Cystic Dystrophy of the Duodenum Due to Heterotopic Pancreas – A Case Report and Review of Literature

Abstract

A 47-year-old male with history of alcohol abuse for 8 to 10 years visited the Department of Gastrointestinal Surgery for recurrent attacks of mild acute pancreatitis since 3 years. He also had recurrent epigastric pain, significant weight loss, early satiety and postprandial fullness. Multiple ultrasonography examinations and Computed Tomography (CT) scans of abdomen done in the past suggested chronic pancreatitis for which patient was advised pancreatic enzyme supplementation, alcohol abstinence, analgesics and dietary changes by different physicians, only to recur again.CT scan revealed bulky head and uncinate process of pancreas with a hypodense area between pancreatic head and second part of duodenum [Table/ Fig-1], prominent pancreatic duct, dilated Common Bile Duct (CBD) (15mm), hugely distended gall bladder, irregular wall thickening of first and second part of duodenum with stenosis of second part of duodenum [Table/ Fig-2]. Upper gastrointestinal endoscopy did not reveal significant duodenal obstruction. Duodenal biopsy showed Brunner's gland hyperplasia. Endoscopic Ultrasound (EUS) confirmed the CT findings. An Fine Needle Aspiration Cytology (FNAC) showed only inflammatory infiltrate and no malignancy. Blood level of CA19-9 was normal. Liver function tests were normal.A diagnosis of groove pancreatitis was made. In view of inability to exclude a neoplasm with certainty, the patient was subjected to a pylorus preserving Pancreatoduodenectomy (PD). During surgery a hard mass in the head and the uncinate process of pancreas was felt. There was no pancreatic calcification. A dilated CBD and a fibrotic partially constricting ring in the midportion of second part of the duodenum were noted.Histopathological examination [Table/ Fig-3] revealed islands of heterotopic pancreatic tissue in the muscularis propria of the duodenum. The lobules comprised of unremarkable pancreatic acini, ducts and few clusters of islet cells. The acini were atrophic in places and few aggregates of ductules were seen. Cystic dilatation of ductules was seen in the duodenal wall with mild to moderate inflammatory infiltrate. Extensive brunner gland hyperplasia was seen in mucosa and submucosa of the duodenum leading to widening of submucosa. The duodenal muscle was markedly hypertrophied, especially in its medial wall, which contained the heterotopic pancreatic tissue. Both these features probably led to narrowing of the duodenal lumen. The head of pancreas showed focal evidence of chronic pancreatitis. The postoperative course was uneventful. The patient is asymptomatic five years after the surgery. A diagnosis of groove pancreatitis was made. The patient was subjected to a pylorus preserving PD. Histopathological examination showed cystic dystrophy of duodenum due to heterotopic pancreas. The patient is doing well at 5 years of follow-up.[

Groove pancreatitis: a challenging imaging diagnosis

“…These CT signs and a medical history of alcohol abuse are highly suggestive of GP. In a case report (46), the patient, having a 10-year history of alcohol abuse, and history of recurrent acute pancreatitis was examined by CT.…”

Could it be groove pancreatitis? A frequently misdiagnosed condition with a surgical solution

“…In our systematic review of 17 studies (Table 3) with 146 patients of which 143 (98%) underwent PD, complete resolution of symptoms was achieved in more than 80% of patients whilst the remaining 20% achieved some improvement of their preintervention symptoms over a 31 month follow up. 7,9,11,[14][15][16][17][18][19][20][21][22][23][24][25][26][27] The major morbidity and mortality rates were 5.5% and 2% respectively, with a clinically significant pancreatic fistula rate of 3.5%. This demonstrates that PD can be performed safely, with acceptable outcomes despite a higher technical difficulty.…”

“…Seventeen studies were included for final review, comprising 146 patients with histopathologically confirmed GP. 7,9,11,[14][15][16][17][18][19][20][21][22][23][24][25][26][27] Mean age was 42.1 years; 79% of patients were male. 9,11,[15][16][17][18][19][20][22][23][24][25][26][27] A smoking history was significant in 71% of patients (95% CI: 46%-88%).…”

“…7,9,11,[14][15][16][17][18][19][20][21][22][23][24][25][26][27] Mean age was 42.1 years; 79% of patients were male. 9,11,[15][16][17][18][19][20][22][23][24][25][26][27] A smoking history was significant in 71% of patients (95% CI: 46%-88%). 9,15,16,19,[24][25][26] Alcohol misuse was present in 75% of patients (95% CI: 60%-86%).…”

Paraduodenal Pancreatitis: Many faces of the Same Diagnostic Challenge.

“…It is still unclear and contentious whether Groove pancreatitis and cystic duodenal dystrophy are distinct entities or part of the same spectrum. Hence, the term "Paraduodenal Pancreatitis (PDP)" has been proposed to include Groove pancreatitis (solid subtype), cystic dystrophy of the duodenum and paraduodenal cysts (cystic subtype, [15,16]).…”