Heterotopic pancreas (HP) is an aberrant anatomic malformation that occurs most commonly in the upper gastrointestinal tract. While the majority of heterotopic pancreatic lesions are asymptomatic, many manifest severe clinical symptoms which require surgical or endoscopic intervention. Understanding of the clinical manifestations and symptoms of HP is limited due to the lack of large volume studies in the literature. The purpose of this study is to review symptomatic cases at a single center and compare these to a systematic review of the literature in order to characterize common clinical manifestations and treatment of this disease.

To classify the common clinical manifestations of heterotopic pancreas.

A retrospective review was conducted of pathologic samples containing heterotopic pancreas from 2000-2018. Review was limited to HP of the upper gastrointestinal tract due to the frequency of presentation in this location. Symptomatic patients were identified from review of the medical records and clinical symptoms were tabulated. These were compared to a systematic review of the literature utilizing PubMed and Embase searches for papers pertaining to heterotopic pancreas. Publications describing symptomatic presentation of HP were selected for review. Information including demographics, symptoms, presentation and treatment were compiled and analyzed.

Twenty-nine patient were identified with HP at a single center, with six of these identified has having clinical symptoms. Clinical manifestations included, gastrointestinal bleeding, gastric ulceration with/without perforation, pancreatitis, and gastric outlet obstruction. Systemic review of the literature yielded 232 publications detailing symptomatic cases with only 20 studies describing ten or more patients. Single and multi-patient studies were combined to form a cohort of 934 symptomatic patients. The majority of patients presented with abdominal pain (67%) combined with one of the following clinical categories: (1) Dyspepsia, (n = 445, 48%); (2) Pancreatitis (n = 260, 28%); (3) Gastrointestinal bleeding (n = 80, 9%); and (4) Gastric outlet obstruction (n = 80, 9%). The majority of cases (n = 832, 90%) underwent surgical or endoscopic resection with 85% reporting resolution or improvement in their symptoms.

Heterotopic pancreas can cause significant clinical symptoms in the upper gastrointestinal tract. Better understanding and classification of this disease may result in more accurate identification and treatment of this malformation.

• Heterotopic pancreas
• Ectopic pancreas
• Aberrant pancreas
• Pancreatic rest
• Groove pancreatitis

• Choristoma/pathology
• Duodenum/pathology
• Gastric Outlet Obstruction/etiology
• Gastrointestinal Hemorrhage/complications
• Humans
• Pancreas/pathology
• Pancreas/surgery
• Pancreatitis/complications
• Upper Gastrointestinal Tract/pathology

• Aberrant pancreas
• Esophageal atresia
• Pancreatic rest
• Trisomy 21

Cystic dystrophy of the duodenal wall is a rare form of the disease which was described in 1970 by French authors who reported the presence of focal pancreatic disease localized in an area comprising the C-loop of the duodenum and the head of the pancreas. German authors have defined this area as a “groove”. We report our recent experience on cystic dystrophy of the paraduodenal space and systematically review the data in the literature regarding the alterations of this space. A MEDLINE search of papers published between 1966 and 2010 was carried out and 59 papers were considered for the present study; there were 19 cohort studies and 40 case reports. The majority of patients having groove pancreatitis were middle aged. Mean age was significantly higher in patients having groove carcinoma. The diagnosis of cystic dystrophy of the duodenal wall can now be assessed by multidetector computer tomography, magnetic resonance imaging and endoscopic ultrasonography. These latter two techniques may also add more information on the involvement of the remaining pancreatic gland not involved by the duodenal malformation and they may help in differentiating “groove pancreatitis” from “groove adenocarcinoma”. In conclusion, chronic pancreatitis involving the entire pancreatic gland was present in half of the patients with cystic dystrophy of the duodenal wall and, in the majority of them, the pancreatitis had calcifications.

• Pancreatitis
• Cystic dystrophy of duodenal wall
• Therapy
• Outcome

Cystic dystrophy of the duodenal wall (CDDW) is a complication of heterotopic pancreatic tissue located in the wall of the gastrointestinal tract, characterized by the presence of multiple small cysts, usually found in the wall of the second part of the duodenum. Gastrointestinal hemorrhage due to CDDW is a rare complication. We report the case of a 50-year-old man who was admitted to our hospital for persistent vomiting. The imaging tests confirmed the diagnosis of CDDW. During his stay in hospital, the patient had a gastrointestinal hemorrhage secondary to this disorder, which made it necessary to perform a Roux-en-Y gastrojejunostomy (Billroth III).

• Cystic dystrophy
• Hemorrhage
• Heterotopic pancreas
• Paraduodenal pancreatitis