Outcome of esophageal atresia/tracheoesophageal fistula in extremely low birth weight neonates (<1000 grams)

Abstract

Purpose: To review the outcomes of extremely low birth weight (ELBW, <1000 g) infants with esophageal atresia/tracheoesophageal fistula (EA/TEF).

Methods: Health records of ELBW EA/TEF infants treated at our institution from 2000 to 2014 were reviewed (REB1000046653). Demographics, operative approach and postoperative complications were analyzed. Data are reported as median (range).

Results: Of 268 EA/TEF infants, 8 (3 %, five females) were ELBW (930 g, 540-995). Gestational age was 28 weeks (23-32). Seven had type-C EA/TEF and one type B.

Outcomes: One trisomy 18 infant received no treatment and died; one initially diagnosed as type A had primary repair at 126 days of life (DOL); six underwent TEF ligation (three trans-pleural) with primary repair in one and delayed anastomosis in two (DOL 120 and 178). The remaining three died (gastrostomy dehiscence and peritonitis, liver hemorrhage during peritoneal drain insertion, severe chronic lung disease and brain hemorrhages). At a median follow-up of 3 years (range 15 months-5 years), all survivors are thriving.

Conclusion: ELBW infants with EA/TEF are rare and result in high morbidity and mortality. Mortality is mainly related to complications not associated with EA/TEF repair. Fistula ligation followed by delayed anastomosis seems to achieve a better outcome in ELBW infants.

Keywords: Collis; Esophageal lengthening; Foker; Isolated esophageal atresia; Long gap esophageal atresia; Thoracoscopy; Trisomy 14; Trisomy 18.