An association of congenital abnormalities that consists of vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia.
* Vertebral anomalies * Ventricular septal defects * Cardiac defects * Anal atresia * Anal fistula * Tracheoesophageal fistula * Esophageal atresia * Radial dysplasia * Thumb dysplasia * Radial hypoplasia * Preaxial polydactyly * Syndactyly * Renal anomaly * Single umbilical artery * Failure to thrive * Slow infant developmental progress * Vertebral defects * Renal dysplasia * Tracheoesophageal fistula * Radial dysplasia * Imperforate anus
Tracheoesophageal fistula and esophageal atresia result from failure of the embryonic esophagus and trachea to develop and separate correctly. Respiratory system development begins at about day 26 of gestation. Abnormal development of the septum during this time can lead to tracheoesophageal fistula. The most common abnormality is type C tracheoesophageal fistula with esophageal atresia, in which the upper section of the esophagus terminates in a blind pouch, and the lower section ascends from the stomach and connects with the trachea by a short fistulous tract.
Correction of esophageal atresia alone requires anastomosis of the proximal and distal esophageal segments in one or two stages. End-to-end anastomosis commonly produces postoperative stricture; end-to-side anastomosis is less likely to do so. If the esophageal ends are widely separated, treatment may include a colonic interposition (grafting a piece of the colon) or elongation of the proximal segment of the esophagus by bougienage. About 10 days after surgery, and again 1 and 3 months later, X-rays are required to evaluate the effectiveness of surgical repair. Postoperative treatment includes placement of a suction catheter in the upper esophageal pouch to control secretions and prevent aspiration, maintaining the infant in an upright position to avoid reflux of gastric juices into the trachea, I.V. fluids (nothing by mouth), gastrostomy to prevent reflux and allow feeding, and appropriate antibiotics for pneumonia.