on and truncus arteriosus in a patient

hya,

Palatal perforation

ronh asrepatio

orderrom thst desease pd has a

been reported up to date in the literature (Kinnear, 1985; Ghandourand Issa, 1991; Ernst et al., 1993; Pochon et al., 1995; Ferjaoui et al.,1999; Kriskovich et al., 2000; Ulnick and Perkins, 2001; Venail et al.,

A 28-year-old girl with the diagnosis of CD was admitted to ourclinic with complaints of dyspnoea, cyanosis, halitosis, depressionof nose (Fig. 1) and chronic non-bloody diarrhoea.

Her initial presentation 8 years previously consisted of gastro-enteritis andhematochezia. Six years earlier shehadbeendiagnosedwith Crohns disease following analysis of intestinal biopsies. Hersymptoms at the timewere controlledwith systemic corticosteroidsand second-line immunosuppressants. Two years before her

* Corresponding author. Dumlupinar Universitesi, Tip Fakultesi Hastanesi, MerkezKampus, Tavsanli Yolu, 10 km, 43270 Kutahya, Turkey. Tel.: 90 505 726 73 75.

Contents lists availab

Journal of Cranio-Ma

e:

Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 17e19E-mail address: foghan2001@yahoo.co.uk (F. Oghan).females (Karlinger et al., 2000; Podolsky, 2002; Jacobsen et al.,2006; Palamarus et al., 2008). Common clinical ndings of CD areabdominal pain, diarrhoea, and loss of appetite, weight loss, fever,hematochezia, nausea, vomiting, and perianal disease (Podolsky,2002; Jacobsen et al., 2006; Fatahzadeh, 2009). Besides gastroin-testinal manifestations, extraintestinal involvement may beobserved in one third of patients (Karlinger et al., 2000; Podolsky,2002; Jacobsen et al., 2006; Eloy et al., 2007). The mucocutaneousmanifestations of Crohns disease in the orofacial region aremultiple, including oral Crohns disease, metastatic Crohns diseasein sites noncontiguous with the bowel system, and reactive disor-ders such as pyoderma gangrenosum (Fatahzadeh et al., 2009).Nasal manifestations are extremely rare and only a few cases have

1 relates to the presence of a partially separate pulmonary trunk ofvariable length; type 2 is present when two pulmonary arteriesarise separately but close to the other from the posterior aspect ofthe truncus; type 3 when two pulmonary arteries take off fromeach side of the truncus and type 4when no true branch pulmonaryartery arises from the truncus (Friedman, 1997).

CD with nasopalatal stula and persistent truncus arteriosus-type 4 with the peculiarities of long survival is extremely rare. Webelieve this to be the rst such case described in the literature.

2. Case report1. Introduction

CD is a chronic inammatory disany part of the alimentary tract f(Chermesh and Eliakim, 2008). Fir(1932) as regional ileitis, the dispeople, often in early adulthood, an1010-5182/$ e see front matter 2011 European Assdoi:10.1016/j.jcms.2011.01.010of the bowel, involvinge mouth to the anuscribed by Crohn et al.rimarily affects whiteslight predilection for

2002; Merkonidis et al., 2005; Kryssia and Henry, 2006; Sari et al.,2007; Michailidou et al., 2009).

Persistent truncus arteriosus is an uncommon congenitalcardiac malformation accounting for approximately 1.2% of allcongenital heart malformations (Friedman, 1997; Williams et al.,1999). Several classication systems have been used to describethe anatomy of persistent truncus arteriosus (Friedman,1997). TypeBowel diseaseCrohns diseasePersistent truncus arteriosusCase report

Saddle nose deformity, palatal perforatiwith Crohns disease

Fatih Oghan a,*, Gurel Pekkan b, Olcay Ozveren c

aDumlupinar University, Faculty of Medicine, Department of Otorhinolaryngology, KutabDumlupinar University, Department of Dentistry, Kutahya, Turkeyc Yeditepe University, Faculty of Medicine, Department of Cardiology, Istanbul, Turkey

a r t i c l e i n f o

Article history:Paper received 19 November 2010Accepted 6 January 2011

Keywords:Nose deformity

a b s t r a c t

Crohns disease (CD) is a chextraintestinal organs sucquite rare in CD. This casecollapse and palatal perfortruncus arteriosus-type 4.

journal homepagociation for Cranio-Maxillo-FacialTurkey

ic granulomatous inammatory bowel disease which may also involve thejoints, liver, skin and perianal tissue. Involvement of the nasal cavity isort presents a 28-year-old girl with CD and saddle nose deformity, alarn as extraintestinal manifestations of the disease in addition to persistent

2011 European Association for Cranio-Maxillo-Facial Surgery.le at ScienceDirect

xillo-Facial Surgery

www.jcmfs.comSurgery.

axilF. Oghan et al. / Journal of Cranio-M18presentation she had experienced an exacerbation of her intestinalsymptoms and soon afterwards developed nasal symptoms. She hadrecognised a depression in her palatal vault one and half years agoand experienced increasing nasal blockage and alar collapse.

