Budd-chiari syndrome complicating restorative proctocolectomy for ulcerative colitis

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.     

Acute Budd-Chiari syndrome, portal and splenic vein thrombosis in a patient with ulcerative colitis associated with antiphospholipid antibodies and protein C deficiency

We report the case of a female patient who had severe thrombotic complications in peripheral (V. jugularis, subclavia, brachialis, poplitea) and visceral (portal and splenic) veins 4 years after the first diagnosis of severe ulcerative pancolitis. A thrombolysis therapy for subclavian and jugular vein thrombosis was performed without complication, but she soon developed acute thrombosis of the hepatic veins (acute Budd-Chiari syndrome). She quickly recovered after liver transplantation and now - 6 years later - she lives a normal life with continuous anticoagulation and medical therapy of the colitis.3 possible causes for the severe coagulation defect in this patient can be supposed: Thrombocytosis, protein C deficiency and an antiphospholipid antibody syndrome.     

Budd-Chiari syndrome and Crohn's disease

Hepatic vein thrombosis (Budd-Chiari syndrome) is a rare complication of ulcerative colitis and has not previously been reported in association with Crohn's disease. We here report the first known case of Crohn's disease presenting as the Budd-Chiari syndrome.     

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.     

A case of pulmonary thromboembolism in active ulcerative colitis]

Thromboembolic disease is a significant cause of morbidity and mortality in patients with inflammatory bowel disease. The reported incidence is 1-6%. The most common thromboembolic complications are deep venous thrombosis of legs and pulmonary thromboembolism. Cerebral thrombosis, portal vein thrombosis, retinal venous thrombosis and arterial thrombosis were also reported. We experienced a case of ulcerative colitis complicated with pulmonary thromboembolism. The patient was a 70-year-old woman who was diagnosed as ulcerative colitis on colonoscopy. We used prednisolone and sulfasalazine for the treatment of ulcerative colitis. Twenty five days later, she complained of abrupt dyspnea and chest pain. Chest CT and ventilation-perfusion scan revealed a thromboembolism in both lung. After the treatment of heparin & warfarin therapy, follow-up chest CT showed much regressed pulmonary thromboembolism. We report a 70-year-old woman with ulcerative colitis complicated with pulmonary thromboembolism and treated with heparin & warfarin therapy successfully.     

A case of Budd-Chiari syndrome and superior mesenteric vein thrombosis in ulcerative colitis]

Patients with inflammatory bowel disease are at increased risk for thromboembolic complications. Its prevalence rate ranges from 1% to 7% in clinical studies and reaches 39% in autopsy series. The cause of thrombotic complications in inflammatory bowel disease is generally considered to be associated with hypercoagulability. We experienced a case of ulcerative colitis associated with Budd-Chiari syndrome and superior mesenteric vein thrombosis. This rare complication of ulcerative colitis was successfully managed by conventional treatment for ulcerative colitis and anticoagulation therapy.     

Portal vein thrombosis successfully treated with a colectomy in active ulcerative colitis

Portal vein thrombosis is a rare complication in ulcerative colitis. We present a patient with portal vein thrombosis in ulcerative colitis who was successfully treated with colectomy. A 38-year-old Japanese female was admitted to our hospital because of an exacerbation of colitis. Abdominal ultrasonography performed because of liver dysfunction showed the thrombus in an umbilical portion of the portal vein. The patient underwent a subtotal colectomy and ileostomy because her colitis did not respond to intensive intravenous therapy. Although portal vein thrombus was treated with an intravenous infusion of urokinase before the operation, no change in the thrombus size was found. Approximately three months after the colectomy, the thrombus of the portal vein disappeared without anticoagulant therapy. Although a resection of an inflamed colon may be theoretically effective in the thrombosis in the inflammatory bowel disease, its benefit has not been confirmed. Our case suggests that the resection of the diseased bowel may have a favorable effect on the course of portal vein thrombosis in acute attacks of ulcerative colitis.     

Budd-Chiari syndrome in a patient with ulcerative colitis: association with anticardiolipin antibodies

This is the first reported case of a young patient with ulcerative colitis who developed acute Budd-Chiari syndrome and was found positive for anticardiolipin antibodies. Although an association between ulcerative colitis and Budd-Chiari syndrome, and between the latter and antiphospholipid antibodies, has been shown in the literature, the coexistence of these three conditions has never been reported. The patient we describe did poorly and was considered as a candidate for liver transplantation in spite of prompt heparin therapy, probably because of the presence of multiple risk factors.     

