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.     

Thrombophilic abnormalities of natural anticoagulants in patients with ulcerative colitis

BACKGROUND/AIMS: Ulcerative colitis patients have increased risk for thromboembolic events. Factors predisposing to thrombosis in ulcerative colitis are poorly defined. The aim of this study was to evaluate possible thrombophilic abnormalities in patients with ulcerative colitis. METHODOLOGY: Fifty-one patients with ulcerative colitis and 51 healthy controls were studied. Disease activity, clinical and endoscopic, was assessed by standard criteria. Plasma levels of antithrombin, protein C, free protein S and activated protein C resistance were determined in both study groups. Genetic test for factor V Leiden was performed in cases with abnormal activated protein C resistance. Parameters of inflammation and fibrinogen were additionally measured in ulcerative colitis patients. RESULTS: Mean values of free protein S were significantly lower in ulcerative colitis patients (84.01 +/- 21.57) compared to healthy controls (100.17 +/- 24.7) (p < 0.001). Mean values of protein C were higher in ulcerative colitis patients (124.6 +/- 39.03) than healthy controls (100.19 +/- 19.86) (p < 0.001). No other significant differences were observed, but there was a trend towards higher prevalence of low values for antithrombin (9.8% vs. 0%, p = 0.056) and free protein S (19.6% vs. 5.9%, p = 0.072) in ulcerative colitis patients. Three ulcerative colitis patients and three healthy controls had low activated protein C resistance ratio. All these subjects were heterozygous for factor V Leiden. No correlation was observed between abnormalities in thrombophilic parameters and clinical, endoscopic or inflammatory parameters in ulcerative colitis group. CONCLUSIONS: Abnormalities in natural anticoagulants are more common in ulcerative colitis patients compared to healthy controls, irrespective of disease activity. Low activated protein C resistance ratio due to factor V Leiden is not more common in ulcerative colitis patients than in healthy controls.     

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.     

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.     

Cerebral venous sinus thrombosis and ulcerative colitis]

Hypercoagulability is a extraintestinal manifestation of inflammatory bowel disease, which could provoke thromboembolic phenomena. Central nervous system venous thrombosis is a rare complication and could be unnoticed. We report on a patient with ulcerative colitis who presented cerebral venous sinus thrombosis as the first manifestation of a hypercoagulable state. We review the literature and discuss about the pathogenic mechanisms of such complication.     

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.     

Arterial thrombosis in ulcerative colitis: Case report

Arterial thrombosis is a rare complication of ulcerative colitis with many difficulties in its treatment. The pathogenesis of thrombosis is not well known and many factors are implicated such as elevated fibrinogen, increased platelet count, and decreased antithrombin III levels. We report a case of a 43-year-old man with ulcerative colitis and sudden and refractory thrombosis of the brachial and radial artery. Despite efforts to restore the blood flow, the final and effective means was the resection of the large bowel.     

Emergency thrombectomy of the inferior vena cava and total colectomy in a patient with ulcerative colitis.

Patients with chronic inflammatory bowel disease, particularly ulcerative colitis, have a greater thrombotic tendency. An effective management of a patient with established ulcerative colitis complicated with acute arterial occlusion of the right lower extremity and inferior vena cava thrombosis is presented. The episodes of thrombosis usually occur in the active phase of the disease. It is suggested that in patients with diseases such as UC, predisposing to thrombosis, the identification of abnormalities in the coagulation profile may predict those particularly at risk.     

Two thrombotic complications in a patient with active ulcerative colitis

A 30-year-old woman is described with both a cerebral venous thrombosis and a deep vein thrombosis of her leg in association with an exacerbation of her ulcerative colitis. Laboratory investigations revealed a transient functional APC resistance, without Factor V mutation, and a mild hyperhomocysteinaemia. After colectomy the APC ratio was normal. These findings may form a partial explanation of the thrombophilia seen in some patients with active ulcerative colitis.     

Budd-Chiari syndrome in a patient with ulcerative colitis and no inherited coagulopathy

We report a case of 27 year old female patient who was admitted to the hospital with an acute flare up of ulcerative colitis.The patient presented with complaints of persistent abdominal pain and bloody diarrhea despite aggressive therapy for her ulcerative colitis.A CT scan of the abdomen on admission revealed hepatic vein thrombosis,suggesting a diagnosis of Budd-Chiari syndrome.Significantly,an associated thrombosis of the inferior mesenteric vein was also detected.Based on imaging data and clinical assessment,the patient was started on anticoagulation therapy and an extensive work-up for hypercoagulability was initiated.Up to the time of publication,no significant findings suggesting this patient has an underlying coagulation disorder have been found.Based on our search of PUBMED,this report is one of only five reported adult cases of Budd-Chiari Syndrome associated with ulcerative colitis in the English literature in living patients without evidence of a co-existing coagulation disorder.This case highlights the potential for thrombosis at unusual sites in ulcerative colitis patients even in the absence of classical coagulation abnormalities.In addition to the case presented,we provide a brief review of previously reported cases of Budd-Chiari Syndrome occurring in patients with inflammatory bowel disease.     

Superior mesenteric arterial thrombosis complicating ulcerative colitis: A case report]

36-year-old men with ulcerative colitis was attacked by the colic. The thrombus in superior mesenteric artery was revealed by computed tomography. Because the effect of the thrombolysis under intrarterial angiography was insufficient, thrombectomy was enforced under the laparotomy in the same day. The arterial thrombosis is extremely rare in the complication of ulcerative colitis. The activity of ulcerative colitis as one of factors of the appearance of thrombus was suggested.     

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.     

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.     

