Oesophageal atresia with very low birth weight: Clinical characteristics and long-term outcome

Abstract

Aim of the StudyAn assessment of the clinical data and outcome of patients with oesophageal atresia (OA) with very low birth weight (VLBW) was completed. MethodWith ethical consent, we reviewed the records of 327 successive patients with OA from 1980 to 2020. Main outcome measures survival and oesophageal repair were compared between patients with VLBW(≤1500 g) and with BW>1500 g. ResultsThirty-four (10%) patients had VLBW. Gross types of OA in VLBW were similar as in other patients: A (15%/7%), B (3%/3%), C (78%/82%), D (3%/4%), E (0%/7%), F (0%/1%) (p = 0.16–0.99). In VLBW the incidence of congenital heart disease (CHD) (47%) and trisomy 13/18 and Cri du Chat (15%) were higher than in BW>1500 g (23% and 1%), (p = 0.001 both). In VLBW one-month mortality was 35% vs 4% in patients with BW>1500 g (p < 0.001), overall mortality 56% and 8% (p < 0.001), respectively. Cause of one-month mortality in VLBW (12 patients) were CHD w/wo chromosomal abnormality (n = 7), cerebral hemorrhage (n = 2), gastric perforation (n = 1), anastomotic leakage (n = 1) and pulmonary hemorrhage (n = 1). Of VLBW patients 79% and of other patients 99% underwent oesophageal repair (p < 0.001). Repair in VLBW patients included early (n = 18) or delayed (n = 5) end-to-end anastomosis and reconstruction (n = 4). Anastomotic complications occurred in 24% of patients with VLBW and in 17% with BW>1500 g, (p = 0.31). From 1980–2000 to 2001–2020 survival in VLBW changed from 11% to 81% (p = 0.002). During 2001 – 2020 all VLBW patients underwent repair. ConclusionOA with VLBW had high incidence of life-threatening associated anomalies and decreased survival. Recently survival and rate of oesophageal repair have improved significantly.