diagnostic imaging of congenital pulmonary abnormalities
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Chest
Congenital Pulmonary Abnormalities
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Mohamed ZaitounAssistant Lecturer-Diagnostic Radiology Department , Zagazig University HospitalsEgyptFINR (Fellowship of Interventional Neuroradiology)-Switzerlandzaitoun82@gmail.com
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Knowing as much as possible about your enemy precedes successful battle and learning about the disease process precedes successful management
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Congenital Pulmonary Abnormalities1-Bronchopulmonary Foregut Malformations2-Congenital Lobar Emphysema3-Pulmonary Underdevelopment4-Scimitar Syndrome5-Bronchial Atresia6-Congenital Diaphragmatic Hernia7-Kartageners Syndrome
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1-Bronchopulmonary Foregut Malformations :-Is a term that encompases :1-CCAM2-Pulmonary Sequestration3-Foregut Duplication Cysts
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1-Congenital Cystic Adenomatoid Malformation (CCAM) :a) Incidenceb) Pathologyc) Typesd) Locatione) Clinical Picturef) Radiographic Featuresg) Differential Diagnosis
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a) Incidence :-They account for approximately 25% of congenital lung lesions
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b) Pathology :-The condition results from failure of normal broncho-alveolar development with hamartomatous proliferation of terminal respiratory units in a gland-like pattern (adenomatoid) without proper alveolar formation
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c) Types :1-Macrocystic (Stocker types 1 and 2) :-Single cyst or multiple cysts >5 mm confined to one hemithorax ; better prognosis ; common2-Microcystic (Stocker type 3) :-Homogeneous echogenic mass without discernible individual cysts ; closely resembles pulmonary sequestration or intrathoracic bowel from a diaphragmatic hernia ; less common
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d) Location :-Lesions are usually unilateral and involve a single lobe-They appear less frequently in the right middle lobe
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e) Clinical Picture :-The diagnosis is usually either made on antenatal ultrasound or in the neonatal period on investigation of progressive respiratory distress-If large , they may cause pulmonary hypoplasia with resultant poor prognosis
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f) Radiographic Features :1-Antenatal Ultrasound2-Plain Radiography3-CT
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1-Antenatal Ultrasound :-These lesions appear as an isolated cystic or solid intrathoracic mass-There can be mass effect where the heart may appear displaced to the opposite side-Hydrops fetalis orpolyhydramniosmay develop
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Longitudinal prenatal sonogram of the right thorax obtained at 27 weeks of amenorrhea , A large typical type II CCAM (c) is shown in the right lung associated with marked ascites (a) ; d indicates diaphragm
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2-Plain Radiography :-Multiple cystic pulmonary lesions of variable size-The cysts may be completely or partially fluid filled in which case the lesion may appear solid or with air fluid levels -Variable thickness of cyst wall
3-CT :-The same as Plain Radiography
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Plain chest radiograph in this newborn shows three separated cysts in the right hemithorax , CT shows a large septated cyst containing air and fluid
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g) Differential Diagnosis :-General imaging differential considerations include :1-Bronchogenic cyst2-Pulmonary sequestration3-Congenital diaphragmatic herniation4-Congenital lobar emphysema
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2-Pulmonary Sequestration :a) Incidenceb) Pathologyc) Typesd) Locatione) Clinical Picturef) Radiographic Featuresg) Differential Diagnosis
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a) Incidence :-The estimated incidence is at 0.1% -The age of presentation is dependent on the type of sequestration-ELS more commonly presents in newborns whereas ILS presents in late childhood or adolescence
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b) Pathology :-Also called(accessory lung)-Refers to aberrant formation of segmental lung tissue that has no connection with thebronchial tree-The anomalous lung tissue has a systemic arterial supply which is usually a branch of the aorta-Venous supply is variable and dependant on the type of sequestration : 1-ILS : Via the pulmonary veins 2-ELS : Through the systemic veins (IVC , Azygos , Portal) into the right atrium
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c) Types :1-Intralobar Sequestration (ILS)2-Extralobar Sequestration (ELS)
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1-Intralobar Sequestration (ILS) :-Accounts for the majority (75-85% of all sequestrations-Present later in childhood with recurrent infections2-Extralobar Sequestration (ELS) :-Less common (15-25% of all sequestrations)-Usually present in the neonatal period with respiratory distress , cyanosis and / or infection-More in males , M:F ratio 4:1-Can be infra diaphragmatic in 10 % of cases
