Chiari's disease, due to non-inflammatory thrombosis of the hepatic veins without involvement of the main trunks.
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Hepatic Veins↗
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Hepatic transplantation with perioperative and long term anticoagulation as treatment for Budd-Chiari syndrome.
Conventional medical and surgical management of Budd-Chiari syndrome is often unsuccessful. In this communication, we report the results of 19 hepatic transplants done for 17 patients suffering from Budd-Chiari syndrome. The first patient who had a transplant did not receive anticoagulant therapy during the postoperative period, and recurrent thrombosis of the hepatic veins in the newly transplanted liver rapidly developed. Sixteen patients who subsequently underwent transplantation were managed using a philosophy of early postoperative anticoagulant treatment when feasible. Using this approach, the cumulative proportion of patients surviving at one and three years was 88 per cent, and no recurrent hepatic vein thrombosis occurred during a mean follow-up period of 28.2 months. Forty-four per cent of the patients who were deliberately given anticoagulant medication experienced a hemorrhagic complication in the postoperative period, but there were no associated deaths. In spite of anticoagulant therapy, 31 per cent of the patients on anticoagulantion medication experienced a thrombotic complication that did not involve the hepatic veins; one early and one late death resulted, and a third patient required urgent retransplantation. Despite the difficulties in patient management, a carefully selected population of patients with hepatic failure secondary to Budd-Chiari syndrome appear to be well served by hepatic transplantation with early postoperative and long term anticoagulant therapy.
Adolescent↗
[Color duplex sonography of liver transplantation. The vascular status].
The present study deals with the value of CDS in liver transplantation. Between September 1988 and October 1991 CDS of hepatic vessels was carried out in 194 liver transplants. Flow direction and flow velocity in the portal vein could be demonstrated in 97%. Measurement of flow velocity indicates the extent of portal hypertension; this can be confirmed by determining flow direction in the splenic and superior mesenteric veins. In 47 of 179 patients venous collaterals were demonstrated and in 15 of 179 patients there was retrograde flow in the splenic vein. In 95% of patients CDS indicated flow direction in the hepatic artery and in 93% in the hepatic veins. Thrombosis of liver vessels (portal and hepatic veins) was demonstrated in 11 out of 13 patients by means of CDS.
Budd-Chiari Syndrome↗
Rapidly progressing Budd-Chiari syndrome complicated by hepatocellular carcinoma.
Budd-Chiari syndrome (BCS) is a disorder caused by occlusion of the hepatic vein or inferior vena cava. The clinical presentation include abdominal pain, hepatomegaly, ascites, leg edema, collateral venous dilatation of the body trunk, and portal hypertension. In addition, BCS can cause hepatocellular carcinoma (HCC) in some patients, although its pathogenesis is not yet completely understood. The average reported time lag from diagnosis of BCS to full-blown HCC ranges from several years to several decades. Hepatic carcinogenesis in patients with BCS perhaps reflects a prolonged and persistent liver injury in that it occurs in the primary inferior vena cava obstruction rather than the primary hepatic vein thrombosis. Among patients with BCS, membranous obstruction of the vena cava (MOVC) usually presents an insidious and chronic illness, whereas primary hepatic vein thrombosis presents an acute or subacute illness. We experienced a case of a patient with BCS, which progressed rapidly that HCC developed only nine months after the diagnosis of BCS. The factors causing this rapid progression are still unclear and remain to be investigated.
Adult↗
Portal vein thrombosis in a 17-year-old female adolescent with essential thrombocytosis.
A 17-year-old girl known to suffer from essential thrombocytosis (ET) was admitted with severe abdominal pain that was due to sonographically verified portal and hepatic vein thrombosis. ET is a well-documented disease and portal vein thrombosis has often been reported in adults but not in children and adolescents suffering from ET. Coincidence of ET, urinary tract infection, oral contraception, and cessation of acetylsalicylic acid therapy might be responsible for the early manifestation of portal and hepatic vein thrombosis in our patient.
Adolescent↗
Portal vein thrombosis following hepatic portoenterostomy.
Life-threatening ascites developed in a 4-month-old infant with biliary atresia 1 month after hepatic portoenterostomy. Although initially ascribed to progressive liver failure, the ascites was subsequently found to be partially caused by portal vein thrombosis. Documented recanalization of the portal venous system was accompanied by resolution of the ascites. Refractory ascites in patients with biliary atresia generally signals end-stage cirrhosis and consideration of liver transplantation. Portal vein patency should be evaluated before referral of such patients for this procedure.
