Hepatic vein thrombosis (Budd-Chiari syndrome)

Hepatic vein thrombosis is caused by one or several thrombogenic conditions, of which myeloproliferative disorders are the most frequent. Thrombosis and its fibrous sequelae can affect the veins diffusely or locally. Severity is determined by the extent and velocity of the thrombotic process. Development of venous collateral vessels is an important compensatory mechanism. Some patients can be totally free of symptoms. Major complications are intractable ascites, liver insufficiency, and gastrointestinal bleeding. Diagnosis can be made via ultrasonography or magnetic resonance imaging in a majority of cases. The main prognostic factors are age, Child-Pugh score, and response of ascites to diuretics. Medical therapy includes control of causal factors, anticoagulation, and nonspecific treatment of complications. Procedures aiming to restore outflow of hepatic blood are indicated in patients with uncontrolled manifestations. Percutaneous angioplasty, followed by portosystemic shunt (including a transjugular intrahepatic portosystemic stent shunt) and eventually liver transplantation can be proposed in a graded manner. The current 10-year survival rate is about 75%.     

Metastatic angiosarcoma of the scalp causing Kasabach-Merritt syndrome

We report a case of thrombocytopenia caused by Kasabach-Merritt syndrome (KMS) in a 69-year-old man with a metastatic angiosarcoma of the scalp. A review of the literature revealed that only 4 cases of KMS associated with angiosarcoma have been previously reported. All these cases occurred with a solitary large lesion. This is the first case of thrombocytopenia caused by KMS associated with small metastatic lesions of angiosarcoma that developed after the primary lesion has been completely removed.     

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.     

Surgical repair for entire inferior vena cava occlusion in Budd-Chiari syndrome

Successful repair of thrombotic occlusion of the entire inferior vena cava, including the iliac and hepatic veins, was performed on a 51-year-old man with Budd-Chiari syndrome associated with severe liver dysfunction.     

球囊扩张合并支架置入治疗肝静脉阻塞型布-加综合征疗效观察

目的 观察球囊扩张合并支架置入治疗单纯肝静脉阻塞型布-加综合征(Budd-Chiari-syndrome,BCS)的疗效.方法 2001年7月至2006年9月采用多普勒超声引导下经皮经肝穿刺肝静脉并肝静脉造影术、经颈静脉行肝静脉成形术、肝静脉球囊扩张及支架置入术治疗肝静脉阻塞型BCS患者43例.结果 术前43例肝静脉平均压力为32.5 cm H2O(1 cm H2O=0.098 kPa),支架置入后立刻下降为20 cm H2O(t=11.5,P＜0.01),再次肝静脉造影显示肝静脉支架通畅,肝内肝静脉侧支消失.其中38例症状立刻消失,5例部分好转.术后43例随访期间(1～62个月,平均31.5个月),1例出院后1个月因上消化道大出血死亡,其余42例症状体征均无再发,术后多普勒超声检查肝静脉均通畅.治疗成功率为100%,所有患者均未出现严重并发症.结论 多普勒超声引导下经皮经肝穿刺肝静脉和经颈静脉途径开通肝静脉,球囊扩张成形及支架置入治疗单纯肝静脉阻塞型BCS安全、有效.     

Brown tumor in secondary hyperparathyroidism causing acute paraplegia

The first known case of a "brown tumor" associated with secondary hyperparathyroidism causing paraplegia is described. A 69-year-old white woman with chronic renal failure due to hypertension was admitted for back pain, and while she was under observation, paraplegia developed. A complete block was demonstrated by myelography. Computed tomography confirmed a mass at the level of obstruction, and results of biopsy were consistent with "brown tumor." Neurologic symptoms were markedly improved with high-dose corticosteroids and a debulking procedure. This entity is important to recognize because prompt treatment of the hyperparathyroidism or decompression of the tumor mass by surgical means or corticosteroid administration can provide marked improvement in symptoms.     

del11(p11-13) with overexpression of Wilms' tumor gene during leukemic transformation of myelodysplastic syndrome

We report a case of leukemic transformation from myelodysplastic syndrome (MDS) with a sole chromosome abnormality of del11(p11-13). The patient had been diagnosed as having MDS (refractory anemia with excess of blast cells, RAEB) in May 1998. At that time, cytogenetic analysis of bone marrow cells showed a normal karyotype. The patient received sequential chemotherapy with low-dose cytosine arabinoside (AraC) and macrophage colony-stimulating factor (M-CSF). Complete remission was obtained with this treatment, but the disease gradually progressed after June 1999. Cytogenetical analysis showed del11(p11-13) in 6 of 40 cells analyzed at that time, and the disease had evoluted to overt leukemia in December 1999 with a gradual increase in the abnormal clone. Furthermore, mRNA of the WT1 gene located at chromosome 11p13 was overexpressed during leukemic transformation, whereas it was not detected at the time of the initial diagnosis of MDS (RAEB) in May 1998. It was thought that this chromosome deletion and overexpression of WT1 resulted in the leukemic transformation in this patient. This is the first case report of del11(p11-13) being considered to be the primary cause of leukemic transformation from MDS.     

