RT Book, Section A1 Patel, Nishant D. A1 Meneshian, Avedis A2 Yuh, David D. A2 Vricella, Luca A. A2 Yang, Stephen C. A2 Doty, John R. SR Print(0) ID 1104586107 T1 Congenital Esophageal Anomalies T2 Johns Hopkins Textbook of Cardiothoracic Surgery YR 2014 FD 2014 PB McGraw-Hill Education PP New York, NY SN 978-0-07-166350-2 LK accesssurgery.mhmedical.com/content.aspx?aid=1104586107 RD 2024/11/07 AB EpidemiologyEsophageal atresia and tracheoesophageal fistula (EA–TEF) occurs in 1 in 3400. Esophageal duplication cysts represent 10 to 15 percent of foregut duplication cysts and 5 to 10 percent of mediastinal cysts. The true incidence of esophageal duplication cysts is unknown. Congenital esophageal stenosis and webs occur in 1 in 25,000 to 50,000 live births.ClassificationAnatomic variations of EA–TEF include isolated EA, EA with distal TEF, EA with proximal TEF, EA with distal and proximal TEF, or N-type TEF. EA with distal TEF is the most common. Esophageal duplication cysts can be cervical, thoracic, or abdominal. Esophageal stenosis and webs can be characterized on the basis of etiology into fibromuscular thickening, trachobronchial remnants, and membranous web.Clinical featuresEA–TEF is suggested by polyhydramnios, and infants present with drooling, rhonchi, aspiration with subsequent respiratory distress. Esophageal duplication cysts are often asymptomatic. Symptoms due to esophageal duplication cysts are secondary to mass effect and include dysphagia and respiratory insufficiency. Congenital esophageal stenosis and webs present in infancy with dysphagia and vomiting of solid food.DiagnosisEA–TEF is readily diagnosed with passage of a 10-F esophageal tube and gentle insufflation, and plain chest radiograph. The presence of air in the stomach confirms a distal TEF, and its absence suggests isolated EA. Esophageal duplication cysts are also diagnosed on plain film and confirmed with esophagography and CT. Esophageal stenosis and web are diagnosed with esophagography and endoscopy.TreatmentSurgical repair of EA–TEF consists of a single-layer primary anastomosis of the proximal and distal esophageal segments and ligation of any TEF. Esophageal duplication cysts are resected. Esophageal stenosis and webs are initially managed conservatively with dilatation. Surgical treatment is reserved for stenosis refractory to dilatation and consists of resection of the stenotic segment and primary anastomosis.ComplicationsOutcomes following repair of EA–TEF are good. Early complications include anastomotic leak and subsequent sepsis. Mid-to-late-term complications include stricture, recurrent fistula, and gastroesophageal reflux.