Acute Budd-Chiari Syndrome Research Articles

Acute Budd–Chiari syndrome due to a simple liver cyst

Simple liver cysts are common, rarely causing significant morbidity or mortality. Budd–Chiari syndrome (BCS) is caused by obstruction of hepatic venous outflow and is the leading cause of postsinusoidal liver failure. We present a rare case of BCS caused by a simple hepatic cyst.A 16cm × 16cm liver cyst was found on computed tomography of a 66-year-old woman presenting with abdominal pain. The cyst had become infected, thus enlarged, exerting mass effect with almost complete compression of the inferior vena cava. Shortly after admission, the patient developed acute liver failure, with deranged clotting and hepatic encephalopathy requiring full organ support on the intensive care unit. Cardiac output studies showed a low cardiac index of 1.4l/min/m2.An emergency laparotomy with fenestration of the cyst and drainage of 2l of purulent material led to a full recovery. Intraoperative cystic fluid aspirates later confirmed no evidence of Echinococcus. Histology confirmed a simple cyst. Liver biopsies showed severe, confluent, bridging necrosis, without background parenchymal liver disease.Acute BCS due to rapid compression of all major hepatic veins leading to fulminant hepatic failure is rare. Our case highlights a clinically significant complication of a simple liver cyst of which clinicians should be aware when managing these ‘innocent’ lesions.

Comparative study on MRI features of acute and chronic Budd-Chiari syndrome

Objective To investigate the features of Magnetic resonance imaging (MRI) and analyze the diagnostic value of MRI on acute and chronic Budd-Chiari syndrome (BCS). Methods Eleven patients with acute BCS who were treated,while other 42 patients with proven chronic BCS were taken as chronic group.All patients in our study underwent MRI examinations.MRI findings were retrospectively studied.Comparison between groups was made by Wilcoxon W rank sum test for frequency of ascites and Fisher Exact Test for the percentage of intrahepatic venous collaterals and extrahepatic venous collaterals. Results Hepatic venous thrombosis with high signal on T 2WI was demonstrated in 11 patients with acute BCS,and 3 of them also had inferior vena cava thrombosis.Compared with the central liver,the periphery of the liver of all patients were moderately hypointensive on T 1WI and slightly hyperintensive on T 2WI,and the enhancement degree of liver periphery in venous phase and arterial phase was relatively low.In chronic group,9 of them had hepatic venous obstruction,3 had inferior vena cava obstruction,and 30 had both.All chronic BCS patients had the features of stasis cirrhosis.There was no signal difference between the periphery and the central of the liver on precontrast and postcontrast images.In acute group,all of them suffered from ascites (2 patients with a moderate amount of ascites,and 9 patients with massive ascites),2 of Key words: Budd-Chiari syndrome; Magnetic resonance imaging; Acute; Chronic; Hepatic vein thrombosis

Budd–Chiari Syndrome Following Laparoscopic Cholecystectomy

Patients with thrombophilic disorder while undergoing intra-abdominal surgery may develop splanchnic vein thrombosis which can have dire consequences. Here we report a case of a 38-year-old female who developed acute Budd-Chiari syndrome after a laparoscopic cholecystectomy. She had polycythemia vera which was not diagnosed before surgery. In this report we want to highlight presurgical evaluation of routine biochemical tests and ultrasonography suggestive of myeloproliferative disorders were missed which led to the Budd-Chiari syndrome. We recommend a meticulous look at the routine evaluation done prior to cholecystectomy is essential.

G67(P) Dent’s Disease Complicated by an Acute Budd-Chiari Syndrome: Case Report

We present the case of a young boy with Dent’s disease, identified as having a mutation in the kidney-specific chloride-proton antiporter CLCN5 during investigation for nephrotic range proteinuria. He went...

