Tip 3 Spinal Muskuler Atrofili Olguya Anestezik Açıdan Yaklaşım

Abstract

Özet Spinal musküler atrofi (SMA); çoğunlukla otozomal resesif geçişli, 1/10.000 sıklıkta görülen, spinal kord ön boynuz hücreleri ve beyin sapı motor nukleuslarının dejenerasyonu sonucu oluşan kalıtsal bir hastalıktır. Hastalık klinik olarak alt ekstremitelerin proksimal kas güçsüzlüğünden, disfaji ve solunum yetmezliğine kadar ilerleyebilen bir seyir gösterebilmektedir. Bu hastaların anestezi uygulamaları; zor havayolu yönetimi, nöromüsküler bloke edici ajanlara karşı aşırı duyarlılık, kas güçsüzlüğü ve respiratuar kapasitedeki bozukluk nedeniyle genellikle zordur. Bu olgu sunumunda; femur fraktürü nedeniyle cerrahi geçirecek Tip 3 SMA’lı bir hastaya anestezi ve postoperatif analjezi amacıyla başarılı bir kombine spinal-epidural anestezi uygulamasının sunulması amaçlandı.

Keywords

• spinal muskuler atrofi
• rejyonel anestezi
• kas hastalıkları

Anesthetic Approach to the Patient with Type 3 Spinal Muscular Atrophy

Abstract

Abstract Spinal muscular atrophy (SMA); It is an inherited disease with mostly autosomal recessive inheritance, seen with a frequency of 1/10000, resulting from degeneration of spinal cord anterior horn cells and brainstem motor nuclei. Clinically, the disease can progress from proximal muscle weakness of the lower extremities to dysphagia and respiratory failure. Anesthesia applications of these patients; difficult airway management is often difficult due to hypersensitivity to neuromuscular blocking agents, muscle weakness and impaired respiratory capacity. In this case report; It was aimed to present a successful combined spinal-epidural anesthesia for anesthesia and postoperative analgesia to a patient with Type 3 SMA who will undergo surgery for a femur fracture.

Keywords

• spinal musculer atrophy
• regional anesthesia
• muscle diseases

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