Characteristics and treatment of congenital esophageal stenosis: A retrospective collaborative study from three Japanese children's hospitals

Abstract

Background/Purpose: There is no consensus on treatment strategy of congenital esophageal stenosis (CES). This study aimed to assess appropriateness of the treatment we have provided to patients with CES over the past four decades.Methods: We carried out a retrospective chart review of 83 CES patients treated at three children's hospitals between 1973 and 2015. Each patient underwent an initial treatment with either surgery or a series of dilation that was followed by surgery if dilation failed to improve esophageal transit. Demographic data, course of treatment, outcomes, and complications were analyzed.Results: During this initial treatment, 19 and 64 patients underwent surgery and dilation, respectively. Out of the 64 patients who underwent dilations as an initial treatment, 26 patients eventually required surgery. Out of all patients who required surgery (19 initial treatments + 26 failed dilations), 29 had tracheobronchial remnants and 16 had fibromuscular hypertrophy. Six patients experienced esophageal perforation during dilation and ten experienced anastomotic leakage after surgery. No patients had swallowing difficulties at the latest follow up, 141(9-324) months.Conclusions: Dilation is recommended as an initial therapy, especially if histological diagnosis of CES is uncertain. Persistent swallowing difficulties after 2 series of dilation may be an indication for surgery.Levels of evidence: level IV