Late-presenting congenital diaphragmatic hernia: a possible cause of sudden respiratory distress in children

Abstract

OBJECTIVE: To emphasize that congenital defects of the diaphragm should be considered in the differential diagnosis of sudden onset of respiratory distress in children. MATHOD: Two illustrative cases of children (aged 2 years) with late presenting congenital diaphragmatic hernia who developed sudden respiratory distress and were managed at the University Hospital of Santa Maria are reported. The medical literature was reviewed to identify cases reports and other articles related to late presenting congenital diaphragmatic hernia. A computerized search was performed using the MEDLINE data base to identify papers published within the last 25 years. RESULTS: In both cases the initial clinical examination and plain chest X-rays were misinterpreted as another pleuropulmonary disease like pneumothorax/pneumatoceles. One of the children underwent to emergency chest tube placement without clinical improvement. The absence of respiratory symptoms that could justify this kind of pathology motivated a more careful investigation, involving fiberoptical endoscopy and intestinal seriography. This subsequent investigation revealed that the cause was late-presenting congenital diaphragmatic hernia. CONCLUSION: Although many congenital diaphragmatic hernias that present late have a history of chronic respiratory symptoms, some of them present with acute symptoms. A higher degree of attention is required to avoid confusion with other intrathoracic conditions, such as pneumatoceles and pneumothorax, if an incorrect diagnosis is to be avoided. Misinterpretation of the radiographs is likely if the possibility of a congenital diaphragmatic hernia is not considered, and may result in increased morbidity. Gastric atony may occur in the early postoperative period.