Acute iatrogenic Budd-Chiari syndrome following hepatectomy for hepatolithiasis: A report of two cases

Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow obstruction at any level from the small hepatic veins to the junction of the inferior vena cava (IVC) and the right atrium, regardless of the cause of obstruction. We present two cases of acute iatrogenic BCS and our clinical management of these cases. The first case was a 43-year-old woman who developed acute BCS following the implantation of an IVC stent for the correction of stenosis in the IVC after hepatectomy for hepatolithiasis. The second case was a 61-year-old woman with complete obstruction of the outflow of hepatic veins during bilateral hepatectomy for hepatolithiasis. Acute iatrogenic BCS should be considered a rare complication following hepatectomy for hepatolithiasis. Awareness of potential hepatic outflow obstructions and timely management are critical to avoid poor outcomes when performing hepatectomy for hepatolithiasis.

Improved Outcomes of Budd-Chiari Syndrome in Paroxysmal Nocturnal Hemoglobinuria with Eculizumab Therapy

Abstract 3478Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal disorder of hematopoietic stem cells characterised by deficiency of cell membrane glycosylphosphatidylinositol-anchored proteins, rendering the cells susceptible to complement attack. Budd-Chiari syndrome (BCS) describes obstruction of the hepatic venous outflow tract. Complications such as ascites and varices often occur at the time of acute thrombosis. Conventional management includes anticoagulation, transjugular intrahepatic portosystemic shunt (TIPSS) placement, peripheral/local thrombolysis and orthotopic liver transplantation (OLT). The morbidity and mortality of PNH patients with BCS is particularly high: a new thrombotic event or thrombosis extension in anticoagulated patients is reported in 27%; a significant proportion of TIPSS fail due to recurrent thromboses; historically, mortality rates of 67% have been reported; hematopoietic stem cell transplantation and OLT have unacceptably high transplant-related mortalities. Eculizumab prevents cleavage of C5 and reduces risk of thrombosis in PNH. The role of this monoclonal antibody in the management of BCS with PNH has yet to be evaluated. Nineteen PNH patients (12 female, 7 male) with a BCS diagnosis who were subsequently given eculizumab, were identified from the Leeds PNH Service. Patients were diagnosed with PNH between 1991–2012 (median follow up from PNH diagnosis 7.5 yrs, range 2 mths – 21 yrs; from BCS diagnosis 3.5 yrs). Median age at PNH diagnosis was 30 yrs (range 19–87) and at BCS 32 yrs (range 19–87). The mean granulocyte clone size at PNH diagnosis (n=14) was 86.3% (range 59.7–99%) and at BCS (n=12) was 93.1% (range 80.9–99.6%). BCS was the presenting feature of PNH in 9 (47.4%). Six were diagnosed as part of diagnostic work-up during the acute BCS. In 3, a diagnosis of PNH was made 1, 6 and 26 months post BCS. Ten (52.6%) were already known PNH at the time of their BCS. None were receiving eculizumab. Median time from PNH to BCS was 30 mths, range 3–132. Seven were anticoagulated (5 primary prophylaxis) prior to BCS. Eight PNH patients (Group 1) developed BCS before the availability of eculizumab and remained alive long enough to receive therapy. Six in Group 1 (75%) developed BCS complications prior to commencing eculizumab including development of varices (n=1), progression of thrombus despite full anticoagulation (n=1), ascites necessitating TIPSS (n=2), hepatomegaly (n=3) and splenomegaly (n=3). Two did not endure BCS complications but suffered cerebral vein thrombotic events on anticoagulation. The median time to first dose of eculizumab therapy in Group 1 was 3 yrs (range 2 mths – 11 yrs). All patients remain on eculizumab (median follow-up 6.9 yrs, range 2.8 – 7.1); there have been no further thrombotic events in this cohort. Group 2 consists of 11 PNH patients in whom BCS was diagnosed during the availability of eculizumab. Four received this less than 14 days from onset of BCS without any subsequent complications. The remaining 7 started eculizumab 2 or more weeks from probable onset of BCS. Complications included ascites necessitating TIPSS and/or gastric/esophageal varices. TIPSS procedures were effective in all patients once on eculizumab with no occlusions. Importantly, 3 of the 19 patients have stopped secondary anticoagulation after starting eculizumab (due to thrombocytopenia, hemorrhage or perceived bleeding risk due to varices). There have been no further thrombotic episodes in these patients. This case series is the first to document 19 patients, managed with eculizumab with both PNH and BCS. Rapid intervention with eculizumab in addition to anticoagulation when safe to administer, appears to prevent the development of BCS associated complications. If complications are established at diagnosis, eculizumab can prevent progression and reoccurrence; it also facilitates successful use of TIPSS. We can conclude: a) primary prophylaxis is not sufficient to prevent BCS in patients with PNH b) prompt treatment with eculizumab may negate long-term complications of BCS and allows effective and safe use of TIPSS c) in selected cases, eculizumab therapy may facilitate the withdrawal of anticoagulation without increasing subsequent risk of thrombosis and d) survival for this cohort is currently 100%. The management of BCS in PNH with immediate commencement of eculizumab and, where appropriate, use of TIPSS, is associated with improved outcomes. Disclosures:Kelly:Alexion Pharmaceuticals, Inc.: Consultancy, Honoraria. Richards:Alexion Pharmaceuticals, Inc.: Consultancy, Honoraria. Arnold:Alexion Pharmaceuticals, Inc: Honoraria. Hillmen:Alexion Pharmaceuticals, Inc: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees. Hill:Alexion Pharmaceuticals, Inc.: Consultancy, Honoraria.

