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

To evaluate long-term outcomes of pure esophageal atresia (EA) repair with preservation of native esophagus.

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).

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.

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.