Delayed presentation of congenital diaphragmatic hernia: diagnosis, management and outcome

ABSTRACT

Although over 90% of cases with congenital diaphragmatic hernia [CDH] are diagnosed antenatally or early at neonatal age by severe respiratory distress, some cases are diagnosed at an older age. The aim of the work was to study the clinical presentation, the pathological findings and the operative results in children with delayed presentation of CDH. Between January 2001 to September 2003, at Menoufiya University Hospitals, 14 infants [12 males and 2 females] with CDH were included in this study. The mean age was 5 +/- 2,3 months [Range, 3-9mdnths]. The presenting symptoms and signs, diagnostic procedures, operative findings, and postoperative outcome were assessed. Accidental diagnosis after routine chest X-ray for repeated attacks of chest infection was the commonest presentation in 9 patients [64.35%]; severe respiratory distress and cyanosis in 2 patients [14.3%], vomiting in 4 patients [28.6%], and weight loss in 6 patients [42.9%]. Physical signs included the absence of breathing sounds in the chest in 8 patients [57.2%], and hyper resonance in 4 [28.6%]. Sex infants [42.9%] were under weight, three of them had marasmus. A posterolateral [Bochdalek] defect of diaphragm was found in the left side in 12 infants [85.3%] and in the right side in 1 infant [7.15%], central CDH was found in 1 case [7.15%]. A hernial sac was found in I infant [7.15%]. Organs protruding into thorax included intestinal loops in 11[78.5%] children, spleen in 6 [42.6%] individuals, stomach in 10 [71.5%] and kidney, pancreas and omentum in one each [7.15%]. The associated anomalies include Ladd's band and malrotation in 4 children [28.6%], lobulated spleen in I patient [7.15%], and mild degree of pulmonary hypoplasia were found in 9 patients [64.35%]. Omphalocele, right ventricular diverticulum, and Fallot's tetralogy were found in I case [7.15%]. The defect in diaphragm was occluded by a primary repair in 13 patients, while a patch of Polypropylene mesh was used once. One infant suffered from adhesive intestinal obstruction 2 months after repair and died after re-exploration and adhesolysis. The other infants were cured up without complications. The congenital diaphragmatic hernia should be considered in differential diagnosis of every infant with unusual respiratory or gastrointestinal symptoms and abnormal X-ray picture of thorax