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Pancreatic adenomas in infants and children: current surgical management
Insights
Pediatric islet cell adenomas cause hyperinsulinism-induced hypoglycemia. Surgical intervention, particularly in infants, can be curative, but prompt diagnosis and treatment are crucial to prevent complications like brain damage.
Area of Science:
- Pediatric endocrinology
- Surgical oncology
- Metabolic disorders
Background:
- Islet cell adenomas are a significant cause of hyperinsulinism in infants and children.
- Hyperinsulinism can lead to severe hypoglycemia, posing risks for neurological complications.
Purpose of the Study:
- To evaluate the surgical management of islet cell adenomas in pediatric patients with hyperinsulinism.
- To assess the outcomes of surgical intervention and develop a diagnostic and treatment protocol.
Main Methods:
- Retrospective review of 32 pediatric patients with hyperinsulinism treated between 1965 and 1977.
- Surgical exploration and pancreatectomy for diagnosed islet cell adenomas.
- Development of a combined medical and surgical protocol.
Main Results:
- Sixteen patients underwent surgery, with eight diagnosed with single pancreatic adenomas.
- Subtotal pancreatectomy was successful in removing adenomas in 4 patients.
- Surgical cure was achieved in 7 patients; 6 had normal outcomes, 1 experienced brain damage, and 1 developed diabetes.
Conclusions:
- Early surgical intervention for islet cell adenomas in children with hyperinsulinism is critical for successful treatment.
- A combined medical-surgical protocol aids in rapid diagnosis and patient selection for surgery.
- Infants unresponsive to diazoxide and older children with hyperinsulinism should be considered for surgical exploration.
Abstract:
Islet cell adenomas are an important consideration in infants and children with hypoglycemia due to hyperinsulinism. Between 1965 and 1977, 32 patients with hyperinsulinism were seen at the Children's Hospital of Philadelphia. Sixteen of these patients underwent surgery. Eight patients had single pancreatic adenomas, 5 of whom were infants under 1 yr of age. In 3 infants an adenoma was recognized and removed at the time of surgery. In 4 patients a subtotal pancreatectomy was successful in removing the adenoma. One infant is whom the adenoma was not included in the subtotal pancreatectomy died postoperatively with intractable hypoglycemia. Of the 7 patients cured by surgery, 6 are normal and 1 has brain damage due to delay in treatment of hypoglycemia. One child has mild diabetes. A combined medical and surgical protocol has been developed that permits rapid diagnosis of hyperinsulinism and selection of candidates for surgery. Infants under 1 yr of age who fail to respond to diazoxide should be explored. In children whose hyperinsulinism appears beyond 1 yr of age, surgery is indicated, since an adenoma is likely.