Long gap esophageal atresia: an Australian experience

BackgroundThe diagnosis of long gap esophageal atresia (LGEA) may preclude immediate primary anastomosis. We reviewed our experience with this entity for a period of 10 years.MethodsA retrospective review was undertaken of the medical records of all patients managed for esophageal atresia (EA)/tracheoesophageal fistula (TEF) during the period from 1991 to 2001 at the Children's Hospital at Westmead, Sydney, Australia. Esophageal atresia was defined as long gap when primary repair was considered technically impossible by the surgeon. Also, a questionnaire was sent to all the general pediatric surgeons in Australia to explore their attitude toward LGEA management.ResultsOne hundred three patients with EA were managed for that period, 17 (16%) of them were defined as LGEA, with mean gap of 5 cm (SD, 1cm). Eight patients (47%) had TEF. Sixteen patients had gastrostomy tube (GT) insertion at a mean age of 4 days. Six patients had esophagostomy at a mean age of 27 days. Thirteen patients had EA repair at a mean age of 146 days. Four patients died before repair and 2 after repair secondary to associated anomalies. Fifty percent of Australian Pediatric Surgeons (APS) responded to the questionnaire. Forty percent defined LGEA as a gap more than 3 to 4 vertebral bodies, whereas 24% considered the absence of TEF as an indication of LGEA. Fifty-six percent of APS will measure the gap on preoperative chest x-rays, and 80% will assess the gap at thoracotomy for ligation of TEF. Ninety-two will measure the gap by inserting a bougie into the upper pouch and into the lower esophagus via the GT. If LGEA was diagnosed, all APS will perform GT with delayed repair. Seventy-two percent of APS will attempt delayed primary repair within 3 to 6 months of age. Seventy-six percent will perform hiatal dissection, and 48% will use upper pouch myotomies. Forty-eight percent will perform gastric pull up, and 32% will use gastric tube for esophageal replacement.ConclusionsLong gap esophageal atresia represents a surgical challenge. Mortality rate is high secondary to associated anomalies. There is no consensus among APS regarding the definition of LGEA. In general, the consensus of APS would be that the preservation of the patient's own esophagus should be attempted before considering the use of an esophageal replacement.