Congenital paraesophageal hernia: The Montreal experience

Congenital paraesophageal hernia (CPEH) is the least common congenital diaphragmatic hernia. We performed an extensive review to further define this entity.

A retrospective study of children with CPEH (types II-IV hiatal hernias) treated at two children's hospitals over a 25-year period (1988-2013) was performed.

Fourteen patients were diagnosed at a median age of 35 days (range 0-500), with one prenatal diagnosis. The most common symptoms were emesis in type II hernias (50%), and respiratory distress in types III and IV hernias (75% and 50%, respectively). Gastroesophageal reflux was concomitantly diagnosed in 50%, while concurrent congenital anomalies existed in 36%. A correct initial diagnosis was made in only 29% of cases. The diagnosis was most often established by UGI study (64%). The method of repair was laparoscopy in 21%, and laparotomy in 71%. An antireflux procedure was done in 13 (93%) patients. Long-term follow up data was available for 86% of patient. During follow-up, one patient recurred and one required sequential pneumatic esophageal dilations. There were no mortalities.

CPEH is a rare entity often associated with gastroesophageal reflux disease and other congenital anomalies. Prognosis is excellent, but awareness of this anomaly may lead to earlier diagnosis.