Budd-Chiari Syndrome: Illustrated Review of Current Management

John D. Horton; Francisco L. San Miguel; Jorge A. Ortiz

Liver International. 2008;28(4):455-466.

Management Strategies

Treatment of BCS generally follows a least invasive to most invasive strategy. However, this paradigm is not absolute and circumstances may dictate immediate surgery or even transplantation depending on the presentation. Algorithms for management of BCS have been reported.^[26,27]

Plessier's algorithm, which was applied to 51 patients over a 7-year period, resulted in overall 1-, 3- and 5-year survival rates of 96%, 89% and 89% respectively.^[27] This algorithm included medical management, recanalization, TIPS and liver transplant. Surgical shunts were not part of the algorithm.

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The views expressed in this document are those of the author(s) and do not reflect the official policy of William Beaumont Army Medical Center, the Department of the Army, the Department of Defense, or the United States Government.

Cite this: Budd-Chiari Syndrome: Illustrated Review of Current Management - Medscape - Apr 01, 2008.

References

Liver Transplantation For Budd-Chiari Syndrome
Author	Year	Country	Years Included	# Patients	PreTx Shunt	PreTx TIPS	1-year Patient Survival (%)	3-year Patient Survival (%)	5-year Patient Survival (%)	Recurrence	% Retransplant
Campbell	1988	UK	1976-1986	17	NS	NS	88	88	NS	1	12
Halff	1990	US	1974-1988	23	5	0	69	45	45	3	13
Jamieson	1991	UK	1986-1990	26	3	0	69	69	50	2	8%
Shaked	1992	US	1984-1990	14	2	NS	86	76	NS	0	21
Knoop	1994	Germany	1988-1994	8	NS	NS	88	NS	NS	1	25
Sakai	1994	Canada	1977-1990	11	3	NS	64	64	64	0	0%
Ringe	1995	Germany	1981-1993	43	4	0	69	69	69	0	21
Hemming	1996	Canada	1987-1995	10	3	NS	NS	NS	59	NS	10
Rao	2000	Hong Kong	1986-1997	8	3	NS	NS	63	NS	0	0%
Srinivasan	2002	UK	1988-1999	19	5	1	95	95	95	2	21
Ulrich	2002	Germany	1988-2001	27	NS	NS	NS	NS	87	NS	15
Attwell	2004	US	1992-2001	10	0	5	90	80	80	0	0%
Cruz	2005	US	1988-2002	11	1	4	81	NS	65	3	9%
Mentha	2006	Europe	1988-1999	248	49	11	76	71	68	6	15
Yamada¹	2006	Japan	1990-2005	9	0	1	88	71	67	2	0
Plessier	2006	France	1997-2004	11	0	2	91	91	NS	1	9
Segev	2006	US	2002-2006*	100	NS	23	88	85	NS	NS	NS

¹ All procedures were live donor liver transplants.
* Time period only includes patients from the MELD era.
NS, not stated; MELDS, model for end-stage liver disease; TIPS, transjugular intrahepatic portosystemic shunt.

Liver Transplantation For Budd-Chiari Syndrome
Author	Year	Country	Years Included	# Patients	PreTx Shunt	PreTx TIPS	1-year Patient Survival (%)	3-year Patient Survival (%)	5-year Patient Survival (%)	Recurrence	% Retransplant
Campbell	1988	UK	1976-1986	17	NS	NS	88	88	NS	1	12
Halff	1990	US	1974-1988	23	5	0	69	45	45	3	13
Jamieson	1991	UK	1986-1990	26	3	0	69	69	50	2	8%
Shaked	1992	US	1984-1990	14	2	NS	86	76	NS	0	21
Knoop	1994	Germany	1988-1994	8	NS	NS	88	NS	NS	1	25
Sakai	1994	Canada	1977-1990	11	3	NS	64	64	64	0	0%
Ringe	1995	Germany	1981-1993	43	4	0	69	69	69	0	21
Hemming	1996	Canada	1987-1995	10	3	NS	NS	NS	59	NS	10
Rao	2000	Hong Kong	1986-1997	8	3	NS	NS	63	NS	0	0%
Srinivasan	2002	UK	1988-1999	19	5	1	95	95	95	2	21
Ulrich	2002	Germany	1988-2001	27	NS	NS	NS	NS	87	NS	15
Attwell	2004	US	1992-2001	10	0	5	90	80	80	0	0%
Cruz	2005	US	1988-2002	11	1	4	81	NS	65	3	9%
Mentha	2006	Europe	1988-1999	248	49	11	76	71	68	6	15
Yamada¹	2006	Japan	1990-2005	9	0	1	88	71	67	2	0
Plessier	2006	France	1997-2004	11	0	2	91	91	NS	1	9
Segev	2006	US	2002-2006*	100	NS	23	88	85	NS	NS	NS

Murad's Budd-Chiari Syndrome Prognostic Classification
	Score*	% of Cohort (%)	5-year Survival (%)	% With Surgical Shunt
Class I	0-1.1	27	89	29
Class II	1.1-1.5	46	74	55
Class III	>1.5	27	42	60

* Equation: 1.27 × encephalopathy + 1.04 × ascites + 0.72 × prothrombin time + 0.004 × bilirubin.
Ascites and hepatic encephalopathy were scored as present (1) or absent (0) and prothrombin time as higher (1) or lower (0) than 2.3 INR. Bilirubin was included as a continuous variable.
INR, international normalized ratio.

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