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Surgical management of islet cell dysmaturation syndrome in young children
H S Bjerke1, R E Kelly, M E Geffner
1Division of Pediatric Surgery, University of California, Los Angeles Center for Health Sciences.
Insights
Early surgical intervention for islet cell dysmaturation syndrome (ICDS) in infants with hyperinsulinemic hypoglycemia can prevent severe complications. Aggressive pancreatic resection leads to normoglycemia and reduces neurological deficits.
Area of Science:
- Pediatric Surgery
- Endocrinology
- Pathology
Background:
- Islet cell dysmaturation syndrome (ICDS) causes infantile hyperinsulinemic hypoglycemia.
- Histological findings include islet cell hyperplasia, pancreatic adenomatosis, and nesidioblastosis.
Purpose of the Study:
- To evaluate the efficacy of surgical resection for severe, medically unresponsive ICDS.
- To assess outcomes, including glycemic control and neurological development, after pancreatic resection.
Main Methods:
- Retrospective review of 11 infants with ICDS undergoing 14 pancreatic resections between 1965 and 1990.
- Surgical approaches included 80% and 90-95% pancreatic resections.
- Histological analysis confirmed nesidioblastosis, islet cell hyperplasia, or adenoma.
Main Results:
- Infants undergoing 90-95% resection required no reoperation or postoperative medication and achieved normoglycemia.
- 80% resection in neonates sometimes required reoperation; older infants required short-term diazoxide.
- Preoperative neurological impairment improved postoperatively, but only one infant normalized.
Conclusions:
- Early and aggressive surgical resection is crucial for managing ICDS.
- Surgical intervention can effectively resolve hypoglycemia and minimize neurological complications.
- Near-total pancreatectomy offers the best outcome for ICDS patients.
Abstract:
Islet cell dysmaturation syndrome (ICDS) encompasses the causes of infantile hyperinsulinemic hypoglycemia histologically described as islet cell hyperplasia, pancreatic adenomatosis and nesidioblastosis. Eleven infants underwent 14 pancreatic resections for ICDS from 1965 to 1990 at the University of California at Los Angeles Medical Center for severe hypoglycemia unresponsive to medical therapy. Seizures were the presenting symptoms of hypoglycemia in eight infants. Six patients had nesidioblastosis, four had islet cell hyperplasia and one patient had an adenoma with histologically normal pancreatic islet cells. Four neonates underwent 80 per cent pancreatic resection; three with nesidioblastosis required reoperation (90 to 95 per cent resection). Four older infants underwent 80 per cent pancreatic resection but required diazoxide for less than six months postoperatively. Three infants underwent 90 to 95 per cent pancreatic resection. None have required reoperation or postoperative medications. All infants are normoglycemic without pancreatic exocrine insufficiency and none had postoperative complications. Five infants had preoperative neurologic impairment, with three having severe retardation; all showed some improvement postoperatively, but only one infant now has normal findings on neurologic examination. Early diagnosis and aggressive surgical resection should minimize neurologic complications of the ICDS.