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Congenital lung abnormalities
- 1. Congenital lung abnormalities Dr Aftab Qadir
- 2. 1.TRACHEOBRONCHIAL ABNORMALITIES • Tracheal agenesis • Tracheal stenosis • Tracheomalacia • Tracheo-oesophageal fistula (TOF) • Bronchial atresia • Tracheal bronchus (PIG bronchus)
- 3. 2.PULMONARY UNDER DEVELOPMENT • Lung agenesis • Lobar under development • Scimitar syndrome
- 4. 3.Bronchopulmonary foregut malformation • Bronchogenic cysts • Enteric cysts • Neuroenteric cysts • CCAM • Pulmonary sequestration
- 5. 5.DIAPHRAGMATIC ABNORMALITIES • Congenital diaphragmatic hernia • Congenital eventeration of the diaphragm
- 6. Tracheal agenesis • Rare • Commonly associated with maternal polyhydramnios • Presentation is immediate and acute with severe respiratory distress, absent cry and inability to intubate the airway. • There are three main forms of tracheal agenesis:
- 8. Tracheal stenosis • Congenital stenosis due to complete cartinogenous rings is rare. • 50% focal, 30% generalised ,20% funnel shaped • 90% of affected children present in the first year of life with biphasic stridor. • CT is useful in assessing the anatomy and has the added advantage of angiographic capabilities. • MRI and bronchoscopy
- 15. Tracheomalacia • Softening of the tracheal wall, due to cartilaginous abnormalities. • Commonest type is secondary to tracheostomy, oesophageal atresia/TOF • Chronic inflammation(associated with cystic fibrosis, recurrent aspiration, immuno-deficiency) • Extrinsic compression (vascular rings, slings or aberrancy) • Neoplasia
- 16. Causes expiratory wheeze • Fluoroscopy shows an exaggerated decrease in the sagital width of the trachea during expiration. • Dynamic CT can be useful to assess the cross- sectional anatomy and compliance of the trachea.
- 19. Tracheo-oesophageal fistula (TOF) • Majority of cases are associated with the presence of oesophageal atresia. • May present with choking, cyanosis, coughing at the time of feeding. • Contrast oesophagram is used to demonstrate the presence of a fine hair-like structure connecting the oesophagus and trachea with linear opacification of the posterior tracheal wall
- 24. Bronchial atresia • The upper lobe bronchi are more frequently affected by congenital atresia of lobar or segmental bronchi. • May be associated abnormalities such as bronchogenic cyst, intralobar sequestration or cystic adenomatoid malformation.
- 27. Tracheal bronchus ( Pig bronchus) • Incidence is 1 % of the normal population • Right upper lobe bronchus arises directly from the trachea
- 31. PULMONARY UNDER DEVELOPMENT • Agenesis, aplasia and hypoplasia. • Agenesis is complete absence of a lung or lobe with absent bronchi • Aplasia is absence of lung tissue but the presence of a rudimentary bronchus • Hypoplasia is the presence of both bronchi and alveoli in an underdeveloped lobe
- 34. Lung agenesis • Recognizable with a small opaque hemithorax, displacement of mediastinal structures towards that side. • Bronchography or bronchoscopy confirms the absent main stem bronchus • Angiography shows no pulmonary or bronchial arterial circulation.
- 39. Scimitar syndrome • Unique form of lobar agenesis or aplasia • Common feature hypoplasia or aplasia of one or more lobes of the right lung. • The hemithorax is small, with obscuration of the heart border and a retrosternal soft-tissue density • Anomalous vein has the appearance of a Turkish scimitar, which normally drains to the IVC • The right pulmonary artery may be absent • Systemic vessel arising from the lower thoracic or upper abdominal aorta supplying the right lower lobe.