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d) Location :-Overall , sequestration preferentially affects the lower lobes-60% of ILS affect theleft lower lobe and 40%theright lower lobe-ELS almost always affect the left lower lobe , however approximately 10% of ELS can be sub-diaphragmatic
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e) Clinical Picture :-Recurrent pulmonary infections
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f) Radiographic Features :1-Ultrasound2-Plain Radiography3-CT4-MRI5-Angiography
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1-Ultrasound :-The sequestrated portion of lung is usuallymore echogenicthan the rest of the lung-ELS may be seenas early as 16 weeks gestation and typically appears as a solid well defined triangular echogenic mass-Colour Doppler may identify a feeding vessel (in-utero cases) from the aorta-If the sequestration is sub diaphragmatic , it may appear as an echogenic intra abdominal mass
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2-Plain Radiography :-Will often show an opacity in the affected segment-Recurrent infection can lead to the development of cystic areas within the mass-Both ILS and ELS can rarely have air bronchograms as they may be connected with the GIT
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CXR shows opacity in the posterior segment of the left lower lobe behind the cardiac silhouette , projecting over the spinal column and not bordering the left diaphragm
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Homogenous dense opacity (red arrow) in right cardiophernic angle
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Well-defined mass in the left lower lobe which determines loss of volume of the left lung and mediastinal shift to the right , the lesion corresponded to an extralobar sequestration
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CT shows area of lobulated opacity in the posterior segment of the left lower lobe , note in the 2D reconstruction the thoracic aorta branch directed towards the opacity
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3-CT :-Large solid mass that may be homogeneous or heterogeneous , sometimes with cystic changes (>5 cm) near diaphragm-Air fluid levels if infected-Surrounding pulmonary consolidation-Sequestration may communicate with GIT-3D reconstructions can beparticularly helpfulin detecting :a) Anomalous arterial vesselsb) Concurrent anomalous veinsc) Differentiating between intra-lobarand extra-lobar sequestrations
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CT+C shows an ILS , the yellow arrow in upper figures shows a hyperdense region in the left lower lobe of the lung with small cystic lesions containing air within it the red arrows in the lower figures show a contrast enhanced vessel arising from the aorta and supplying the area of hyperdensity in the lung
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CT+C shows a homogeneous mass in the posterior segment of the left lower lobe , feeding artery (arrow) is seen arising from the aorta , a finding that is diagnostic for sequestration
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ELS
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4-MRI :*T1 :-The sequestrated segment tends to be of comparatively high signal to normal lung tissue*T2 :-Also tends to be of comparatively high signal*MRA :-Can be helpful in demonstrating anomalous arterial supply
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5-Angiography :-Not part of routine investigation but is the gold standard in determining arterial supply
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g) Differential Diagnosis :-See CCAM
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3-Foregut Duplication Cysts :a) Bronchogenic Cystb) Esophageal Duplication Cystc) Neuroenteric Cyst
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a) Bronchogenic Cyst :1-Incidence2-Pathology3-Location4-Clinical Picture5-Radiographic Features6-Differential Diagnosis
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1-Incidence :-Bronchogenic cysts are rare congenital lesions accounting for only 5-10% ofpediatric mediastinal masses
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2-Pathology :-Bronchogenic cysts form as a result of abnormal budding of the bronchial tree during embryogenesis (between 4th-6thweeks) and as such they are lined by secretory respiratory epithelium (cuboid or columnar ciliated epithelium)
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3-Location :-The most common location is the middle mediastinum-subcarinal , right paratracheal and hilar locations most common
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4-Clinical Picture :-In many instances , bronchogenic cysts are asymptomatic and are found incidentally when the chest is imaged-When large , mass effect may result in bronchial obstruction leading to air trapping and respiratory distress-An alternative presentation may occur when the cyst becomes infected
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5-Radiographic Features :a) Plain Radiographyb) CT
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a) Plain Radiography :-Findings are nonspecific , mediastinal masses should be evaluated further using CT scanning or MRI to confirm the presence of fluid -The cysts usually appear as soft-tissue density rounded structures
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The chest X-ray showed a non calcified homogeneous opacity lateral to the trachea with a well defined interface with the lung , cervicothoracic sign indicated the posterior localisation of the mass.