A case of portal vein thrombosis in patient with ulcerative colitis

Portal vein thrombosis is a rare and serious complication in ulcerative colitis (UC). We report a patient with UC who developed portal vein thrombosis with persistent ascites which was successfully managed with total colectomy. A 46-year-old man was admitted complaining of bloody stool. UC had been diagnosed 11 years previously. He required subtotal colectomy because his colitis did not respond to conservative therapy and worsened with suspected peritonitis. Although the portal vein thrombosis was diagnosed after surgery and the systemic anti-coagulant therapy was started, this was stopped after 2 days because of massive rectal bleeding. Fortunately, sufficient hydration with intravenous infusion and re-infusion of concentrated ascites led to portal vein thrombolysis successfully after 28 postoperative days. This case suggests that colectomy and sufficient hydration may have a favorable effect on treatment of portal vein thrombosis in patients with UC.     

Ulcerative colitis complicated by Budd-Chiari syndrome.

We present two cases of ulcerative colitis associated with a chronic pattern of hepatic vein thrombosis (Budd-Chiari syndrome). Progression of the hepatic disorder appeared to be unrelated to the activity of the colitis. Liver function tests were normal until a late stage in the disease except for a high serum alkaline phosphate and a low serum albumin. This combination of abnormalities together with ascites merit further investigation and should raise the suspicion of hepatic vein thrombosis complicating ulcerative colitis.     

Portal vein thrombosis in Crohn's disease

Thromboembolic complications of inflammatory bowel disease are a well-recognized occurrence. Portal vein thrombosis has been reported in patients with ulcerative colitis but to date has not been reported in patients with Crohn's disease. We present the first case of portal vein thrombosis in a patient with Crohn's disease.     

PORTAL VEIN THROMBOSIS ASSOCIATED WITH ACTIVE ULCERATIVE COLITIS: PERCUTANEOUS TRANSHEPATIC RECANALIZATION

A patient with severe ulcerative colitis developed portal vein thrombosis, Intraportal infusion of plasminogen activators through a percutaneous transhepatic catheter produced an effective thormbolysis without increasing rectal bleeding. This is the first report in which portal vein thrombosis associated with active ulcerative colitis was treated successfully.     

Gastrointestinal bleeding due to chronic portal vein thrombosis in ulcerative colitis

Summary The authors report the case of a patient suffering from ulcerative colitis, who had several episodes of digestive hemorrhage due to portal hypertension. Portal hypertension was secondary to chronic portal vein thrombosis. This diagnosis was made on the venous phase of celiac and mesenteric angiography. The authors review the published cases of ulcerative colitis with portal vein thrombosis and discuss the possible etiologic factors: hypercoagulability, thrombocytosis, and intraabdominal sepsis.     

Severe Ulcerative Colitis, Dural Sinus Thrombosis, and the Lupus Anticoagulant

Thrombnembolic disease is a well-recognized but very uncommon complication of inflammatory bowel disease. The mechanisms of the increased risk of thrombosis are not well understood: although several coagulation abnormalities have been described in inflammatory bowel disease patients, it is not clear whether they actually contribute to hypercoagulation or whether they are nonspecific markers of inflammation. Antiphospholipid antibodies (anticardiolipin antibodies and/or lupus anticoagulant) have recently been associated with an increased risk of thrombosis, particularly cerebrovascular disease in young patients. We report the case of a 33-yr-old female with severe ulcerative colitis at first attack who developed thrombosis of the superior and inferior longitudinal dural sinuses. No risk factors for thrombosis or coagulation abnormalities were observed; however, lupus anticoagulant was detected in the serum. The patient was successfully treated with osmotic agents, prophylactic anticonvulsant, and antiplatelet therapy, combined with i.v. steroids. After 6 months, the colitis is in remission, and the neurological recovery is good even if not yet complete.     

A patient who survived total colonic ulcerative colitis surinfected by cytomegalovirus complicated by toxic megacolon and disseminated intravascular coagulation

The authors report the case of a patient aged 60-year-old who survived ulcerative colitis complicated by toxic megacolon and disseminated intravascular coagulation. This patient was not known for this ulcerative colitis and was first hospitalised for a suspicion of diverticulitis. The admission symptoms were fever, abdominal pain and bloody diarrhoea. The evolution was defavorable under antibiotics and sulfasalazine. The patient was readmitted 5 days after he left hospital, and the diagnosis of UC was based on colon biopsy made during the first hospitalisation. A treatment with methylprednisolone was started and the patient worsened day by day with apparition of toxic megacolon and disseminated intravascular coagulation. Subtotal colectomy was performed for degradation of general status and coagulation factors. Pathological findings confirmed ulcerative colitis with toxic megacolon. Cytomegalovirus inclusions were demonstrated on the colonic specimen and confirmed by PCR. In this report the authors discuss the etiology of toxic megacolon and disseminated intravascular coagulation in ulcerative colitis surinfected by cytomegalovirus. Mortality of these pathologies is high necessitating rapid diagnosis of cytomegalovirus infection by sigmoid biopsy. Management requires immunosupression interruption and ganciclovir therapy, or surgery in unsuccessful medical treatment.     

