Serhat ÇETİN, Ender Cem BULUT, Ahmet OLGUN, Bora KÜPELİ

Osteogenezis imperfekta tanılı pediatrik hastada ultra-mini perkütan nefrolitotomi

Osteogenezis imperfekta (Oİ), genetik ve klinik çeşitlilik gösteren nadir, kalıtsal bir hastalık grubudur. Tip 1 kollajende defekt vardır, kemik frajilitesinde artış, osteopeni ve iskelet deformiteleri görülebilmektedir. Oİ hastalarında cerrahi uygulamalar, kemik yapılarda yaralanma ve kırık gibi komplikasyonlara neden olabilir. Bu nedenle pediatrik ürolitiyazis yönetiminde girişim kararı dikkatle alınmalı, güvenli ve işlem süresini kısaltacak teknikler tercih edilmelidir.Perkütan nefrolitotomi (PCNL) operasyonu erişkin hastalarda büyük böbrek taşlarında ilk tercih olarak kullanılmaktadır. Teknolojik gelişmelerle birlikte mini-PCNL, ultra mini-PCNL ve mikro-PCNL gibi daha minimal invaziv yaklaşımlar ortaya çıkmıştır ve bu yöntemler düşük morbiditeleri sayesinde çocuk hastalarda başarıyla kullanılmaktadır.Olası riskleri nedeniyle literatürde Oİ hastalarına yönelik üriner taş cerrahisi nadir olarak bildirilmiştir. Bu raporda böbrek taşına ultra mini-PCNL uyguladığımız Oİ tanılı 4 yaş 4 aylık çocuk hasta sunulmuştur.

Anahtar Kelimeler:

endoüroloji, ürolitiyazis, pediatrik üroloji, ultra mini pcnl

Ultra-mini percutaneous nephrolithotomy surgery in a pediatric patient with osteogenesis imperfecta

Osteogenesis imperfecta (OI) is a rare, inherited disease group with genetic and clinical diversity. There is a defect in the type 1 collagen structure. Increasing bone fragility, osteopenia, and skeletal deformities can be seen. Surgical applications in patients with OI may cause complications such as spinous process injury in vertebrates or bone fracture. Therefore, in the management of pediatric urolithiasis, the decision of surgery should be carefully considered, and safe techniques that shorten the procedure time should be preferred.Percutaneous nephrolithotomy (PCNL) operation is the first choice for large kidney stones in adult patients. Along with the technological advances, more minimally invasive approaches such as mini-PCNL, ultra-mini-PCNL, and micro-PCNL have been developed, and these methods are safely used in pediatric patients due to their low morbidity.Because of its potential risks, urinary stone surgery in OI patients has rarely been reported in the literature. This study presents a 4-year-4-month-old girl with OI and kidney stone, whom we performed ultra-mini-PCNL.

Keywords:

endourology, urolithiasis, pediatric urology, ultra mini pcnl,

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