Bile Ducts↗
Split liver transplantation in Europe--1988 to 1993.
The shortage of liver grafts results in the fact that 8% of potential recipients die before receiving a graft. Liver graft division has therefore been proposed to maximize the current available liver graft pool. However, the question of benefit or additional risk for the recipient that this technique might carry remains unanswered. The European Split Liver Registry was opened in March 1993 and reviewed retrospectively the clinical experience obtained at nine European centers regarding the use of split liver transplants, during the five year period from March 1988 to March 1993. From 50 donor livers, 100 grafts were prepared: 2 grafts were discarded and the other 98 were transplanted in 53 children (2 times in 3 children) and 42 adults (2/42 in heterotopic position). Sixty-three grafts were implanted in an urgent recipient (half of whom had acute hepatic failure). Portal vein thrombosis, hepatic artery thrombosis, biliary complications, and retransplantation rates were 4%, 11.5%, 18.7%, and 18.7%, respectively. Most of these complications were unrelated to the technique itself. Actual 6-month graft survivals of elective and urgent orthotopic transplants were 80% and 61.3% in children, and 72.2% and 55.6% in adults; actual 6-month patient survival rates for similar groupings were 88.9% and 61.1%, and 80% and 67.7%, respectively. Similar rates are reported after conventional transplants in Europe. It is concluded that split liver transplantation is an efficient transplant technique that benefits both urgent patients who otherwise could have died before getting a graft in time and elective patients.
Adolescent↗
Outcome of liver transplantation for children with liver disease.
INTRODUCTION: The advent of liver transplantation has revolutionised the outcome of children with both acute liver failure and chronic end-stage liver disease. The aim of this study was to review the outcome of all paediatric liver transplants performed since the National Liver Transplant Programme began in 1990. METHODS: A retrospective review of all paediatric liver transplants from 1990 to December 2004 was performed. RESULTS: 46 liver transplants were performed in 43 children, of whom 23 (53.3 percent) were female. Median age at transplant was 21 months (range 11 months to 14 years). The most common indication for liver transplant was biliary atresia (71.7 percent). Living-related transplants accounted for 63 percent (29). Re-transplant rate was 6.5 percent with allograft loss as a result of hepatic artery thrombosis (two) and hepatic vein thrombosis (one). Tacrolimus was the primary immunosuppressive agent used in 89 percent of patients, with a 19.6 percent incidence of acute allograft rejection within the first six months. There were nine deaths. They were related to portal vein thrombosis (three), chronic rejection (one), sepsis (two), post-transplant lymphoproliferative disease (two) and primary graft non-function (one). Overall actuarial one- and five-year survival rate was 85.7 percent and 81.8 percent, respectively. CONCLUSION: Liver transplantation is an established form of intervention for end-stage liver disease and a variety of liver-related metabolic disease. Our results are comparable to those of well-established liver transplant centres.
Adolescent↗
Catheter-directed thrombolysis and thrombectomy for the Budd-Chiari syndrome in paroxysmal nocturnal hemoglobinuria in three patients.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic stem cell disorder characterized by hemolytic anemia, hemoglobinuria, bone marrow failure, and hypercoagulability. Thrombosis is the leading cause of mortality and occurs in one-half of PNH patients, with the hepatic veins being the most common site. Patients with hepatic vein thrombosis (Budd-Chiari syndrome) can present with abdominal pain, hepatomegaly, jaundice, and ascites. Prognosis is poor for these patients; death may occur from liver failure, vessel rupture, intestinal ischemia, infarction, necrosis, or sepsis. The authors report three consecutive cases of successful treatment with catheter-directed thrombolysis and thrombectomy directly in the hepatic veins in patients with PNH who developed acute hepatic vein thrombosis. This treatment represents a potential bridge toward more curative therapies such as allogeneic bone marrow transplant.
Adolescent↗
Noncavernomatous portal vein thrombosis in hepatic cirrhosis: treatment with transjugular intrahepatic portosystemic shunt and local thrombolysis.
