Retrospective cohort study of long-gap oesophageal atresia

Aim: Long-gap oesophageal atresia (LGOA) remains a rare variant of a relatively common congenital malformation. Objectives of this study were to review the short-term results including survival, length of stay and post-operative complications for infants with LGOA managed at a single centre in addition to their growth and neurodevelopmental assessment.

Methods: Retrospective review of the case notes of all infants admitted with oesophageal atresia to our institution from January 2001 to May 2011. Infants with LGOA were selected based on pre-defined criteria. Demographic and clinical variables and details of follow-up visits including developmental assessments were extracted from their case notes.

Results: Of 101 infants with oesophageal atresia, 15 fulfilled the criteria for LGOA. Overall survival was 80%. Median length of stay was 83 days. Additional congenital anomalies were present in nine (60%). A fall in weight centile during hospitalisation or outpatient follow-up signifying growth failure was seen in a majority with 11 of 13 patients showing this phenomenon. Follow-up at our institution ranged from 6 months to 9 years. Developmental assessments (Bayley-III) commenced in August 2006 were available in four patients (age 5-13 months) and were abnormal in all, with particular delay in the gross motor domain.

Conclusions: Infants with LGOA spend a long time in hospital. They remain at significant risk of growth failure during hospitalisation and following discharge. There appears to be a risk of developmental delay that warrants close monitoring.