). The lesion showed inhomoge-neous enhancement after intravenous contrast administration, excluding a bronchogenic cyst with dense contents (Fig. B). An FDG PET-CT scan showed isolated increased tracer uptake in the lesion (Fig. C). The imaging findings were indeterminate and a differential diagnosis of pulmonary carcinoid or large hamartoma was sug-gested. After complete surgical excision of the lesion, histological analysis revealed a sclerosing hemangioma.

Comment

Sclerosing hemangioma is a rare benign lung tumor with female predominance. Although rare, sclerosing hemangiomas are the second most common benign lung tumor, after hamartomas. Because of the histological appearance with predominantly hemor-rhagic component, these lesions were initially interpreted as hem-angiomas. More recent immunochemical analysis demonstrates that these neoplasms are derived from primitive respiratory epithe-lium. Therefore, sclerosing hemangioma is in fact a misnomer, whereas sclerosing pneumocytoma is more appropriate. In rare cases, malignant degeneration of these lesions has been described, without apparent effect on long term outcome.

Clinically, these lesions are most often incidentally found in as-ymptomatic patients. Possible symptoms such as cough, dyspnea, pain or hemoptysis are caused by local mass effect on adjacent structures.

Imaging typically demonstrates a well described round or ovoid lesion of variable size. A CT scan of the chest may demonstrate varying areas of attenuation in the lesion and in some cases there are peripheral calcifications, but en-hancement is typical. These findings can also be seen in carcinoid, hamartoma, teratoma or inflammatory lesions. On FDG PET imaging, sclerosing hemangiomas show low to moderate FDG uptake, relative to the size of the lesion. The FDG PET findings can be misleading and can be interpreted as malignant, especially in patients with already docu-mented malignancy or high risk patients.

The diagnosis can be suggested on basis on clinical presentation and imaging, but sclerosing hemangioma is essentially a histological diagnosis. A limited but complete resection of the lesion is the management of choice.

Reference

1. Keylock J.B., Galvin J.R., Franks T.J.: Sclerosing hemangioma of the lung. ArchPathol Lab Med, 2009, 133: 820-825.

JBR–BTR, 2015, 98: 57.

IMAGES IN CLINICAL RADIOLOGY

1. Dpt of Radiology and Medical Imaging, UZ Gent, Ghent, Belgium.

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Progressive quadriplegia resulting from septic facet joint arthritis

S. Raeymaeckers1, K. Nieboer1, J. De Mey1

A 70-year-old male patient presents with pain in the face and neck, persisting for one week. For one day he has also noted muscular weakness in the left arm. The patient presents with fever of unknown origin and remembers being scratched by a cat some days before. The diagnosis of a cerebral vascular inci-dent was initially withheld and a CT-scan of the brain was performed. This showed no abnormalities. The pain gradually became even more excruciating and an MRI of the cervical spine was performed to exclude spondylodiscitis. Since it was impossible for the patient to lie still, the exam had to be aborted. A contrast- enhanced CT-scan of the cervical spine was performed instead, which could not rule out spondylodiscitis. The patient was started on intravenous anti-biotics. Over the following hours the patient progressively developed quadriple-gia. MRI of the cervical spine was attempted again, this time with moderate suc-cess. On the fat-saturated sagittal T2 image (Fig. A) we can see a fluid-infiltration in the posterior soft tissues of the neck (arrows). A slight heterogeneous signal of the medulla may well indicate edema. On the fat-saturated axial T1 image after administration of contrast (Fig. B) we can see enhancement of the posterior mus-culature (arrows), as well as the left facet joint C5-C6 (arrowhead). On the non fat-saturated contrast-enhanced axial T1 image (Fig. C) we can see an enhancing epidural collection on the left posterior side of the spinal canal (arrow), causing a shift of the medulla. The patient was rushed to the OR for decompressive lami-nectomy. Streptococcus mitis was cultivated from samples of the collection, the antibiotic therapy was then changed to clindamycine. During the following months progressive recuperation of function was observed, with a persistent deficit in motor function in the left arm.