- 45. Congenital lobar over inflation/emphysema • Characterised by progressive over distension of a lobe • Aetiology is unknown in 50% of cases • Male to female ratio is 3:1 • Associated anomalies include the patent ductus arteriosus, ventricular septal defect and tetralogy of Fallot • The upper lobes, or right middle lobe, are commonly involved.
- 48. Bronchopulmonary foregut malformation • Bronchogenic cysts • Enteric and • Neurenteric cysts • Cystic hamartomatous (adenomatous) malformation • Pulmonary sequestration.
- 49. Bronchogenic cysts • 50% of all congenital thoracic cysts • May be intrapulmonary or mediastinal bronchogenic • Can be paratracheal (usually right sided, carinal or hilar) • Carinal location is most common. • Bronchogenic cysts do not usually communicate with the tracheobronchial tree
- 55. Enteric cysts • Located in the posterior mediastinum. • If present in the oesophageal wall these are referred to as oesophageal cysts or duplication cysts. • Mediastinal uptake of 99 Tc-MDP (pertechnetate)contain gastric mucosa.
- 58. Neuroenteric cysts • Present as posterior mediastinal masses with associated vertebral abnormalities. • MRI is the most useful tool for evaluating the thoracic and spinal components of neuroenteriec cysts
- 61. CCAM • Hamartomatous proliferation of terminal bronchioles • Composed of both solid and cystic tissue. • Malformations are classified on the basis of clinical, radiographic and histological features: • Type 1,2 and 3
- 68. Pulmonary sequestration • Congenital mass of aberrant pulmonary tissue • No normal connection with the bronchial tree or with the pulmonary arteries. • Usually located in one of the basal segments of the lower lobe. • Intralobar sequestration (ILS) is contained within the lung with no separate pleural covering
- 71. CHARACTERSTIC INTRALOBAR EXTRALOBAR Incidence More common ( 75 %) Less common( 25 %) Gender predisposition Equal Men 4: 1 Pleural investment Shares visceral pleura of parent lobe Separate visceral pleura Location Posterior basal segments (Approx. 60% on left) Above, below or within diaphragm (Approx. 90% on left) Venous Drainage Pulmonary venous Systemic venous (azygos, IVC, portal) Presentation Early adulthood with a history of pulmonary infection, chronic cough, or asthma. Asymptomatic mass (15%) Mostly present during first 6 months of life due to respiratory or feeding problems Radiographic Features Homogeneous consolidation with irregular margins or uniformly dense mass with smooth or lobulated contours. Single well defined, homogeneous, triangular shaped opacity in the lower thorax. May present else where in the thoracic cavity.
- 76. Pulmonary arteriovenous malformations • Congenital or acquired. • The acquired connections arc called pulmonary fistulas. • Congenital arteriovenous malformations are abnormal communications between pulmonary arteries and veins • No intervening capillary bed and are often clinically silent, • 60% are in the lower lobes • Typical appearances are of a well-defined pulmonary mass which is often lobulated.
- 80. DIAPHRAGMATIC ABNORMALITIES • Hernia • Eventeration • Agenesis
- 81. Congenital diaphragmatic hernia • Boakdalek hernia-Posteropleuoperitoneal foramen • Causes severe respiratory distress in the neonate • Involves the left pleuroperitoneal foramen in 75 % of cases. • The prognosis correlates with the degree of underlying lung hypoplasia
- 82. • Neonatal radiograph shows a left-sided large intrathoracic mass of soft-tissue density • There is absence of the normal gas-containing bowel in the abdomen.
- 87. Congenital eventeration of the diaphragm • Either partial or complete • Often right sided, due to hypoplasia of the diaphragmatic muscle. • Most eventerations are minor, transitory, local diaphragmatic elevations found incidentally within the first few years of life • Disappear with age.