Side to Side Cavocavostomy in Piggy Back Adult Liver Transplantation

Aims: Retrospective study designed to describe results of the side-to-side cavo-cavostomy (STSCC) anastomosis through caval reconstruction, with particular emphasis on complications associated with this technique. All within a single Institution experience. Patients and methods: From June 1998 to January 2012, 577 cadaver liver transplants were performed at our Institution. 575 of which underwent STSCC in accordance with Belghiti's technique described elsewhere. Temporary porto-caval shunt was coupled to STSCC in 38 cases. Routine intraoperative Doppler ultrasonography was performed at the end of every procedure. Results: STSCC was feasible in all but 2 cases (99.66%) regardless of recipient's vena cava, anatomic factors, graft size or re-transplant status. Mean operative time for STSCC was 13 minutes (6-30 minutes). Though not necessarily considered a complication, there were 3 cases of caval stump thrombosis (0,52%). Complications strictly associated with this particular technique were limited to 4 patients (0,69%). The most significant one was the case of a patient that presented with acute post-operative Budd-Chiari syndrome that developed progressive deterioration due to liver failure. The patient could not be rescued with re-transplantation due to progressive brain damage and eventual cardiac arrest. The other three patients that developed direct complications attributed to STSCC were managed successfully. In one patient that developed outflow obstruction due to torsion from donor graft/recipient mismatch, the outflow was surgically improved by falciform ligament fixation. The second was only evident on biopsy by sinusoidal congestion and was managed non-operatively with measures to decrease central venous pressure. The third patient developed a pulmonary distress due to a decreased cardiac return consequence of a large sized graft, with subsequent severe haemodynamic instability requiring significant amount of pressors. The patient was managed non-operatively through a “positional” approach, by turning him 45 degree to the left in order to improve venous return to the heart. Twenty-five cases were done uneventfully with STSCC in patients undergoing re-transplantation. Conclusions: Side-to-Side Cavo-Cavostomy during piggyback liver transplantation has proven to be a safe and straightforward procedure, which can be performed even in a re-transplantation setting. In our experience this technique has shown a very low incidence of venous outflow obstruction. Maximal preservation of recipient inferior vena cava and temporary porto-caval shunt, improves decompression of inferior and splanchnic venous systems during the an-hepatic phase. In our opinion, STSCC helps overcome the outflow complications associated with traditional piggyback technique by using the orifices of one or more recipient hepatic veins as needed. Our study also indicates that donor graft to recipient mismatch is not an absolute contraindication when proper body size match is considered. A wide anastomosis with typical recipient hepatic veins inclusion and routine post-anastomotic Doppler Ultrasonography measurements are warranted.