Comment

Septic facet joint arthritis is a suppurative bacterial infection of a facet joint and the adjacent soft tissues. It remains an uncommon but probably under-estimated entity, we estimate 79 cases have been reported. The infection is most often caused by hematogenous spreading, but can also be caused by direct in-oculation (such as surgery or infiltration procedures) and can even extend from an adjacent infection in the soft tissues. The highest incidence rate is found on the lumbar level where the infection is even known to spread from an accompa-nying appendicitis or diverticulitis. It occurs typically in the 6th-7th decade of life, predisposing factors are IV drug use, immunocompromised state and diabetes, cirrhosis, chronic kidney failure and other chronic illnesses. Septic facet joint arthritis can be associated with spondylodiscitis and formation of epidural abscesses, which can give rise to cord compression and/or foraminal narrowing. Also paravertebral abscesses may be seen, this however does not indicate a worse prognosis. Treatment relies primarily on the use of intravenous antibiot-ics, but percutaneous drainage may be indicated. Decompressive laminectomy is

generally indicated when an epidural abscess and neurological deficits are present. MRI is the imaging method of choice. Abnormal enhancement can be seen within the facet joint and the adjacent bone marrow as well as edema in the adjacent soft tissues. This occurs typically on a single level and unilaterally, with widening of the facet joint in question and erosion of the cortex. Sagittal STIR or FSE T2 sequences with fat saturation are most sensitive for bone marrow edema and epidural involvement. An axial T1 weighted sequence after administration of contrast with fat saturation is better suited to delineate the extent of involvement of the infection and to exclude epidural and para-spinal abscesses.

Reference

1. Muffoletto A.J., Ketonen L.M., Mader J.T., Crow W.N., Hadjipavlou A.G.: Hematogenous pyogenic facet joint infection. Spine, 2001, 26 (14): 1570-1576.

JBR–BTR, 2015, 98: 58.

IMAGES IN CLINICAL RADIOLOGY

1. Department of Radiology, UZ Brussel, Brussels, Belgium.

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Serpiginous cholesteatoma mimicking a vascular channel

A. Karandikar1 , S.Ch. Loke1, J. Goh1, S.B. Yeo2, T.Y. Tan3

A 36-year-old male who had history of hearing loss since childhood presented with a chronically discharging right ear.

HRCT temporal bones revealed non-dependent opacifica-tion in the epi- and meso-tympanum with medially displaced head of malleus, erosion of incus and the tegmen tympani suspicious for an attic cholesteatoma. In addition, there was a serpiginous structure seen extending across the posterior aspect of the mastoid temporal bone which was thought to represent a trans-mastoid emissary vein. MRI subsequently performed showed both the lesions (in the middle ear cavity and the serpiginous structure in the mastoid) were hyperin-tense on Propeller DW (b value 1000 sec/mm2) sequence suggesting 2 cholesteatomas. The serpiginous structure in the mastoid did not show post contrast enhancement thus excluding a vascular lesion. A canal wall down mastoidec-tomy was done which confirmed both the findings.

Comment

The imaging appearance of cholesteatomas has been ex-tensively discussed in the literature. On HRCT, cholesteato-ma generally presents as locally aggressive soft tissue mass causing ossicular and bony erosions. It is classically known to be hyperintense on Propeller Diffusion weighted (type of Non-EPI sequence) sequence and do not show post contrast enhancement.

Imaging of the soft tissue in the middle ear cavity in our case was classical for an acquired cholesteatoma on CT and MRI.

The serpiginous structure in mastoid bone had well- defined scalloped margins on HRCT prompting it to represent a vascular channel. MRI subsequently performed and mainly the Propeller DW sequence had classical features for choles-teatoma thus changing the surgical plan. Given the lack of local aggressiveness and its posterior location, it may have represented a congenital cholesteatoma.

A serpiginous cholesteatoma is an extremely rare form of presentation. We suggest that in cases of atypical findings

on HRCT, radiologists must avail MR imaging if possible, as it can alter treatment options, as in this case.

Reference

1. Barath K., Huber A.M., Stampfli P., et al.: Neuroradiology of cholesteatomas. AJNR Am J Neuroradiol, 2011, 32: 221-229.

JBR–BTR, 2015, 98: 59.

IMAGES IN CLINICAL RADIOLOGY

1. Department of Radiology, Tan Tock Seng Hospital, Singapore, 2. Department of Otorhinolaryngology, Tan Tock Seng Hospital, Singapore, 3. Department of Radiology, Changi General Hospital, Singapore.

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A rare case of ischemic stroke following occlusion of the artery of Percheron

H. Dechamps1, P. Gillardin1, R. De Potter2, D. Dewilde1, M. Lemmerling1

A 57-year-old man was admitted to the ER after falling on the back of his head without any prodromi. No loss of consciousness was noted. The patient was responsive and able to walk into the ambulance without any help. Besides alcohol and nicotine abuse, medical history was blank.