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Azygoesophageal recess reflection , (a) PA chest radiograph shows the azygoesophageal line (arrowheads) , (b)CT shows the azygoesophageal recess (white arrow) formed by the esophagus anteriorly (black arrow) and the azygos vein posteriorly (arrowhead) , the azygoesophageal line inarepresents the interface between this recess and the lung
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(a)PA chest radiograph demonstrates a subcarinal abnormality with increased opacity (*) , splaying of the carina and abnormal convexity of the upper and middle thirds of the azygoesophageal line (arrowheads) , (b)Corresponding CT scan helps confirm a subcarinal mass (arrow) which proved to be a bronchogenic cyst
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(a)PA chest radiograph shows the posterior junction line (arrow) projecting through the tracheal air column , (b)CT shows the posterior junction line (arrow) which is formed by the interface between the lungs posterior to the mediastinum and consists of four pleural layers
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(a)PA chest radiograph shows a mass (arrow) obliterating the posterior junction line , note that the mass extends above the level of the clavicle and has a well demarcated outline due to the interface with adjacent lung (arrowhead) , (b)CT helps confirm the posterior location of the mass (arrow), which proved to be a bronchogenic cyst
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Bronchogenic cyst , conventional radiograph demonstrates a right paratracheal mass
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Bronchogenic cyst , conventional radiograph shows a subcarinal mass
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Bronchogenic cyst , conventional radiograph demonstrates a thin walled cyst in the left lower lobe with an air fluid level
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b) CT :-Typically appear as well circumscribed spherical or ovoid masses of variable attenuation-Approximately 50% are fluid density (0-20 HU) , however a significant proportion are of soft tissue density (>30 HU) or even hyperdense to surrounding mediastinal soft tissues -CT is better able to detect calcium oxalate (milk of calcium) layering dependently-Usually no solid contrast enhancement
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CT+C : well circumscribed unilocular water attenuation cyst in the middle mediastinum , the cyst has smooth imperceptible walls with no enhancement
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Bronchogenic cyst , CT shows a subcarinal mass with fluid density
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CT with and without contrast media showed a large homogeneous mass of low attenuation arising in the right paratracheal space extending posteriorly , thin wall and absence of enhancement suggested the diagnosis of Bronchogenic cyst
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6-Differential Diagnosis :-From oesophageal duplication cysta) Clinical Picture :-Asymptomatic bronchogenic cyst-Symptomatic oesophageal cyst in case of peptic ulceration
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b) Plain Radiography :-Bronchogenic cysts appear at a subcarinal location (most commonly)-Oesophageal cysts appear more tubular and in close relation to the esophagusc) CT :-Thin walled bronchogenic cyst-Thick walled oesophageal cyst (due to presence of smooth muscle)
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Bronchogenic Cyst (subcarinal) Esophageal Duplication Cyst ( in close relation to esophagus)
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Bronchogenic Cyst Esophageal Duplication Cyst
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b) Oesophageal Duplication Cyst :1-Incidence2-Clinical Picture3-Radiographic Features4-Differential Diagnosis
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1-Incidence :-It is the second most common GIT duplication after that of the ileum-As a congenital abnormality , if symptomatic , it is usually identified soon after birth
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2-Clinical Picture :-Presentation of large duplication cysts is usually in the newborn or infant with symptoms referable to pressure on the adjacent lung or esophagus leading to :1-Respiratory difficulties2-Dysphagia3-Vomiting-Smaller cysts can be asymptomatic and only found incidentally at any time
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3-Radiographic Features :a) Plain Radiographyb) Barium Swallowc) CTd) MRI
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a) Plain Radiography :-They are usually seen as rounded fluid / soft tissue densityposterior mediastinal masses
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Esophageal duplication cyst in a 3 year old girl with cough and dyspnea , (a) Chest radiograph shows homogenous opacification of the right hemithorax (arrows) at the time of first admission , (b) Follow-up CT 3 years later shows a large cystic periesophageal mass (arrows)
