Delayed presentation of congenital diaphragmatic hernia

ABSTRACT

The late-presenting congenital diaphragmatic hernia [CDH] represents a considerable diagnostic challenge. This study was undertaken to define various patterns of delayed presentations, and to analyze the pitfalls in the diagnosis and treatment of these patients. Thirty-three children with CDH were treated between 1993 and 2000; fifteen of these children [45.5%] were diagnosed after age of 2 months form the basis of this report. Each child was evaluated as to age. sex, side of herniation, presenting symptoms, physical and roentgenographic findings, management and outcome. Thirteen patients had Bochdalek hernia, and 2 had Morgagni hernia. The diaphragmatic defect was right- sided in 6 cases, and left sided in 9. The ages ranged from 2 months to 14 years with a median of 2,5 years. Five patients presented acutely, three with respiratory distress, and two with gastrointestinal [GI] obstruction. The remaining 10 patients presented with chronic respiratory or GI complaints. Inappropriate insertion of chest drain occurred in 3 patients misdiagnosed as having pleural effusion [2 cases], and pneumothorax [one patient]. Two patients had previously reported normal chest radiographs. Plain radiographs were sufficient to make a definitive diagnosis in only 6 patients, but GI contrast studies were necessary for confirmation of the diagnosis in the other 9 patients. All patients were treated through abdominal approach with primarily closure of the diaphragmatic defects without patch. A distinct hernial sac was present in 6 cases, and associated malrotation in 6 patients. All patients except one survived the operation with rapid improvement of their GI and respiratory symptoms. Late-presenting CDH should be included in the differential diagnosis of any child with persistent GI or respiratory problems associated with abnormal chest x-ray film. Nasogastric tube placement must be considered as an early diagnostic or therapeutic intervention when the diagnosis is suspected, and GI contrast studies should be part of the diagnostic work-up of these cases