ESPE Abstracts (2015) 84 P-3-1074

Congenital Hyperinsulinism in Siblings: Case Report

Agnieszka Brandt, Marta Buraczewska & Małgorzata Myśliwiec

Clinic of Pediatrics, Diabetology and Endocrinology, Medical University of Gdansk, Gdansk, Poland

Background: Congenital hyperinsulinism (CHI) is the most common cause of hypoglycemia in childhood, and diagnosis and treatment of CHI is one of the most difficult aspects of modern endocrinology and diabetology. In half of infants suffering from congenital hyperinsulinism, which may require resection of pancreatic, potentially curable focal form can be found. Recently introduced diagnostic imaging using 18F-DOPA-PET allows for differentiation as a diffuse and focal form and determines further treatment. Identification of the exact location of lesions is used in preoperative planning, which involves resection limited to the focus and as a result it leads to a reduction in postoperative complications.

Case report: Case report of the two sisters, who were diagnosed with congenital focal form of hyperinsulinism and the impact of performed diagnostic test for the treatment and the occurrence of postoperative complications. Congenital hyperinsulinism in older sister was diagnosed when diagnostic imaging with 18F-DOPA-PET was not yet available, which led to subtotal pancreatectomy and ultimately resulted in diabetes and pancreatic enzyme deficiency symptoms after operation. In the postoperative examination of the removed tissue a single focus of hyperinsulinism was found. In the second, younger sister during diagnosis it was possible to perform the study with 18F-DOPA-PET, and there was identified an isolated focus of hyperinsulinism, which was resected during operation. In this patient none postoperative complications were observed and complete resolution of the symptoms of hypoglycemia. In both sisters genetic tests were performed for searching the reason of familial from of CHI.

Conclusions: It is important to perform diagnostic imaging with 18F-DOPA-PET in children with CHI before planning pancreatectomy in CHI.

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