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

Abstract

BackgroundHeterotopic 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 presentationHere 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.ConclusionsUpon 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.

Rare case of ectopic pancreas presenting with persistent umbilical discharge

“…Zhao et al . reviewed 18 patients with ectopic pancreas at the umbilicus and reported that most of them presented with umbilical discharge . A definitive preoperative diagnosis is difficult to obtain, because ectopic pancreas is hard to detect.…”

“…Zhao et al . suggested that biopsy was useful for deciding on appropriate treatment for persistent umbilical discharge . For example, measuring the pancreatic enzymes in the liquid from the umbilicus may aid in diagnosis.…”

Ectopia of the pancreas in the loop of the small intestine in an 8-y-o child

“…В исследованиях Z. Zhao и соавт. [6] обнаружено, что лишь 14 из 60 обследованных пациентов были в возрасте до 18 лет, а подавляющее большинство -взрослые [6].…”

“…Основной метод верификации диагноза -гистологическое [2]. Не следует забывать, что эктопированная поджелудочная железа может стать причиной для малигнизации, исходящей из этой ткани [6,14]. Поэтому к этой группе пациентов следует относиться с онкологической настороженностью и полностью резецировать измененные отделы.…”

Spectrum of Omphalomesenteric Duct Related Anomalies and Their Surgical Management in Children

“…Heterotrophic tissue is also known to be associated with OMD remnants, particularly with Meckel’s diverticulum. This predisposes to local hyperacidity and mucosal ulceration, causing recurrent abdominal pain or per rectal bleeding in children [ 6 , 7 ]. Various surgical options have been described in the literature, ranging from wedge or segmental resection in case of Meckel’s diverticulum to umbilical exploration in case of umbilical discharge.…”