Congenital oesophageal stenosis in oesophageal atresia: underrecognised and often missed?

Purpose Congenital oesophageal stenosis (COS) is characterised by an intrinsic oesophageal narrowing that is present, but not necessarily symptomatic at birth. Small studies report an association of COS with oesophageal atresia (OA) in up to 14% of OA cases. Although OA is usually appreciated shortly after birth, the diagnosis of a concomitant COS is frequently delayed. This risk may be increased with the current movement away from routine postoperative upper gastrointestinal (GI) contrast study following OA repair. We performed a systematic review of the literature to assess the timing of diagnosis of COS in patients with COS and OA and how this impacted on patient outcomes. Methods A systematic review in accordance with PRISMA guidelines was undertaken. Only patients with OA associated with COS were included. Delayed diagnosis was defined as presentation > 1 month of age. Results 14 full-text studies with a total of 131 patients were included. Diagnosis of COS was delayed in 62/131 (47%) patients. These children presented with symptoms of dysphagia and aspiration at a median age of 13.5 months (IQR 7–30 months). In total, 18/131 patients were identified at the initial operation, due to difficulty passing a tube distally into the stomach. The data on timing of contrast studies were provided in 60/131 (46%) patients. A routine postoperative contrast study was performed in 39/60 (65%) of these, of which COS was identified immediately in 28/39 (72%). A diagnosis of COS could also be made on retrospective review of the early contrast study in a further 6/39 patients, giving an overall sensitivity of 87%. Conclusion The association of COS and OA may be underrecognised and diagnosis delayed if routine contrast study is not performed. Contrast studies, performed in the neonatal period are effective at detecting a concomitant COS (sensitivity > 87%). This review supports routine early contrast study after OA repair with specific consideration of the presence of COS.