Introduction. Congenital hyperinsulinism (CHI) is an inherited disease characterized by severe persistent hypoglycemia in the neonatal period due to insulin hypersecretion and rapid development of neurologic and systemic disorders. There are two main CHI forms: a diffuse one when hyper functioning of beta-cells affect the whole pancreas, and a focal one, characterized by an area of intact parenchyma. Currently, surgery is an acknowledged approach in focal forms, while in diffuse CHI forms surgical treatment is prescribed only in severe pharmacoresistant cases.Material and methods. A total of 11 patents with CHI, aged 1.5 - 26 months, had laparoscopic pancreatic resections at the department of pediatric surgery of Russian Medical Academy of Continuous Professional Education. All patients were examined , treated and prepared for surgery at the Endocrinology Research Centre ( Moscow). The focal CHI form was diagnosed in 3 children; the diffuse one - in 8 patients. In the focal CHI form, a focal resection was done: laparoscopic corpocaudal resection of the pancreas – 2 patients; resection of the pancreatic head with Roux–en-Y pancreaticojejunostomosis - 1 child. All children with diffused disease had laparoscopic near-total (98%) pancreatectomy.Results. In all cases, there were no intraoperative complications. Stenosis of the distal common bile duct developed in one child after the resection of pancreatic head which required a laparoscopic cholecystoduodenoanastomosis. There were no other complications. Two children with focal CHI were reported to recover completely; one patient suffered of hypoglycemia attacks not related to the increased insulin production. In diffuse cases, a stable euglycemia was achieved in 2 patients; 3 children had recurrent hypoglycemia attacks which required Somatostatin analogue therapy. One patient developed diabetes mellitus. In this case, follow-up period lasted for 2 months which is too short to assess the endocrinological outcome.Conclusion. The article presents the first Russian experience of laparoscopic pancreas resection in children with CHI. The laparoscopic approachcan be successfully applied in the surgical treatment of children with CHI.
Представлено клиническое наблюдение инсулиномы у девочки 16 лет, у которой отмечены жалобы на приступы потери сознания с последующей ретроградной амнезией, сопровождавшиеся слабостью и головокружением. Больная был переведена в эндокринологическое отделение, где при регулярном контроле гликемии зафиксированы низкие значения (2,0-2,5 ммоль/л). Топический диагноз инсулиномы был установлен с помощью лучевых методов -мультиспиральной компьютерной томографии с контрастным усилением и магнитно-резонансной томографии, при которых в хвосте поджелудочной железы выявлена овальной формы опухоль размерами 12 × 10 мм. Девочке было проведено молекулярно-генетическое исследование гена MEN1 -мутаций в исследованном гене не выявлено. На период предоперационной подготовки с целью профилактики гипогликемического синдрома был назначен прогликем (диазоксид) в дозе 100 мг/сут, на фоне которого отмечалась стабилизация показателей гликемии. В дальнейшем была выполнена лапароскопическая дистальная резекция поджелудочной железы с опухолью. После операции достигнута стойкая эугликемия. При микроскопическом и иммуногистохимическом исследовании подтверждена нейроэндокринная опухоль поджелудочной железы G1. В обсуждении представлены данные об эпидемиологии, диагностике и хирургическом лечении инсулином у детей и взрослых. Ключевые слова: инсулинома, поджелудочная железа, лапароскопия, дистальная резекция поджелу-дочной железы с сохранением селезенки.We present a clinical case of insulinoma in 16 years-old girl. The patient complained about episodes of loss of consciousness, followed by retrograde amnesia and accompanied by weakness and dizziness. The patient was transferred to the Department of Endocrinology, where hypoglycemia was detected (2.0-2.5 mmol/l). Topical diagnosis of insulinoma was evidentiated with the help of radiological methods -multislice CT and contrast-enhanced MRI, which revealed an oval tumor 12 × 10 mm. The molecular genetic analysis of MEN1 gene was performed -no mutations were revealed. Prescribed Proglikem (diazoxide) therapy (100 mg/day) on pre-operative period was effective, patient's glycemia values stabilized. Further the laparoscopic distal pancreatectomy with tumor removal was performed. After the surgery we achieved stable euglycemia. On microscopic and immunohistochemical studies pancreatic neuroendocrine tumor G1 was confirmed. The discussion presents data on the epidemiology, diagnosis and surgical treatment of insulinomas in children and adults.
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