Shirai et al. Journal of Medical Case Reports 2011, 5:124 http://www.jmedicalcasereports.com/content/5/1/124 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access Salvage living donor liver transplantation after percutaneous transluminal angioplasty for recurrent Budd-Chiari syndrome: a case report Yusaku Shirai1, Hitoshi Yoshiji1*, Saiho Ko2, Masaharu Yamazaki1, Yasuhide Ikenaka1, Ryuichi Noguchi1, Chie Morioka1, Kosuke Kaji1, Yosuke Aihara1, Keisuke Nakanishi1, Junichi Yamao1, Masahisa Toyohara1, Akira Mitoro1, Masayoshi Sawai1, Motoyuki Yoshida1, Masao Fujimoto1, Masahito Uemura1, Yoshiyuki Nakajima2 and Hiroshi Fukui1 Abstract Introduction: Budd-Chiari syndrome is a very rare pathological entity that ultimately leads to liver failure. Several therapeutic modalities, including percutaneous transluminal angioplasty, have been attempted to save the life of patients with Budd-Chiari syndrome. Few reports have described a salvage living donor liver transplantation performed after percutaneous transluminal angioplasty in a patient with acute Budd-Chiari syndrome. Case presentation: A 26-year-old Japanese man developed severe progressive manifestations, such as massive ascites and hematemesis due to rupture of esophageal varices. After making several investigations, we diagnosed the case as Budd-Chiari syndrome. We first performed percutaneous transluminal angioplasty to dilate a short- segment stenosis of his inferior vena cava. The first percutaneous transluminal angioplasty greatly improved the clinical manifestations. However, after a year, re-stenosis was detected, and a second percutaneous transluminal angioplasty failed to open the severe stricture of his inferior vena cava. Since our patient had manifestations of acute liver failure, we decided to perform salvage living donor liver transplantation from his brother. The transplantation was successfully performed and all clinical manifestations were remarkably alleviated. Conclusion: In cases of recurrent Budd-Chiari syndrome, the blocked hepatic venous outflow is not always relieved, even with invasive therapies. We have to take into account the possibility of adopting alternative salvage therapies if the first therapeutic modalities fail. When invasive therapy such as percutaneous transluminal angioplasty fails, liver transplantation should be considered as an alternative option. Introduction Budd-Chiari syndrome (BCS) is a rare clinical condition that results from obstruction of the hepatic venous out- flow and has a predisposition to thrombosis of the major hepatic veins and/or inferior vena cava (IVC) at points proximal to the right atrium [1]. The manifesta- tions of BCS are variable, such as hepatic enlargement, pain, tenderness, and portal hypertension [2]. When the occlusion is rapid, the patient may present with acute portal hypertensive manifestations, such as progressive massive painful refractory ascites and rupture of * Correspondence: yoshijih@naramed-u.ac.jp 1Third Department of Internal Medicine, Nara Medical University, Nara, Japan Full list of author information is available at the end of the article esophageal varices. This clinical situation is called acute BCS, and several therapeutic options, including medical treatments (for example, anticoagulants and diuretics) and invasive management, have been employed [2]. Sev- eral invasive therapeutic modalities for BCS have been reported, such as percutaneous transluminal angioplasty (PTA), transjugular intrahepatic portosystemic shunt (TIPS), and orthotopic liver transplantation [3]. For acute BCS, these invasive therapeutic options are likely to be the most appropriate modalities [3]. Even with these invasive therapies, however, recovery of the obstructed hepatic venous outflow is not always achieved. In that case, sequential salvage therapeutic modalities should be considered since acute BCS © 2011 Shirai et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.