|Year : 2018 | Volume
| Issue : 1 | Page : 54-56
Cerebral venous thrombosis in inflammatory bowel disease
Sunil Mathew1, Reddy Ravikanth2
1 Department of Anatomy, St. John's Medical College, Bengaluru, Karnataka, India
2 Department of Radiology, St. John's Medical College, Bengaluru, Karnataka, India
|Date of Web Publication||12-Jan-2018|
Department of Radiology, St. John's Medical College, Bengaluru - 560 034, Karnataka
Source of Support: None, Conflict of Interest: None
Patients with inflammatory bowel disease (IBD) have a higher risk of venous thrombosis compared with the general population. The most common sites of venous thrombosis in IBD patients are the deep veins of the legs, pulmonary system, and portal and mesenteric veins. However, cerebral venous thrombosis (CVT) is rarely associated with IBD. In patients with IBD complicated by CVT, favorable outcomes are possible with early diagnosis and appropriate treatment. However, when patients are not treated, the mortality rate can be as high as 50%. Here, we present a 32-year-old man with the complaints of headache and seizures earlier diagnosed with ulcerative colitis. A diagnosis of CVT was made based on imaging findings. Thromboembolism is an extraintestinal complication of IBD, more frequently venous thrombosis and pulmonary thromboembolism.
Keywords: Cerebral venous thrombosis, inflammatory bowel disease, thromboembolism
|How to cite this article:|
Mathew S, Ravikanth R. Cerebral venous thrombosis in inflammatory bowel disease. CHRISMED J Health Res 2018;5:54-6
|How to cite this URL:|
Mathew S, Ravikanth R. Cerebral venous thrombosis in inflammatory bowel disease. CHRISMED J Health Res [serial online] 2018 [cited 2020 Aug 7];5:54-6. Available from: http://www.cjhr.org/text.asp?2018/5/1/54/223120
| Introduction|| |
Patients with inflammatory bowel disease (IBD) have an elevated risk of venous thromboembolism compared with the general population. Although cerebral venous thrombosis (CVT) is a rare complication in CD patients, the CVT is sometimes very serious, leading to mortality. The pathophysiology of thrombosis in IBD patients is not yet fully understood. However, it has been suggested that CVT is associated with several coagulation problems that develop during an IBD flare-up; these include thrombocytosis, increased levels of fibrinogen, fibrinopeptide A, factors V and VIII, the antithrombin factor III deficiency as well as a deficiency of free protein S. This case report describes the case of a 32-year-old male in whom CVT occurred during active phase of IBD.
| Case Report|| |
A 32-year-old man with a history of increased frequency of diarrhea, weight loss, and diffuse abdominal pain for 2 months. The patient was diagnosed to have ulcerative colitis after sigmoidoscopy and biopsy and was started on treatment. Two days later, the patient developed headache and seizures. Routine laboratory tests, including tests for thrombophilia, serum levels of antiphospholipid antibodies and homocysteine, were performed. Blood investigations for thrombophilia were negative, and antiphospholipid antibodies were negative with high serum homocysteine levels. Liver function, renal function, and serum electrolytes were within the normal limits. Noncontrast magnetic resonance venogram (MRV) of the brain demonstrated non-visualization of the superior sagittal sinus [Figure 1] suggesting thrombosis with regions of Gradient blooming in the corona radiata suggesting venous infarct and gradient blooming in the superior sagittal sinus suggesting thrombosis [Figure 2]. Contrast-enhanced computed tomography (CT) of the abdomen showed gross wall thickening of the distal ileum, ascending, and transverse colon [Figure 3] with mesenteric hypervascularity [Figure 4]. Esophagoduodenoscopy and histopathology confirmed IBD. Treatment with sulfasalazine, low-molecular weight heparin, and intravenous dexamethasone was started, and the patient showed neurological improvement in 4 days.
