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PubMed2005

Serious intestinal bleeding from vascular ectasia secondary to portal thrombosis after living-related liver transplantation in a child.

Serious intestinal bleeding from vascular ectasia secondary to extrahepatic portal thrombosis is much less frequent than variceal bleeding, and its treatment is not clearly defined. We describe a 4-year-old girl with repeated intestinal bleeding from vascular ectasia, without any varix, with late extrahepatic portal vein thrombosis (PVT) and late hepatic artery thrombosis (HAT) after living-related liver transplantation. The bleeding stopped after simple splenectomy. She has presented neither bleeding nor any serious complications related to splenectomy for 1 year to date. We think uncontrollable hemorrhage from gastrointestinal vascular ectasia secondary to extrahepatic portal thrombosis in a pediatric patient can and should be treated by simple splenectomy, because patients with this complication usually have a normally functioning liver. However, it is not clear whether this procedure is effective for variceal bleeding.

Child, Preschool↗
PubMed1993

Portal vein thrombosis in a child with homozygous sickle-cell disease.

A 13-year-old boy with homozygous sickle-cell (SS) disease died suddenly at home following a short history of abdominal pain. Autopsy revealed venous thrombosis of the hepatic, portal, superior mesenteric and splenic veins. Venous thrombosis is rare in SS disease and thrombosis of mesenteric vessels is most frequently seen in chronic myeloproliferative disorders. Its occurrence in SS disease raises the possibility of a common pathogenesis and adds another pathology to the causes of the abdominal painful crisis.

Abdominal Pain↗
PubMed2003

Safety and necessity of including the middle hepatic vein in the right lobe graft in adult-to-adult live donor liver transplantation.

OBJECTIVE: To evaluate the safety of donors who have donated the middle hepatic vein in right lobe live donor liver transplantation (LDLT) and to determine whether such inclusion is necessary for optimum graft function. SUMMARY BACKGROUND DATA: The necessity to include the middle hepatic vein in a right lobe graft in adult-to-adult LDLT is controversial. Inclusion of the middle hepatic vein in the graft provides uniform hepatic venous drainage but may lead to congestion of segment IV in the donor. METHODS: From 1996 to 2002, 93 right-lobe LDLTs were performed. All right-lobe grafts except 1 contained the middle hepatic vein. In the donor operation, attention was paid to preserve the segment IV hepatic artery and to avoid prolonged rotation of the right lobe. The middle hepatic vein was transected proximal to a major segment IVb hepatic vein whereas possible to preserve the venous drainage in the liver remnant. RESULTS: There was no donor death. Two donors had intraoperative complications (accidental left hepatic vein occlusion and portal vein thrombosis) and were well after immediate rectification. Twenty-four donors (26%) had postoperative complications, mostly minor wound infection. The postoperative international normalized ratio on day 1 was better in the donors with preservation of segment IVb hepatic vein than those without the preservation, but, in all donors, the liver function was largely normal by postoperative day 7. The first recipient had severe graft congestion as the middle hepatic vein was not reconstructed before reperfusion. In 7 other recipients, the middle hepatic vein was found occluded intraoperatively owing to technical errors. The postoperative hepatic and renal function of the recipients with an occluded or absent middle hepatic vein was worse than those with a patent middle hepatic vein. The hospital mortality rate was also higher in those with an occluded middle hepatic vein (3/9 vs. 5/84, P = 0.028). CONCLUSIONS: Inclusion of the middle hepatic vein in right-lobe LDLT is safe and is essential for optimum graft function and patient survival.

Adult↗
PubMed2006

Retinal vein thrombosis associated with pegylated-interferon and ribavirin combination therapy for chronic hepatitis C.

