A Difficult Case of a Very Low Birth Weight Infant With Severe Congenital Diaphragmatic Hernia

Abstract

The prognosis of congenital diaphragmatic hernia (CDH) is improving due to perinatal management such as gentle ventilation and operation after stabilization. However, we often experience difficult cases in long-term management. We report a case of a 1,222g very low weight infant boy with left congenital diaphragmatic hernia, born at 38 weeks' gestation. Sonographic examination of the fetus at 35 weeks' gestation had revealed a lung-to-head ratio of 0.66, a lung-to-thorax ratio of 0.04, and liver intrusion to the thorax. After birth, the general examination revealed bilateral cloudy corneas, a cleft palate, and hypospadias. Left diaphragmatic repair was performed at 6 days of age. Due to perforation of the intestine, drainage was performed at 7 days and jejunostomy was placed at 16 days. After 2 months of respirator support, the baby was extubated and oxygen was supplied by a nasal canula. However his pulmonary hypertension got worse from around 12 months of age. After several reintubataion for respiratory deficiency, he died at 18 months due to severe pulmonary hypertension and circulatory failure.