On physical examination she had saddle nose deformity, palatalperforation (Fig. 2), alar collapse (Fig. 3), and clubbing of the ngers.Intraoral examination revealed that she had a palatal perforation

Fig. 1. Saddle nose deformity.

Fig. 2. Palatal perforation approximately 2 cm in diameter.

Fig. 3. Alar collapse.approximately 2 cm in diameter with the upper left central andlateral, bilateral premolar and molar teeth missing (Fig. 2). She hada six-unit metal-ceramic xed dental prosthesis between her uppercanine teeth. She had all the mandibular teeth in place. No otheroral lesions were found. Nasal examination revealed intranasalpurulent necrotic crusting and total nasal perforation.

A systolic ejection murmur was heard at the left sternal border.Histopathologic examination of the biopsy of the columella showednonspecic inammatory granulomatous tissue. No microorgan-isms were identied by Gram, fungal, and acid-fast bacilli stains.Cultures for bacteria, acid-fast bacilli, and fungi were also negative.In order to differentiate other diseases that lead to nasal septalperforation and chronic bowel disease, antinuclear antibody, anti-bodies to double-stranded DNA (anti-dsDNA), protoplasmic-stain-ing antineutrophil cytoplasmic antibodies (p-ANCA), classicalantineutrophil cytoplasmic antibodies (c-ANCA), venereal diseaseresearch laboratory test (VDRL), Treponema pallidum haemag-glutination assay (TPHA), enzyme multiplied immunoassay tech-nique (EMIT), complement levels, rheumatoid factor, humanimmunodeciency virus (HIV) serology and Gliadin antibodies(immunoglobulin (Ig)G and IgM), anti-endomysial antibody (EMA)were carried out, yielding negative results in all tests.

The patient had type 4 persistent truncus arteriosus. A tele-cardiogram showed cardiomegaly. and a transthoracic echocar-diogram revealed a hypertrophied right ventricle, absence of thepulmonary valve and artery, overriding of both ventricles anda large single subtruncal ventricular septal defect. She was takinglow-dose diuretic treatment, systemic steroids, metronidazole,loperamide and cyclophosphamide.

3. Discussion

The aetiology of nasal septal perforations includes nasal septalsurgery, epistaxis, cryotherapy, nose picking, nasotracheal intuba-tion, nasal fractures, penetrate injuries, septal haematoma, nasaltampons, chemical irritants, nasal sprays including vasoconstrictoragents and steroids, cocaine, neoplastic diseases and midlinegranulomatous diseases, vasculitis, Wegeners granulomatosis,sarcoidosis, syphilis, tuberculosis, typhus, diphtheria, leprosy, rhi-nosclerosis, fungal diseases (Ulnick and Perkins, 2001; Merkonidiset al., 2005; Wanyura et al., 2006; Andre et al., 2006; Dosen andHaye, 2007; Eloy et al., 2007; Butt et al., 2010). Nasal ndings in CDmay be chronic mucosal inammation, nasal obstruction, epistaxis,and rarely septal perforation (Kriskovich et al., 2000; Kryssia andHenry, 2006; Sari et al., 2007). In this patient, CD caused a septalperforation that caused a saddle nose deformity and palatal perfo-ration. The authors believe that granulomatous inammation of thenose led to the formation of deep mucosal ulceration and eventuallyto saddle nose deformity as nasal symptoms of obstruction,discharge and cast production preceded the saddle nose deformity.