Paroxysmal nocturnal hemoglobinuria revealed by hepatic vein thrombosis (Budd-Chiari syndrome) during Infliximab therapy

We report the case of a 41-Year-old man presenting with hepatic vein thrombosis (Budd-Chiari syndrome) during Infliximab therapy for ankylosing spondylitis. The systematic work-up revealed paroxysmal nocturnal hemoglobinuria. One Year later the patient was receiving anticoagulation therapy and was in good condition. The role of Infliximab in the development of thrombosis in this patient with rare underlying thrombophilia is discussed.     

Transhepatic fibrinolysis of mesenteric and portal vein thrombosis in a patient with uIterative colitis: A case report

AIM: To present a case of acute mesenteric and portal vein thrombosis treated with thrombolytic therapy in a patient with ulcerative colitis in acute phase and to review the literature on thrombolytic therapy of mesenteric-portal system. Treatment of acute portal vein thrombosis has ranged from conservative treatment with thrombolysis and anticoagulation therapy to surgical treatment with thrombectomy and/or intestinal resection. METHODS: We treated our patient with intraportal infusion of plasminogen activator and then heparin through a percutaneous transhepatic catheter. RESULTS: Thrombus resolved despite premature interruption of the thrombolytic treatment for neurological complications, which subsequently resolved. CONCLUSION: Conservative management with plasminogen activator, could be considered as a good treatment for patients with acute porto-mesenteric thrombosis.     

Acquired protein C and S deficiency,inflammatory bowel disease and cerebral arterial thrombosis

Summary Thromboembolic complications may occur in inflammatory bowel disease. Recently, we had the opportunity to observe a case of a cerebral arterial thrombosis in a young patient with active ulcerative colitis. Investigation of blood coagulation revealed a temporary Protein C, Protein S and Factor II deficiency. To our knowledge, this is the first reported case of a temporary Protein C and S deficiency in a patient with thrombosis and inflammatory bowel disease.     

Transhepatic fibrinolysis of mesenteric and portal vein thrombosis in a patient with ulcerative colitis： A case report

AIM: To present a case of acute mesenteric and portal vein thrombosis treated with thrombolytic therapy in a patient with ulcerative colitis in acute phase and to review the literature on thrombolytic therapy of mesenteric-portal system. Treatment of acute portal vein thrombosis has ranged from conservative treatment with thrombolysis and anticoagulation therapy to surgical treatment with thrombectomy and/or intestinal resection. METHODS: We treated our patient with intraportal infusion of plasminogen activator and then heparin through a percutaneous transhepatic catheter. RESULTS: Thrombus resolved despite premature interruption of the thrombolytic treatment for neurological complications, which subsequently resolved. CONCLUSION: Conservative management with plasminogen activator, could be considered as a good treatment for patients with acute porto-mesenteric thrombosis.     

Superior Mesenteric Vein Thrombosis After Colectomy for Inflammatory Bowel Disease

PURPOSE: Thromboembolism is a significant cause of morbidity and mortality in inflammatory bowel disease. Several prothrombotic conditions have been investigated in inflammatory bowel disease. The aim of this study was to evaluate the incidence of symptomatic postoperative superior mesenteric vein thrombosis in inflammatory bowel disease patients undergoing colonic resections and to identify and characterize their clinical presentation. METHODS: Between January 1999 and December 2001, 83 consecutive patients undergoing total colectomy for inflammatory bowel disease were studied retrospectively. Patients who developed new-onset postoperative acute abdominal pain were evaluated by CT scan of the abdomen. A complete coagulation profile, including thrombin time, platelet count, protein C, protein S, antithrombin III, homocysteine level, factor V Leiden mutation, plasminogen, and prothrombin G20210A mutation, was obtained in patients diagnosed with superior mesenteric vein thrombosis. RESULTS: Four patients (4.8 percent; 3 females; 3 patients with ulcerative colitis and 1 with Crohn's colitis) developed symptomatic postoperative superior mesenteric vein thrombosis. Two of these patients had extension of the clot into the portal vein. Their presenting symptom was abdominal pain, with a median interval of ten days from the index surgery. The hematologic workup was negative in three patients, with one heterozygous for prothrombin G20210A mutation. All patients were treated with systemic anticoagulation for at least six months. One ulcerative colitis patient was diagnosed after abdominal colectomy and underwent an uneventful ileal pouch-anal anastomosis after systemic anticoagulation. CONCLUSION: Postoperative superior mesenteric vein thrombosis is a more frequent occurrence than previously reported in patients with inflammatory bowel disease. Direct surgical trauma to the middle colic veins, with resulting thrombosis, is likely to be the precipitating factor in a borderline intrinsically hypercoagulable environment. All patients became asymptomatic after systemic anticoagulation and recovered uneventfully.