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Management of Budd Chiari Syndrome by liver transplantation: A case report

Year 2013, Volume 35, Issue 4 (2013): Supplement, 6 - 9, 14.02.2014

Abstract

Abstract

Budd-Chiari syndrome (obstruction of venous drainage of the liver) may be idiopathic, due to thrombosis of the hepatic veins in thrombogenic states or be due to extrinsic compression. It is rare, but life-threatening, if left untreated. For patients with end-stage liver disease, liver transplantation should be considered. Orthotopic liver transplantation (OLT) is associated with the potential for massive blood loss, necessitating rapid infusion of large quantities of blood products. In these patients, hemodynamic instability can be dramatically diminished by inferior vena cava cross-clamping during OLT. There are few reports about living donor transplantations in Budd-Chiari case. We present a case of 27-year-old man, with a diagnosis of end-stage liver disease secondary to Budd-Chiari syndrome and underwent living OLT. Anaesthesia management, and follow-up of patients affected by this condition represent a challenge for anesthesiologists. The aim of this report is to discuss the potential options in the intraoperative management of patients with Budd-Chiari syndrome, who will undergo living OLT.

Keywords: Budd-Chiari syndrome, orthotopic liver transplantation, anaesthesia management

 

References

  • Choi GS, Park JB, Jung GO, Chun JM, Kim JM, Moon JI, Kwon CH, Kim SJ, Joh JW, Lee SK. Living donor liver transplantation in Budd-Chiari syndrome: A single-center experience. Transplant Proc 2010; 42: 839.
  • Yoshimura N, Okajima H, Ushigome H, Sakamoto S, Fujiki M, Okamoto M. Current status of organ transplantation in Japan and worldwide. Surg Today 2010; 40: 514-25.
  • Soyama A, Eguchi S, Yanaga K, Takatsuki M, Hidaka M, Kanematsu T. Living donor liver transplantation with extensive caval thrombectomy for acute-onchronic Budd-Chiari syndrome. Surg Today 2011; 41: 1026.
  • Yamada T, Tanaka K, Ogura Y, Ko S, Nakajima Y, Takada Y, Uemoto S. Surgical techniques and long-term outcomes of living donor liver transplantation for Budd-Chiari syndrome. Am J Transplant 2006; 6: 2463-9.
  • Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med 2004; 350: 578-85.
  • Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger MH, Condat B, Farges O, Chagneau C, de Ledinghen V, Francoz C, Sauvanet A, Vilgrain V, Belghiti J, Durand F, Valla D. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology 2006; 44: 1308-16.
  • Valla DC. Hepatic vein thrombosis (Budd-Chiari syndrome). Semin Liver Dis 2002; 22: 5-14.
  • Cazals-Hatem D, Vilgrain V, Genin P, Denninger MH, Durand F, Belghiti J, Valla D, Degott C. Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: A study in 17 explanted livers. Hepatology 2003; 37: 510

Budd Chiari Sendromu’nda karaciğer transplantasyon yönetimi: Bir olgu sunumu

Year 2013, Volume 35, Issue 4 (2013): Supplement, 6 - 9, 14.02.2014

Abstract

Budd-Chiari sendromu (karaciğer venöz drenaj tıkanması) idiyopatik, hepatik venin trombojenik trombozu veya ekstrensek basısına bağlı gelişebilir. Nadir görülür, fakat tedavi edilmez ise yaşamı tehdit edicidir. Son dönem karaciğer hastalığı olan hastalar için, karaciğer transplantasyonu düşünülmelidir. Ortotopik karaciğer transplantasyonu (OKT), masif kan kaybı potansiyeli ve kan ürünlerinin büyük miktarlarda hızlı infüzyon gereksinimi ile ilişkilidir. Bu hastalarda OKT sırasında vena kava inferiora kros klempaj uygulanmasıyla hemodinamik instabilite önemli ölçüde azaltılabilir. Budd-Chiari ile birlikteliği olan karaciğer donör transplantasyonu olguları hakkında az sayıda rapor bulunmaktadır. Bu yayında, Budd-Chiari sendromuna sekonder olarak son dönem karaciğer hastalığı tanısı alan ve OKT yapılan 27 yaşındaki bir erkek hasta sunulmaktadır. Bu hastaların anestezi yönetimi ve takibi anesteziyolojistler için önemli bir sorun oluşturmaktadır. Bu raporun amacı Budd-Chiari sendromu ile birlikte OKT uygulanması planlanan hastaların intraoperatif yönetiminde potansiyel seçenekleri tartışmaktır.

References

  • Choi GS, Park JB, Jung GO, Chun JM, Kim JM, Moon JI, Kwon CH, Kim SJ, Joh JW, Lee SK. Living donor liver transplantation in Budd-Chiari syndrome: A single-center experience. Transplant Proc 2010; 42: 839.
  • Yoshimura N, Okajima H, Ushigome H, Sakamoto S, Fujiki M, Okamoto M. Current status of organ transplantation in Japan and worldwide. Surg Today 2010; 40: 514-25.
  • Soyama A, Eguchi S, Yanaga K, Takatsuki M, Hidaka M, Kanematsu T. Living donor liver transplantation with extensive caval thrombectomy for acute-onchronic Budd-Chiari syndrome. Surg Today 2011; 41: 1026.
  • Yamada T, Tanaka K, Ogura Y, Ko S, Nakajima Y, Takada Y, Uemoto S. Surgical techniques and long-term outcomes of living donor liver transplantation for Budd-Chiari syndrome. Am J Transplant 2006; 6: 2463-9.
  • Menon KV, Shah V, Kamath PS. The Budd-Chiari syndrome. N Engl J Med 2004; 350: 578-85.
  • Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger MH, Condat B, Farges O, Chagneau C, de Ledinghen V, Francoz C, Sauvanet A, Vilgrain V, Belghiti J, Durand F, Valla D. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology 2006; 44: 1308-16.
  • Valla DC. Hepatic vein thrombosis (Budd-Chiari syndrome). Semin Liver Dis 2002; 22: 5-14.
  • Cazals-Hatem D, Vilgrain V, Genin P, Denninger MH, Durand F, Belghiti J, Valla D, Degott C. Arterial and portal circulation and parenchymal changes in Budd-Chiari syndrome: A study in 17 explanted livers. Hepatology 2003; 37: 510
There are 8 citations in total.

Details

Primary Language English
Journal Section Case Reports
Authors

Mustafa Aydoğan

Cuneyt Kayaalp

Murat Bıçakcıoğlu

Yusuf Çolak

Mahmut Durmuş

Publication Date February 14, 2014
Published in Issue Year 2013Volume 35, Issue 4 (2013): Supplement

Cite

AMA Aydoğan M, Kayaalp C, Bıçakcıoğlu M, Çolak Y, Durmuş M. Management of Budd Chiari Syndrome by liver transplantation: A case report. CMJ. February 2014;35(4):6-9.