Primary Budd-Chiari syndrome - a single center experience.

Budd-Chiari syndrome (BCS) is recognized as a clinical manifestation of various prothrombotic conditions which may be lethal within 3 years of the onset of symptoms if untreated. This study is a retrospective analysis of patients with BCS managed between 2004 and 2011. The diagnosis was confirmed with contrast CT-angiography and/or Doppler ultrasound. BCS was diagnosed in 20 patients (11 females and 9 males), median age 38 years (ranging from 18 to 56). Twelve patients were referred as acute BCS for the liver transplant (LTx) assessment. Thrombosis of the hepatic veins was caused by myeloproliferative disorders (n=8), end-stage liver disease (n=4), protein C deficiency (n=3), paroxysmal nocturnal hemoglobinuria (PNH) (n=1), antiphospholipid syndrome (n=1) and secondary poliglobulia (n=1). In two patients the origin of BCS could not be established despite appropriate screening. Median follow-up was 29 months. Low molecular heparin with subsequent conversion to vitamin K antagonists was routinely applied in all patients. Two patients underwent TIPS procedure with good long term outcome and 10 subjects received LTx; 1 patient was lost to follow-up and 1 died of chest infection 9 years since the diagnosis of BCS was made; 14 patients, including those who received LTx, were alive and well at least one year after BCS diagnosis. All survivors remain stable and are followed-up on a regular basis. Strict adherence to the diagnostic and therapeutic guidelines plays a crucial role in the management of BCS patients. Our results confirm the efficacy of anticoagulation as well as TIPS and/or OLT in treatment of this rare condition.

Recurrent acute Budd–Chiari syndrome after right hepatectomy: US color-Doppler vascular pattern and left hepatic vein stenting for treatment

After extended right hepatectomy remnant liver can be affected by outflow obstruction due to torsion of the inferior vena cava or kinking of the left hepatic vein. Remnant liver fixation is therefore suggested to avoid postoperative acute Budd-Chiari syndrome. Despite remnant liver reposition during surgery, a 76-years-old woman developed complete outflow obstruction. This clinical situation, due to left hepatic vein kinking, was suspected by US examination and confirmed by CT scan that showed a pathological intrahepatic vascular pattern. Patient required urgent relaparotomy and the liver was replaced in normal position. However, recurrence of outflow obstruction occurred and it was ultimately treated by inferior vena cava angiogram with left hepatic vein stenting.

Systemic thrombolysis for acute, severe Budd-Chiari syndrome

Budd-Chiari syndrome (BCS) or hepatic venous outflow obstruction, is a rare and complex clinical entity, with diverse aetiology and a wide range of presentations. Its rarity and heterogeneity create significant challenges for evidence-based clinical decision-making, particularly in the even more infrequent setting of acute BCS presentation. Here we present the case of a young woman with acute, severe BCS, where treatment alternatives were limited due to a significant IVC thrombus burden. A clearly defined acute onset allowed the prompt use of systemic thrombolysis, conducted in consultation with a multi-disciplinary team of clinicians, with close monitoring for both treatment response and complications. Her treatment resulted in resolution of hepatic vein occlusion and prevention of the potentially significant clinical sequelae. We review the literature and discuss current opinion in the management of BCS.

