A case of persistent hyperinsulinemic hypoglycemia of infancy successfully treated with spleen preserving distal pancreatectomy allowing discontinuation of octreotide

Abstract

A 5-year-old girl was diagnosed as having persistent hyperinsulinemic hypoglycemia of infancy (PHHI) after birth. Medical treatment with octreotide prevented severe hypoglycemic attacks, but daily frequent blood sugar tests and supplementary snacks were needed to control the irregular onset of hypoglycemia. The patient was obese and had severe fatty liver due to the over-nutrition related to her hyperinsulinemia. The mapping of insulin levels by percutaneous transhepatic portal venous sampling revealed that the hyper-secretion of insulin occurred predominantly in the body to tail of the pancreas. She underwent a spleen-preserving distal pancreatectomy (SPDP). Diffuse type of nesidioblastosis was diagnosed. After surgery the octreotide could be discontinued, and her weight became nearly normal ; she did not have diabetes mellitus. In the treatment of PHHI, long-term medical control increases the risk of adverse drug effects ; on the other hand, a 95% subtotal pancreatectomy for PHHI refractory to medical control induces diabetes mellitus after a long period of time. Thus, a SPDP might have the potential to effectively deal with these issues.