• Bronchopulmonary abnormalities – Heterogenous group of entities, originate from primitive foregut or lung bud – Interfere normal develompent 3rd-24th weeks of gestation • Failure of development of lung bud agenesis • Incomplete development pulmonary hypoplasia • Majority manifested in early childhood (occasionally first recognized in adolescents or adults) • This chapter limits discussion to developmental in adults Anomalous Tracheobranchial Branching • Tracheal bronchus – An anomalous bronchus arises from the right lateral wall of the trachea – Less than 2 cm from carina – Supplies apical segment of RUL / entire lobe (pig bronchus or bronchus suis) – Normal vascular supply – End blindly tracheal diverticulum – Asymptomatic incidentally • Recurrent RUL pneumonia • Air trapping or atelectasis of apical segment or RUL • Focal emphysema • Bronchiectasis • A Small bronchus from right tracheal wall • B and C supplying the apical segment of RUL • A 3D VRT shows TB from Trachea • B virtual bronchoscopy show TB,R,L from level of lower trachea • C coronal 3D shows tracheal bronchus entering right lung • Accessory Cardiac Bronchus – Supernumerary bronchus that arises from the medial wall of right main or intermediate bronchus opposite the origin of the RUL bronchus – Courses medially and caudally – Ends blindly – Asymptomatic; cough and hemoptysis to infection / Ca – CT airway originates from medial wall of mainbronchus or bronchus intermediate cephalad to the origin of the middle lobe bronchus • A and B. Cardiac bronchus from medial wall of bronchus intermedius, ending blind • C (MIP) and D (3D VRT) show a medial and inferior course of cardiac bronchus, ends blindly Congenital Bronchial Atresia • Rare congenital anomaly characterized by short- segment obliteration of a lobar, segmental, or subsegmental bronchus at or near its origin • Most common : Apicoposterior segment of LUL segment bronchi of RUL middle lobe lower lobe • Airway and airspaces distal to obstruction develop normally collateral channels • Asymptomatic; recurrent pneumonia • Patophysiology – Bronchial tree peripheral to point of obliteration is patent + normal number of airways and airspaces accumulation mucus a mucocele (distal to atresia) – Alveoli in affected area ventilated by collateral pathways air trapping with hyperinflation • Radiography area of pulmonary hyperlucency and a hilar nodule or mass – Hyperlucency = oligemia + increase in volume of air (air trapping) – Adjacent normal lung and or mediatinum compressed and displaced – Mucus ovoid, round, or branching opacities near the hilum – Expiratory CXR air trapping • CT – Mucoid impaction w/ bronchial dilatation (bronchele) distal to atresia – Bronchial occlusion – Decreased vascularity and attenuation and increased volume of affected segment • MRI – Mucoid impaction • Variable signal at T1 • Very high signal at T2 • A. increased lucency in left middle and upper lung zone • B. CT marked decreased attenuation and vascularity of superior segment of LLL + anterior displacement of left major fissure + bronchocele distal to atretic bronchus (arrow) CT demonstrates decreased attenuation and vascularity + bronchial atresia (arrow) + pectus excavatum • CT modality of choice • DD/ – Foreign body – Endobronchial tumor
• 3D VRT shows atresia of RUL
bronchus + abnormal branching pattern of right middle lobe bronchus Bronchogenic Cyst • Abnormal separation of localized portions of the tracheobronchial tree from the adjacent airways • 75% at mediastium and 25% at lung • Asymptomatic; compression trachea/bronchi/esophagus • Thin walled, unilocular, roughly spherical, mucoid or serous • Radiography – Round or oval masses in right paratracheal or subcarinal region – Sharply circumscribed, solitary • CT – Homogenous cystic mass with thin smooth wall – Near water density (-10 - +10 HU) – No enhancement • A. focal convexity subcarinal with mild displacement of bronchus intermedius • B. Cystic mass in subcarinal region • C. Mass homogenous high signal fluid (MRI T2) • MRI – Low signal at T1 – High signal at T2 • Imaging algorithm CT or MRI recomended • DD – Lung tumor – Abscess – Congenital cystic adenomatoid malformation Congenital Cystic Adenomatoid Malformation • Multicystic mass of pulmonary tissue with abnormal proliferation of bronchial structures • Neonatal Respiratory distress • Adult incidental; recurrent infection • Type – Type I: large, multiloculated cysts > 2 cm (adults) – Type II : more uniform small cysts < 2 cm – Type III : solid-appearing lesions that microscopically demonstrate tiny cysts • Radiography – Unilocular or multiloculated cyst or as a complex soft tissue and cystic mass – Contralateral shift of mediastinum • CT – Unilocular or multiloculated cyst or as a complex soft tissue and cystic mass from 4-12 cm • Imaging algorithm CT • Large cystic lesion in the RLL, increasing in size. • Thin-walled cyst with septation Intralobar and Extralobar Sequestration • Malformation in which a portion of lung is detached from the remaining normal lung and receives its blood supply from a systemic artery • Intralobar bronchial obs and chronic infection; the detached portion is contiguous with normal lung parenchyma and contained within the same visceral pleural envelope • Ekstralobar congenital; separate from normal lung and enclosed within its own pleural membrane • Asymptomatic; superimposed infection • DD/ bronchiectasis and lung abscess • Radiography – Intralobar homogenous opacity in posterior basal segment of a lower lobe, almost invariably contiguous with the hemidiaphragm – Extralobar sharply defined, triangular-shaped opacity in the posterior costophrenic angle, adjacent to the left hemidiaphragm – Both most frequently in left lower • PA and lateral CXR show an area of homogenous increased opacity in the posterior basal region of the left lower lobe, abuts the diaphragm • CT – Intralobar : focal areas of lucency or irregular cystic spaces w/ or w/out fluid – Extralobar : homogenous opacity or well circumscribed mass • MRI – Differentiating cystic, solid, hemorrhagic, and mucous components of mass • Aortography – Feeding vessels, esp extralobar • Imaging algorithm – MRI free radiation + MRA = excellent visualization of vessels – CT exclude DD • DD/ – Pneumonia – Bronchial obstruction – Recurrent atelectasis – Lung abscess – Bronchogenic cyst – CPAM • A subtle area of increased lucency in the left lower lung • CT sharply defined focal area of decreased attenuation in left lower lung Enhance CT a large vessel originating from aorta and coursing toward the sequestered lung • Aortogram large aberrant vessel originating from the aorta Pulmonary Agenesis, Aplasia and Hypoplasia • Agenesis : complete absence of one lung • Aplasia : suppresion of all but a rudimentary bronchus that ends in a blind pouch, with no evidence of pulmonary vascularture or parenchyma • Hypoplasia : reduction in the number of size of airways, vessels, and alveoli • Most die in neonatal period • Radiography – Total or almost total absence of aerated lung in one hemithorax – Elevation ipsilateral hemidiaphragm – Ipsilateral shift of the mediastinum and the ribs • CT – Aplasia : rudimentary bronchus – Hypoplasia : patent bronchus, pulmonary artery, and hypoplastic lung • DD/ – Pneumonectomy – Atelectasis – Bronchiectasis – Fibrothorax Akhirnyaa……..