Esophageal atresia and tracheoesophageal fistula: Management of the uncommon types

Abstract

Summary During the past six years, among 108 hospital admissions for esophageal atresia, 31 (29 per cent) have had one of the four unusual varieties, i.e., Type A, B, D, or E (“H”). In five patients with an initial diagnosis of atresia without fistula (Type A), primary esophageal anastomosis was performed following a planned delay of from four to ten weeks. This was accompanied in three infants by an elongation procedure to lengthen the proximal esophageal segment. This regimen of delayed primary anastomosis, following proximal pouch elongation, has replaced colon interposition in the management of Type A atresia in this center; and is probably the procedure of choice in premature infants with Type C atresia. Recognition of the upper tracheoesophageal fistula has proved difficult in five infants admitted with double fistula (Type D); and anastomotic leak has resulted in a high morbidity and mortality. When lying high in the neck, division of the upper fistula through a cervical incision as a separate procedure may be preferable to complete repair via thoracotomy. Major diagnostic problems are presented by the tracheoesophageal fistula without atresia, Type E (“H”). When this anomaly is seen in newborn infants with respiratory distress, the diagnosis is most readily established by the use of contrast studies of the upper esophagus. Reduced motility in the distal esophagus is a suggestive sign. In older infants and children presenting with intestinal (or abdominal) symptoms, in contrast, endoscopic studies are most helpful. All such fistulae have been divided through cervical incisions.