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b) Barium Swallow :-In cystic esophageal duplication , the oesophagogram shows the esophagus to be displaced to the side opposite the mass
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c) CT :-Duplication cysts appear as is sharply marginated masses with homogeneous fluid density-No enhanced after intravenous contrast administration is visible
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d) MRI :-MRI demonstrates features of a cyst*T1 :-Low signal*T2 :-High signal*T1+C :-No solid enhancement
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T1 T2
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4-Differential Diagnosis :-From Bronchogenic cyst-See before
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c) Neuroenteric Cyst :1-Incidence2-Radiographic Features
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1-Incidence :-Neurenteric cysts are rare posterior mediastinal lesions that may be connected to the meninges through a midline defect in one or more vertebral bodies-This abnormality may be associated with vertebral anomalies such as hemivertebra , butterfly vertebra or spina bifida
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2-Radiographic Features :a) Plain Radiographyb) CTc) MRI
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a) Plain Radiography :-Well circumscribed rounded mediastinal mass
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Neurenteric cyst in a 30 year old female with flank pain , (a) Chest radiograph shows a well defined round mass (arrow) in the lower thoracic region , (b- c) Both axial T1 and coronal T2 show a large mass that is of homogenous high signal intensity in the right paravertebral region (arrow) , the cyst presumably contains proteinaceous fluid
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b) CT :-The cyst is seen as hypoattenuating lesion which may show soft tissue attenuation
c) MRI :-The appearance depends on the variable protein content :*T1 & T2 : of variable signal intensities
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2-Congenital Lobar Emphysema :a) Incidenceb) Pathologyc) Associationsd) Locatione) Clinical Picturef) Radiographic Featuresg) Differential Diagnosis
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a) Incidence :-More common in males (M:F = 3:1)
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b) Pathology :-Is acongenital lung abnormalitythat results in progressive overinflation of one or more lobes of a neonate lung-Idiopathic , 50%-Obstruction of airway with valve mechanism , 50% :1-Bronchial cartilage deficiency or immaturity2-Mucus3-Web , stenosis4-Extrinsic compression
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c) Associations :-May be associated withcongenital heart defectssuch as :1-Ventricular septal defect (VSD)2-Patent ductus arteriosus (PDA)3-Tetralogy of Fallot
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d) Location :-Left upper lobe : most common 40-45%-Right middle lobe : 30%-Right upper lobe : 20%-May involve more than a single lobe in 5%-Much rarer in the lower lobes-Therefore despite the left upper lobe being most commonly affected , the right hemithorax is the most common side to be affected
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e) Clinical Picture :-Patients typically present with respiratory distress most commonly in the neonatal period and usually within the first 6 months of life
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f) Radiographic Features :1-Plain Radiography2-CT
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1-Plain Radiography :a) Immediate postpartum period :-The affected lobe tends to appear opaque and homogeneous because of fetal lung fluid or it may show a diffuse reticular pattern that represents distended lymphatic channels filled with fetal lung fluid
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b) Later Findings :-Appears as an area of hyperlucency in the lung with a paucity of vessels-Mass effect with mediastinal shift and hemidiaphragmatic depression-Lateral film may demonstrate posterior displacement of the heart
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Chest radiographs demonstrates increased translucency of the left lung with mild mediastinal shift to the right side , on the lateral view the hyperlucency is confined to the left upper lobe, shifting the oblique fissure posteroinferiorly
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Low density and expanded left lung with important mass effect (mediastinal shift to the right , contralateral atelectasis and increased of the left intercostal space)
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A large hyperlucent area (red arrow) in the left upper , mid and lower zone with attenuated vascular markings within the lucency (green arrow)
