Late-Presenting Congenital Diaphragmatic Hernia in Children

Abstract

Congenital diaphragmatic hernia (CDH) is a typical neonatal emergency. There is, however, a subset of patients who manifest this congenital anomaly in infancy and early childhood. Late-presenting diaphragmatic hernia is characterized by a variety of gastrointestinal and respiratory symptoms with acute or chronic modes of manifestation. The aim of the study. Analysis of the clinical course, diagnosis, and surgical management of latepresenting CDH in children. Material and methods. A retrospective study of medical charts and diagnostic studies was performed for children older than 1 month of age with CDH, treated in the University Department of Pediatric Surgery and Urology in Wrocław between 1975 and 2005. Results. In a 30-year period, 23 children with late-presenting CDH were treated. In one case, paraoesophageal hernia was diagnosed; Morgagni hernia was diagnosed in 2 cases. Among 20 children with postero-lateral CDH, 12 were younger than 1 year of age at diagnosis. In this group, left-sided CHD was noted in 17 children, while a right-sided defect was noted in 3. Nine infants with left CDH presented mainly respiratory symptoms; in 3 cases gastrointestinal symptoms prevailed. In the group of children beyond 1 year of age, 6 manifested chronic respiratory symptoms and 2 had mainly acute gastrointestinal complaints. In all children, CDH was diagnosed preoperatively. The most accurate diagnostic modality in 17 cases was a contrast upper GI study. Intraoperative findings revealed the presence of a hernial sac in only 3 patients. A complicated course of CHD with torsion of the stomach was noted in 2 children; intestinal necrosis was seen in one patient. Small and large bowel were the organs most commonly displaced into the thoracic cavity (noted in 12 and 11 children, respectively). In all children, surgical management of CDH was successful. Conclusions. Lack of typical clinical signs and symptoms of late-presenting CDH seems to be a factor responsible for delayed diagnosis of this anomaly in older children. Late presentation of CDH should be taken into differential diagnosis in all children presenting with gastrointestinal or respiratory symptoms. Late-presenting diaphragmatic hernia is an unequivocal indication for surgical management as soon as its diagnosis has been established.