An estimated 300 million people worldwide suffer from chronic hepatitis C with a prevalence of 0.8%-1.0% of the general population in Canada. An increasing pool of evidence exists supporting the use of pegylated- interferon (pegIFN) and ribavirin combination therapy for hepatitis C. We report a 49-year old male of North American aboriginal descent with chronic hepatitis C (genotype 2b). Biopsy confirmed that he had cirrhosis with a 2-wk history of left eye pain and decreased visual acuity. He developed retinal vein thrombosis after 16 of 24 wk of pegIFN-alpha 2a and ribavirin combination therapy. He was urgently referred to a retinal specialist and diagnosed with non-ischemic central retinal vein occlusion of the left eye. PegIFN and ribavirin combination therapy was discontinued and HCV RNA was undetectable after 16 wk of treatment. Hematologic investigations revealed that the patient was a factor V Leiden heterozygote with mildly decreased protein C activity. Our patient had a number of hypercoagulable risk factors, including factor V Leiden heterozygosity, cirrhosis, and hepatitis C that alone would have most likely remained clinically silent. We speculate that in the setting of pegIFN treatment, these risk factors may coalesce and cause the retinal vein thrombosis.

Antiviral Agents↗
PubMed1992

Duplex assessment of the portal venous system.

The clinical applications of duplex ultrasound and color Doppler imaging in the evaluation of the portal venous system and hepatic vasculature are quite diverse and widespread. These include identification of portal vein thrombosis, portal venous hypertension, and hepatic veno-occlusive disease as well as a role in the preoperative and postoperative treatment of patients who are candidates for portosystemic shunts or for liver allografts. As technological improvements continue, the current applications will almost certainly be refined, with resultant improvements in sensitivity, specificities, and accuracy rates.

Aneurysm↗
PubMed2002

Complexities in ETS-domain transcription factor function and regulation: lessons from the TCF (ternary complex factor) subfamily. The Colworth Medal Lecture.

The ETS-domain transcription factor family can be divided into a series of subfamilies. Elk-1 represents the founding member of the ternary complex factor (TCF) subfamily. By focusing on the TCF subfamily, we can demonstrate the complexities that exist in the function and regulation of ETS-domain transcription factors. This article focuses on Elk-1 in detail and summarizes the functions of other TCFs. The key themes covered include the domain structure of the TCFs, the mechanisms of complex formation with serum response factor, regulation of TCFs by mitogen-activated protein kinase cascades, and transcriptional regulatory properties of the TCFs. Finally, the emerging role of the TCFs in vivo is discussed. A picture is developing indicating that, while these proteins exhibit significant sequence and functional conservation, key differences in their structure and regulation are being identified which may relate to unique functions of these proteins in vivo.

Amino Acid Sequence↗
PubMed2006

Acute partial Budd-Chiari syndrome and portal vein thrombosis in cytomegalovirus primary infection: a case report.

BACKGROUND: Splanchnic vein thrombosis may complicate inherited thrombotic disorders. Acute cytomegalovirus infection is a rare cause of acquired venous thrombosis in the portal or mesenteric territory, but has never been described extending into a main hepatic vein. CASE PRESENTATION: A 36-year-old immunocompetent woman presented with acute primary cytomegalovirus infection in association with extensive thrombosis in the portal and splenic vein. In addition, a fresh thrombus was evident in the right hepatic vein. A thorough evaluation for a hypercoagulable state was negative. The clinical course, biological evolution, radiological and histological findings were consistent with cytomegalovirus hepatitis complicated by a partial acute Budd-Chiari syndrome and portal thrombosis. Therapeutic anticoagulation was associated with a slow clinical improvement and partial vascular recanalization. CONCLUSION: We described in details a new association between cytomegalovirus infection and acute venous thrombosis both in the portal vein and in the right hepatic vein, realizing a partial Budd-Chiari syndrome. One should be aware that this rare thrombotic event may be complicated by partial venous outflow block.

Acute Disease↗
PubMed2004

Budd-Chiari syndrome and portal vein thrombosis due to right atrial myxoma.

Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction. Atrial myxomas account for 35% to 50% of primary cardiac tumors and are usually located in the left atrium. We describe a patient who presented with hepatic inferior vena cava and portal vein thrombosis due to a large myxoma of the right atrium. After the successful removal of the tumor, ascites resolved completely. We conclude that right atrial myxoma is a rare but potentially curable cause of Budd-Chiari syndrome.

Anticoagulants↗
PubMed1984

Veno-occlusive liver disease after dacarbazine therapy (DTIC) for melanoma.

