Yasmine Yousef1, Caroline Lemoine2, Dickens St-Vil2, Sherif Emil3. 1. Division of Pediatric General and Thoracic Surgery, The Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada. 2. Division of Pediatric General Surgery, Centre Hospitalier Universitaire Sainte Justine, Montreal, Quebec, Canada. 3. Division of Pediatric General and Thoracic Surgery, The Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada. Electronic address: Sherif.Emil@McGill.ca.
Abstract
INTRODUCTION: Congenital paraesophageal hernia (CPEH) is the least common congenital diaphragmatic hernia. We performed an extensive review to further define this entity. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
INTRODUCTION:Congenital paraesophageal hernia (CPEH) is the least common congenital diaphragmatic hernia. We performed an extensive review to further define this entity. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.