Acute Budd Chiari and Underlying JAK 2 Mutation Requiring TIPS Placement

Purpose: The JAK2V617F mutation is strongly implicated in the pathogenesis of myeloproliferative disorders (MPD) which account for at least 50% of cases of Budd-Chiari Syndrome (BCS). MPD is a common cause of splanchnic vein thrombosis in young women. Patients with BCS and underlying JAK2 mutation have severe hepatic outflow obstruction with worse Child-Pugh scores, and often require decompressive procedures such as transjugular intrahepatic portosystemic shunts (TIPS) placement or liver transplantation compared to those without the mutation. A 19 year old Caucasian woman with chronic abdominal pain, nausea and vomiting was found to have splenic vein thrombus on ultrasound. An initial hypercoagulable workup was negative. She was instructed to discontinue her oral contraceptives and started on anticoagulation with warfarin. Imaging studies two months later showed resolution of the thrombus. She was noncompliant with her anticoagulation and presented six months later with similar symptoms. A repeat ultrasound of the abdomen showed thrombosis of two of her hepatic veins, right portal vein and splenic vein. She was immediately restarted on anticoagulation. She was noted to have a mildly elevated ALT in the 50-60 range but her other liver function tests remained normal. She also had a normal CBC except for mild anemia and a normal INR. Further workup revealed a positive JAK2 mutation. She was admitted one month later with worsening abdominal pain, new large ascites and occlusion of all three hepatic veins despite anticoagulation. She was diagnosed with acute BCS. Hepatic venography demonstrated “spider web” collaterals of the right and left hepatic veins consistent with BCS. She underwent a large volume paracentesis and successful TIPS placement with decrease in her hepatic venous pressure gradient from 27 to 5 mm Hg. Liver biopsy showed evidence of moderate to severe sinusoidal dilation in zone 3 consistent with BCS. She recovered well with resolution of her ascites and abdominal pain. She was discharged home with the anticipation of lifelong anticoagulation. Patients with newly diagnosed BCS should be routinely screened for JAK2 mutations. Unlike the majority of patients with MPD, this subgroup of patients have normal hemoglobin levels, platelet counts and erythropoietin levels making diagnosis difficult with laboratory values alone. These patients present with severe hepatic outflow obstruction which portends a worse clinical outcome, and thus should be aggressively intervened upon at time of presentation to ensure long-term survival.

A Rare Case of Inferior Vena Cava Sarcoma Presenting as Acute Fulminant Budd-Chiari Syndrome

A Rare Case of Inferior Vena Cava Sarcoma Presenting as Acute Fulminant Budd-Chiari Syndrome

Portosystemische Shuntchirurgie zwischen TIPSS und Lebertransplantation

Portosystemic shunt surgery in addition to transjugular intrahepatic portosystemic shunt (TIPS) insertion must still be regarded as a current treatment option for portomesenteric decompression in patients with pharmacological and endoscopic treatment failure, where liver transplantation is not imminent. This applies to secondary prophylaxis of rebleeding from varices in patients with well preserved liver function, e.g. liver cirrhosis CHILD A or extrahepatic portal vein thrombosis. Even if emergency endoscopy represents the treatment of choice in the acute bleeding situation, latest data from San Diego on emergency portacaval shunt surgery are encouraging. Likewise, portacaval shunt procedures can be an attractive alternative to TIPS or liver transplantation for acute Budd-Chiari syndrome or veno-occlusive disease.This article is an update on the systematics and methodology of portacaval shunt surgery, emphasizing the significance of this treatment option based on latest studies.

Thrombocytopenia and acute Budd-Chiari syndrome after liver transplantation: think about heparin-induced thrombocytopenia

Thrombocytopenia and acute Budd-Chiari syndrome after liver transplantation: think about heparin-induced thrombocytopenia