Several cases of nasal involvement in CD have been published,the rst in 1985 (Kinnear, 1985; Ghandour and Issa,1991; Ernst et al.,1993; Pochon et al.,1995; Ferjaoui et al.,1999; Kriskovich et al., 2000;Ulnick et al., 2001; Venail et al., 2002;Merkonidis et al., 2005; Kryssiaand Henry, 2006; Sari et al., 2007; Michailidou et al., 2009). Saddlenose deformity in a patient with CD was rst described byMerkonidis et al. (2005). Eloy et al. (2007) reported two CD patientswith laryngeal ulceration and sinonasal involvement. Kriskovich et al.(2000) reported a 12 years of childwith activated CD and nasal septalperforation. They emphasised that only three cases of nasalinvolvement in CD had been reported by Kriskovich et al. (2000).

Type 4 persistent truncus arteriosus is the least frequent form ofthis malformation accounting in a previous series for about 1% of allcases of persistent truncus arteriosus (Williams et al., 1999). 80% or

lo-Facial Surgery 40 (2012) 17e19more children born with persistent truncus arteriosus die within

the rst year of life without surgical intervention (Williams et al.,1999; Bodi et al., 2002), although some cases of long survival inunrepaired patients have been described (Hicken et al., 1966; Bodiet al., 2002). In 2002, Bodi et al. reported a case of persistentunrepaired truncus arteriosus-type 4 in a 54-year-old womanwiththe peculiarities of long survival, absence of pulmonary arteries andgood clinical status. In our case, the patient was a 28-year-old girl.Although, in this case, the patients nasal and palatal lesions arevery severe, the coincidental type 4 persistent truncus arteriosuswas more severe. According to a literature review, a CD case withoro-nasal manifestations as well as type 4 persistent truncusarteriosus has not been described previously.

The management of Crohns disease consists of surgical andmedical treatment comprising aminosalicylates, prednisolone andmild cytotoxic drugs such as 6-mercaptourine and azathioprine

Eloy P, Leruth E, Goffart Y, Nollevaux MC, Fridman V, Dartevelle M, et al: Sinonasalinvolvement as a rare extraintestinal manifestation of Crohns disease. Eur ArchOtorhinolaryngol 264: 103e108, 2007

Ernst A, Preyer S, Plauth M, Jenss H: Polypose pansinusitis als eine ungewohnlicheextraintestinale Manifestation des Morbus Crohn. [Polypoid pansinusitis in anunusual extraintestinal manifestation of Crohns disease]. HNO 113: 813e815,1993

Fatahzadeh M: Inammatory bowel disease. Oral Surg Oral Med Oral Pathol OralRadiol Endod 108: e1ee10, 2009

Fatahzadeh M, Schwartz RA, Kapila R, Rochford C: Orofacial Crohns disease: an oralenigma. Acta Dermatovenerol Croat 17: 289e300, 2009

Ferjaoui M, Koli H, Najed D, Hajri H: Nasal localization of the Crohns disease. RevLaryngol Otol Rhinol (Bord) 120: 341e342, 1999

FriedmanWF: Congenital heart disease in infancy and childhood. In: Saunders (ed.),Heart disease. A textbook of cardiovascular medicine. Philadelphia: Braunwald,877e962, 1997

Ghandour K, Issa M: Oral Crohns disease with late intestinal manifestations. OralSurg Oral Med Oral Pathol 72: 565e567, 1991

Hanauer S, Meyers S: Management of Crohns disease in adults. Am J Gastroenterol92: 559e566, 1997

F. Oghan et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 17e19 19(Rampton,1999). Oralmetronidazole for a restricted period has alsobeen shown to prolong remission (Hanauer and Meyers, 1997).Granulomatous nasal lesions may response to sulfasalazine andazathioprine in CD (Kryssia and Henry, 2006). The patient in thisstudy was taking low-dose diuretic treatment, systemic steroids,metronidazole, loperamide and cyclophosphamide when sheadmitted to our clinic.

Clinicians should be familiar with the extraintestinal manifes-tations of Crohns disease and include this important and oftenserious disease in the evaluation of patients with selected orofacialdisorders (Fatahzadeh et al., 2009). Otolaryngologists and dentistsshould be aware of such an association and consider the diagnosisof CD in atypical cases of nasal disease and palatal involvement.

4. Conclusion

In this case, a patient with CD and nasopalatal ndings andpersistent truncus arteriosus-type 4 with the peculiarities of longsurvival is presented. The recognition of orofacial manifestations inCDmay help prevent misdiagnosis and unnecessary treatment, andfacilitates timely diagnosis, palliation and denitive therapy.

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Saddle nose deformity, palatal perforation and truncus arteriosus in a patient with Crohns disease1 Introduction2 Case report3 Discussion4 ConclusionReferences