PURPOSE: To evaluate the use of the transjugular intrahepatic portosystemic shunt (TIPS) and local, low-dose thrombolysis in the treatment of complete, noncavernomatous portal vein occlusion. MATERIALS AND METHODS: TIPS implantation and portal vein recanalization was attempted in seven patients with noncavernomatous portal vein obstruction and recurrent variceal bleeding. TIPS placement was followed by thrombolytic therapy to restore portal venous blood flow. Hemodynamic effects and clinical characteristics after the procedure and during follow-up were assessed. RESULTS: The implantation of TIPS and the recanalization of the portal vein trunk were successful in all patients. The treatment reduced the portal venous pressure gradient and restored portal blood flow. No bleeding complications were observed. CONCLUSION: TIPS placement and recanalization of the main portal vein is a safe and effective treatment option for patients with liver cirrhosis and noncavernomatous portal vein occlusion.
Combined Modality Therapy↗
[Intestinal obstruction caused by non-absorbable tablets and Budd-Chiari syndrome in a patient with Crohn's disease].
Crohn's disease is a chronic inflammatory granulomatous disorder affecting any part of the gastrointestinal tract, particularly the terminal ileum and the colon. Familiar complications are strictures, fistulae, perforation, haemorrhages and malabsorption due to multiple resections. A patient with two rare complications of Crohn's disease is described. A 16-year-old female with ileocaecal Crohn's disease presented with anaemia and ileus. This ileus was caused by some 40 tablets of ferrosulphate with a non-soluble matrix, in the presence of an existing stenosis of the ileum due to Crohn's disease. An ileocaecal resection was performed. During an exacerbation of Crohn's disease she developed hepatic vein thrombosis with a Budd-Chiari syndrome (upper abdominal pain, hepatomegaly and ascites). Prescription of tablets with a non-soluble matrix is contraindicated in patients with a partial stenosis of the intestine. Patients with active Crohn's disease are predisposed to thromboembolic complications. Hepatic vein thrombosis in our patient may have been the result of hypercoagulability during the exacerbation of her disease.
Budd-Chiari Syndrome↗
Budd-Chiari syndrome and portal vein thrombosis associated with myeloproliferative disorders: diagnosis and management.
The improvement observed in the prognosis of Budd-Chiari syndrome (BCS) since 1985 may be related to a better identification of underlying prothrombotic states. In a recent investigation of the currently identified prothrombotic disorders, one or several prothrombotic disorders were evidenced in 72% of patients with portal vein thrombosis (PVT) and 87% of patients with hepatic vein thrombosis. According to the different criteria used for their diagnosis, myeloproliferative disorders (MPDs) were reported in 25 to 65% of patients with splanchnic vein thrombosis (STV). In a single-center retrospective study of 128 patients with STV, clusters of abnormal megakaryocytes in bone marrow biopsy combined with endogenous erythrocyte colony formation were used as reference standards for a diagnosis of MPD. These two criteria allowed the diagnosis of MPDs at risk of aggravation in 25% (26 of 103) of patients with STV (41% in BCS; 23% in PVT). After a mean follow-up of 6.09 +/- 6.6 years, hematologic progression was confirmed in 23% of patients with the two criteria both positive, whereas neither progression nor features suggestive of MPD were observed in patients with the same concordant negative criteria. The combination of marked splenomegaly and platelet count > 200 x 10 (9)/L that was never observed in patients without MPD, constitutes a simple index for the suspicion of an underlying MPD, but the absence of an obvious splenic enlargement and normal or decreased platelet numbers may even be observed in patients with MPD-associated STV. In the absence of reliable indicators of the exact expansion of the myeloproliferative clone, the therapeutic management of STV-associated MPD is difficult to standardize. Owing to the suspected incidence on the prognosis of BCS of an associated MPD, the present watchful-waiting attitude proposed in MPD without obvious hematologic expression may be challenged.
Budd-Chiari Syndrome↗
Portal vein thrombosis complicating neonatal hepatic abscess.
Hepatic abscess in a neonate is a rare but serious disorder. Diagnosis of hepatic abscess requires a high index of suspicion in any septic neonate. CT scan and ultrasound of liver are the most sensitive diagnostic tests in detection of hepatic abscess. Portal vein thrombosis and portal cavernoma formation is hitherto unreported complication of neonatal hepatic abscess in English literature. Present case report highlights the difficulty in diagnosis of neonatal hepatic abscess and describes the development of portal vein thrombosis and cavernoma during its treatment.
Female↗
Budd-Chiari syndrome complicating restorative proctocolectomy for ulcerative colitis: report of a case.
