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Congenital and developmental anomalies of the lung

Congenital and developmental anomalies of the lung -...

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CONGENITAL AND CONGENITAL AND DEVELOPMENTAL ANOMALIES OF DEVELOPMENTAL ANOMALIES OF THE LUNG Karan Madan Deptt. Of Pulmonary & Critical care Medicine PGIMER Chandigarh
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Seminar outline Introduction Embryology of lung development Classification Individual anomalies
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Introduction Developmental anomalies of the lung are usually detected in the neonatal period and in early hildh d childhood. Some are not encountered until later childhood or adulthood. Some can be confused with more sinister abnormalities. An understanding of their imaging features & presentation important for the physician.
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Relevant Embryology Intrauterine development: Embryonic, pseudoglandular, canalicular & saccular (alveolar). 26th day gestation Ventral diverticulum of the foregut 26th day gestation - Ventral diverticulum of the foregut Next 2 days, the right and left lung buds arise from this Next 2 days, the right and left lung buds arise from this outpouching f f Respiratory portion of the gut becomes separated from the esophageal portion by tracheoesophageal septum. Lung buds elongate into primary lung sacs & the 5 lobar bronchi appear.( Upto 5th week – Embryonic phase)
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Relevant Embryology 5 lobar bronchi branch in a dichotomous fashion By 16 th week, virtually all of the conducting airways are present. Airways are blind tubules lined by columnar or cuboidal epithelium Airways are blind tubules lined by columnar or cuboidal epithelium – Pseudoglandular (5 -16 th week) Canalicular period (17th to 25th 28th weeks) Canalicular period (17th to 25th–28th weeks) Saccular (alveolar) period – Alveoli demonstrated as early as 30 k t ti weeks gestation. Final period of normal intrauterine lung development from 36 k t t P lifi d l t f l li weeks to term - Prolific development of alveoli. Postnatal period - Alveolar development continues
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CLASSIFICATION BRONCHOPULMONARY COMBINED LUNG & VASCULAR (LUNG BUD) ANOMALIES COMBINED LUNG & VASCULAR ANOMALIES ANOMALIES A C it l b hi l A H ti l A Ab f i A. Congenital bronchial atresia B. Congenital lobar h A. Hypogenetic lung (scimitar) syndrome B. Bronchopulmonary t ti b th A. Absence of a main pulmonary artery B. Anomalous origin of the l ft l t f emphysema C. Congenital cystic adenomatoid malformation sequestration, both intralobar and extralobar left pulmonary artery from the right C. Anomalous pulmonary venous drainage (partial or D. Pulmonary bronchogenic cysts (BPFM) E Tracheal bronchus venous drainage (partial or complete) D. Pulmonary arteriovenous malformation E. Tracheal bronchus F. Accessory cardiac bronchus G. Tracheomalacia H. Tracheal stenosis I. Pulmonary underdevelopment
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Bronchial atresia Focal obliteration of a proximal segmental or subsegmental bronchus. Lacks communication with the central airways Development of distal structures is normal.
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