Abstract
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.
References
- Russell SH, Hirsch NP. Anaesthesia and myotonia. Br J Anaesth 1994; 72(2): 210-6.
- Cruz Guzmán Odel R, Chávez García AL, Rodríguez-Cruz M. Muscular dystrophies at diffe- rent ages: metabolic and endocrine alterations. Int J Endocrinol
- doi:10.1155/2012/485376.
- Gurnaney H, Brown A, Litman RS. Malignant hy- perthermia and muscular dystrophies. Anesth Analg 2009; 109(4): 1043-8.
- Birnkrant DJ, Panitch HB, Benditt JO, Boitano LJ, Carter ER, Cwik VA, Finder JD, Iannaccone ST, Ja- cobson LE, Kohn GL, Motoyama EK, Moxley RT, Schroth MK,Sharma GD, Sussman MD. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest 2007; 132(6): 1977- 86.
- Muenster T, Mueller C, Forst J, Huber H, Schmitt HJ. Anaesthetic management in patients with Duc- henne muscular dystrophy undergoing orthopaedic surgery: a review of 232 cases. Eur J Anaesthesiol 2012; 29(10): 489-94.
- Sener EB. [The Use of Neuromuscular Agents in Patients with Myasthenia Gravis, Myotonia, Muscu- lar Dystrophy, First and Second Motor Neuron Dise- ases and Burns]. Turkiye Klinikleri J Anest Reanim- Special Topics 2011; 4(2): 36-46.
- Buyukkocak U. [The use of neuromuscular bloc- king agents in neuromuscular diseases]. Turkiye Klinikleri J Anest Reanim 2005; 3: 148-56.
- Mori K, Mizuna J, Naqaoka T, Harashima T, Mori- ta S. [Combined spinal-epidural anesthesia for cesa- rean section in a parturient with myotonic dyst- rophy]. Masui 2010; 59(8): 1000-3. ss 2012:485376.
Abstract
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.
References
- Russell SH, Hirsch NP. Anaesthesia and myotonia. Br J Anaesth 1994; 72(2): 210-6.
- Cruz Guzmán Odel R, Chávez García AL, Rodríguez-Cruz M. Muscular dystrophies at diffe- rent ages: metabolic and endocrine alterations. Int J Endocrinol
- doi:10.1155/2012/485376.
- Gurnaney H, Brown A, Litman RS. Malignant hy- perthermia and muscular dystrophies. Anesth Analg 2009; 109(4): 1043-8.
- Birnkrant DJ, Panitch HB, Benditt JO, Boitano LJ, Carter ER, Cwik VA, Finder JD, Iannaccone ST, Ja- cobson LE, Kohn GL, Motoyama EK, Moxley RT, Schroth MK,Sharma GD, Sussman MD. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest 2007; 132(6): 1977- 86.
- Muenster T, Mueller C, Forst J, Huber H, Schmitt HJ. Anaesthetic management in patients with Duc- henne muscular dystrophy undergoing orthopaedic surgery: a review of 232 cases. Eur J Anaesthesiol 2012; 29(10): 489-94.
- Sener EB. [The Use of Neuromuscular Agents in Patients with Myasthenia Gravis, Myotonia, Muscu- lar Dystrophy, First and Second Motor Neuron Dise- ases and Burns]. Turkiye Klinikleri J Anest Reanim- Special Topics 2011; 4(2): 36-46.
- Buyukkocak U. [The use of neuromuscular bloc- king agents in neuromuscular diseases]. Turkiye Klinikleri J Anest Reanim 2005; 3: 148-56.
- Mori K, Mizuna J, Naqaoka T, Harashima T, Mori- ta S. [Combined spinal-epidural anesthesia for cesa- rean section in a parturient with myotonic dyst- rophy]. Masui 2010; 59(8): 1000-3. ss 2012:485376.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Research Article |
| Authors | |
| Publication Date | May 1, 2014 |
| Published in Issue | Year 2014 Volume: 3 Issue: 2 |