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Congenital hyperinsulinism and the surgeon: lessons learned over 35 years
H N Lovvorn1, M L Nance, R J Ferry
1Department of Surgery, The Children's Hospital of Philadelphia, The University of Pennsylvania School of Medicine, 19104, USA.
Insights
Near-total pancreatectomy (95%) is recommended for congenital hyperinsulinism, offering better glycemic control with no increased risk of complications or diabetes mellitus. This approach improves outcomes for affected newborns and infants.
Area of Science:
- Pediatric Surgery
- Endocrinology
- Medical Genetics
Background:
- Congenital hyperinsulinism causes severe hypoglycemia in infants, potentially leading to brain damage.
- Pancreatectomy is considered for medically refractory cases, with near-total resection (95%) emerging as a preferred option.
- The impact of pancreatectomy extent on congenital hyperinsulinism management and outcomes requires further definition.
Purpose of the Study:
- To evaluate the effect of varying degrees of pancreatectomy on congenital hyperinsulinism management.
- To analyze outcomes including glycemic control, reoperation rates, morbidity, mortality, and diabetes development.
- To compare results between subtotal (<95%) and near-total (>=95%) pancreatectomy.
Main Methods:
- Retrospective review of 101 children with congenital hyperinsulinism requiring pancreatectomy (1963-1998).
- Outcome assessment included glycemic response, need for reoperation, surgical morbidity/mortality, and diabetes mellitus development.
- Histopathology categorized lesions as diffuse or focal; complete response defined by discharge without medications or continuous feeding.
Main Results:
- 53% of patients underwent pancreatectomy; 43% achieved a complete response.
- Complete response rates were higher with >=95% pancreatectomy (50%) versus <95% (19%).
- Focal lesions had an 82% complete response rate, compared to 33% for diffuse lesions; diabetes developed in 14% of diffuse cases.
Conclusions:
- Near-total pancreatectomy (>=95%) is associated with better euglycemia restoration in congenital hyperinsulinism.
- The risks of surgical complications and diabetes mellitus do not appear increased with 95% pancreatectomy.
- Recommend 95% pancreatectomy as the initial surgical choice for refractory congenital hyperinsulinism in infants.
Background/Purpose:
Congenital hyperinsulinism induces severe and unremitting hypoglycemia in newborns and infants. If poorly controlled, seizures and irreversible brain damage may result. Subtotal (<95%) or near-total (95% to 98%) pancreatectomy have been performed for glycemic control in babies who do not respond to aggressive medical therapy. Because hypoglycemia often persists after subtotal resection, 95% pancreatectomy has emerged as the procedure of choice. To define the effect of more or less extensive pancreatectomy on the management and outcome of refractory congenital hyperinsulinism, the authors examined our single institutional experience.
Methods:
The records of children treated between 1963 and 1998 for congenital hyperinsulinism, and who required pancreatectomy, were reviewed. Outcome parameters included glycemic response to surgery, need for reresection, surgical morbidity, surgical and long-term mortality, and development of diabetes mellitus (DM). A complete response was defined as discharge to home on no glycemic medications, no continuous feedings, and without DM. Histological reports were reviewed and categorized as either diffuse or focal disease.
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 > or = 95% pancreatectomy (n = 34), but in only 19% having less than 95% resection (n = 16). The remaining three babies had local excision of a solitary focal lesion, and each showed a complete response. Histopathology showed diffuse islet abnormalities in 42 specimens (79%) and solitary focal 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. Surgical morbidity occurred in 13 cases (26%), and the 30-day surgical mortality 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 risks for surgical complications and DM do not appear increased, the authors recommend 95% pancreatectomy as the initial procedure of choice for newborns and infants with congenital hyperinsulinism.