- 89. CYSTIC FIBROSIS • Autosomal recessive trait • Prevalence of approximately 1 in 2500. • Chronic respiratory illness
- 91. Imaging • Atelectasis • Mucoid impaction • Focal or generalized overinflation • Cylindrical or cystic bronchiectasis • Bronchial wall thickening • Hilar adenopathy • Pulmonary arterial hypertension and cor pulmonale • Recurrent pneumonias • Hypertrophic osteoarthropathy • Recurrent pneumothorax is common
- 96. Few cases
- 105. Thank You
Editor's Notes
- Congenital lobar overinflation
- lethal congenital anomaly
- Diagnosis can the confirmed via the cautious injection of water-soluble contrast(gastrographin) into the oesophagus. Type 1. Absent upper trachea, lower trachea connecting to the oesophagus. Type 2. Common bronchus connecting right and left main bronchi to the oesophagus with absent trachea Type 3. Right and left main bronchi arising independently from the oesophagus. There are associated congenital heart, radial ray and duodenal (atresia) anomalies.
- The determination of the cause of a fixed tracheal narrowing in a symptomatic child is crucial, and in showing the presence and extent of tracheal narrowing, MRI is and bronchoscopy may not he adequate alone. Acquired tracheal stenosis occurs as a consequence of long-term intubation or traumatic suctioning. The diagnosis of congenital tracheal stenosis should prompt a detailed search for associated abnormalities (tracheo-oesophageal fistula (TOF), lung agenesis or hypoplasia, pulmonary artery sling and bronchial stenosis). Associated abnormalities
- Tracheal stenosis. (a) Posteroanterior and (b) lateral radiographs of the upper airways show narrowing of the tracheal lumen in the subglottic trachea (arrows) Posteroanterior radiograph of the trachea in another patient, obtained with filtered high-kilovoltage technique, shows two tracheal narrowings (arrows).
- Bronchogram. Water-soluble contrast has been introduced into the trachea and bronchi showing long segment tracheal stenosis. Right apical (pig) bronchus also present. B) Prone oesophagograrm showing good bolus distension of the oesophagus with a hairline communication between the oesophagus and trachea (which contains contrast along its posterior wall) representing the 'N' or 'H' type tracheo-oesophageal fistula (arrow).
- Acquired stenosis: AP chest radiograph (Fig. A) demonstrates focal, short segment, symmetric narrowing of the proximal tracheal air column (arrows). Contrast-enhanced chest CT (Fig. B-E) (lung windows) confirms the presence of marked tracheal narrowing with a reduced coronal diameter and anterior luminal tapering.
- Acquired stenosis due to intubation CXR: Smooth distal tracheal stenosis CT: Smooth distal tracheal stenosis with soft tissue thickening of the tracheal wall.
- Tracheal Stenosis. Coronal and sagittal MPR images demonstrate marked narrowing of the trachea at point A and slightly less narrow extending to point B. The patient had a history of long-term endotracheal use.
- Malacia= Softening Clinical features: Similar to asthma like wheeze, cough, dyspnea. Expiratory wheeze increasing with cry and disappears on rest.
- Tracheomalacia. (a) Radiograph of the trachea in a 2-month-old infant with stridor shows marked diffuse tracheal narrowing during expiration (arrows). (b, c) Radiographs of a 3-week-old infant with Hurler disease.
- Dynamic CT
- Or may present, in a more insidious way, with chronic respiratory infection. In cases associated with oesophageal atresia the diagnosis is more obvious. However, when in isolation, tracheo-oesophageal fistula can be difficult to diagnose.
- Type A - Esophageal atresia without fistula so-called pure esophageal atresia (10%) Type B - Esophageal atresia with proximal TEF Type C - Esophageal atresia with distal TEF (85%) Type D - Esophageal atresia with proximal and distal TEFs Type E - TEF without esophageal atresia or so-called H-type fistula (4%) Type F - Congenital esophageal stenosis
- chest radiograph reveals esophageal atresia and distal tracheoesophageal fistula. Note the Replogle tube in the upper pouch and the GI air below the diaphragm. Type C
- chest radiograph reveals esophageal atresia without tracheoesophageal fistula. Note the absence of gas below the diaphragm.
