Management and Outcomes of Intestinal Atresia: A Single Institution Experience From 1947 to 2019

Abstract

Aim of the study: We aimed to evaluate disease characteristics, associated malformations and surgical outcomes of congenital intestinal atresia. Methods: We identified all patients with jejunoileal (JIA) or colonic atresia (CA) treated at the Helsinki University Children's hospital during 1947–2019 and collected clinical data retrospectively from archived and electronic medical records. Results: Of the 180 included patients, 156 had JIA and 24 CA. Overall survival improved markedly from 34% during 1947–1977 to 93% during 1978–2019. Rate of primary anastomosis (81% in JIA, 21% in CA) remained unchanged while early surgical complications decreased (32% vs 18%, P = 0.04) and prematurity rate increased (21% vs 45%, P = 0.002) among JIA patients over time. Around half of patients had associated, mostly gastrointestinal malformations which occurred comparably in JIA and CA. During 1978–2019, 20 (21%) JIA patients, but none of CA patients, developed short bowel syndrome. Presence of type 3b atresia (n = 16) or gastroschisis (n = 14), but not the extent of intestinal resection or surgical complications, was associated with development of short bowel syndrome without decreasing survival. Shorter primary bowel resection without tapering surgery in JIA predisposed to repeated resections due to obstructive symptoms, and prolonged duration of parenteral nutrition. Conclusion: Survival of patients with JIA and CA has improved remarkably over time and is currently high despite frequent prematurity and associated malformations. In patients with JIA, apple peel atresia and gastroschisis predisposed to short bowel syndrome without jeopardizing high survival rates. Level of Evidence: IV.