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Salvage living donor liver transplantation after percutaneous transluminal angioplasty for recurrent Budd-Chiari syndrome: a case report
- Yusaku Shirai1,
- Hitoshi Yoshiji1Email author,
- 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
© Shirai et al; licensee BioMed Central Ltd. 2011
Received: 22 July 2010
Accepted: 29 March 2011
Published: 29 March 2011
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.
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.
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.
Budd-Chiari syndrome (BCS) is a rare clinical condition that results from obstruction of the hepatic venous outflow and has a predisposition to thrombosis of the major hepatic veins and/or inferior vena cava (IVC) at points proximal to the right atrium . The manifestations of BCS are variable, such as hepatic enlargement, pain, tenderness, and portal hypertension . When the occlusion is rapid, the patient may present with acute portal hypertensive manifestations, such as progressive massive painful refractory ascites and rupture of 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 . Several invasive therapeutic modalities for BCS have been reported, such as percutaneous transluminal angioplasty (PTA), transjugular intrahepatic portosystemic shunt (TIPS), and orthotopic liver transplantation . For acute BCS, these invasive therapeutic options are likely to be the most appropriate modalities . 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 frequently leads to severe life-threatening acute liver failure. We report the case of a patient with recurrent BCS in whom the first PTA was successful, but the second PTA failed to re-open the stenosis of the IVC. Since our patient showed manifestations of acute liver failure, we decided to perform salvage living donor liver transplantation (LDLT) from his brother. The transplantation was successful, and all clinical manifestations were remarkably alleviated.
Laboratory data of the patient
BCS is a clinical condition characterized by hepatic venous outflow obstruction due to various underlying causes . BCS is uncommon, with an incidence rate of about one in two and a half million people per year, and current knowledge about its etiology is still insufficient . Most western cases of BCS have a known etiology such as protein-C or -S deficiency, and myeloproliferative diseases, whereas most Japanese cases are idiopathic including acute BCS . The Japanese Ministry of Health and Welfare Research Committee on Aberrant Portal Blood Flow carried out an epidemiological survey and clinical study on BCS, and reported that the majority of BCS patients in Japan are idiopathic, with an obstructing lesion in the IVC . This was also the case in our patient. It has been reported that stenosis of the IVC at the level of the liver is a common cause of non-cirrhotic ascites, which develop within a short period in developing countries . These patients generally have a poor nutritional status, which is now recognized as one of the causes of an immune-compromised state. Patients with such status frequently have thrombophlebitis of the IVC at the level of the liver, which probably results in stenosis or thrombosis of the IVC. Before the onset of symptoms, our patient suffered from alcohol abuse and did not have sufficient nutrition. It may be possible that he had a similar situation of malnutrition, which led to temporary acute stenosis of the IVC.
Clinically, in most cases of BCS, leg edema, abdominal fullness, and subcutaneous venous dilatation over the trunk gradually appear. In some cases, severe clinical manifestations such as progressive refractory ascites and rupture of esophageal varices occur within a short period; a condition classified as acute BCS . Acute BCS often results in fulminant liver failure due to severe liver congestion. In our case, the clinical manifestations developed within a short period accompanied with severe liver dysfunction, which could be classified as acute BCS. Although the general prognosis of acute BCS is poor, the outcome may be fair with accurate early diagnosis and effective treatment. We were able to make an early diagnosis of acute BCS and identify the stricture lesion of the IVC through a combination of several diagnostic modalities, such as Doppler US, 3-D CT, and MRI. After the accurate diagnosis, optimized therapy should be employed for acute BCS. As in our case, about one third of patients harbor short-segment stenosis of either the hepatic veins or IVC. Since such patients are candidates for PTA , we first performed PTA and achieved good clinical results for one year. However, it has been reported that PTA has a high level of recurrence and sometimes needs to be repeated. About half the patients who underwent PTA developed re-stenosis within one year. Likewise, our patient had re-stenosis approximately one year after the first PTA. Esophageal varices and ascites developed very quickly along with re-stenosis. We attempted PTA again, but the second time was not effective. The IVC was temporarily dilated but soon re-stenosed. It was reported that an expandable metallic stent (EMS) can be useful for overcoming and reducing the occurrence of re-stenosis or obstructions after PTA . However, in our case, the stenosis level of the IVC and debouch of the hepatic veins were very close. The radiologist in our hospital concluded that we could not employ EMS in this case since sufficient long-term blood flow could not be guaranteed.