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2-CT :-Hyperlucent lobe (hallmark)-As Plain Radiography
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g) Differential Diagnosis :1-General Differential Diagnosis includes:a) CCAMb) Pulmonary artery hypoplasiac) Bronchial atresia
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2-Unilateral Hypertransradiant Hemithorax :a) Rotationb) Chest Wallc) Pleurad) Lunge) Pulmonary Vessels
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a) Rotation :-The hypertransradiant hemithorax is the side to which the patient is turned 1-Poor technique2-Scoliosisb) Chest Wall :1-Mastectomy2-Poliomyelitis3-Polands syndrome (unilateral congenital absence of pectoral muscles +/- rib defects)c) Pleura :-Pneumothorax
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d) Lung :1-Compensatory Hyperelaxation :-Following lobectomy2-Air way Obstruction :-Air trapping on expiration results in increased lung volume and shift of the mediastinum to the contralateral side-Foreign body , bronchial atresia , endobronchial mass & bronchial compression3-Unilateral Bullae , pneumatocele4-Swyer-James (McLeod) Syndrome5-Congenital Lobar Emphysema6-Schimitar Syndromee) Pulmonary Vessels :-Pulmonary embolus to a major pulmonary artery
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3-Pulmonary Underdevelopment :a) Typesb) Radiographic Features
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a) Types :1-Pulmonary Agenesis :-Complete absence of the lung parenchyma , bronchus & pulmonary vasculature-Abnormal blood flow in the dorsal aortic arch during the 4th week of gestation (embryonic phase) causes pulmonary agenesis-More than 50% of affected fetuses have other abnormalities involving the cardiovascular (patent ductus arteriosus , patent foramen ovale) , gastrointestinal (tracheoesophageal fistula , imperforate anus) , genitourinary or skeletal (limb anomalies , vertebral segmentation anomalies) system
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2-Pulmonary Aplasia :-Blind ended rudimentary bronchus is present without lung parenchyma or pulmonary vasculature3-Pulmonary Hypoplasia :-Bronchus and rudimentary lung are present , however the airways , alveoli & pulmonary vessels are decreased in size & number
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-Can be primary or secondary , primary pulmonary hypoplasia in which a cause cannot be elucidated is much less common than secondary hypoplasia-The majority of cases of pulmonary hypoplasia are secondary to a process limiting the thoracic space for lung development which can be either intrathoracic or extrathoracic
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-The most common intrathoracic cause is congenital diaphragmatic hernia which is left sided in 75%90% of cases-The most common extrathoracic cause of pulmonary hypoplasia is severe oligohydramnios , skeletal dysplasias in which a small and rigid thoracic cage causes pulmonary hypoplasia
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b) Radiographic Features :1-Pulmonary Agenesis :-Postnatal radiography demonstrates diffuse opacification of the involved hemithorax with ipsilateral mediastinal shift , severe volume loss and opacity on the side of agenesis often with close spacing of the ribs , the bronchus and PA to the affected lung are absent and blood flow to the contralateral lung is increased -CT helps confirm the absence of the lung parenchyma , bronchus and pulmonary artery on the involved side
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Pulmonary Agenesis
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2-Pulmonary Aplasia :-Imaging findings in pulmonary aplasia and agenesis are similar except for the presence of a short blind-ending bronchus in aplasia
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Pulmonary aplasia , (a) Chest radiograph depicts the trachea (white arrow) and the right main bronchus (arrowhead) however , the left main bronchus is not seen , there is leftward mediastinal shift , compensatory hyperinflation of the right middle lobe extending into the left hemithorax is also noted (black arrow) , (b) CT shows a blind-ending left main bronchus (arrowhead) with absence of the left lung parenchyma
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3-Pulmonary Hypoplasia :-The ribs may appear crowded with a low thoracic-to-abdominal ratio -Films may also show features of the neonate's underlying condition-There may be mediastinal shift with a homogenous density on the involved hypoplastic side and compensatory herniation of the contralateral lung across the mediastinum
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Chest radiograph of a newborn with primary pulmonary hypoplasia of the right lung showing shift of the mediastinum to the right hemithorax
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Radiograph showing left pulmonary hypoplasia
