Successful laparoscopic resection of a rare focal form of congenital hyperinsulinism: Case report and literature review

Congenital hyperinsulinism is a severe disease that causes irreversible damage to the cerebral cortex. This article presents a clinical observation of successful surgical treatment for a child with an extremely rare form of hyperinsulinism accompanied by two foci in the opposite ends of the pancreas. The child was in extremely critical condition from birth due to recurrent hypoglycemia and was diagnosed with hyperinsulinism at the age of two weeks. Specific therapy with diazoxide and octreotide was initiated but proven to be ineffective (parenteral glucose administration was still needed, and octreotide only provided partial results). When the child was one month old, positron emission tomography-computed tomography with 18F-DOPA was performed and revealed the focal hyperfixation of the radiopharmaceutical in the tail and head of the pancreas. This finding indicated the presence of two pathological foci or a focus in the tail and the physiological accumulation of the radiopharmaceutical in the head. At the age of one month and 20 days, the child underwent laparoscopic revision of the pancreas to visualize a focus in the tail that was confirmed by express biopsy. The resection of the tail (15%) was performed with confirmation of clear resection margins. During further revision, a focus was detected in the head. Resection was again performed with histological confirmation of complete resection. On the 12^th day after the operation, the child was discharged without therapy with clinical recovery. The child is currently being monitored (for over six months), growing and developing according to age. A fasting test was performed, and the recurrence of hyperinsulinism was ruled out. This article presents a detailed description of the clinical and radiological examination results, the patient’s history, the surgical techniques, and the intervention results. As part of the discussion, a literature review on the behavior of laparoscopic resections of the pancreas in children with congenital hyperinsulinism was carried out.