- Red arrow points to end of NG which is blocked from entering the distal esophagusby the patient's esophageal atresia. Note the lack of gas in the abdomenindicating a fistulous tract does not connect the trachea to the distal esophagus
- In the newborn period this presents as a mass, occupying part or all of an upper lobe due to retention of fetal lung fluid trapped behind the atresia. In later childhood, the fetal lung fluid escapes (via pores or Kohn and canals of Lambert), revealing a round opacity at the site of the atresia central to the air trapping.
- Bronchial atresia is a congenital abnormality resulting from interruption of a bronchus with associated peripheral mucus impaction and associated hyperinflation of the obstructed lung.The hyperinflation of the affected lung segment is caused by collateral ventilation through the pores of Kohn. The characteristic finding is a hyperlucent area of the lung surrounding a branching or nodular opacity that extends from the hilum. central mass surrounded by hyperlucent lung (blue arrow). CT scan in the same patient shows a round opacity at the site of the atresia, medial to the air trapping, representing mucoid impaction just distal to the atresia.
- Types of tracheal bronchi. a) Displaced Type, b) Supernumerary Type, c) Pig bronchus. Clinical features: Asymptomatic, persistent upper lobar pneumonia, atelectasis or air trapping.
- CT section demonstrates a right-upper-lobe bronchus (arrows) arising from the trachea, above the carina. (c) CT scan of the same patient at the level of the carina, 2 cm below the origin of the tracheal bronchus. (d) Bronchogram helps confirm the diagnosis and shows the origin of the tracheal bronchus. Rib alterations in the right hemithorax are secondary to prior thoracotomy.
- Displaced type tracheal bronchus (white arrow) in coronal (A1) and axial (A2) plane. Pig bronchus (white arrow) in coronal (B1) and axial (B2) plane.
- Pulmonary underdevelopment including absent lung is fairly common.
- Right lung agenesis has a higher mortality rate, possibly because of the higher incidence of cardiovascular abnormalities Associated abnormalities include the Vater syndrome (vertebral segmentation anomalies, anorectal atresia, tracheo-oesophageal fistula, oesophageal atresia, radial ray and renal anomalies).
- Chest X-ray showing radio opaque right hemi thorax with shift of mediastinum,overinflated left lung in a neonate with crowding of the ribs and opacification of the right hemithorax due to agenesis of the right lung.
- Chest X-ray showing hypoplasia of the right lung with mediastinal shift to the right. (B, C) VQ scans show reduced ventilation and perfusion to the abnormal hypoplastic right lung (posterior view).
- Pulmonary hypoplasia. Axial CT of the same patient from shows mediastinal shift to the right and reduction in volume of the right lung. Both main bronchi are visible shows a reduction in the caliper of the right pulmonary artery (arrow) compared to the left pulmonary artery
- (congenital pulmonary venolobar syndrome) May be inherited with an autosomal dominant inheritance with variable expression. associated with other abnormalities of pulmonary vessels and the thorax. The variable components including partial anomalous pulmonary venous return from the abnormal lung (often seen as a scimitar-shaped vein; absent or small pulmonary artery perfusing the abnormal lung; arterial supply to the abnormal segment of lung partly or wholly from the thoracic aorta, abdominal aorta or coeliac axis; ipsilateral hemidiaphragm anomalies; absent IVC and anomalies of the bony thorax with excessive extrapleural areolar tissue). The anomalous vein may drain to the portal vein, hepatic veins or the right atrium. This may be associated with a mass of abnormal lung tissue in the right lower lobe (pulmonary sequestration).
