Tip 3 Spinal Muskuler Atrofili Olguya Anestezik Açıdan Yaklaşım

Yıl 2021, Cilt: 8 Sayı: 1, 191 - 193, 31.12.2020

Ecem Aydın Koçoğlu İsmet Topçu Gönül Tezcan Keleş Tülün Öztürk Emin Alp Yentur Hüseyin Serhat Yercan

https://doi.org/10.34087/cbusbed.804790

Öz

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.

Anahtar Kelimeler

spinal muskuler atrofi, rejyonel anestezi, kas hastalıkları

Anesthetic Approach to the Patient with Type 3 Spinal Muscular Atrophy

Öz

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.

Anahtar Kelimeler

spinal musculer atrophy, regional anesthesia, muscle diseases

Kaynakça

• 1. Lunn MR, Wang CH. Spinal muscular atrophy. Lancet 2008;371(9630):2120-33.
• 2. Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds A, Wong B, et al.; Participants of the International Conference on SMA Standard of Care. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol 2007;22(8):1027-49.
• 3. J B Strober GIT. Progressive Spinal Muscular Atrophies. J Child Neurol 1999 Nov;14(11):691-5.
• 4. Rudnik-Schöneborn S, Goebel HH, Schlote W et al. Classical infantile spinal muscular atrophy with SMN deficiency causes sensory neuronopathy. Neurology 2003; 60: 983–987.
• 5. Graham RJ AU, Laubach AE, Sethna NF. Anesthesia and perioperative medical management of children with spinal muscular atrophy. Paediatr Anaesth 2009;19:1054-1063.
• 6. Islander G. Anesthesia and spinal muscle atrophy. Paediatr Anaesth 2013;23:804-816.
• 7. Messina S PM, De Rose P, et al. Feeding, problems and malnutrition in spinal muscular atrophy type II. Neuromuscul Disord 2008;18:389-393. 8. Bach JR. The use of mechanical ventilation is appropriate in children with genetically proven spinal muscular atrophy type 1: the motion for. Paediatr Respir Rev 2008;9:45-50.
• 9. Maruotti GM AR, Scanni E, et al. Anesthetic management of a parturient with spinal muscular atrophy type II. J Clin Anesth 2012;24:573-577.
• 10. Talbot K. Progressive Spinal Muscular Atrophy. J Inher Metab Dis 1999;22: 545-554.
• 11. Neumann MM DM, Macknet MR, Applegate RL 2nd. Dexmedetomidine for awake fiberoptic intubation in a parturient with spinal muscular atrophy type III for cesarean delivery. Int J Obstet Anesth 2009;18(4):403-7.
• 12. Özhan MÖ SM, Eşkin MB, Çaparlar C, Atik B, Fener N. Tip III spinal musküler atrofili bir gebenin sezaryen operasyonunda kombine spinal epidural anestezi uygulaması. Anestezi Dergisi 2012;20: 119-122.
• 13. Beach TP SW, Hamelberg W. Circulatory collapse following succinylcholine: report of a patient with diffuse lower motor neuron disease. Anesth Analg 1971;50:431-437.
• 14. Habib AS HS, Millar S, Deballi P 3rd, Muir HA. Anesthesia for cesarean section in a patient with spinal muscular atrophy. J Clin Anesth 2004;16(3):217-9.