GKD Anest Yoð Bak Dern Derg. 2015; 21(2): 121-124 | DOI: 10.5222/GKDAD.2015.121

Miyotonik Distrofi Tip 1 ve Tip 2’li iki hastada spinal anestezi deneyimlerimiz: olgu sunumu

Meltem Kipri, Mesut Þener, Esra Çalýþkan, Anýþ Arýboðan
Baþkent Üniversitesi Týp Fakültesi, Anesteziyoloji Ve Reanimasyon Ana Bilim Dalý, Ankara

Giriþ: Miyotonik Distrofiler (MD), konjenital baþlangýçlý Tip I ve eriþkin baþlangýçlý Tip II olarak 2 grupta incelenmektedir. MD’li hastalarda miyotoninin þiddetinden baðýmsýz olarak tüm organ sistemleri etkilenebilmektedir. Bu nedenle perioperatif dönemde artmýþ anestezi riski mevcuttur. Olgu 1: Tip I MD’li 26 yaþýnda erkek hastaya spinal anestezi altýnda pilonidal sinüs cerrahisi yapýlmasý planlandý. Operasyon odasýnda spinal anestezi uygulandýktan sonra baþarýlý bir cerrahi iþlem uygulandý. Olgu 2: Tip II MD’li 31 yaþýnda kadýn hastaya spinal anestezi altýnda açýk miyomektomi yapýlmasý planlandý. Operasyon odasýnda spinal anestezi uygulandýktan sonra baþarýlý bir cerrahi iþlem uygulandý. Her iki hastada da perioperatif dönemde herhangi bir komplikasyonla karþýlaþýlmadý. Tartýþma: MD’li hastalarda yaygýn kullanýlan anestezik ilaçlara karþý yanýt normal populasyonda beklenenden farklýdýr. Ýntravenöz (ÝV) anesteziklere karþý artmýþ hassasiyet söz konusudur. Perioperatif opioid uygulamalarý sýrasýnda solunum depresyonu riski yüksektir. Hastalarda kas gevþeticilere duyarlýlýk da artmýþtýr, süksinilkolin uygulamasý miyotoniyi tetikleyebilir. Bununla birlikte propofol enjeksiyon aðrýsý, operasyon odasýnda aðrý, stres ve hipoterminin indüklediði miyotoni görülebildiðinden bu durumlara karþý önlem alýnmalýdýr. Uygun olgularda rejyonel anestezi yöntemlerinin, gerektiðinde düþük dozda ÝV sedatif ilaçlarla kombine edilerek uygulanmasý önerilmektedir. Nörostimulasyon sýrasýnda miyotoni gözlenebileceði ve kas hipertrofisi sonucunda ultrason ile siniri ayýrt etmek zorlaþacýðýndan, santral nöroaksiyel bloklar periferik sinir boklarýna göre þimdilik daha iyi bir tercih olarak görülmektedir.

Anahtar Kelimeler: Miyotonik Distrofi, Spinal Anestezi, Preoperatif Deðerlendirme, Anestezi Yönetimi

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Spinal Anesthesia in two cases with Myotonic Dystrophy type 1 and type 2: Case Reports

Meltem Kipri, Mesut Þener, Esra Çalýþkan, Anýþ Arýboðan
Department of Anesthesiology and Reanimation, Baskent University, Ankara, Turkey

Introduction: Myotonic dystrophy (MD) is classified into two groups: TypeI with congenital onset, typeII with adult onset. All organ systems can be affected regardless severity of myotonia among patients with MD. Frequency of anesthesia-related complications are increased during perioperative period. Case1: 26-year-old man with MD typeI had pilonidal sinus to be operated under spinal anesthesia. Spinal anesthesia was performed in the operating room and successful surgical procedure was performed. Case2: 31-year-old woman with MD typeII was planned to have open myomectomy under spinal anesthesia. Spinal anesthesia was performed in the operating room and successful surgical procedure was performed. Neither of two cases had any complications in perioperative period. Discussion: Response to commonly used anesthetic drugs is different than expected in MD patients when compared to normal population. There is an increased sensitivity to intravenous (IV) anesthetic agents. Risk of respiratory depression during perioperative period is also high with opioid injections. Sensitivity to muscle relaxants is also higher and succinylcholine can trigger myotonia. Propofol injection pain, stress, hypothermia and pain in the operating room may also induce myotonia, necessary precautions must be taken. Since malignant hyperthermia risk is not high in patients with MD compared to normal population, volatile anesthetics should be preferred. In suitable cases, regional anesthesia combined with low dose IV sedative medications are recommended. Since myotonia may be observed during neuro-stimulation, and may be difficult to distinguish the nerve with ultrasonography after muscle hypertrophy, central neuraxial blocks might be preferred over peripheral nerve blocks in current practice.

Keywords: Distrophia Myotonica, Spinal Anesthesia, Preoperative Assessment, Anesthetic Management

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