- Scimitar syndrome. Chest radiograph shows displacement of the heart toward the right side because of right lung hypoplasia and the anomalous pulmonary vein paralleling the right heart border (arrows). Axial CT of the same patient shows hypoplasia of the right lung with ipsilateral mediastinal shift
- Scimitar syndrome. Coronal CT reconstructions (A, B and C) show abnormal systemic arterial supply to the right lower lobe from a branch of the abdominal aorta (arrows). Axial CT shows the scimitar vein entering the inferior vena cava (arrow).
- but is probably related to obstruction of the bronchus by a ball valve mechanism. or occasionally two lobes, with emphysema being a misnomer, as there is no alveolar wall destruction. Postulated reasons for this include abnormal bronchial cartilage deficiency/dysplasia, inflammatory changes, inspissated mucus, mucosal folds or webs, bronchial stenosis and extrinsic vascular or mass compression. Histological examination of the `emphysematous' lobes reveals distended alveoli with thin septa, which may sometimes he associated with an increase in the alveolar number (polyalveolar type). and other associated anomalies occur in up to 50% of children. (Distribution left upper = 43%, right middle = 32%, right upper = 20( : and lower l obes = 5%.)
- Congenital lobar emphysema/overinflation. Chest X-ray shows gross overinflation of the right lung which is hypovascular with marked shift of the mediastinum to the left and herniation of the lung into the left hemithorax (arrow). (B) CT of right middle lobe congenital lobar overinflation/emphysema causing shift of the mediastinum to the left with marked distortion of the pleural reflections to the left of the midline.
- During the first few days of life, lung fluid may become trapped Definitive treatment for congenital lobar emphysema (which is compromising the patient) is surgical resection of the involved lobe with an excellent prognosis.
- This refers to a number of developmental abnormalities resulting from abnormal budding of the embryonic foregut and trachco-bronchial tree. The latter are more common and represent an earlier budding abnormality: intrapulmonary bronchogenic cysts result from later budding defects and the cysts are all lined by ciliated epithelium. They may contain smooth muscle and cartilage.
- but instrumentation of the cyst or infection may lead to an air-filled cyst or an air-fluid level. The differential diagnosis includes an acquired cyst or one of the extremely rare but potentially malignant cystic mesenchymal hamartomas.
- imaging Evaluation of intrathoracic foregut cysts, localising and defining the extent in relation to other structures and characterising the intrinsic density. Cysts may be watery or viscous and attenuation values are variable.
- Bronchogenic cyst. (a) Chest radiograph of an asymptomatic 5-year-old girl shows a large soft-tissue mass in the right hemithorax (arrows). (b) Contrast material-enhanced CT scan through the upper lobes shows a well-defined water-density homogeneous mass (*) with no contrast enhancement.
- Pulmonary bronchogenic cyst. Lung (A) and mediastinal (B) windows of an axial enhanced-CT show a sharply marginated oval non-enhancing opacity in the posterior segment of the right upper lobe (arrows). This measured 10 HU in attenuation. The appearance is compatible with a fluid-filled bronchogenic cyst
- The images show a well defined lesion of water attenuation in close proximity to the trachea or bronchus, which is typical for a bronchogenic foregut cyst.
- form earlier in embryogenesis Duplication cyst and present early with acid secretion which may rupture into the traeheobronchial tree and cause haemoptysis
- Enteric foregut cyst The images show a well defined lesion of water attenuation in the lower mediastinum in close proximity to the esophagus, which is typical for an enteric foregut cyst.
- Esophageal Duplication Cyst. White arrows point to a middle mediastinal soft tissue mass with sharp margins on the conventional frontal radiograph. On the CT scan, the white arrow points to a fluid-filled, cystic mass to the right of the location of the esophagus. The mass measured water density.
