Surgical problems in patients with VATER-associated anomalies.

From 1960 through 1980, 14 patients with esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) with two or more other associated anomalies of VATER have been seen. Seventy percent of patients were preterm or low in birth weight. In addition to the esophageal abnormality, vertebral anomalies were found in 7 patients, anorectal anomalies in 11, limb anomalies in 9 and renal anomalies in 9 patients. Three patients had associated duodenal atresia and 4 had cardiac anomaly. Eight patients are dead, 5 are living, and 1 is lost to follow-up. Six of the eight deaths occurred during 1960 to 1970. Preliminary gastrostomy was done in all patients. Primary esophageal repair was carried out in 9 patients--5 died and 4 survived. Three patients had delayed esophageal repair and two of these patients survived. Cervical esophagostomy was done in one patient with EA without TEF after unsuccessful repeated bouginage for elongation of the upper esophageal pouch. Three of the survivors showed growth retardation--below 5th percentile. Aggressive nutritional support and management of chronic problems related to the anomalies are important.