Recovery from hepatic vein thrombosis (Budd-Chiari syndrome) complicating ulcerative colitis

Summary A 22-year-old female with active ulcerative colitis developed massive ascites, hypoalbuminemia, and hepatomegaly compatible with thrombosis of the hepatic veins. The diagnosis of Budd-Chiari syndrome was confirmed by ultrasonography, computed tomography, and by liver biopsy. A search of the literature disclosed only three previous reports of Budd-Chiari syndrome occurring in patients with ulcerative colitis. All patients have been young females with active colitis and no other known risk factor for the development of hepatic vein thrombosis. Our patient, unlike the previously reported patients who died, recovered sufficiently to be discharged from the hospital.     

Hepatic-pericardio-phrenic collateral (natural shunt) in a patient with Budd-Chiari syndrome

Hepatic venous outflow tract obstruction, Budd-Chiari syndrome (BCS), leads to portal hypertension and to the development of collaterals that bypass the obstruction. Described here is a BCS patient with an unusually large transdiaphragmatic collateral between the left hepatic and left innominate veins, which decompressed the oesophageal varices. This has not been reported earlier in the literature.     

Leiomyosarcoma of inferior vein cava presenting as acute Budd-Chiari syndrome.

We report a 49-year-old lady who presented with acute Budd-Chiari syndrome. Spiral CT scan showed inferior vein cava (IVC) tumor and ischemia of the right liver secondary to hepatic vein blockage. These were confirmed by MRI scan and IVC gram, at which time tissue diagnosis was obtained. At surgery, the tumor was seen to originate from the infrahepatic IVC and extended to the level of the diaphragm, blocking the hepatic vein outflow. The tumor was excised completely. Histology confirmed it to be leiomyosarcoma of the IVC. The patient is well, without recurrence of symptoms or tumor, 10 months later.     

TIPS for acute and chronic Budd-Chiari syndrome: a single-centre experience

BACKGROUND/AIMS: Transjugular intrahepatic portosystemic shunt (TIPS) is a technically challenging but feasible treatment for Budd-Chiari syndrome (BCS). However, information about the outcome, particularly in patients with liver failure, is scarce. We report our experience of TIPS for BCS. METHODS: Fifteen patients with BCS underwent TIPS. Eight had hepatic failure and seven underwent TIPS for BCS uncontrolled by medical treatment. RESULTS: Fourteen out of 15 had successful TIPS placement. Out of the eight hepatic failure patients, four died soon after TIPS: one liver rupture, one portal vein rupture, one liver failure and one pulmonary oedema. Another patient had a significant intrahepatic haematoma, which resolved with conservative management. TIPS was successfully placed in all of the seven patients with chronic BCS, in whom there was an average follow-up of 20 months. Ascites resolved and liver function improved in all. One patient died after 18 months from the original hepatic metastatic disease. Four patients have had evidence of TIPS dysfunction requiring three balloon dilatations and one restenting. No patient has required liver transplantation. CONCLUSIONS: TIPS should be the first line treatment for BCS uncontrolled by medical therapy. However, mortality in BCS with hepatic failure is high and liver transplantation could be a better option.     

经皮经肝肝静脉开通术治疗布加氏综合征

目的：肝静脉阻塞是目前临床治疗的难点，本研究目的是探讨新的安全有效的治疗方法。方法：肝静脉型和混合型布加氏综合征８例，超声引导下，经皮经肝肝静脉穿刺、球囊扩张及内架置入。使闭塞的肝静脉再通．整个操作均在超声监视下进行。结果：术后８例患者原闭塞处肝静脉内径平均达０．８１ｃｍ，平均肝静脉压由３．６７ｋＰａ降至１．３２ｋＰａ（Ｐ＜０．０１）。肝静脉均恢复正向回心血流。短期内病人腹胀、腹水消失，肝脾肿大缓解。观察２．６－１６个月。病情无反复。并发腹腔出血１例，无其它严重并发症。结论：超声引导下经皮肝静脉开通术，操作简便直观，疗效肯定，是肝静脉阻塞安全、合理的非手术治疗方法。