- Most bronchogenic, enteric and neuroenteric cysts are filled with serous or mucoid fluid and are typically solitary and unilocular. Regardless of the CT density, lack of enhancement is expected but complex cysts (following infection may show wall enhancement on T2 weighted image show high signal intensity, both ct and mri exclude the vascular lesions such as pulmonary artery slings Neurenteric Cyst Neurogenic cysts contain neural and gastrointestinal element. They are commonly associated with vertebral anomalies and scoliosis. The cyst does not communicate with CSF. The cyst is well demarcated and has a near water attenuation value on CT and water signal intensity on MRI
- Neuroenteric 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-weighted – and coronal T2-weighted MR images show a large mass that is of homogenous high signal intensity in the right paravertebral region (arrow). The cyst presumably contains proteinaceous fluid. (d) The photograph of the resected tumor shows a thick-walled encapsulated cystic mass.
- Hamartomas result from an abnormal formation of normal tissue Congenital cystic adenomatoid malformation at the expense of alveolar development and lesion The cysts are lined by respiratory epithelium and usually communicate with the trachcobronchial tree with a slight predilection for the upper lobes. Type 1. This is the most common type (50%) composed of variable cysts with at (one dominant cyst (greater than 2 cm in diameter). Prognosis is excellent and there is infrequent association with other congenital abnormalities (5% ).
- Imaging Type 1 lesions have one or more dominant cysts with adjacent smaller cysts. Type 2 lesions display more heterogeneous and smaller cysts. Type 3 CCAM appear as a solid mass with mass effect and a ground-glass appearance on CT Air-fluid levels may he seen with or without superadded infection.
- Chest X-ray: right cystic hamartomatous/adenomatous malformation type 1 with multiple cystic lesions in the right lower lobe showing air-fluid levels consistent with infection. Axial CT scan through the lung bases show the thick-walled cysts in the right lower lobe.
- Type 1 congenital cystic adenomatoid malformation (CCAM). Axial CT images (A and B) show a large multicystic mass (arrows) that occupies the entire right lower lobe of an infant and contains cysts larger than 2 cm in diameter. Air-fluid levels are visible.
- Type 2 congenital cystic adenomatoid malformation (CCAM). Axial (A) and coronal (B) CT images show a multicystic mass (arrows) in the right lower lobe of an infant containing multiple air-filled cysts less than 2 cm in diameter.
- Chest X-ray: cystic hamartomatous/adenomatous malformation type 3. Extensive ground-glass shadowing with gross overinflation of the right lung and herniation across the midline due to the presence of a CCAM type 3. (B) CT scan of the same patient with extensive overexpansion of the right lung and ground-glass shadowing due to microcysts beyond the resolution of the CT.
- The sequestration is usually supplied by an anomalous artery arising from the aorta and its venous drainage is via the azygos system. The pulmonary veins or the inferior vena cava. Although frequently asymptomatic, children with sequestrations usually present because of super added infection Bronchograms show normal bronchi draping around the sequestration and aortography demonstrates one or more systemic vessels entering the mass, usually arising from the aorta at or below the diaphragm Venous drainage is usually via the pulmonary veins. and is intimately connected to adjacent lung.
- Imaging is directed to identification of sequestrated or dysplastic lung tissue, Identification of aberrant arterial and venous connections and evaluation of Possible bronchial or gastrointestinal connections, and exclusion of other associated Icing anomalies such as horseshoe lung or hypoplasia, and assessment of diaphragmatic integrity. The imaging modality of choice for sequestration is ultrasound, especially in the newborn period presenting with a mass adjacent to the liver or diaphragm. Antenatal sonography often chest mass suggests the diagnosis of extralobar sequestration. Doppler ultrasound readily demonstrates the vascular connections to the sequestration . CT of the lung in the intralobar sequestration localises and shows the extent of the abnormality, showing a multicystic mass at the lung base. The extralobar sequestration is a solid soft-tissue mass with avid contrast enhancement adjacent to the diaphragm. MRI be useful for identifying the pulmonary abnormality and vascular connections in a multiplanar fashion.
- Intralobar sequestration. Coronal CT reconstruction shows a mass lesion in the medial aspect of the left lower lobe (black arrow). Systemic arterial supply comes from the descending thoracic aorta (red arrow). Venous drainage is into a left lower lobe pulmonary vein (blue arrow).