A case of veno-occlusive disease of the liver with fatal outcome after dacarbazine (DTIC) therapy for melanoma is reported. There was a fulminant clinical course from start of symptoms until death. At autopsy the liver was enlarged and firm with signs of venous congestion. Small- and medium-sized hepatic veins were blocked by thrombosis. Eosinophilic infiltrations were found around the vessels. Published cases from the literature are reviewed and pertinent features discussed.

Budd-Chiari Syndrome↗
PubMed2002

Hepatic arterial infusion chemotherapy for advanced hepatocellular carcinoma with portal vein tumor thrombosis: analysis of 48 cases.

BACKGROUND: The prognosis of patients with hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) is extremely poor. The aim of this study was to elucidate the efficacy of hepatic arterial infusion chemotherapy (HAIC) for patients with advanced HCCs. METHODS: Forty-eight HCC patients with PVTT were treated by HAIC via a subcutaneously implanted injection port. Of these, 14 had PVTT in the second portal branch and 34 patients had PVTT in the first portal branch or in the main portal trunk. One course of chemotherapy consisted of daily cisplatin (7 mg/m(2) for 1 hour on Days 1-5) followed by 5-fluorouracil (170 mg/m(2) for 5 hours on Days 1-5). Patients were scheduled to receive four serial courses of HAIC. Responders were defined as having either a complete response (CR) or partial response (PR) and nonresponders were defined as exhibiting stable disease or progressive disease. The prognosis after HAIC and factors related to survival were analyzed. RESULTS: Following HAIC, 4 and 19 patients exhibited a CR and PR, respectively (response rate = 48%). The 1, 2, 3, and 5-year cumulative survival rates of 48 patients treated with HAIC were 45%, 31%, 25%, and 11%, respectively. Median survival periods for 23 responders and 25 nonresponders were 31.6 (range, 8.3-76.9) months and 5.4 (1.9-29.0) months, respectively. Therapeutic effect (P < 0.001) and hepatic reserve capacity (P = 0.021) were identified as significant prognostic factors by univariate analysis. Multivariate analysis identified only therapeutic effect as being significantly related to survival. CONCLUSIONS: HAIC using low-dose cisplatin and 5-fluorouracil may be a useful therapeutic option for patients with advanced HCC with PVTT. HCC patients with PVTT who respond to HAIC could certainly have survival benefits.

Aged↗
PubMed1986

Vascular complications after liver transplantation: a 5-year experience.

During the past 5 years, 104 angiographic studies were performed in 87 patients (45 children and 42 adults) with 92 transplanted livers for evaluation of possible vascular complications. Seventy percent of the studies were abnormal. Hepatic artery thrombosis was the most common complication (seen in 42% of children studied, compared with only 12% of adults) and was a major complication that frequently resulted in graft failure, usually necessitating retransplantation. In six children, reconstitution of the intrahepatic arteries by collaterals was seen. Three survived without retransplant. Arterial stenosis at the anastomosis or in the donor hepatic artery was observed in 11% of patients. Portal vein thrombosis or stenosis occurred in 13% of patients. Two children and one adult with portal vein thrombosis demonstrated hepatopetal collaterals that reconstituted the intrahepatic portal vessels. Uncommon complications included anastomotic and donor hepatic artery pseudoaneurysms, a hepatic artery-dissecting aneurysm, pancreaticoduodenal mycotic aneurysms, hepatic artery-portal vein fistula, biliary-portal vein fistula, hepatic vein occlusion, and inferior vena cava thrombosis.

Adolescent↗
PubMed2005

Severe exudative ascites as an initial presentation of Crohn's disease.

Ascites in the course of Crohn's diseases is rarely seen and usually is a result of a concomitant malignancy, infection or thrombosis of portal or hepatic veins. In this article, a 55-year-old female patient suffering from severe ascites and finally diagnosed as Crohn's disease is presented. Further investigations revealed no malignancy, peritoneal infection or vein thrombosis. Treatment of Crohn's disease with steroids and 5-aminosalicylic acid also resulted in the disappearance of the ascites.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2005

[Liver abscesses with portal and mesenteric vein thrombosis in combination with late onset of appendicitis].

