Ahmet ŞEN, Gülşah BALIK, Başar ERDİVANLI, Sertan KANTAROĞLU

Musküler distrofi miyotonik distrofi tanılı over kanseri vakasında kombine spinal epidural anestezi

Sıklıkla yaşamın ikinci dekadında gözlenen otozomal dominant geçişli miyotonik distrofi tip 2, kardiyomyopati, entelektüel ve emosyonel anormalliklerle seyreder, beşinci dekadda ölüm sıktır. Genel anestezide kardiyopulmoner mortalite yüksektir. Bu nedenle total abdominal histerektomi yapılacak miyotonik distrofili olguda rejyonal anestezinin perioperatif etkinliğinin tartışılması amaçlandı. Beş yıldır miyotonik distrofi tip II tanılı ve daha önceden ameliyat öyküsü olmayan 48 yaşında kadın olguda, over kanseri tanısıyla total abdominal histerektomi planlandı. Preoperatif değerlendirmede, ekstremitelerde motor güçsüzlük, derin tendon reflekslerinde azalma, ekokardiyografide hafif sol ventrikül hipertrofisi saptandı. Laboratuar değerleri ve vital bulguları normaldi. Kas güçsüzlüğü göz önüne alınarak kombine spinal epidural anesteziyle sürekli epidural analjezi uygulandı. Beş saat süren ameliyat süresince ve sonrasında epidural analjezi ile vital bulguları stabil seyreden hasta, postoperatif 10. günde taburcu edildi. Olgumuzda perioperatif kardiyopulmoner ve uzamış derlenme benzeri komplikasyonlar gelişebileceğinden, rejyonel anestezi ve analjezi tercih edildi. Rejyonel teknik ile miyotonik distrofili hastalarda mortalite ve morbiditenin azaltılabileceğini düşünüyoruz.

Combined spinal and epidural anesthesia for ovarian cancer in a patient with muscular dystrophy

Myotonic dystrophy type 2 is an otosomal dominant disease, observed during the second decade of life. It is characterized by cardiomyopathy, intellectual/emotional anomalies, death at the fifth decade, cardiopulmonary mortality due to general anesthesia. Therefore we discussed regional anesthetic management of a 48-years old female with a history of myotonic dystrophy, scheduled for total abdominal hysterectomy due to ovarian cancer. She had no previous surgery, presented with stable vital signs, generalized weakness of limbs, depressed deep tendon reflexes, mild left ventricular hypertrophy and normal laboratory values. We proceeded with combined spinal epidural anesthesia and epidural analgesia, due to concerns of postoperative respiratory muscle weakness. The patient was stable during and after the surgery lasting for 5 hours and was discharged at the tenth postoperative day. We preferred regional anesthetic technique to prevent perioperative cardiopulmonary complications and delayed neuromusclular recovery. We suggest that mortality and morbidity in myotonic dystrophy may be decreased by regional anesthesia.

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