- Extralobar sequestration. Coronal (A) and sagital (B) CT reconstructions show a well-defined mass in the posterior basal segment of the left lower lobe (arrows).
- Coronal ultrasound examination of a pulmonary sequestration (arrow) in left lower lobe. The Doppler scan shows a large systemic vessel arising from the aorta to supply the sequestration. (B) Axial ultra-sound shows the origin of systemic vessel (arrow) from aorta, confirmed at aortography.
- Axial contrast-enhanced CT scan through the lung bases with a large systemic vessel arising from the left side of the aorta (arrow A) supplying a very vascular left-sided extralobar sequestration (ELS) (arrowB). (B) Corona CT multiplanar reconstruction (MPR) showing the normal lung and beneath this (arrow) the left-sided basal ELS with a draining vein entering the azygous system below the diaphragm.
- associated with either liver disease, cyanotic chronic pulmonary infection or emphysema however cyanosis, polycythacmia. dyspnoea and digital clubbing sometimes develop. Multiple lesions are common (33-50%). as are bilateral lesions (8-20% ). and 6% occur in patients with the autosomal dominant disorder hereditary haemorrhagic telangiectasia.
- Simple pulmonary arteriovenous malformation. Coronal CT shows a subpleural oval opacity in the left lower lobe (arrow) with a large feeding vessel. Marked scoliosis is visible.
- may be associated with lung malformations and cause severe respiratory symptoms.
- and is one of the commonest congenital anomalies of the thorax The diagnosis is now frequently made prenatally at antenatal ultrasound or MRI
- which is usually scaphoid on examination. and other developmental abnormalities of the lung. The differential diagnoses are of other cystic-appearing intrathoracic masses in the newborn Lobar emphysema, Cystic adenomatoid malformations. Sequestration, bronchogenic cysts
- A chest X-ray taken at 2 days of age showing a left-sided congenital diaphragmatic hernia with loops of bowel in the left hemithorax and shift of the heart and mediastinum to the right. The stomach is delineated by the presence of the nasogastric tube below the level of the diaphragm.
- Congenital absence of diaphragm
- CT scout image reveals indistinct right diaphragmatic copula with right paracardiac soft tissue shadow. CT images show a defect of the right crus of the diaphragm with herniation of the stomach, the first part of the duodenum and part of the left lobe of the liver into the right hemithorax.
- Symptomatic patients require surgical plication. The differential diagnosis of diaphragmatic herniation is sometimes difficult
- Frontal and lateral chest x-ray shows smooth elevation of right hemidiaphragmmedially and anteriorly consistent with an eventration of the hemidiaphragm
- With the exception of asthma, The cystic fibrosis gene is located on chromosome 7 and is inherited as an. Gene carriage rate is approximately 1 in 25 individuals of Northern European extraction This is often initially thought to be related to viral pneumonitis and early radiological appear- ances are identical Infants with cystic fibrosis are prone to develop atelectasis, focal or generalised overinflation with mucus plugging partially obstructing the airway producing secondary hyperinflation.. In older children chest radiographs show overinflation with bronchial wall thickening, dilatation and bronchial mucus plugging with frank bronchiectasis. Bronchiectatic cavities may contain air-fluid levels Eventually hilar adenopathy or pulmonary hypertension resulting in large central pulmonary arteries develops.
- HRCT is sensitive with extensive central bronchiectasis the hallmark feature. Complications such as pneumothorax are not uncommon. Haemoptysis uncommon can be a devastating complication, and bleeding sites may be demonstrated by selective bronchial arteriography and embolisation in the intractable cases. Corpulmonale and pulmonary hypertension eventually develop, and the definitive treatment at the present time is lung/heart-lung transplantation. Patients also have sinus, pancreatic and gut disease simultaneously.