Pyogenic liver abscesses are caused by appendicitis in less than 10%. Also the ascending septic inflammation of portal vein (pylephlebitis) could be a serious complication of intra-abdominal infection. Although pylephlebitis is not frequent today, its' mortality and morbidity rates remain high. We describe a case of young man with fever, abdominal pain, and multiple hepatic abscesses. After the symptomatic relief due to antibiotic therapy the pain returned as a result of the development of portal and mesenteric vein thrombosis. The cause of either hepatic abscesses or thrombosis was not clear at the time of dismissal. 2 months later the patient underwent acute abdominal revision with appendectomy for acute phlegmonous and gangrenous appendicitis. Since that time he has been without any clinical symptoms.

Abdomen, Acute↗
PubMed1998

Vascular complications of liver transplantation: evaluation with gadolinium-enhanced MR angiography.

PURPOSE: To evaluate use of gadolinium-enhanced magnetic resonance (MR) angiography in detection of vascular complications of liver transplantation. MATERIALS AND METHODS: Thirteen liver transplant recipients suspected to have vascular complications were evaluated with gadolinium-enhanced MR angiography by using a three-dimensional spoiled gradient-echo breath-hold technique during the arterial and venous phases of a high-dose (42 mL) bolus injection of gadolinium contrast material. Conventional angiography (n = 11) and surgery (n = 3) were used as the standard of reference. The transplant hepatic artery, celiac trunk, superior mesenteric artery, portal vein, superior mesenteric vein, splenic vein, hepatic veins, and inferior vena cava (IVC) were evaluated for thrombosis or stenosis by two radiologists. RESULTS: Ten vascular complications were identified with conventional angiography or surgery: transplant hepatic artery thrombosis (n = 3) or stenosis (n = 3), portal vein stenosis (n = 1) or occlusion (n = 2), and suprahepatic IVC stenosis (n = 1). All 10 complications were correctly diagnosed with MR angiography. There was agreement between results of MR angiography and conventional angiography or surgery in 58 of 62 vessels evaluated (94%). There was minor disagreement in four vessels (6%). CONCLUSION: Three-dimensional gadolinium-enhanced MR angiography may have the potential to enable accurate diagnosis of vascular complications of liver transplantation.

Adolescent↗
PubMed1995

Partial nodular transformation of the liver with portal vein thrombosis. A report of two autopsy cases.

Partial nodular transformation (PNT) of the liver is a rare condition in which nonfibrous nodules composed of hyperplastic hepatocytes replace the hepatic parenchyma around the hepatic hilus. We report two autopsy cases involving PNT of the liver with portal vein thrombosis. Case 1 was a 27-year-old man with malignant lymphoma. Ascites gradually increased, and he died 4 years after the onset of his illness. Case 2 was a 73-year-old woman treated for cirrhosis for 4 years who died of renal failure. Postmortem examination of these two cases revealed numerous coalescent nodules in the hilus of the liver as well as portal vein thrombus in the hilus. Microscopically, these nodules in the perihilar area were composed of hyperplastic hepatocytes without fibrous rim, and the peripheral parenchyma showed atrophy to some extent. The portal vein thrombi in the hilus and large portal tracts were mainly fresh and partially organized. Portal vein branches in the peripheral small portal tracts were devoid of significant pathologic changes. We suggest that PNT of the liver in our cases occurred as the result of uneven blood supply to the perihilar parenchyma due to portal vein thrombosis in the hepatic hilus.

Adult↗
PubMed1984

Budd-Chiari syndrome with inferior vena cava obstruction associated with systemic lupus erythematosus.

The Budd-Chiari syndrome is an uncommon condition in which hepatic venous outflow is obstructed by thrombosis of the major hepatic veins. Many of the cases are idiopathic, but it has been described in association with vena caval webs, abdominal trauma, retroperitoneal neoplasms, and hypercoagulable states. A patient with systemic lupus erythematosus (SLE) who developed the Budd-Chiari syndrome with inferior vena cava thrombosis led to a review of the possible association between SLE and the Budd-Chiari syndrome. The therapy of the Budd-Chiari syndrome with associated vena cava thrombosis is also discussed.

Adult↗