Acil Laparotomi Gereken Xeroderma Pigmentosumlu Pediatrik HastadaZor Entübasyon ve Genel Anestezi Yönetimi

Xeroderma pigmentosum (XP) nadir görülen nörokutanöz sendromlardandır. Genellikle ultraviyolenin yol açtığı DNA hasarını onaran sistemdeki bozukluk sonucu oluşan lezyonlar nedeniyle cerrahi gereksinimleri olmaktadır. Bu hastalarda uygulanacak genel anestezi konusunda bir standardizasyon geliştirilememiştir. Sevofluranın nörolojik durumda kötüleşme yapıp yapmadığı konusunda netlik yoktur. Zor entübasyon ve ekstübasyonda yaşanabilecek sıkıntılar da mevcuttur. Genel anestezi altında acil laparotomi geçiren XP’li pediatrik hasta sevofluran, videolaringoskopi ve sugammadeksin başarılı kullanıldığını bildirilen ilk vaka raporu olduğunu düşünmekteyiz.

Difficult Intubation and General Anesthesia Management in a Pediatric Patient with Xeroderma Pigmentosum Requiring Emergency Laparotomy

Xeroderma pigmentosum (XP) is one of the rare neurocutaneous syndromes. The lesions occurring due to an impairment in the DNA repair mechanisms caused by ultraviolet light often require surgical interventions. There is no standardized general anesthesia procedure to be used in these patients. Besides, it is not clear whether sevoflurane leads to deterioration in the neurological condition. There are also other problems that may be encountered during difficult intubation and extubation. This may be the first reported pediatric patient with XP that underwent emergency laparotomy under general anesthesia with the successful use of sevoflurane, videolaryngoscopy, and sugammadex.

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