Congenital esophageal stenosis owing to ectopic tracheobronchial remnants

Congenital esophageal stenosis owing to tracheobronchial remnants (TBR) is a rare condition. Inappropriate treatment often is carried out before the correct diagnosis is established. For a better understanding and management of this disease, patients with TBR treated at our hospital and collected from the literature are reviewed to evaluate the course of onset, associated anomalies, methods of diagnosis and treatment, and outcomes.

Three patients treated at our hospital and 59 patients identified from the literature were included in the study. Gender, age at onset of symptoms, age at definitive treatment, esophagographic findings, pathology, methods of treatment, and outcomes of the 62 patients were recorded and analyzed.

Boys slightly predominated (33:28, 1 unknown gender). Symptoms of dysphagia and regurgitation developed at the mean age of 3.2 +/- 4.5 months. Definitive treatment was carried out at the mean age of 2.6 +/- 3.0 years with a time lag of 2.0 +/- 2.5 years from the onset of symptoms. Twenty-five patients had associated anomalies with esophageal atresia being the most prevalent. Esophagography showed segmental stenosis over the distal third of the esophagus in all patients except one. The esophagographic findings could be classified into 3 types: type Ia, 10 cases; Ib, 15 cases; type II, 14 cases; type III, 3 cases. A nonyielding esophageal stenosis without inflammation was the characteristic esophagoscopic finding. Esophagoscopic dilatation of the stenosis was attempted in 26 patients, but all failed, and 2 patients suffered esophageal perforation. Surgical resection was mandatory for all patients to restore their esophageal continuity. Postoperative complications included anastomotic stenosis, anastomotic leakage, hiatal hernia, and gastroesophageal reflux.

TBR should be suspected in patients who present with a typical history of dysphagia after ingestion of solid food and have characteristic esophagographic and esophagoscopic findings. It has a strong tendency to occur with esophageal atresia. Esophagoscopic dilatation is ineffective and may render the patient at risk for esophageal perforation. Operation is the treatment of choice and carries little morbidity and mortality.