36th Annual Congress of Turkish Pediatric Surgical Association and 3rd Annual Congress of IPEG-MEC

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Video Presentation - 10

Laparoscopic Pancreatectomy of a Treatment-Resistant Rare Hyperinsulinism Case with Glucokinase Gene Mutation: Video Presentation

M Saraç*, T Tartar*, Ü Bakal*, K Karabulut**, A Kazez*
*Firat University, Faculty of Medicine, Department of Pediatric Surgery, Elazig, Turkey
**Firat University, Faculty of Medicine, Department of General Surgery, Elazig, Turkey

Aim: We aim to present a subtotal pancreatectomy with laparoscopic splenic preservation for a hyperinsulinism case with glucokinase gene mutation, which failed in medical treatment.

Case Report: Hyperinsulinism was detected in a 10-year-old male patient who has been followed up due to autism and epilepsy since the age of four. In genetic study, c.271 G> C (p.V91L) heterozygous mutation of his glucokinase gene was determined. Subtotal pancreatectomy was proposed by the pediatric endocrinology clinic to the patient whose hypoglycemia attacks did not improve with medical treatment. Subsequently, subtotal pancreatectomy with laparoscopic splenic preservation (85%) was performed. Insulin was applied to the patient who had hyperglycemia for his first two postoperative days. After the insulin was stopped for the patient whose hypoglycemia partially continued, diazoxide and sandostatin were started. Blood glucose levels were taken under control. The patient was discharged without any problems on the 7th postoperative day.

Conclusion: It may be sufficient to remove less pancreatic tissues for the hyperinsulinism cases with glucokinase gene mutation. Therefore subtotal pancreatectomy can be applied more advantageously with laparoscopy.

Glukokinaz Gen Mutasyonlu, Tedaviye Dirençli Nadir Bir Hiperinsülinizm Olgusunda Laparoskopik Pankreatektomi: Video Sunumu

M Saraç*, T Tartar*, Ü Bakal*, K Karabulut**, A Kazez*
*Fırat Üniversitesi Tıp Fakültesi Çocuk Cerrahisi AD, Elazığ
**Fırat Üniversitesi Tıp Fakültesi Genel Cerrahi AD, Elazığ

Amaç: Medikal tedavide başarısız olunan glukokinaz gen mutasyonlu hiperinsülinizm olgusunun laparoskopik dalak koruyucu subtotal pankreatektomi işlemini sunmak.

Olgu sunumu: Dört yaşından beri otizm ve epilepsi tanıları ile izlenen 10 yaşındaki erkek hastada hiperinsülinizm tespit edilmiş. Genetik çalışmasında glukokinaz geninde c.271G>C (p.V91L) heterozigot mutasyon tespit edildi. Medikal tedavi ile hipoglisemi atakları düzelmeyen hastaya pediatrik endokrinoloji kliniği tarafından subtotal pankreatektomi önerildi. Bunun üzerine laparoskopik dalak koruyucu subtotal pankreatektomi (%85) yapıldı. Postop ilk iki gün hiperglisemik seyreden hastaya insülin başlandı. Sonrasında hipoglisemileri kısmen devam eden hastada insülin kesilip diazoksit ve sandostatin başlandı. Kan şekeri düzeyleri kontrol altına alındı. Hasta postop 7. gün sorunsuz taburcu edildi.

Sonuç: Glukokinaz gen mutasyonlu hiperinsülinizm olgularında daha az pankreas dokusunun çıkarılması yeterli olabilir. Bu nedenle subtotal pankreatektomi laparoskopi ile daha avantajlı olarak yapılabilir.

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