Congenital hyperinsulinism and the surgeon: Lessons learned over 35 years

Background/Purpose: Congenital hyperinsulinism induces severe and unremitti ng hypoglycemia in newborns and infants. If poorly controlled, seizures and irreversible brain damage may result. Subtotal (<95%) or near-total (95% t o 98%) pancreatectomy have been performed for glycemic control in babies wh o do not respond to aggressive medical therapy. Because hypoglycemia often persists after subtotal resection, 95% pancreatectomy has emerged as the pr ocedure of choice. To define the effect of more or less extensive pancreate ctomy on the management and outcome of refractory congenital hyperinsulinis m, the authors examined our single institutional experience. Methods: The records of children treated between 1963 and 1998 for congenit al hyperinsulinism, and who required pancreatectomy, were reviewed. Outcome parameters included glycemic response to surgery, need for reresection, su rgical morbidity, surgical and long-term mortality, and development of diab etes mellitus (DM). A complete response was defined as discharge to home on no glycemic medications, no continuous feedings, and without DM. Histologi cal reports were reviewed and categorized as either diffuse or focal diseas e. Results: Of 101 children treated for congenital hyperinsulinism during this period, 53 (50%) required pancreatectomy for glucose control. Mean follow- up for the study population was 9.8 +/- 1.1 years. Overall, 23 children (43 %) showed a complete response, occurring in 50% of patients having greater than or equal to 95% pancreatectomy (n = 34), but in only 19% having less t han 95% resection (n = 16). The remaining three babies had local excision o f a solitary focal lesion, and each showed a complete response. Histopathol ogy showed diffuse islet abnormalities in 42 specimens (79%) and solitary f ocal lesions in 11 (21%). A complete response was observed for 82% of focal but only 33% of diffuse lesions. Eight patients (15%) required reresection for persistent hypoglycemia, seven having diffuse lesions and one focal. S urgical morbidity occurred in 13 cases (26%), and the 30-day surgical morta lity rate was 6%, each death (n = 3) occurring before 1975. DM developed in seven children (14%), each having diffuse lesions, and was independent of resection type. Conclusion: Because euglycemia is more readily restored, and because the ri sks for surgical complications and DM do not appear increased, the authors recommend 95% pancreatectomy as the initial procedure of choice for newborn s and infants with congenital hyperinsulinism. Copyright (C) 1999 by W.B. S aunders Company.