Clinical features and outcomes of total pancreatic lipomatosis with chronic pancreatitis: a case series

Background: Fatty changes in the pancreas are common, whereas total pancreatic lipomatosis (PL) is rare. Commonly associated with various components of metabolic syndrome and metabolic-associated steatotic liver disease, total PL can have various etiologies and can manifest with severe pancreatic exocrine insufficiency.

Method: We retrospectively analysed the clinical profile and management outcomes of 8 patients (mean age: 37.1 years; 5 male) with total PL seen at a tertiary care center over the last 15 years.

Results: All patients presented with abdominal pain and had coexistent chronic pancreatitis, while 5/8 (62%) patients had metabolic syndrome. None of the patients had a history of acute pancreatitis or congenital syndromes, nor developed pancreatic carcinoma in the follow up. Seven (87%) patients had pancreatic ductal dilatation and calcification. All patients had pancreatic exocrine insufficiency, while 5/8 (52%) patients had endocrine insufficiency. Six (75%) patients were successfully managed with pancreatic endotherapy.

Conclusions: Pancreatic endotherapy is safe and effective in the treatment of abdominal pain in patients who have chronic pancreatitis with total PL. These patients have a high frequency of pancreatic exocrine as well as endocrine insufficiency.