Aim of the study:
Gastro-oesophageal reflux following oesophageal atresia (OA) repair may induce pathological oesophageal mucosal changes including Barrett’s oesophagus, a known malignant precursor. We aimed to determine the incidence of Barrett’s oesophagus and oesophageal cancer, following OA repair, to quantify the magnitude of this association and inform the need for, and design of, a screening program.
Systematic review of published literature in December 2017. Included papers were published in English and reported either: (a) ≥1 case of either Barrett’s oesophagus (gastric or intestinal metaplasia) or oesophageal cancer; or (b) studies reporting long term (>2 years) follow-up of OA. Duplicate cases published in multiple reports from the same centre were excluded. Data are median (range).
From total 1586 studies retrieved from database searches, 207 full texts were reviewed and 45 papers were included.
There were 9 reported cases of oesophageal cancer (3 adenocarcinoma, 6 squamous cell carcinoma) reported in 5 papers from 4 centres in 3 countries. Median age at diagnosis 44 years (20-46). At last recorded follow-up, 6 patients were alive and receiving treatment and 3 had died.
248 cases of Barrett’s oesophagus were reported in 39 papers from 23 centres in 15 countries (intestinal metaplasia [n=46], gastric metaplasia [n=123], metaplasia type not specified [n=79]). Age at diagnosis median 8 years (7 months-30 years). Prevalence of Barrett’s following OA in series reporting long term endoscopic follow-up was 10% (2.5-36%)
There is a notable global prevalence of Barrett’s oesophagus following OA repair in screened populations. Precise quantification is challenging due to a lack of standardised follow-up and reporting. Oesophageal malignancy is rare following OA repair but has a younger age at presentation compared to non-OA cases. These data may be used to inform the case for routine endoscopic screening of the oesophagus following OA repair.