Haemodynamic response to abdominal decompression in acute Budd–Chiari syndrome

Intra-abdominal hypertension (IAH) and abdominal compartment syndrome commonly occur in patients with liver disease. We compared haemodynamic variables pre- and post-abdominal decompression in patients with acute Budd-Chiari syndrome (BCS) and patients with chronic liver disease (CLD), ascites and IAH. Patients with IAH admitted to the Liver ICU, King's College Hospital were studied. Transpulmonary thermodilution cardiac output (CO) monitoring was performed with the PiCCO(®) system. Ten patients with decompensated BCS (median age 39 years, 20-52) and eight patients with CLD (59 years, 33-65) and tense ascites requiring paracentesis were studied. Intra-abdominal pressure (IAP) was raised in both groups pre-intervention (BSC 23 mmHg, 17-40; CLD 26, 20-40). Intrathoracic blood volume (ITBVI) was persistently low in the BCS group (632 ml/m(2) , 453-924) despite volume resuscitation. Post-intervention, reduction in IAP was noted in both groups (BCS P<0.001, CLD P<0.0001). The ITBVI increased (P=0.001) in the BCS group only. An increase in cardiac index (CI) and stroke volume index (SVI) was noted in both groups (BCS: CI P=0.003, SVI: P=0.007; CLD: CI P=0.005, SVI P=0.02). The central venous pressure did not change in either group and did not correlate with markers of flow (CI, SVI) or IAP. Both groups demonstrated an inverse relationship between IAP, CI and SVI. Patients with BCS and IAH have evidence of central hypovolaemia. In addition to raised IAP, hepatic venous obstruction and caudate lobe hypertrophy limit venous return in patients with BCS. Reduction in IAP and re-establishment of caval flow restores preload with improvement in CO.

Intravenous leiomyomatosis presenting as acute Budd-Chiari syndrome

Intravenous leiomyomatosis is a rare condition that has been described as being associated with venous occlusion by direct intravascular tumor extension, typically from a pelvic organ. We report an exceedingly rare case of intravenous leiomyomatosis extending into the inferior vena cava, leading to pulmonary embolism, hepatic venous outflow obstruction, and an acute Budd-Chiari syndrome. This is the second reported patient with intravenous leiomyomatosis with Budd-Chiari syndrome and, to our knowledge, the first reported patient who survived with surgery. Correlative images, illustrating computed tomography and magnetic resonance imaging findings characteristic of intravenous leiomyomatosis with secondary Budd-Chiari syndrome, are presented and discussed.

Salvage living donor liver transplantation after percutaneous transluminal angioplasty for recurrent Budd-Chiari syndrome: a case report

IntroductionBudd-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.Case presentationA 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.ConclusionIn 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.

A Simple and New Device to Avoid Hepatic Venous Outflow Obstruction in Adult Liver Transplantation

Hepatic venous drainage in liver transplantation may be reduced to the level of caval anastomosis producing an obstruction degree and leading to serious vascular complication such as the acute Budd-Chiari syndrome, which may result in organ loss. Outflow obstruction may be caused by lack of technique in caval anastomosis or by allograft malposition as a consequence of anatomical graft and recipient conditions. Fixation of the round ligament, placement of bowel loops and use of tissue expanders have been described to stabilize graft position during liver transplantation with related procedure complications. We report our experience of a simple homemade device using a surgical glove expander that allowed us to successfully avoid outflow obstruction in all of nine treated patients. No device related complications occurred. In malposed liver allografts, we strongly suggest the use of this simple and safe device to avoid hepatic venous outflow obstruction on condition that the device is early removed within 48 hours.

