Recurrent Jejunal Intussusception From Heterotopic Pancreas

Abstract

Introduction Heterotopic or ectopic pancreas is usually asymptomatic. We present a unique case of heterotopic pancreas of the jejunum causing recurrent jejunal intussusception. Case description A 39-year-old male presented locally with a one-year history of recurrent left mid abdominal discomfort. Prior contrast-enhanced computed tomography (Figure 1) was reviewed and showed two areas of jejunal intussusception (arrows). Subsequent diagnostic laparoscopy done at his local hospital was unrevealing. Given ongoing recurrent pain, he came for further evaluation. On examination at our institution, the patient did have pain to palpation in the left mid abdomen without an appreciated mass. Laboratory evaluation revealed a normal complete blood count, liver test and chromogranin A. Capsule endoscopy did visualize a submucosal jejunal lesion. Double balloon enteroscopy showed a 3-centimeter distal jejunal submucosal umbilicated mass (Figure 2). The biopsy of the lesion showed normal jejunal mucosa without dysplasia. Small bowel resection was recommended for definitive diagnosis and to treat the probable recurrent intussusception. Pathologically, a solid 1.2 x 0.8 x 0.6 cm submucosal mass was found. The biopsy result showed benign jejunum with pancreatic glands and ducts extending from the mucosal surface to the muscularis propria consistent with heterotopic pancreatic glands (Figure 3, HE, x10). He has had no further episode following the surgery.FigureFigureFigureDiscussion We present a rare case of heterotopic pancreas of the jejunum causing recurrent jejunal intussusception. Heterotopic (or ectopic) pancreas is a rare congenital anomaly defined as pancreatic tissue that lacks anatomical or vascular communication with the normal body of the pancreas. It is usually found in the stomach, duodenum and jejunum. It generally has a diameter of 1-4 cm and is commonly submucosal in nature. Heterotopic pancreas is commonly asymptomatic but can cause pancreatitis, abscess, pseudocyst, malignant transformation, or local complications such as obstruction or intussusception. The diagnosis of this lesion can be challenging. Endoscopic features include umbilication and duct opening, but these findings are non-specific. Endoscopic biopsies are generally superficial and not diagnostic. As the diagnosis is challenging, surgical or endoscopic excision is recommended for both diagnosis and treatment.