- Atelectasis Discoid, segmental, lobar with RUL predominance Mucoid impaction Nodular and fingerlike densities along bronchovascular bundle Peribronchial cuffing Clubbing and hypertrophic osteoarthropathy can occur
- Frontal chest x-ray in cystic fibrosis shows diffuse interstitial disease with bronchiectasis and nodular densities of mucoid impaction
- Chest x-ray of patient with cystic fibrosis showing the lungs hyperexpanded with flat diaphragms (red arrows). A port catheter (yellow arrow) is in place to administer chronic IV medicine. Within the lungs (green ovals) the white lines are dilated airways, filled with mucous. Axial computed tomography (CT) of the upper chest showing a port catheter (yellow arrow) in place to administer chronic IV medicine. Dilated airways (red arrows) are seen as black areas within the lungs (green ovals), some with thickened walls (blue arrows). Mucous (purple arrow), which is white in appearance, is also seen in some of the airways. Axial computed tomography image of the lower lungs showing thickened dilated airways with mucous filling. Coronal computed tomography (CT) of the chest shows dilated airways (red arrows) seen as black areas within the lungs, some with thickened walls (blue arrows). Mucous (purple arrow), which is white in appearance, is also seen in some of the airways.
- CT scan at the level of the upper lobes in a 4-year-old boy demonstrates mild signs of bronchiectasis (short arrows), bronchial wall thickening (arrowhead), and mosaic perfusion. CT scan at the level of the lower lung zones in a 26-year-old woman. In contrast to the usual severe findings in this age group, this CT scan demonstrates mild signs of bronchiectasis, mosaic perfusion (∗), and a small area of consolidation (arrow).
- There is hyperlucency and overinflation of the left upper lobe. This child has congenital lobar overinflation. The left upper lobe is the most commonly involved and as a result of air-trapping, the lobe becomes progressively overinflated. Mass effect on local structures causes progressive clinical deterioration.
- Bronchial atresia. Coronal CT shows a hyperlucent and hypovascular left lower lobe. A branch of the left lower lobe bronchus is dilated and contains a mucous plug (arrow)
- Pulmonary hypoplasia. Chest radiograph shows opacification of the right hemithorax with mediastinal shift to the right
- Schmitar syndrome
- Bronchogenic cyst in an asymptomatic 50-year-old man. (a) Chest radiograph shows a sharply defined lesion of increased opacity in the right cardiophrenic angle (arrow). (b) Contrast enhanced CT shows a well -circumscribed low- attenuation lesion in the posterior mediastinum.Note posterior mediastinal mass extending into the azygo-esophageal recess and pericardium. (c) Axial T1-weighted MR image shows a lesion (M) of increased signal intensity compared to CSF. (d) Axial T2- weighted MR image reveals that the lesion is of similar signal intensity as that of CSF, which suggests a cyst. The cyst capsule is seen as a well-defined low-signal intensity rim (arrow) on T2-weighted MR image. The cystic lesion exhibits septations or mural nodules. (e) Photograph of the cystic lesion.
- Single chest x-ray in of a 14 year old female demonstrates a Port-a-cath ® in situ and typical finding of advanced bronchiectasis: ring shadows and tram-track opacities are seen throughout both lungs, particularly well in the uppers zone.
- A: axial, end-inspiratory CT image of the trachea at the level of the arch vessels demonstrates a normal trachea. Right, B: axial, dynamic expiratory CT image of the trachea at the same level shows excessive collapse of the airway with anterior bowing of the posterior membranous airway wall resulting in a crescent-shaped appearance to the trachea, findings consistent with severe tracheomalacia.
- Multiple pulmonary nodules are seen on the chest radiograph, the largest in the right mid-zone. There is a prominent vessel leading to this nodule. Intravenous contrast enhanced CT demonstrates multiple pulmonary arteriovenous malformations ranging in size from small to moderately large.