Childhood chronic pancreatitis is a rare disorder, which can lead to a chronically debilitating condition. The etiology of recurrent hereditary pancreatitis, which previously was classified as idiopathic, has now been attributed in certain cases to specific genetic mutations including abnormalities in the PRSS gene. We describe here the use of total pancreatectomy and autoislet transplant in a 5-year-old with chronic pancreatitis. This represents the youngest patient undergoing the procedure at our institution. The early successful outcomes for this patient, both of symptom relief and glycemic control are detailed.
