Short-term Outcomes of Pancreatectomy in Congenital Hyperinsulinism: A Retrospective Multi-center Study

Background: Congenital hyperinsulinism (CH) is a well-known cause of the persistent neonatal hypoglycemic state that may lead to irreversible neurological damage. While medical therapy can improve the condition in some cases, refractory cases require further investigation to identify focal or diffuse pancreatic lesions. Surgery is the main treatment for refractory cases and can improve the neurological and glycemic status of patients. The study aimed to evaluate the short-term outcomes of surgical intervention in cases of CH who underwent surgical treatment. Methods: A multicenter cross-sectional survey was conducted to review cases of CH that underwent surgery between ۲۰۱۸ and ۲۰۲۰. Focal cases were treated with simple enucleation or distal pancreatectomy, while diffuse cases underwent near-total pancreatectomy. The glycemic and neurological states of the patients were evaluated and the results were analyzed. Results: Among the ۵۶ neonates who underwent pancreatic surgery, ۴۸ (۸۵%) had diffuse disease, and the remaining cases suffered from focal lesions. All focal cases achieved normoglycemia, while ۲۴ (۵۰%) of the diffuse cases achieved normal glycemic levels (P≤۰.۰۰۳). Additionally, the incidence of irreversible neurological deficits was higher in the diffuse group (P=۰.۰۲۹). Conclusion: The focal form of CH seems to be totally curative by surgical operations. However, the proper management of diffuse form is still demanding. Although in our study, we had an acceptable success rate in the short-term, lifelong euglycemia may not be obtainable in these patients.

Keywords:

Congenital Hyperinsulinism , Neonates , Neurologic Disorder , Pancreatectomy