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Fatal Outcome of Undetected Congenital Diaphragmatic Defect: Two Autopsy Cases
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Congenital diaphragmatic hernia is one of the most frequently occurring anomalies of the thorax and seen once in every 2 000 to 5 000 births. Herniation of the abdominal organs into thoracic cavity through the defect in the diaphragm takes place early in gestation resulting in varying degrees of pulmonary hypoplasia. The defect is usually in the posterolateral aspect of the diaphragm. The size and the location of the defect affect the degree of pulmonary hypoplasia and clinical presentation. In 65 to 80 % of the cases the defect is in the right hemidiaphragm.
The mortality rate of congenital diaphragmatic hernia is high despite advances in neonatal intensive care and the overall mortality rate from multiple studies is approximately 50 %.
We report a 4-year-old girl and a newborn boy died of complications resulting from congenital unilateral diaphragmatic defect. On forensic autopsy, parts of the abdominal organs were found to be in the left chest cavity and there was a defect on the left leaflet of the diaphragm in both cases. The deaths of the child and newborn were attributed to the complications of the congenital diaphragmatic defect.
These cases emphasize the importance of complete evaluation of fetuses before and after birth and follow-up of all "healthy" children.
Keywords
- Smith NP, Jesudason EC, Losty PD. Congenital diaphragmatic hernia. Pediatric Respiratory Reviews 2002, 3:339-348
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