Heterotopic pancreas in the omphalomesenteric duct remnant in a 9-month-old girl: a case report and literature review

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Heterotopic pancreas in the omphalomesenteric duct remnant in a 9-month-old girl: a case report and literature review Zitong Zhao1, Chiang Khi Sim2 and Sangeeta Mantoo1*

Abstract Background: Heterotopic pancreas most commonly occurs in the upper gastrointestinal tract of adults, usually as an incidental finding. It seldom occurs at the umbilicus, and even rarely in the pediatric age group. Case presentation: Here we present a case of heterotopic pancreatic tissue in the omphalomesenteric duct remnant of a 9-month-old baby girl. She presented with redness at the base of the umbilicus associated with occasional mild wetness. A urachal fistula was suspected by ultrasound. Histology from subsequent resection revealed fibrous tissue with heterotopic pancreatic tissue and accompanying small bowel mucosa. The patient’s umbilical redness resolved after the surgery. Conclusions: Upon literature search, we found only 17 other cases of heterotopic pancreas reported in the umbilicus. They described a high male to female ratio, frequent association with omphalomesenteric duct remnant and presentation of umbilical discharge. The Heinrich system is frequently used to classify heterotopic pancreas into 3 types, based on the presence of acini, islets and ducts. Several mechanisms have been proposed on the pathogenesis of heterotopic pancreas, including misplacement, metaplasia and totipotent cell theories. Heterotopic pancreas can manifest clinically with diseases of the pancreas, including malignant transformation, reported as high as 12.7% in a series. Awareness of this finding in the biopsy aids the suitable treatment decisions for the patient. Keywords: Heterotopic pancreas, Omphalomesenteric duct remnant

Background Umbilical discharge is a common pediatric problem. An underlying congenital anomaly should always be considered. Heterotopic pancreas is the second most common congenital anomaly of the pancreas, most commonly occurring in the upper gastrointestinal tract of adults. It seldom occurs at the umbilicus, and even rarely in the pediatric age group. Here we present a 9-month-old baby girl with heterotopic pancreatic tissue in the omphalomesenteric duct (OMD) remnant. Literature review of other 17 cases of heterotopic pancreas reported at the umbilicus has been performed [1–12]. * Correspondence: [email protected] 1 Department of Anatomical Pathology, Division of Pathology, Singapore General Hospital, Singapore, Singapore Full list of author information is available at the end of the article

Case presentation Clinical presentation

A 9-month-old baby girl presented with redness at the base of the umbilicus since her umbilical cord fell off, without accompanying discharge or smell. However, there was mild wetness in the umbilicus when she cried. The mother experienced an uneventful pregnancy and delivery. No congenital anomalies were discovered. There was no other medical history or family history of medical issues. Ultrasound revealed a tubular structure at the umbilicus with po