Congenital diaphragmatic hernia: a retrospective autopsy study.

ABSTRACT

Congenital diaphragmatic hernia is one of the acute pediatric surgical emergencies and an important cause of perinatal mortality. Its incidence varies from 1 in 2000 to 1 in 5000 live-births, with a greater incidence in stillbirths and abortions. Non-operative mortality is generally reported as 100% and early diagnosis is imperative. A retrospective study of all infant and neonatal autopsies done during last 30 years (1960 to 1989) was conducted. Ten cases of congenital diaphragmatic hernia were encountered among 588 autopsies and its incidence was 1.7% of all infant and neonatal autopsies, dying due to varying causes. The malefemale ratio was 73, while maternal factors showed no consistent relationship. All cases had left-sided posterolateral diaphragmatic defects. Dyspnea, cyanosis and dextrocardia was the classical triad present in more than 50% cases. The commonest herniated contents were the small intestinal loops and the left lobe of liver. Associated congenital multiple anomalies related to neural axis, skull, vertebral column and cardiovascular system were noted in 30% cases and were major contributory factors for perinatal mortality.