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Chest Radiograph of a 3-month-old infant with primary pulmonary hypoplasia of the right lung
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White left lung with diffuse interstitial opacity and hyperexpansion of the right lung , the trachea is deviated to the left as is the cardiac silhouette
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A chest radiograph of a newborn with diaphragmatic hernia in the right hemithorax shortly after birth , CT shows the presence of abdominal contents in the right hemithorax , note the presence of the left lower bronchus and its main branches (horizontal arrow) and absence of the right lower lobe bronchus , the liver in the right hemithorax is indicated by the upper arrow
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AP and lateral chest radiographs in a patient with skeletal dysplasia show the short dysplastic ribs and narrowed thorax
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A chest radiograph of a newborn with achondroplasia and small chest causing hypoplasia of both lungs
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4-Scimitar Syndrome :a) Pathologyb) Locationc) Associationsd) Radiographic Features
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a) Pathology :-Also known as Hypogenetic Lung Syndrome , Pulmonary Venolobar Syndrome-Characterized by a hypoplastic lung that is drained by an anomalous vein into the systemic venous system (IVC)-A combination ofpulmonary hypoplasia and partial anomalous pulmonary venous return (PAPVR)
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b) Location :-It almost exclusively occurs on the right side
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c) Associations :1-Accessory diaphragm , diaphragmatic hernia2-Bony abnormalities : hemivertebrae , rib notching & rib hypoplasia3-CHD : ASD , VSD , PDA & tetralogy of Fallot
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d) Radiographic Features :1-Plain Radiography2-CT
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1-Plain Radiography :-Small lung with ipsilateral mediastinal shift and in one third of cases the anomalous draining vein may be seen as a tubular structure paralleling the right heart border in the shape of a Turkish sword (scimitar)-The right heart border maybe blurred
2-CT :-As Plain Radiography
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(a) CXR shows volume loss in the right hemithorax with rightward mediastinal shift , the right heart border is not well seen , an anomalous vessel (arrowheads) is seen in the right cardiophrenic angle , this vessel increases in caliber in the caudal direction (scimitar sign) , (b) CT+C shows the lower lobe pulmonary vein (scimitar vein) draining into the intrahepatic IVC (arrows) , (c) Volume-rendered CT clearly depicts the anomalous vein (arrow)
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5-Bronchial Atresia :a) Pathologyb) Locationc) Radiographic Featuresd) Differential Diagnosis
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a) Pathology :-Bronchial atresia is a rare anomaly resulting from focal obliteration of a segmental , subsegmental or lobar bronchus-The bronchi distal to the stenosis are dilated and filled with mucus with mild hyperinflation of the adjacent lung due to air trapping
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b) Location :-Commonly occurs at the apico-posterior segment of the left upper lobe
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c) Radiographic Features : HRCT-Atretic bronchial stump(s) often become mucus plugged and can give afinger in gloveappearance-Distal lung parenchyma supplied by the atretic segment can be emphysematous due to air trapping
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Central mass surrounded by hyperlucent lung (blue arrow)
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Bronchial atresia , CT shows mucoid impaction (arrow) just distal to bronchial atresia in the right upper lobe , distal air trapping is also noted
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CXR in inspiration/expiration show hyperlucency of the left lung , the heart is displaced towards the right and there is shifting of the mediastinum towards the right on expiration confirming air trapping of the left lung , in addition , a tubular structure is visible in the left lower lung (arrow) , CT shows hyperlucency of the left lower lung limited by normal upper lung (white arrows) , there is a non-enhancing tubular structure in the centre of the left lower lung (red arrows) , the displacement of the heart towards the right is due to the expanded left lung plus a marked pectus excavatum
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CXR shows an area of hyperlucency in the left upper lung with branching serpiginous shadows within (arrows) , CT confirms the unenhanced mucous plug within the area of hyperlucency , expiratory film confirms air trapping
