Use of hyperglycemic clamp to assess pancreatectomy and islet cell autotransplant in patient with heterotaxy syndrome and dorsal pancreas agenesis leading to chronic pancreatitis

Patients with heterotaxy syndrome (HS) can present with an associated complete dorsal pancreas agenesis (DPA). They are considered to be at increased risk for developing diabetes due to a reduced functional beta cell mass (FBM) as well as for chronic pancreatitis leading to unmanageable pain. We report the case of a young female with chronic pancreatitis due to HS and associated DPA. She presented with a severe persisting upper abdominal pain refractory to non-surgical treatment. Unlike in previously reported cases, she had a high FBM (i.e. 150% of normoglycemic controls) as determined by hyperglycemic clamp. She underwent a total pancreatectomy followed within 24 hours by an intraportal autologous islet cell transplant containing 4 x 106 beta cells (4,700 IEQ)/kg bodyweight. After surgery, the pain resolved, eliminating the need for analgesics. The intraportal implant established an adequate FBM (72% of controls at posttransplant month 2) achieving glycemic control without need for insulin administration. A hyperglycemic clamp can assess utility and efficacy of an intraportal islet cell autotransplant following total pancreatectomy in patients with HS and complete DPA.