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Insulinomas first presenting as refractory seizure disorders are well documented in adulthood but rarely found in children. Only a few cases of childhood insulinoma have been reported so far. We report on two adolescents with hyperinsulinaemic hypoglycaemia, initially misdiagnosed as epilepsy and migraine accompagnée, and compare those to other cases published. Localization of insulinoma was challenging and, in one patient, angiography with selective arterial calcium stimulation and hepatic venous sampling in addition to CT and MRI was necessary. In these patients, long-term recovery was achieved by laparoscopic distal pancreatic resection in one and by conventional enucleation in the pancreatic head in the second patient. In contrast to adults, macrosomy and a decrease in school performance were the main symptoms and, during fasting, impaired cognitive function occurred after a relatively short period and at a higher glucose threshold or lower insulin/glucose ratio, respectively. Neuroglycopenic signs may be attributed to behaviour abnormalities or seizure disorders but in children and adolescents may already be caused by insulinoma. In these cases, timely diagnosis as well as tumour resection ensure long-term cure.