Haemorrhagic cerebral sinus thrombosis associated with ulcerative colitis: a case report of successful treatment by anticoagulant therapy

A 29-year-old man with ulcerative colitis was admitted to our hospital because of convulsions and a headache. Before admission, oral prednisolone had been administered due to his ulcerative colitis relapse. Computed tomography revealed a low-density area in the right frontal pole suggestive of a venous infarction. Once his headache and convulsions improved after the administration of an antiepileptic drug, he began to complain of right arm numbness and right hemianopsia again. An urgent magnetic resonance imaging angiograph showed extensive thrombosis in the superior sagittal sinus. We finally used the anticoagulant agents, heparin and urokinase, which eased his complaints and prevented the development of bloody stools. He was discharged with no neurological symptoms 25 days after admission. This is a rare case of sinus thrombosis complicated by ulcerative colitis, in which anticoagulant therapy was successful. Magnetic resonance imaging angiography was useful for the diagnosis and for evaluating the therapeutic effect.     

Glucose intolerance and diabetes mellitus in ulcerative colitis: Pathogenetic and therapeutic implications

Diabetes mellitus is one of the most frequent co-morbidities of ulcerative colitis patients.The epidemiological association of these diseases suggested a genetic sharing and has challenged gene identification.Diabetes co-morbidity in ulcerative colitis has also relevant clinical and therapeutic implications,with potential clinical impact on the follow up and outcome of patients.These diseases share specific complications,such as neuropathy,hepatic steatosis,osteoporosis and venous thrombosis.It is still unknown whether the coexistence of these diseases may increase their occurrence.Diabetes and hyperglycaemia represent relevant risk factors for postoperative complications and pouch failure in ulcerative colitis.Medical treatment of ulcerative colitis in patients with diabetes mellitus may be particularly challenging.Corticosteroids are the treatment of choice of active ulcerative colitis.Their use may be associated with the onset of glucose intolerance and diabetes,with difficult control of glucose levels andwith complications in diabetic patients.Epidemiologic and genetic evidences about diabetes co-morbidity in ulcerative colitis patients and shared complications and treatment of patients with these diseases have been discussed in the present review.     

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.     

Paradoxical Response to Heparin in 10 Patients with Ulcerative Colitis

Objectives : A patient with ulcerative colitis refractory to standard therapy was treated with heparin for a deep vein thromhosis. Paradoxically, rectal bleeding did not increase; instead, his colitis rapidly went into remission. The same effect occurred when this patient was later treated for a pulmonary embolism. On the basis of these observations and reports of a hypercoagulable state in ulcerative colitis, heparin was tested as a therapeutic agent in nine additional patients. Methods : Nine of the 10 patients had ulcerative colitis poorly controlled on sulfasalazine and prednisolone. Two had associated thromboembolic disease, and one was on no medication. Patients were started on heparin in hospital, taught to self-inject subcutaneously, and discharged to continue on 10.000 U of unfractionated heparin twice daily. Current doses of sulfasalazine were maintained; prednisolone was tapered and stopped. Patients were carefully monitored for adverse side-effects. Sections of colonic mucosa from nine patients were examined for intravascular thrombosis of the mucosal blood vessels. Results : Nine patients became asymptomatic (normal stool frequency, no rectal bleeding) on combined heparin and sulfasulazine therapy; one patient had a partial improvement in symptoms. Highly significant statistical differences between pre- and posttreatment mean scores were found for all disease parameters. Intravascular fibrin thrombi were identified in sections from six of nine patients. No serious complications were associated with this use of heparin. Conclusions : The heparin-linked remission of ulcerative colitis, observed by chance in our first patient, was followed by simitar responses in eight of nine further patients. This suggests that, used as described, heparin may have a role in treating refractory ulcerative colitis.     

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.     

The role of CD40 in ulcerative colitis: histochemical analysis and clinical correlation

OBJECTIVES : CD40 co-stimulator seems to be implicated in the loss of tolerance against self-antigens in many autoimmune diseases. The evidence suggests that in the pathogenesis of ulcerative colitis there is an activity state against self-antigens of the gut wall and flora. The aim of this study was to analyse the expression of CD40 in ulcerative colitis, comparing it with Crohn's disease and nonspecific inflammation of the colon and to determine whether there is a relationship between its expression and the activity stage of the disease. METHODS : The expression of CD40 in the colonic samples of 51 patients (30 ulcerative colitis, 9 Crohn's disease and 12 nonspecific inflammation) was analysed by immunohistochemistry. Twenty-four patients with ulcerative colitis were scored according to clinical, endoscopic and histological classification. RESULTS : The mean percentage of CD40+ cells per field in the colonic mucosa was: ulcerative colitis 21 +/- 11%, Crohn's disease 24 +/- 9%, nonspecific inflammation 7 +/- 7%. The ulcerative colitis patients were statistically significantly different compared to the patients with nonspecific inflammation (P < 0.005), even when comparing the patients in remission (P < 0.05). The expression in Crohn's disease was similar to that in ulcerative colitis. The expression of CD40 in ulcerative colitis was directly proportional to the state of activity of the disease according to the clinical (P < 0.02), endoscopic (P < 0.01) and histological (P < 0.02) criteria. CONCLUSIONS : The expression of CD40 in the colonic mucosae of patients with ulcerative colitis is significantly increased and is proportional to the state of activity. The results seem to confirm the hypothesis that a loss of tolerance could be involved in the pathogenesis of this disease.     

Cerebral venous thrombosis associated with ulcerative colitis

Thromboembolic complications, such as deep venous thrombosis and pulmonary embolism, are well recognized in patients with inflammatory bowel disease (IBD). We describe three cases of cerebral venous thrombosis complicating ulcerative colitis. Cerebral venous thrombosis is a rare but potentially devastating complication of IBD, and the diagnosis needs to be considered in any patient with IBD presenting with neurological symptoms.