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Surgical treatment of hyperinsulinaemic hypoglycaemia in infancy and childhood
L Spitz1, R K Bhargava, D B Grant
1Hospital for Sick Children, Department of Paediatric Surgery, London.
Insights
Hyperinsulinaemic hypoglycaemia can cause mental retardation. Early surgical intervention, typically a 95% pancreatectomy, is recommended for patients unresponsive to medical management to prevent neurological damage.
Area of Science:
- Pediatric Endocrinology
- Surgical Gastroenterology
- Metabolic Disorders
Background:
- Hyperinsulinaemic hypoglycaemia (HH) is a condition where the pancreas produces excess insulin, leading to dangerously low blood sugar levels.
- Despite increased awareness, a significant proportion of patients develop mental retardation by the time HH is diagnosed.
- Diagnosis relies on detecting elevated plasma insulin during hypoglycaemic episodes.
Purpose of the Study:
- To evaluate the efficacy of surgical intervention for hyperinsulinaemic hypoglycaemia in patients refractory to medical management.
- To emphasize the importance of early surgical referral to prevent long-term neurological sequelae.
Main Methods:
- Retrospective analysis of 21 infants and children with hyperinsulinaemic hypoglycaemia who underwent surgical treatment.
- Primary surgical approach involved a 95% pancreatectomy.
- Management of recurrent hypoglycaemia post-resection was also assessed.
Main Results:
- Surgical treatment was pursued in patients who failed to respond to optimal medical therapy.
- A 95% pancreatectomy is the standard surgical procedure.
- One patient experienced recurrent hypoglycaemia after a less extensive resection, necessitating a secondary extended resection.
Conclusions:
- Early surgical referral for hyperinsulinaemic hypoglycaemia unresponsive to medical treatment is crucial.
- Delaying surgery increases the risk of permanent neurological damage, including mental retardation.
- A 95% pancreatectomy is an effective surgical option for managing severe hyperinsulinaemic hypoglycaemia.
Abstract:
Despite a greater awareness of hyperinsulinaemic hypoglycaemia, one in three patients has some degree of mental retardation by the time the diagnosis is made. The diagnosis is established by demonstrating high plasma insulin concentrations during an episode of hypoglycaemia. Twenty one hyperinsulinaemic infants and children were referred for surgical treatment after failing to respond to medical management. The surgical procedure of choice is a 95% pancreatectomy. Recurrence of the hypoglycaemia may develop after less radical resections as occurred in one patient who then underwent an extended resection 72 hours postoperatively. Patients who fail to respond to optimal medical treatment should be referred for surgery early and not as a last resort if permanent neurological damage is to be avoided.