Stent angioplasty of narrowed portocaval shunt in Budd Chiari syndrome: a case report

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Abstract

Background

Hepatic vein thrombosis (Budd-Chiari Syndrome) is a rare disorder resulting from an obstruction to the outflow of blood from the liver. Early decompression is needed to prevent liver dysfunction and death. Radiological intervention includes angioplasty of stenosis and webs and the placement of transjugular intrahepatic portosystemic shunts (TIPPS). Side-to-side portacaval shunt (SSPCS) remains the gold standard for achieving good long-term results.

Case presentation

A 37-year old lady underwent side-to-side portacaval shunt for Budd Chiari syndrome. She had early shunt blockage and this was successfully treated with the placement of a metallic stent across the shunt.

Conclusion

At five years, she remains asymptomatic, with normal liver functions, no ascites, and normal flow through the stent on Colour Doppler examination.

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Most cited references 9

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Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients.

M Gebel, B Ringe, J. Borst … (1995)

The optimal treatment of Budd-Chiari syndrome (BCS) remains an open question. It is still a matter of controversial discussion whether venous decompression or liver transplantation is superior. To elucidate the role and prognosis of both surgical options in our own experience, a consecutive series of 50 patients treated between 1981 and 1993 was retrospectively analyzed. Twelve patients had different types of portosystemic shunts or local decompressive procedures, and transplantation was performed in 43 cases, including five with previous conventional surgery. The overall mortality of 18 of 50 was conventional surgery. The overall mortality of 18 of 50 was concentrated within the early postoperative period, with no patient lost after 1 year. In the venous decompression group, the success rate was only 29%, and treatment failure was closely related to the finding of cirrhosis or technical problems like vascular thrombosis. After transplantation, early complications were rejection, primary nonfunction, or graft necrosis, and contributed significantly to the risk of sepsis. Thirty of 43 liver recipients are currently alive, including four rescued after failed decompressive surgery, with 1- and 10-year survival of 69%, and excellent recurrence-free rehabilitation. These results clearly indicate that patient selection plays a dominant prognostic role in the treatment of BCS. Venous decompression and liver transplantation should both be integrated in a common therapeutic concept, and the individual decision for the preferred approach must be based on the leading clinical symptom: portal hypertension or liver failure, together with the assessment of reversibility of hepatic damage, and the potential of cure of the underlying disease.

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Budd-Chiari syndrome: current management options.

Daniel J. Cameron, D Slakey, A Venbrux … (2001)

To assess the outcomes of current treatment strategies for Budd-Chiari syndrome. Budd-Chiari syndrome, occlusion or obstruction of hepatic venous outflow, is a disease traditionally managed by portal or mesenteric-systemic shunting. The development of other treatment options, such as catheter-directed thrombolysis, transjugular portosystemic shunting (TIPS), and liver transplantation, has expanded the therapeutic algorithm. The authors reviewed the medical records of all patients diagnosed with Budd-Chiari syndrome at the Johns Hopkins Hospital during the past 20 years. A total of 54 patients were identified: 13 (24%) male patients and 41 (76%) female patients, ranging in age from 2 to 76 years (median 33 years). Twenty-one (39%) had polycythemia vera, 3 (5.6%) used estrogens, 11 (20%) had a myeloproliferative or coagulation disorder, and in 7 (13%) the cause remained unknown. Forty-three patients were treated with surgical shunting, 24 mesocaval and 19 mesoatrial. Actuarial survival rates at 1, 3, and 5 years after shunting were 83%, 78%, and 75%, respectively. Of 33 patients surviving more than 4 years, 28 (85%) had relief of clinical symptoms. Five patients required shunt revision and eight had radiologic procedures to maintain shunt patency. Primary and secondary shunt patency rates were 46% and 69% respectively for mesoatrial shunts and 70% and 85% respectively for mesocaval shunts. Clot lysis was successful as primary treatment in seven patients. TIPS was performed in three patients, one after a failed mesocaval shunt. During an average of 4 years of follow-up, these patients required multiple procedures to maintain TIPS patency. Six patients underwent liver transplantation. Of these, three had previous shunt procedures. Five of the transplant recipients are alive with follow-up of 2 to 9 years (median 6). Both shunting and transplantation can result in a 5-year survival rate of at least 75%, and other treatment modalities may be appropriate for highly selected patients. Optimal management requires that treatment be directed by the predominant clinical symptom (liver failure or portal hypertension) and anatomical considerations and be tempered by careful assessment of surgical risk.

