Heterotopic Pancreas Located at the Gastroesophageal Junction in a Hiatal Hernia: A Case Report

Abstract

Heterotopic pancreas, commonly referred to as pancreatic rest or ectopic pancreas, is a congenital anomaly in which pancreatic tissue is anatomically separate from the main pancreatic gland without continuity of a duct system or vascularity. It is commonly found in the upper gastrointestinal tract, specifically in the stomach and small intestine. To date, only about 18 adult cases of heterotopic pancreas in the esophagus have been reported in the English medical literature; seven of which were in women, and five of which originated at the gastroesophageal junction (GEJ). Of these five cases, only two occurred in a hiatal hernia. We report the third case of the heterotopic pancreas at the GEJ in a hiatal hernia discovered in a 62-year-old Caucasian female who presented to the outpatient clinic complaining of worsening reflux characterized as burning retrosternal chest pain. The patient failed maximum medical therapy and was referred to general surgery for laparoscopic hiatal hernia repair with Toupet fundoplication to prevent further reflux and damage to the esophagus. The reflux symptoms persisted after the procedure. Follow-up esophagogastroduodenoscopy with biopsy of the GEJ revealed a small focus of heterotopic pancreas tissue, confirmed by histopathology. The management of heterotopic pancreas differs throughout the literature depending on the size, symptomatology, and potential for malignancy. Management in cases of pancreatic rest, specifically at the GEJ, ranges from observation with conservative medical therapy, resection, or esophagectomy. With this case, we aim to contribute to the literature with the third case of pancreatic rest in the GEJ of a hiatal hernia.

Pancreatic Heterotopia at the Gastroesophageal Junction: A Case Report and Review of the Literature

“…Pancreatic heterotopia is defined as pancreatic tissue that lacks direct anatomical or vascular continuity with the body of the pancreas but displays a similar physiological function [ 2 ]. It has a high prevalence in middle-aged males and is normally identified within the submucosal layer of the gastrointestinal tract in the stomach, duodenum, or jejunum with rare cases at the gastroesophageal junction [ 5 , 6 ].…”

Expression and clinicopathological characteristics of PDX1, PTF1A, and SALL4 in large and small ducts of ectopic pancreas located in gastro-duodenum and jejunum

“…The treatment of ectopic pancreas varies with symptomatology, size, and potential malignancy. Management of pancreatic rest, especially at the gastroesophageal junction, including observation of conservative medical therapy, resection, or esophagectomy [ 3 ]. In recent years, the treatment of ectopic pancreas has shifted from open to laparoscopic surgery and more recently to robotic surgery [ 4 ].…”