Type D congenital esophageal atresia associated with omphalomesenteric duct remnant

Abstract

Esophageal atresia is a relatively common congenital malformation occurring in 1 per 2500-3000 live births. The classification by Gross is used. 1 Esophageal atresia with proximal and distal tracheo-esophageal fistula (Gross D, <1%) is a rare anomaly. Anomalies of the omphalomesenteric duct remnant occur in approximately 2% of the population. We present herein the case of a patient with Gross type D congenital esophageal atresia and omphalomesenteric duct remnant.A girl was born at gestational week 37 via normal spontaneous vaginal delivery, weighing 2128 g. Apgar scores were 8 and 9 at 1 and 5 min, respectively. After delivery, a nasogastric tube could not be passed into the stomach. Radiography showed the nasogastric tube coiled in the upper esophagus with bubbles in the stomach and abdomen. Gastrostomy was performed at 2 days old, with division of proximal and distal tracheo-esophageal fistulae and esophago-esophagostomy at 4 days old. Gastric and oral feedings started on day 11 and 19, respectively. The abdomen became distended after oral feeding, however, and plain abdominal radiography demonstrated a mildly dilated bowel. Abdominal ultrasonography identified a mass (26 mm ¥ 32 mm) in the right upper quadrant. At 21 days old the patient underwent laparotomy. A marked small-bowel dilation was noted and a fibrous band connecting the umbilicus to the small bowel was identified as the focal point for small-bowel stenosis. A 10 cm section consisting of both stenosed and dilated small intestine was resected. Oral feeding was restarted 6 days postoperatively. At 53 days old, balloon dilatation was needed due to anastomotic stricture using 8 mm ¥ 4 cm balloon. Clinical course has since been uneventful and the patient was discharged free of complaints at 90 days old.Chittmittrapap et al. reviewed the records of 253 patients with esophageal atresia with or without tracheo-esophageal fistula. One hundred and twenty-two (48%) of 253 patients had a total of 213 other anomalies. Most commonly affected were the cardiovascular (29%), anorectal (14%), and genitourinary (14%) systems. 2 Omphalomesenteric duct remnant, including Meckel's diverticulum, is found in approximately 2% of the population. Complications from this abnormality are most often seen in childhood, with intestinal bleeding as perhaps the most common complication. Obstruction, another frequent complication, may arise from various mechanisms. A fibrous band develops when the omphalomesenteric duct fails to regress completely. In the present case this band attached to the back of the umbilicus, causing stricture and ileus of the small bowel. As the quantity of lactation increases, obstructive symptoms might result.Embryologically, the primitive foregut divides into the trachea and esophagus during gestational week 4. If this division fails to occur, esophageal atresia and tracheo-esophageal fistula result. 3 The omphalomesenteric duct in the embryo represents a communication between the yolk sac and primitive midgut. This duct begins to close by gestational wee...

Diagnosis and management of congenital type D esophageal atresia

“…Type D EA is characterized by both a distal and proximal TEF and accounts for only 1% of all cases of EA. A few case reports of type D EA are present in the literature [ 5 – 7 ].…”