Marfan Sendromlu Gebede Anestezi Yönetimi

Year 2015, Volume: 17 Issue: 3, 39 - 45, 05.01.2016

Gülçin Aydın İşın Gençay Ferda Yaman Selim Çolak Cemile Sayan Gülcan Bakkal Ünase Büyükkoçak

https://doi.org/10.24938/kutfd.124929

Abstract

Marfan Sendromu (MS) 1/5000 oranında otozomal dominant geçiş gösteren bir bağ doku hastalığıdır. Fibrillin-1 (FBN1) geninin 15q21 kromozomunda mutasyon vardır. İskelet sistemi anormallikleri, lens dislokasyonu, dural ektazi ve aort dilatasyonu ile karakterizedir. Hastaların %80’ninde kardiyovasküler sistemde birtakım değişiklikler mevcut olup bunlar; aort dilatasyonu, aort yetmezliği, mitral-triküspit valv prolapsusu ve regürjitasyonudur. Majör ölüm nedenleri arasında aortik anevrizma rüptürü ve diseksiyonu yer alır. Gebelikteki fizyolojik değişiklikler aort diseksiyonu patogenezi ve progresyonuna katkıda bulunmaktadır.

Gebelikte artmış aort kompliyansı ve bunun sonucunda aort dilatasyonu sözkonusudur. Gebelik ve doğum MS’lu hastalarda yaşamı tehdit eden diseksiyonlara yol açar; özellikle de aort çapı 40 mm’ den geniş ise. MS’lu gebelerde lumbal spinal deformiteler ve yaygın artrodezisler, epidural ve spinal anestezi başarısızlığını artırır. Benzer şekilde dural ektaziler dural delinme riskinin artmasına neden olur. Bu olgu sunumuyla MS’lu gebe kadında elektif sezaryen operasyonunda genel anestezi uygulamamızı sunmayı amaçladık.

Keywords

Marfan Sendromu, diseksiyon, genel anestezi

References

• Keane MG, Pyeritz RE. Medical management of Marfan syndrome. Circulation. 2008; 117(21): 2802-13.
• Cox DA, Ginde S, Kuhlmann RS, Earing MG. Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation. Archives of Gynecology and Obstetrics 2014; 290(4): 797-802.
• Loeys BL, Dietz HC, Braverman AC, Callewaert BL, De Backer J, Devereux RB, Hilhorst-Hofstee Y, Jondeau G, Faivre L, Milewicz DM. The revised Ghent nosology for the Marfan syndrome. Journal ofMedical Genetics. 2010; 47(7): 476-85.
• Dietz HC, Cutting CR, Pyeritz RE, Maslen CL, Sakai LY, Corson GM, Puffenberger EG, Hamosh A, Nanthakumar EJ, Curristin SM. Marfan syndrome caused by a recurrent de novo missense mutation in the fibrillin gene. Nature. 1991; 352(6333): 337-9.
• Kielty CM, Shuttleworth CA. Fibrillin-containing microfibrils: structure and function in health and disease. The İnternational Journal of Biochemistry and Cell Biology. 1995; 27(8): 747-60.
• Hollister DW, Godfrey M, Sakai LY, Pyeritz RE. Immunohistologic abnormalities of the microfibrillar-fiber system in the Marfan syndrome. New England Journal of Medicine. 1990; 323(3): 152-9.
• Rossiter JP, Repke JT, Morales AJ, Murphy EA, Pyeritz RE. A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. American Journal of Obstetrics and Gynecology. 1995; 173(5): 1599-606.
• Pacini L, Digne F, Boumendil A, Muti C, Detaint D, Boileau C, Jondeau G. Maternal complication of pregnancy in Marfan syndrome. International Journal of Cardiology. 2009; 136(2): 156-61.
• Earing MG, Webb GD. Congenital heart disease and pregnancy: maternal and fetal risks. Clinics in Perinatology. 2005; 32(4): 913-9.
• Barrett JM, Van Hooydonk JE, Boehm FH. Pregnancy-related rupture of arterial aneurysms. Obstetrical and Gynecological Survey. 1982; 37(9): 557-66.
• Saeki N, Taguchi S, Kawamoto M. Successful management of a patient with Marfan syndrome complicated with acute aortic dissection using landiolol during Cesarean section. Journal of Anesth. 2010; 24(2): 277-9.
• McNeilly G, Nicholl A, Broadway J, Rao S. Dural ectasia in Marfan’s syndrome: magnetic resonance imaging appearances and anaesthetic experience of three deliveries. International Journal of Obstetric Anesth. 2013; 22(4): 337-9.
• Buser RT, Mordecai MM, Brull SJ. Combined spinal-epidural analgesia for labor in a patient with Marfan's syndrome. Int J Obstet Anesth. 2007; 16(3): 274-6.
• Kuczkowski KM. Labor Analgesia for the Parturient With an Uncommon Disorder: A Common Dilemma in the Delivery Suite. Obstetrical and Gynecological Survey. 2003; 58(12): 800-3.
• Castellano JM, Silvay G, Castillo JG. Marfan Syndrome: Clinical, Surgical, and Anesthetic Considerations. In: Seminars in cardiothoracic and vascular anesth: 2013; SAGE Publications. 2013; 1089253213513842.
• Allyn J, Guglielminotti J, Omnes S, Guezouli L, Egan M, Jondeau G, Longrois D, Montravers P. Marfan’s syndrome during pregnancy: anesthetic management of delivery in 16 consecutive patients. Anesth and Analg. 2013; 116(2): 392-8.
• Fattori R, ANienaber C, Descovich B, Ambrosetto P, Reggiani LB, Pepe G, Kaufmann U, Negrini E, von Kodolitsch Y, Gensini GF. Importance of dural ectasia in phenotypic assessment of M arfan's syndrome. The Lancet. 1999; 354(9182): 910-3.
• Ahn NU, Sponseller PD, Ahn UM, Nallamshetty L, Kuszyk BS, Zinreich SJ. Dural ectasia is associated with back pain in Marfan syndrome. Spine. 2000; 25(12): 1562-8.
• Singh SI, Brooks C, Dobkowski W. General anesthesia using remifentanil for Cesarean delivery in a parturient with Marfan’s syndrome. Canadian Journal of Anaesth. 2008; 55(8): 526-31.
• Dean JC. Management of Marfan syndrome. Heart. 2002; 88(1): 97-103.