Adverse outcome of congenital diaphragmatic hernia is determined by diaphragmatic agenesis, not by antenatal diagnosis

Abstract

Background/purpose: The authors studied their congenital diaphragmatic hernia (CDH) cases retrospectively to ascertain if classical CDH and diaphragmatic agenesis (DA) have separate clinical manifestations, whether antenatally diagnosed cases behave differently from those not diagnosed antenatally, and if antenatal diagnosis before 25 weeks carries a worse prognosis.

Methods: The authors performed a retrospective review of 23 infants with CDH treated at their institution between January 1996 and March 1999. The patients were divided into 3 groups that were analyzed: DA and classical CDH, antenatally diagnosed and nonantenatally diagnosed, and antenatally diagnosed before 25 weeks and after 25 weeks.

Results: There were 8 cases of DA and 11 cases of classical CDH. Four infants died without operation and could not be classified. Neonates with DA had significantly longer mean duration of preoperative stabilization compared with classical CDH (5.25+/-2.76 days v 1.36+/-1.0 days) and postoperative mechanical ventilatory support (15.7+/-3.0 days v 4.9+/-3.0 days). Fifty percent of DA patients died; all classical CDH patients survived. Twelve cases were diagnosed antenatally, 6 before 25 weeks' gestation. Antenatally diagnosed cases had no statistically significant difference in mortality rates from those not diagnosed antenatally; 50% of those diagnosed before 25 weeks survived.

Conclusions: DA cases require more preoperative preparation and postoperative ventilation and have a bad prognosis compared with classical CDH. Antenatal diagnosis of CDH does not convey a different prognosis. Fifty percent of CDH patients with antenatal diagnosis before 25 weeks survive.