A STUDY ON CONGENITAL DIAPHRAGMATIC DEFECTS IN INFANCY AND CHILDHOOD

Abstract

Congenital diaphragmatic defects were a group of defects that result in anatomical and physiological dysfunction of the diaphragm. The reported incidence is 1: 2500-3000 live birth. In more than 80% of cases, the defect is in the form of hernia

that is associated usually with variable degree of pulmonary hypoplasia. Our concern is I mainly in cases of CDH and eventration, which are depicting more than 97% of all defects.

This study included 42 patients in infancy and childhood age periods with congenital diaphragmatic defects that were followed for a period ranged between 6 - 60 months with an average of 30 months. The main presenting symptom was the respiratory distress in 100% of cases, while 71.4% of them presented acutely and 28.6% presented in a delayed course including the 5 cases of eventration. On radiological examination of plain X-ray chest, the most frequent signs were mediastinal shift to the opposite side (97.6%); air filled loops in thorax (83.3%). Persistent fetal circulation resulting in pulmonary hypertension is much more common in high-risk groups; we find it in 74% of high-risk group cases, while it represents 47% of other groups. Every effort must be done for treating this PH. Along many decades of time no actual significant increase in the survival rate among the high-risk group of CDH has been reported, despite the introduction of many new therapeutic modalities.

Nearly 59.3% of cases were operated during the first week after exclusion of delayed­ presenters and non-operated cases from both groups. Incidence of left to right sides among Bochdalek hernia was 2.6: I in both groups. Associated malrotation reported in