Transjugular intrahepatic portosystemic shunt for the management of severe venoocclusive disease following bone marrow transplantation

✍ Scribed by M W Fried; D G Connaghan; S Sharma; L G Martin; S Devine; K Holland; A Zuckerman; S Kaufman; J Wingard; T D Boyer

• Publisher: John Wiley and Sons
• Year: 1996
• Tongue: English
• Weight: 374 KB
• Volume: 24
• Category: Article
• ISSN: 0270-9139
• DOI: 10.1002/hep.510240321

✦ Synopsis

tension with ascites frequently accompanies these histologi-Hepatic venoocclusive disease (VOD) is a common, lifecal changes and contributes to the clinical manifestations of threatening complication of bone marrow transplantathis syndrome. A wedged hepatic venous pressure gradient tion (BMT). Portal hypertension is usually present and greater than 10 mm Hg is highly specific for the diagnosis of accounts for many of the clinical manifestations of this VOD in this setting. 6,7 Similar histological and clinical syndrome. We describe the results of transjugular intrachanges may be seen with other causes of hepatic venous hepatic portosystemic shunt (TIPS) for the management outflow obstruction, i.e., Budd-Chiari syndrome. 8 of VOD after BMT. TIPS was performed in six patients Varying degrees of liver dysfunction occur with VOD. Cowith histologically confirmed VOD who had progressive agulopathy, hepatic encephalopathy, and intractable ascites jaundice and ascites. Portal hypertension was improved requiring large volume paracentesis may occur. Rapidly inby TIPS in all patients (mean portal pressure gradient creasing levels of serum bilirubin and marked weight gain before TIPS, 20.2 { 4.6 vs. 6.7 { 1.9 mm Hg post-TIPS, P are predictors for the development of severe VOD. 9 Mild to õ .004). Three patients who underwent TIPS late in the moderate VOD may spontaneously resolve, whereas severe course of VOD did not demonstrate any clinical improve-

VOD results in the relentless progression to multiorgan failment after TIPS and expired within 2 weeks of the proceure, with a mortality rate approaching 100%. 2 There is no dure. The remaining three patients had less advanced satisfactory treatment for VOD, and management remains disease and demonstrated decreases in serum bilirubin, largely supportive. Infusions of heparin, prostaglandins, or improvement in coagulopathy, and decreased ascites tissue plasminogen activating factor have been used either after TIPS. Two patients subsequently expired, one with prophylactically or as therapy for established VOD in an atpersistent histological changes of VOD. The lone survitempt to improve blood flow through the terminal hepatic vor continues to do well with resolution of ascites, jaunvenule. [10][11][12][13][14] While some of these interventions resulted in a dice, and coagulopathy as of her last outpatient visit.

decreased incidence of VOD or clinical improvement in estab-TIPS was an effective method for portal decompression lished VOD, significant side effects have been reported and, in patients with VOD after BMT, and was associated at this time, none of these modalities are considered to be with clinical improvement in some patients. However, standard therapy for VOD.

these effects may be transient and may not improve

Both VOD and Budd-Chiari syndrome cause centrilobular overall survival. (HEPATOLOGY 1996;24:588-591.) congestion within the liver. In Budd-Chiari syndrome, this congestion is associated with hepatocellular necrosis and pro-Hepatic venoocclusive disease (VOD) is a clinical syndrome gressive liver injury. Relief of the congestion by a side-to-side characterized by jaundice, tender hepatomegaly, and ascites portacaval shunt is associated with complete resolution of that usually occurs within 3 weeks of bone marrow transthe clinical illness. 15 Because of the clinical similarities of plantation (BMT). [1][2][3] The incidence of VOD in this setting VOD and the Budd-Chiari syndrome, it is reasonable to beranges between 10% and 54%, depending on the cytoreductive lieve that decompression of the portal-venous system in VOD regimen and the criteria used to define the syndrome. 2-4 may be beneficial in a manner similar to that shown with The development of VOD has been strongly associated with portacaval shunts for the treatment of Budd-Chiari synhigh-dose, combination cytoreductive therapy, particularly drome. 15,16 In support of this idea are the observations that regimens that involve busulfan, cyclophosphamide, carmusside-to-side portacaval shunt improved clinical and biochemitine, and etoposide, coupled with total body irradiation. 2,3 cal parameters in patients with VOD following BMT. 17,18 Un-The pathophysiology of VOD is not well understood. The fortunately, most patients with severe VOD are pancytopenic hepatic lesion of VOD is characterized by occlusion of termiand critically ill, making the risks of major abdominal surnal hepatic venules, sinusoidal congestion, sinusoidal fibrogery prohibitive. Transjugular intrahepatic portosystemic sis, and hepatocellular necrosis, which occurs predominantly shunt (TIPS) is an attractive alternative for decompressing in the centrizonal region of the hepatic lobule. 5 Portal hyperthe portal system because of its relative technical ease and low rate of complications. 19 We report the use of TIPS for the management of patients with severe VOD following BMT.

Abbreviations: VOD, venoocclusive disease; BMT, bone marrow transplantation; TIPS,

PATIENTS AND METHODS

transjugular intrahepatic portosystemic shunt. From the Divisions of 1 Digestive Diseases and 2 Bone Marrow Transplantation, Depart-A diagnosis of VOD was considered in patients who developed ment of Medicine, and the Departments of 3 Pathology and 4 Radiology, Emory University hyperbilirubinemia and ascites within 4 weeks following BMT. Other