Acute Budd-Chiari Syndrome

1Division of Gastroenterology; 2Department of Laboratory Medicine; 3Department of Surgery, University of British Columbia, Vancouver, British Columbia Correspondence: Dr Eric M Yoshida, Division of Gastroenterology, Vancouver General Hospital, Diamond Health Care Centre, 5153-2775 Laurel Street, Vancouver, British Columbia V5Z 1M9. Telephone 604-875-5371, fax 604-875-5447, e-mail eric.yoshida@vch.ca Received for publication April 8, 2011. Accepted April 11, 2011 CASE PRESENTATION A 23-year-old woman presented to hospital with a two-week history of abdominal pain, nausea and increasing abdominal girth. Her medical history was significant only for migraines and oral contraceptive use. Laboratory values showed an alanine aminotransferase level of 155 U/L, an aspartate aminotransferase level of 85 U/L, an alkaline phosphatase level of 165 U/L, total bilirubin of 12 μmol/L (0.71 mg/dL), an albumin concentration of 27 g/L (2.7 g/dL) and an international normalized ratio of 1.2. An abdominal Doppler ultrasound and a computed tomography angiogram (Figure 1) demonstrated a nonocclusive, intrahepatic inferior vena cava (IVC) clot measuring 3.4 cm × 1.4 cm, as well as marked ascites. The patient subsequently began to develop congestive hepatopathy and was scheduled for emergency placement of a portocaval shunt to protect her liver function. Caudate lobectomy was required to expose the IVC for a polytetrafluoroethylene graft anastomosis. Pathology of the caudate lobe and random biopsies from the right and left lobes showed focal hepatic vein and central venous organizing thrombosis with concomitant sinusoidal dilation (Figure 2). The patient had a relatively uncomplicated postoperative recovery. Portocaval shuntogram and pressure measurements showed a widely patent shunt with no significant pressure gradient. Thrombophilia workup was negative for any identifiable intrinsic clotting disorder. DISCUSSION Budd-Chiari syndrome is caused by thrombosis of the hepatic veins and/or the thrombosis of the intrahepatic or suprahepatic IVC. While an underlying etiology such as malignancy, rheumatological disorder, myeloproliferative disease, inheritable coagulopathy, infection or hyperestrogen state can be identified in many cases, the etiology remains idiopathic in 16% to 35% of cases (1-3). Clinical presentations vary – ranging from acute or fulminant liver failure, to subacute (signs or symptoms for less than six months and no evidence of liver cirrhosis) to chronic (signs or symptoms for more than six months with evidence of portal hypertension and cirrhosis). The goals of therapy are to prevent the propagation of the clot, restore patency of thrombosed veins, decompress liver congestion and manage complications such as ascites and portal hypertension. For acute thrombosis, thrombolytic therapy or radiological procedures (eg, angioplasty, stenting or transjugular intrahepatic portosystemic shunts) can be considered; however, surgical decompression remains the mainstay of treatment. Surgical shunts commonly connect the portal venous system to the IVC, creating a low-pressure route for hepatic arterial blood to drain from the liver. In patients who underwent shunt surgery before development of cirrhosis and whose shunts remain patent, five-year survival rates of as high as 89% have been reported (4-6). Chronic Budd-Chiari syndrome is managed with anticoagulation and treatment of the complications of liver failure. Rarely, liver transplantation may be necessary. IMAGE OF THE MONTH

Anticoagulation and Budd-Chiari syndrome

To the Editor, We read with interest the article by Shukla and Bhatia [1] in a recent issue of the Journal. The authors found that 6 of their patients could not undergo the standard radiological procedures because of technical reasons; reasons for this technical failure were however, not provided. In our experience of adult patients, the TIPSS procedure is usually possible. Of 58 patients who underwent TIPSS, we faced technical difficulty in only one patient who had acute Budd-Chiari syndrome; the massive hepatomegaly caused an increase in distance between the IVC and narrowed portal vein (unpublished data). Technical difficulties usually arise in infants when the vein size is too small for stenting. In 14 children with Budd-Chiari syndrome, we could perform radiological intervention successfully in 11; the procedure failed due to small vessel size in 3 patients [2]. The authors found that those with more severe disease did not do well in the long term with anticoagulation alone. These are precisely the patients who benefit with radiological intervention [3]. We have successfully done TIPSS in a fouryear-old child with Budd-Chiari syndrome who presented with fulminant liver failure [4]. The use of anti-coagulation alone in the treatment of Budd Chiari syndrome is a compromise rather than an alternative treatment.