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A 27-year experience with surgical treatment of Budd-Chiari syndrome.

Mohammed Orloff, B Girard, Johanna P Daily … (2000)

To determine the effects of surgical portal decompression in Budd-Chiari syndrome (BCS) on survival, quality of life, shunt patency, liver function, portal hemodynamics, and hepatic morphology during periods ranging from 3.5 to 27 years. Experiments in the authors' laboratory showed that surgical portal decompression reversed the deleterious effects of BCS on the liver. This study was aimed at determining whether similar benefit could be obtained in patients with BCS. From 1972 to 1999, the authors conducted prospective studies of the treatment of 60 patients with BCS who were divided into three groups: the first had occlusion confined to the hepatic veins treated by direct side-to-side portacaval shunt (SSPCS); the second had occlusion involving the inferior vena cava (IVC) treated by a portal decompressive procedure that bypassed the obstructed IVC; and the third group, who had advanced cirrhosis and hepatic decompensation and were referred too late for treatment by portal decompression, required orthotopic liver transplantation. In the 32 patients with BCS resulting from hepatic vein occlusion alone, SSPCS had a surgical death rate of 3%, and 94% of the patients were alive 3.5 to 27 years after surgery. All 31 survivors remained free of ascites and almost all had normal liver function. No patient with a patent shunt had encephalopathy. The SSPCS remained patent in all but one patient. Liver biopsies showed no evidence of congestion or necrosis, and 48% of the biopsies were diagnosed as normal. Mesoatrial shunt was performed in eight patients with BCS caused by IVC thrombosis. All patients survived surgery, but five subsequently developed thrombosis of the synthetic graft and died. Because of the poor results, mesoatrial shunt was abandoned. Instead, a high-flow combination shunt was introduced, consisting of SSPCS combined with a cavoatrial shunt (CAS) through a Gore-Tex graft. There were no surgical or long-term deaths among 10 patients who underwent combined SSPCS and CAS, and the shunts functioned effectively during 4 to 16 years of follow-up. Ten patients with advanced cirrhosis were referred too late to benefit from surgical portal decompression, and they were approved and listed for orthotopic liver transplantation. Three patients died of liver failure while awaiting a transplant, and four patients died after the transplant. The 1- and 5-year survival rates were 40% and 30%, respectively. SSPCS in BCS with hepatic vein occlusion alone results in reversal of liver damage, correction of hemodynamic disturbances, prolonged survival, and good quality of life when performed early in the course of BCS. Similarly good results are obtained with combined SSPCS and CAS in patients with BCS resulting from IVC occlusion. In contrast, mesoatrial shunt has been discontinued in the authors' program because of an unacceptable incidence of graft thrombosis and death. In patients with advanced cirrhosis from long-standing, untreated BCS, orthotopic liver transplantation is the only hope of relief and results in the salvage of some patients. The key to long survival in BCS is prompt diagnosis and treatment by portal decompression.

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Author and article information

Journal

Journal ID (nlm-ta): Cases J

Title: Cases Journal

Publisher: BioMed Central

ISSN (Electronic): 1757-1626

Publication date Collection: 2009

Publication date (Electronic): 1 January 2009

Volume: 2

Page: 1

Affiliations

[1 ]Department of Gastrointestinal Surgery, Jaslok Hospital & Research Centre, Mumbai, India

[2 ]Department of Gastroenterology, Jaslok Hospital & Research Centre, Mumbai, India

[3 ]Department of Interventional Radiology, Jaslok Hospital & Research Centre, Mumbai, India

Article

Publisher ID: 1757-1626-2-1

DOI: 10.1186/1757-1626-2-1

PMC ID: 2648948

PubMed ID: 19117529

SO-VID: 59810e28-0c23-465b-8fa7-aff3ac74cddf

License:

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.

Stent angioplasty of narrowed portocaval shunt in Budd Chiari syndrome: a case report

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Abstract

Background

Case presentation

Conclusion

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Most cited references 9

Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients.

Budd-Chiari syndrome: current management options.

A 27-year experience with surgical treatment of Budd-Chiari syndrome.

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