Pancreatoduodenectomy due to lipomatous pseudohypertrophy of the pancreas

BACKGROUND: Lipomatous pseudohypertrophy of the pancreas, pancreatic lipomatosis, pancreatic steatosis, non-alcoholic fatty pancreatic disease, or fatty pancreas is an extremely rare disease, characterized by the organ enlargement and a localized or diffuse replacement of pancreatic acinar cells by mature adipose tissue, preserving the pancreatic ductal system and islets of Langerhans. AIMS: To report a rare case of lipomatous pseudohypertrophy of the pancreas in a symptomatic patient and the surgical treatment employed. METHODS: A 24-year-old male patient with weight loss (10 kilograms in 8 months), hyperglycemia, severe and recurrent acute abdominal pain, epigastric discomfort associated with nausea, vomiting, and jaundice for 40 days. Magnetic resonance imaging was performed, revealing an irregular lipomatous pseudohypertrophy of the pancreas, measuring 6.0 x 5.6 cm in the head, uncinate process, and part of the body of the pancreas. The pancreatic duct dilation was diffuse and irregular, associated with atrophy of the remnant parenchyma, particularly in the tail of the pancreas. The patient underwent pancreatoduodenectomy without total mesopancreas excision followed by pancreatojejunostomy. RESULTS: The postoperative course was uneventful, the length of stay in the ICU was two days, and the patient was discharged on the seventh postoperative day. CONCLUSIONS: The disease treatment depends on the signs and symptoms at presentation and a pancreatoduodenectomy is indicated in patients with severe and recurrent abdominal pain.