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d) Differential Diagnosis :1-Congenital Lobar Emphysema2-Allergic Bronchopulmonary Aspergillosis (ABPA) : for finger in glove appearance
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6-Congenital Diaphragmatic Hernia :a) Incidenceb) Associationsc) Types
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a) Incidence :-1 in 2000 to 3000 births-Mortality rate of isolated hernias is 60% (with postnatal surgery) and higher when other abnormalities are present
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b) Associations :1-Pulmonary hypoplasia (common)2-CNS abnormalities :-Neural tube defects : spina bifida , encephalocele-Anencephaly
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c) Types :1-Morgagni Hernia2-Bochdalek Hernia
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1-Morgagni Hernia :a) Incidenceb) Pathologyc) Locationd) Radiographic Featurese) Differential Diagnosis
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a) Incidence :-Is one of the congenitaldiaphragmatic hernias-It is rarer than the Bochdalek herniaand accounts for approximately 2% of all congenital diaphragmatic hernias
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b) Pathology :-It is characterized by herniation through theforamen of Morgagni (anterior opening in the diaphragm that extends between the sternum medially and the eighth rib laterally)-As compared to the Bochdalek hernia , the Morgagni hernia is rare , small & anterior
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c) Location :-This hernia occurs in the anterior midline (retrosternal or parasternal) through the foramen of Morgagni with 90% of cases occurring on the right side-Morgagni hernias typically occur medially
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d) Radiographic Features :-Anterior herniation of bowel loops on a lateral chest radiograph is the typical finding-Other herniated viscera include the liver , spleen and omentum
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Morgagni hernia in a 2 year old child , lateral chest radiograph shows herniation of a bowel loop (arrows) in a classic location through an anteromedial defect
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e) Differential Diagnosis :-Cardiophrenic angle lesions :-Thecardiophrenic spaceis usually filled with fat , however lesions originating above or lower to the diaphragm can present as cardiophrenic angle lesions :1-Pericardial fat pad2-Pericardial cyst3-Pericardial fat necrosis4-Morgagnis hernia5-Lymphadenopathy : metastases , lymphoma6-Pericardial lipomatosis
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2-Bochdalek Hernia :a) Incidenceb) Pathologyc) Locationd) Radiographic Features
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a) Incidence :-Is one of the congenitaldiaphragmatic hernias-More common than Morgagni hernia-It is more common in infants (90%)
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b) Pathology :-They occur posteriorly and are due to a defect in the posterior attachment of thediaphragmwhen there is a failure of pleuroperitoneal membrane closure in utero-Retroperitonealstructures may prolapse through the defect , e.g. retroperitoneal fat ,spleenor leftkidney
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c) Location :-They occur posteriorly-It occurs most frequently on the left side with approximately 80% being left-sided and 20% right-sided
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d) Radiographic Features :1-Plain Radiography2-CT
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1-Plain Radiography :-On conventional radiographs , the hernia may appear as a lung base soft-tissue opacity lesion seen posteriorly on lateral images
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Intrathoracic kidney
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2-CT :-CT usually demonstrates fat above the diaphragm and is extremely beneficial in revealing organ entrapment-Coronal and sagittal reformatted images show the defect to best advantage
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7-Kartageners Syndrome :a) Pathologyb) Clinical Picturec) Radiographic Features
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a) Pathology :-Kartagener's syndrome (immotile cilia syndrome) is due to the deficiency of the dynein arms of cilia causing immotility of respiratory , auditory and sperm cilia
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b) Clinical Picture :-Kartagener syndrome is characterized by the clinical triad of :1-Situs inversus2-Chronic sinusitisand / or nasal polyposis3-Bronchiectasis-Other features include :1-Telecanthus : widened interpupillary distance by a nasal polyp2-Infertility in male3-Subfertility in female
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c) Radiographic Features :-Complete thoracic and abdominal situs inversus-Bronchiectasis-Sinus hypoplasia and mucosal thickening
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