Use of hyperglycemic clamp to assess a pancreatectomy and islet autotransplantation in a patient with dorsal pancreas agenesis and chronic pancreatitis

BACKGROUND Patients with heterotaxy syndrome (HS) can present with an associated complete dorsal pancreas agenesis (DPA). They are considered to be at increased risk for the development of diabetes due to a reduced functional beta cell mass (FBM) as well as for chronic pancreatitis leading to untreatable pain. CASE DESCRIPTION We report the case of a patient that developed chronic pancreatitis due to HS and associated DPA. She presented to our center with severe abdominal pain refractory to non-surgical treatment. Unlike in previous reported cases, this patient had a high FBM (i.e. 150% of normoglycemic controls) determined by a hyperglycemic clamp. She was treated with total pancreatectomy followed by islet auto transplantation, leading to an adequate postoperative FBM (72% of normoglycemic controls) with preserved glycaemic control without need for exogenous insulin. After surgery, the pain disappeared, eliminating the need for opioid and other analgetics. CONCLUSIONS This case report shows that selected patients with HS and complete DPA can be considered for total pancreatectomy followed by islet autotransplantation in the presence of a sufficient FBM before surgery.