Liver transplantation has been used as an alternative to, or after failure of, surgical interventions for BCS . It has been proposed that patients with fulminant hepatic failure consequent to BCS should be listed with the United Network for Organ Sharing system status 1A . However, in Japan, it is extremely difficult to wait for DLDT due to the severe shortage of the number of donors. Although the surgical techniques of LDLT are different from those of DLDT, refined techniques were developed in Japan thanks to the enormous efforts of Japanese surgeons . Although patients with BCS account for only 1-2% of all orthotopic liver transplants, fortunately the department of surgery in our hospital has experience with LDLT. Since our patient's brother suggested becoming a donor for LDLT, we could perform LDLT in the current case. As our patient suffered acute liver failure, all other therapies were less likely to be successful. LDLT was successfully performed, and all the patient's clinical manifestations were significantly alleviated. Therefore, liver transplantation should be considered as one of the alternative options for therapeutic salvage.
We described the case of a patient with recurrent BCS in whom the first PTA was successful, but the second PTA failed to re-open the stenosis of the IVC. We performed salvage LDLT from his brother. The transplantation was successfully performed, and all clinical manifestations were significantly alleviated. In cases of recurrent BCS, the blocked hepatic venous outflow is not always relieved, even with invasive therapies. We have to consider the possibility of adopting alternative salvage therapies if the first therapeutic modalities fail. When invasive therapeutic modalities such as PTA fail, liver transplantation should be considered as an alternative option.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
The authors acknowledge (anonymously) the patient on whom this case report is based.
- Valla DC: Primary Budd-Chiari syndrome. J Hepatol. 2009, 50 (1): 195-203. 10.1016/j.jhep.2008.10.007.View ArticlePubMedGoogle Scholar
- Darwish Murad S, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ, Trebicka J, Morard I, Lasser L, Heller J, Hadengue A, Langlet P, Miranda H, Primignani M, Elias E, Leebeek FW, Rosendaal FR, Garcia-Pagan JC, Valla DC, Janssen HL, EN-Vie (European Network for Vascular Disorders of the Liver): Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med. 2009, 151 (3): 167-175.View ArticlePubMedGoogle Scholar
- Horton JD, San Miguel FL, Membreno F, Wright F, Paima J, Foster P, Ortiz JA: Budd-Chiari syndrome: illustrated review of current management. Liver Int. 2008, 28 (4): 455-466. 10.1111/j.1478-3231.2008.01684.x.View ArticlePubMedGoogle Scholar
- Menon KV, Shah V, Kamath PS: The Budd-Chiari syndrome. N Engl J Med. 2004, 350 (6): 578-585. 10.1056/NEJMra020282.View ArticlePubMedGoogle Scholar
- Okuda H, Yamagata H, Obata H, Iwata H, Sasaki R, Imai F, Okudaira M, Ohbu M, Okuda K: Epidemiological and clinical features of Budd-Chiari syndrome in Japan. J Hepatol. 1995, 22 (1): 1-9. 10.1016/0168-8278(95)80252-5.View ArticlePubMedGoogle Scholar
- Shrestha S: Bacterial peritonitis in hepatic inferior vena cava disease: a hypothesis to explain the cause of infection in high protein ascites. Hepatol Res. 2002, 24 (1): 42-10.1016/S1386-6346(02)00018-9.View ArticlePubMedGoogle Scholar
- Valla DC: The diagnosis and management of the Budd-Chiari syndrome: consensus and controversies. Hepatology. 2003, 38 (4): 793-803.View ArticlePubMedGoogle Scholar
- Shirai Y, Yoshiji H, Fujimoto M, Kojima H, Yanase K, Namisaki T, Kitade M, Yamamoto K, Sakaguchi H, Kichikawa K, Fukui H: Successful treatment of acute Budd-Chiari syndrome with percutaneous transluminal angioplasty. Abdom Imaging. 2004, 29 (6): 685-687. 10.1007/s00261-004-0183-6.View ArticlePubMedGoogle Scholar
- 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 (10): 2463-2469. 10.1111/j.1600-6143.2006.01505.x.View ArticlePubMedGoogle Scholar
- Washburn WK, Gish RG, Kamath PS: Model for end-stage liver disease (MELD) exception for Budd-Chiari syndrome. Liver Transpl. 2006, 12 (12 Suppl 3): S93-94. 10.1002/lt.20964.View ArticlePubMedGoogle Scholar
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