HETEROTOPIC PANCREAS IN THE DUODENAL WALL – A RARE CAUSE OF RECURRENT ACUTE OR CHRONIC PANCREATITIS
Male aged 49, habitual alcoholic and smoker, had recurrent attacks of mild acute pancreatitis (AP) and was hospitalized on4 occasions over 3 years since August 2007. Each time he was treated conservatively and advised abstinence from alcohol and smoking. However he had failed attempts at alcohol abstinence. During the course of the disease over 3 years he had occasional mild abdominal pain in between attacks and was managed on outpatient basis. He had gradual weight loss (15-20Kg) over 2 years and developed diabetes since 1 year. He had a moderate appetite but early satiety and post-prandial fullness. Since last 2 months he had continuous dull aching pain, requiring increasing dosage of analgesics. There was no jaundice, vomiting or GI bleed and physical examination was unremarkable.
Serial investigations from August 2009 to March 2015 were essentially normal. WBC count showed mild leukocytosis during attacks, serum amylase and lipase were elevated during attacks and near normal in intervening period. Liver biochemistry was normal, USG abdomen and CT scan revealed no abnormality. During this period he was under treatment of medical gastroenterologist and the treatment included proton pump inhibitors, pancreaticenzyme supplements, analgesics (NSAIDs, opioids), glycemic control (OHAs) and advice for alcohol abstinence.
Upper GI Endoscopy showed edematous duodenal mucosa but no obvious obstruction. Duodenal biopsy showed Brunner’s gland hyperplasia. MRCP added no further information but confirmed absence of PD or CBD stricture.
The presumptive diagnosis at this stage was chronic pancreatitis with inflammatory head mass or a possibility of groove pancreatitis (uncommon). However an underlying malignancy could not be ruled out.
The dilemmas faced were whether to continue conservative therapy (already on, but no relief), pancreatobiliary endotherapy (not indicated based on MRCP findings), pancreas preserving surgery for inflammatory head mass (most likely choice under the circumstances) or pancreatodudenectomy for a possible underlying malignancy(always a difficult proposition till the final HP & a matter of debate).
After discussing all options with the patient and family, he was scheduled for surgery. In view of inability to excludea neoplasm with certainty, the patient was planned for a Pylorus Preserving Pancreatoduodenectomy (PPPD). At operation a hard mass was felt in the head and uncinate process of pancreas while the rest of the pancreas was firm in consistency indicative of a focal disease. The GB was hugely distended and CBD was dilated. A fibrotic partially constricting ring was felt in mid portion of D2. FNAC showed only inflammatory cells. A PPPD was done.
Postoperative course was uneventful and patient was discharged on 7th postoperative day. Histopathological examination revealed several islands of heterotopic pancreatic tissue in the muscularis propria of the duodenum. The acini were atrophic in places and only 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. The duodenal muscle was markedly hypertrophied, especially in its medial wall, which contained the heterotopic pancreatic tissue. The submucosa was widened due to the Brunner gland hyperplasia. Both these features probably led to narrowing of the duodenallumen. The head of pancreas showed focal evidence of chronic pancreatitis. Fibrosis was seen surrounding dilated intrapancreatic CBD and extending between pancreatic lobules along with focal atrophy, fibrosis and occasional dilated ducts with inspissated secretions in pancreatic head. There was no evidence of malignancy. Lymph nodes were unremarkable.
This patient was transported from India to Singapore on an Air Ambulance flight for higher treatment.
Air Ambulance Singapore