Long-term results of delayed primary anastomosis for pure oesophageal atresia: a 27-year follow up

Abstract

The management of a newborn with pure oesophageal atresia continues to be challenging. We started treating babies with pure oesophageal atresia by delayed primary anastomosis in 1977. The purpose of this study was to review the long-term outcome in infants with pure oesophageal atresia (EA) treated by delayed primary anastomosis with special emphasis on gastroesophageal reflux (GOR) related morbidity. The medical charts of all patients treated by delayed primary anastomosis between 1977 and 2004 were retrospectively reviewed. All survivors were followed up with completion of a questionnaire and personal/phone interviews. There were 26 patients in total admitted during the 27-year study period with the diagnosis of pure oesophageal atresia. Three died prior to surgery due to associated anomalies; two had almost no distal oesophageal segment and underwent oesophageal replacement surgery. The remaining 21 children were treated with delayed primary anastomosis and made up our study group. There were four deaths (19%) in this group, and all were prior to 1980. The median gestational age was 35.5 weeks and the median birth weight was 2.6 kg; median initial gap was 3.7 cm and median preoperative gap was 1.5 cm; median age at operation was 80 days and the median hospital stay was 5.5 months. The median follow-up period was 13.5 years. Fourteen children (66%) developed symptomatic gastroesophageal reflux and nine of these needed fundoplication (43%). Sixteen children developed strictures at the anastomotic site; ten responded to repeated dilatations while six needed resection and reanastomosis. At the time of this study, 15 out of the 17 survivors (88%) were on normal diet with no respiratory problems and 2 (12%) were dependent on gastrostomy feeds. Our long-term follow-up data shows that the delayed primary anastomosis provides excellent functional results in patients born with pure oesophageal atresia. The high incidence of gastroesophageal reflux and associated morbidity requires early intervention to prevent ongoing feeding problems due to oesophagitis and stricture formation.

Surgical outcomes of esophageal atresia without fistula for 24 years at a single institution

“…This series would appear to be representative of that complex subgroup of EA patients, those without a fistula [8,16]. The male predominance, prematurity, frequency of twin gestation, and low birth weights appear characteristic for infants with this abnormality [4,17,18].…”

“…Although a minority of patients may be amenable to primary repair in the neonatal period, most will require a staged approach (involving more than one operative procedure) to the restoration of esophageal continuity [7][8][9]. In many of these patients, esophageal replacement will be required.…”

“…Although diagnosis is usually straightforward, in many cases, surgical repair remains difficult because of the "long gap" between upper and lower esophageal pouches [7,8].…”

Piglet Model for Studying Esophageal Regrowth after Resection and Interposition of a Silicone Stented Small Intestinal Submucosa Tube

“…Of these, approximately 8% have pure esophageal atresia with partial agenesis of the lower segment, known as long-gap esophageal atresia [3,4], making the incidence of long-gap esophageal atresia a rare condition occurring in approximately 0.002% of the population. The current treatment of choice for long-gap esophageal atresia is delayed primary repair [4,5]; however, when primary repair is not possible, a replacement esophagus may be fabricated using part of the stomach or bowel. These replacements give a poorly functioning esophagus, and are also associated with considerable surgical risk [5,6,7,8].…”

“…The current treatment of choice for long-gap esophageal atresia is delayed primary repair [4,5]; however, when primary repair is not possible, a replacement esophagus may be fabricated using part of the stomach or bowel. These replacements give a poorly functioning esophagus, and are also associated with considerable surgical risk [5,6,7,8]. …”