Abstract

Question: A 2-week-old, full-term boy was seen in the clinic with a 3-day history of yellow colored drainage coming from the umbilicus. He was initially seen by his pediatrician and subsequently referred to the tertiary hospital owing to the great concern for drainage noted from the umbilicus. He presented with no recent history of fever, rash, increased fussiness, lethargy, or signs of inflammation around the umbilicus. He was feeding well and seemed appropriate for his age. He did have other congenital anomalies, including mid cleft lip and palate and malformed ears. Physical examination revealed an alert, active, and well-developed infant. He weighed 3.54 kg (∼30th percentile), height 49.0 cm (∼20th percentile), and the head circumference was 34 cm (∼10th percentile). Vital signs were normal. He had midline cleft lip and palate, and mid face hypoplasia. The heart, chest, extremities, skin, and neurologic examination were grossly normal. The abdomen was nontender, nondistended, and without organomegaly. There was a yellow, soft substance emerging from the umbilicus with minimal erythema and tenderness at the umbilicus. A barium enema was obtained to evaluate the intra-abdominal anatomy and the discharge from the umbilicus (Figure A). The retrograde filling of the small bowel with radiopaque contrast material revealed a fistulous connection from the small bowel to the umbilicus. The contrast streamed out of the umbilicus onto the anterior abdominal wall (Figure B). What is the most likely diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The streaming of the contrast from the umbilicus during the barium enema confirmed a enterocutaneous fistula from the small intestines. If the omphalomesenteric (OM) duct is patent from the small bowel to the umbilicus, then fecal umbilical drainage will be noted. The OM duct or the vitelline duct is the remnant of connection between the embryonic midgut and the yolk sac. This duct provides nutrition to the developing embryo until the placenta is established.1Zafer Y. Yigit S. Turken A. et al.Patent omphalomesenteric duct.Turk J Med Sci. 2000; 30: 83-85Google Scholar By the 9th week of gestation, the duct separates from the intestines. However, if this involution fails, various residual malformations can occur. Meckel's diverticulum is the most common associated anomaly. The occurrence of patent OM ducts is rare. A contrast study is the preferred approach when an umbilico-enteric fistula is suspected. This will identify a potential intraperitoneal connection with the bowel or an extraperitoneal communication with the urinary tract.2Konvolinka C.W. Patent omphalomesenteric duct.Surgery. 2002; 131: 689-690Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar Operative resection of the remnant or the umbilical anomaly is the preferred method of treatment.3Carlisle E.M. Mezhir J.J. Glynn L. The umbilical mass: a rare anomaly.Pediatr Surg Int. 2007; 23: 821-824Crossref PubMed Scopus (19) Google Scholar Segmental resection of the intestine containing the fistula or a simple excision of the diverticulum is acceptable. This patient underwent operative repair by identifying the OM remnant leading from the mid jejunum to the underside of the umbilicus. The OM fistula was resected with an end-to-end sutured anastomosis of the jejunum (Figures C and D). Interestingly, tissue pathology reports showed small segments of squamous mucosa contiguous with small bowel mucosa (Figure E) and a focal area of foveolar (gastric) mucosa (Figure F). Both Meckel's diverticulum and OM cyst may contain gastric mucosa that secretes hydrochloric acid;2Konvolinka C.W. Patent omphalomesenteric duct.Surgery. 2002; 131: 689-690Abstract Full Text Full Text PDF PubMed Scopus (12) Google Scholar the presence of gastric mucosa has not been reported in OM fistulas and was identified in this case.

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