Preservation of native esophagus in infants with pure esophageal atresia has good long-term outcomes despite significant postoperative morbidity

Purpose: To evaluate long-term outcomes of pure esophageal atresia (EA) repair with preservation of native esophagus. Methods: Infants with pure EA treated at our institution (2000–2010) and with minimum 5-year follow-up were reviewed (REB:1000046653). Data analysed included demographics, management and outcomes and are reported as mean ± SD/median (range). Results: Of 185 infants with EA, 12 (7 %) had pure EA (gestational age: 36 ± 2.4 weeks, birth weight: 2353 ± 675 g). Ten had associated anomalies, including trisomy-21 (n = 2) and duodenal atresia (n = 1). Surgery: 1 patient (short gap) underwent primary thoracoscopic anastomosis, 11 had gastrostomy (Stamm, n = 5; image-guided, n = 6) as initial procedure. At definitive repair (age: 128 ± 91 days; weight 5.5 ± 2.3 kg): ten had primary anastomosis and 1 had Collis gastroplasty. No patient had esophageal replacement surgery. Outcomes: three patients had gastrostomy dehiscence requiring re-operation. At post-operative esophagram, seven had anastomotic leak successfully treated conservatively. Seven patients developed strictures requiring balloon dilatations (median two dilatations, range 1–10), six received antireflux surgery. At 7-year follow-up (range 5–15 years), all patients had the gastrostomy closed and were on full oral feeds. Conclusions: The management of pure EA continues to be challenging. The preservation of native esophagus is possible with significant morbidity. The long-term outcomes are favourable.