Clinical Aspects of Congenital Diaphragmatic Hernia in the Central Counties of Taiwan

Abstract

SUMMARY Congenital diaphragmatic hernia (CDH) occurs approximately 1 in 3000 live births. Eighty-five percent were left sided and commonly occurred as classic posteriolateral or Bochdalek hernia. Pulmonary hypoplasia and pulmonary hypertension were frequently associated. A reported incidence of 40-50% of CDH patients were associated with other malformations, the most common of which are those involving the central nervous system. Mortality rates are around 40% but some studies reported 16-25% with current advances in treatment. From July 1995 through May 2003, 11 infants of CDH were treated at Changhua Christian Hospital (CCH). Of the infants, 2 were prenatally diagnosed, 7 (64%) were left sided, 3 (27%) were right sided and one (9%) was paraesophageally located. 8 (73%) of the infants expired either days or months after birth. Barotrauma occurred in 4(36%) infants. Three (27%) infants had pulmonary dysplasia and pulmonary hypertension. Four (3 6%) infants associated with intestinal malformation. One infant had major cardiac defects and chromosome anomaly. The mortality rate of CDH was 73%. It was higher than other studies. However, the survival rates in recent 6 years had reached 50%. It is probably related to the improvement of care. INTRODUCTION Congenital diaphragmatic hernia (CDH) is an anomaly occurs in approximately 1/3000 live births 1. The etiology of the disease was not well known. It was postulated that the defect may be related to the failure of the pleuroperitoneal canal to close during the 8th week of gestation. This may only be the explanation for patients with small diaphragmatic defect.