Surg Gynecol Obstet 1992 Jun;174(6):453-9

Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome

Shaked A, Goldstein RM, Klintmalm GB, Drazan K, Husberg B, Busuttil RW
Department of Surgery, University of California, Los Angeles

Abstract
We have analyzed the indications and results of shunt operation versus
orthotopic liver transplantation (OLT) in 22 patients with Budd-Chiari syndrome
(BCS). The underlying cause of the syndrome was similar between the two groups
and was related to myeloproliferative disorders or the use of birth control
pills in 18 of 22 patients. The results of biopsies of the liver showed
centrilobular congestion and necrosis in all candidates who underwent shunting
and the presence of fibrosis and cirrhosis in the OLT candidates. The
indications for shunts included symptoms related to portal hypertension only and
well-preserved synthetic hepatic function. Ten patients were treated with 12
shunt procedures, including mesoatrial (eight patients) and side to side
portacaval shunt (four patients). Significant complications after shunt
procedure included fulminant (one of ten patients) and progressive (one of ten
patients) hepatic failure requiring urgent OLT; one death occurred because of
pulmonary sepsis. Indications for OLT were signs of end stage liver expressed by
severe portal hypertension and variceal bleeding (four of 14 patients),
progressive encephalopathy (seven of 14 patients) and poor synthetic function
(bilirubin greater than 3 milligrams per deciliter in eight of 14 patients and
albumin less than 3.0 grams per liter, or both, in ten of 14 patients). Fourteen
patients were treated with 16 OLT, three patients had retransplantation for
primary nonfunction graft (two of 14 patients) or chronic rejection (one of 14
patients). There were two early deaths in the group. With a follow-up period
between two months to five years, 12 of 14 patients undergoing OLT are alive,
fully functional and have normal liver function tests. Seven of ten patients who
had shunts are alive, six are able to maintain normal activity and one has
progressive end stage hepatic disease and is not a candidate for OLT. However,
the hepatic function continues progressively to be abnormal. Various options are
available for the treatment of the syndrome. Portosystemic decompression is
effective and should be considered at the early stage of the disease, prior to
the development of significant hepatic failure. However, few of the patients
will continue to have slow, but progressive hepatic failure and may require OLT.
The only effective treatment for end stage hepatic disease secondary to the BCS
is OLT