Eralp ÇEVİKKALP, Gonca HAYRAN GÜL, GÖNÜL TEZCAN KELEŞ

RETT SENDROMU VE ANESTEZİ YÖNETİMİ

Rett sendromu gelişimsel ve nörolojik bozuklarla seyreden X kromozomundan kodlanan MECP2 gen mutasyonuyla ortayaçıkan bir hastalıktır. Erken yaşlarda başlayan gelişimsel gerileme, EEG anormallikleri, epileptik nöbet, mental retardasyonun eşlikettiği santral tutulumlu bir hastalıktır. Bunun yanı sıra ortopedik, solunumsal ve kardiyak sorunlarda görülebilmektedir. UzamışQT süresi, skolyoz, apne, kaşeksi bu sendromlu çocuklarda sık görülür ve bu nedenle anestezi uygulamasında özen ve dikkatgerektir. Bu olgu sunumunda; Rett sendromu olan çocuk hastada perioperatif anestezik yaklaşımı, riskleri ve sık karşılaşılabilecekdurumları paylaştık.

RETT SYNDROME AND ANESTHETIC MANAGEMENT

Rett syndrome is a developmental and neurological disease characterized by defective X and is caused by mutations in thegene MECP2 coded chromosomes. It is a central involvement diesase that starts in the early developmental years, together alongwith EEG abnormalities, seizures, mental retardation. Orthopedic, respiratory and cardiac problems can also be seen. ProlongedQT, scoliosis, apnea, cachexia is common in children with this syndrome and requires significant anesthesia care and attention. Inthis case, we emphasized the anesthetic management of a Rett Syndrome child, its possible risks and problems.

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