At ICU admission physical examination showed a patient with impaired consciousness, a Glasgow Coma Scale of 8 and distinct anisocoria with an unresponsive mydriatic left pupil. Vestibulo-ocular reflexes were preserved. The man had appropriate responses to nociceptive stimuli and normoflexia was seen with down going plantar reflexes with Babinski sign negative for both sides. Blood pressure was elevated but further cardiovascular, respiratory and abdominal examination was unremarkable. Apart from the elevated non-sober blood glucose level and hyper-cholesterolemia, all parameters ranged within normal limits.

Chest X-ray depicted an enlarged heart shadow with bilateral perihilar vascular consolidation. Cerebral computed tomography (CT) (Fig. A) showed no obvious brain lesions.

Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain revealed T2-weighted hyperintense signals of the thalami and left mesencephalon (Fig. B), sug-gesting bilateral paramedian thalamic and left mesencephalic infarction. Diffusion weighted MRI also confirmed the suspected diffusion restriction in these regions (Fig. C), highly suggestive for an acute infarction in the artery of Percheron territory. Transcranial Doppler and 3D time-of-flight MR angiography (TOF-MRA) displayed lightgraded ostial occlusion of the internal carotid on both sides, without evidence for carotid or vertebral dissection.

The patient’s condition improved gradually and after 12 days he was transferred for further revalidation. Both electrocardiogram and Holter monitoring demonstrated nothing abnormal. Echocardiography confirmed hypertrophy of the heart with a tricuspid insufficiency grade I and the patient was diagnosed with a hypertensive hypertrophic cardiomyopathy. No evidence was found suggesting a possible extracranial cause for embolism.

Antithrombotic and antihypertensive therapy was initiated at the time of admission, along with insulin to correct hyperglycemia, A statine was proposed because of the hypercholesterolemia. Cognitive revalidation was initiated after his transfer.

Comment

The artery of Percheron (AOP) is an uncommon anatomic variant supplying bilateral parame-dian thalami and sometimes the rostral part of the mesencephalon. Occlusion of the AOP can cause typical neurological, opthalmological and neuropsychological symptoms.

The posterior cerebral artery is divided into four segments. The P1 segment extends from the junction between the basilar artery and the posterior communicating artery. It supplies the medial part of the thalamus on both sides and the rostral part of the mesenchephalic midbrain. The artery of Percheron (AOP) is a variation of the P1 segment where a single prominent perforating branch supplies both thalami and the mesencephalic midbrain. The exact prevalence remains unknown. The anomaly has no significant difference in prevalence between males and females.

The AOP occlusion can result into three typical features. The neurological signs are most preva-lent and have been reported in up to 92% of cases (consciousness impairment, focal neurological signs), opthalmological signs were seen in 64% of cases (ocular motility, abnormal light reflexes, ptosis) and neuropsychological abnormalities up to 42% of cases (executive function disorders, memory deficit or language disorders). Although in most cases coma resolves rapidly, when pres-ent, the neuro(psychological) and opthalmologic signs may persist. In most cases occlusion is due to cardioembolism.

The diagnosis of an AOP occlusion is dependent on imaging studies, mainly MRI, as CT findings may appear normal early on. Though CT is easier to obtain, MRI remains essential when a typical thalamo(mesencephalic) syndrome is suspected. In these cases, ischemic lesions of the paramedian areas of both thalami are seen, with possible rostral mesencephalic involvement. The AOP occlusion can also show a characteristic midbrain “V” sign, appearing as a high-intensity signal on diffusion-weighted and axial FLAIR images along the pial surface of the midbrain in the interpeduncular fossa, and present in 67% of patients. MRI coupled with 3D TOF-MRA of the carotid, vertebral and basilar arteries is preferred as the first choice investigation to rule out a basilar artery occlusion.

Other etiological investigations such as electrocardiography and Holter monitoring can be useful as they may reveal cardiac abnormality or arrhythmia associated with embolism.

The main differential diagnosis is dural venous sinus thrombosis as a subset of cerebral venous thrombosis. Other rarer causes of restricted thalamic diffusion include Creutzfeld-Jakob’s disease, Wernicke encephalopathy and extrapontine myelinolyse.

ICU admission is definitely required in the management of patients with an AOP occlusion due to possible life-threatening complications (increased intracranial pressure, hyperglycemia, deep venous thrombosis, etc.).

Reference

1. Lamboley J.L., Le Moigne F., Have L., et al.: [Artery of Percheron occlusion: value of MRI. A review of six cases]. J Radiol, 2011, 92: 1113-1121.

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