Congenital esophageal stenosis: Therapeutic strategy based on etiology.

BACKGROUND/PURPOSE The diagnosis and treatment of congenital esophageal stenosis (CES) can be a vexing clinical problem. This study aims at determining the ideal therapeutic strategy for the management of CES. METHODS Medical records of patients with CES were reviewed retrospectively with regard to diagnostic method, therapy, and outcome. RESULTS During the last 29 years, 36 patients, aged 1 month to 9 years, were found to have CES. Diagnosis was made by fluoroscopy, esophagoscopy, endoscopic ultrasonography (EUS), and pathologic examination. Of the 36 patients, 15 had tracheobronchial remnants (TBR), 13 had fibromuscular stenosis (FMS), 5 had membranous stenosis (MS), and 3 had multiple stenoses (MPS). EUS was diagnostic in distinguishing TBR from FMS. All patients with TBR underwent operative repair in which 13 patients had resection and anastomosis with or without preoperative dilation. The 14th patient had enucleation and the 15th underwent myotomy. Postoperative dilation was needed 2.2 times for 2.1 months on average in this group of patients. Ten patients with FMS were treated by dilation only (a mean of 4.7 dilations was required for a duration of 2.6 years), whereas 3 patients underwent open surgical intervention. Most patients with MS or MPS were treated by dilation (with or without endoscopic resection of the membranes), whereas 2 patients had surgical intervention. All patients in this study are doing well after a mean follow-up period of 5.3 years (range, 0.5 to 25 years) without any further operative interventions. CONCLUSIONS If the etiologic diagnosis of CES is unclear, EUS is useful in distinguishing TBR from FMS. This distinction is critical, because patients with TBR should undergo surgical resection, whereas most cases of FMS, MS, and MPS can be treated with esophageal dilation alone.