Laryngeal atresia, pleurodesis, and diaphragmatic hypertrophy in a newborn infant with findings relevant to fetal lung development.
A rare congenital malformation, cartilaginous subglottic laryngeal atresia, was found associated with developmental growth disturbances in the lungs (hypoplasia), diaphragm (hypertrophy), and pleural cavities (pleurodesis) in a newborn premature male infant who died immediately after birth. Because of coexistent esophageal atresia, tracheoesophageal fistula, and anal atresia, the lower respiratory tract and gastrointestinal canal together formed a closed system throughout fetal life. A mechanism whereby diaphragmatic hypertrophy and mesenchymal obliteration of the pleural cavities may have evolved during uterine development is suggested. Since pulmonary hypoplasia was also present, an explanation of this unique constellation of development abnormalities has a bearing on normal lung development.
D000015:Abnormalities, Multiple / Q000473:pathology*; D003964:Diaphragm / Q000002:abnormalities* / Q000473:pathology; D004065:Digestive System Abnormalities; D004947:Esophagus / Q000002:abnormalities / Q000473:pathology; D006801:Humans; D006984:Hypertrophy; D007231:Infant, Newborn; D007830:Larynx / Q000002:abnormalities* / Q000473:pathology; D008168:Lung / Q000196:embryology* / Q000473:pathology; D008297:Male; D010994:Pleura / Q000002:abnormalities* / Q000473:pathology; D013909:Thorax / Q000002:abnormalities / Q000473:pathology; D014132:Trachea / Q000002:abnormalities / Q000473:pathology