PURPOSE: This is a case of hepatic vein thrombosis presenting in a delayed fashion after proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis. Search for a causative thrombotic condition resulted in the diagnosis of polycythemia vera, a myeloproliferative disorder associated with hypercoagulability. The polycythemia was masked by an iron deficiency associated with the ulcerative colitis. METHODS: The history, physical, diagnostic modalities, and treatment for this patient are described, and the literature of Budd-Chiari syndrome associated with ulcerative colitis is reviewed. RESULTS: Six cases of Budd-Chiari syndrome in the setting of ulcerative colitis are reported in the literature from 1945 to 1997. CONCLUSIONS: Hepatic vein thrombosis is a rare complication of ulcerative colitis. The diagnosis of Budd-Chiari syndrome demands a thorough search for a hematologic condition predisposing to thrombosis. Our patient had a myeloproliferative disorder, polycythemia vera, that is associated with a hypercoagulable state. The disorder was masked by an iron deficiency associated with the ulcerative colitis. Recognition of the entity will permit successful treatment.
Adult↗
Thrombosis of hepatic veins and inferior vena cava. Relief by thoracic transposition of spleen.
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Adult↗
Autonomic neuropathy in extra-hepatic portal vein thrombosis: evidence for impaired autonomic reflex arc.
BACKGROUND/AIMS: Autonomic dysfunction is common in cirrhosis, and may be associated with increased mortality and hyperdynamic circulatory changes. Our aim was to investigate whether autonomic disturbances occur in extrahepatic portal vein thrombosis and their correlation with hemodynamic abnormalities. PATIENTS AND METHODS: Heart rate variation in response to standing, deep breathing, and Valsalva maneuver, and blood pressure response to sustained handgrip and to standing, were studied in 16 subjects with portal vein thrombosis (10 males, 30.8+/-2.8 years: mean+/-SE), 12 with cirrhosis (7 males, 52+/-2.3 years), and 10 healthy controls (7 males, 30.8+/-3.0 years). Supine resting, and 10- and 30-min standing epinephrine and norepinephrine levels were measured and results correlated with cardiac output. RESULTS: Autonomic dysfunction occurred in 62% of portal vein thrombosis and 75% of cirrhosis subjects, but in no controls (p<0.02). Similarly, postural hypotension occurred in portal vein thrombosis (-10.25+/-0.65 mmHg, p=0.003) and cirrhosis (-7.42+/-0.82 mmHg, p=0.007) but not in controls. All groups had similar baseline epinephrine and norepinephrine concentrations. Epinephrine increased significantly in controls (45%, p<0.01 and 49%, p<0.02) after 10 of 30 min standing but not in the portal vein thrombosis or the cirrhotic group, and norepinephrine increased after 10 and 30 min standing in cirrhotics (128%, p<0.004 and 130%, p<0.008) and controls (129%, p<0.002 and 116%, p<0.004), but not portal vein thrombosis (34.5% and 39%, NS vs baseline). Portal vein thrombosis and cirrhosis groups had increased cardiac output (4441+/-509 and 3262+/-292) vs controls (1763+/-212 ml/min/m2, p<0.002), but there was no correlation with autonomic neuropathy or with catecholamine levels. CONCLUSIONS: Autonomic dysfunction and impaired catecholamine response to orthostatic stress occur commonly in portal vein thrombosis and suggest an impairment of the autonomic reflex arc, but changes do not correlate with hemodynamic abnormalities.
Adult↗
Hepatic portal vein thrombosis diagnosed by transhepatic portography.
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Budd-Chiari Syndrome↗
Extra hepatic portal vein thrombosis in a child associated with lupus anticoagulant.
The presence of lupus anticoagulant has been implicated in venous as well as arterial thrombosis. We report here a 10-year-old boy who presented to us with hematemesis, malaena and splenomegaly. An ultrasound showed a recanalized portal vein with collaterals suggestive of portal vein thrombosis. He had grade IV esophageal varices. The liver function tests were normal. Investigations for prothrombotic factors showed that tests for PNH and for APC resistance were negative. Levels of anti-thrombin II and protein C were normal. There was a prolonged activated partial thromboplastin time with a normal prothrombin time. Presence of lupus anticoagulant was confirmed with dilute Russell viper venom time and platelet neutralization test. Repeat tests after 10 weeks showed persistence of the lupus anticoagulant. ELISA test for anti-phospholipid antibody was negative. The association of lupus anticoagulant with portal vein thrombosis in the pediatric age group is very rare.
Adolescent↗