|Figure 1: Magnetic resonance venogram image showing non-visualization of the superior sagital sinus suggesting thrombosis|
Click here to view
|Figure 2: Axial gradient recalled echo image showing blooming in the region of left corona radiata suggesting infarction and blooming in the superior sagittal sinus suggesting thrombosis|
Click here to view
|Figure 3: Axial contrast-enhanced computed tomography image demonstrating diffuse wall thickening involving the hepatic flexure and transverse colon|
Click here to view
|Figure 4: Axial contrast-enhanced computed tomography image demonstrating circumferential wall thickening with submucosal edema and mesenteric hypervascularity in the region of the ascending colon|
Click here to view
| Discussion|| |
Approximately 1.5%–6% of patients with IBD are complicated by CVT during disease. Increased incidence of thrombotic complications is noted in patients with ulcerative colitis and Crohn's disease, however, cerebral vascular involvement is rare. The cause may be due to transient coagulation abnormalities including increased fibrinogen, elevated factor VII, elevated factor VIII, antithrombin III deficiency, protein C and protein S deficiency. Patients with CVT and IBD comorbidity are significantly younger when compared with patients with CVT patients without IBD.
CVT can be triggered by an imbalance of the body's homeostasis or reduced action of the intrinsic antithrombotic mechanism. Early diagnosis is crucial to prevent such outcomes as hydrocephalus, intracranial hypertension, and further seizures. Occlusion of cerebral venous system impairs blood outflow from the brain, resulting in increased intracranial and capillary pressure and subsequently intracerebral hemorrhage. Although the efficacy of anticoagulation in CVT has not been unequivocally proven, it is widely used as the main-stay therapy. There is a pathophysiological rationale to recommend the use of anticoagulation in CVT. The use of anticoagulation can theoretically prevent thrombus propagation, facilitate recanalization of the occluded venous sinus, and improve venous outflow. Standard medical treatment of CVT includes low-molecular-weight heparin and endovascular thrombolysis.
Understanding the role of risk factors – such as genetic or acquired thrombophilia, pregnancy, use of oral contraceptives, and hyperhomocysteinemia – in CVT development is important. CVT was confirmed by MRV, computed tomographic venography, conventional angiography, surgery, or autopsy. At baseline, all patients received CT or MR imaging (MRI). Repeated cerebral imaging was performed at the discretion of the treating physician, and documentation of new parenchymal lesions was required. Heparin is generally considered the main-stay of treatment, supported by data from a few small trials. More invasive treatment options are available, such as endovascular thrombolysis and – in more severe cases – decompressive hemicraniectomy. The most dreaded complications of CVT are intracranial hypertension and cerebral herniation. The raised intracranial pressure is secondary to obstructed venous drainage leading to cerebral edema and intracranial hemorrhage which is often accompanying this condition. CVT usually starts in the large venous sinuses and may extend into the cortical veins. In a minority of cases, the thrombosis is limited to the cortical or internal cerebral veins. The clinical symptoms depend on the localization and extension of the thrombus and are highly diverse. Collateral venous drainage in the brain is limited, and venous obstruction causes increased capillary pressure, cerebral edema, and eventually venous infarcts.
Although a plain CT or MRI is useful in the initial evaluation of patients with suspected CVT, a negative plain CT or MRI does not rule out CVT. A venographic study (either CT venography [CTV] or MRV) should be performed in suspected CVT if the plain CT or MRI is negative or to define the extent of CVT if the plain CT or MRI suggests CVT. An early follow-up CTV or MRV is recommended in CVT patients with persistent or evolving symptoms despite medical treatment or with symptoms suggestive of propagation of thrombus. In patients with the previous CVT who present with recurrent symptoms suggestive of CVT, repeat CTV, or MRV is recommended. Gradient echo T2 susceptibility-weighted images combined with MR can be useful to improve the accuracy of CVT diagnosis. Catheter cerebral angiography can be useful in patients with inconclusive CTV or MRV in whom a clinical suspicion for CVT remains high. A follow-up CTV or MRV at 3–6 months after diagnosis is reasonable to assess for recanalization of the occluded cortical vein/sinuses in stable patients.
The treatment of cerebrovascular complications in patients with IBD consists in control of the inflammatory process that seems to be the key to the reduction of risk of cerebrovascular complications of IBD; selected medication may directly inhibit platelets activation (5-aminosalicylic acid, azathioprine, and its metabolite, 6-mercaptopurine, and infliximab). Reduction of modifiable risk factors as smoking, contraception, and diet is necessary. Treatment of the condition is by antiedema measures and heparin. Steroids reduce the intracerebral edema and are also indicated in the active cases of ulcerative colitis. Anticoagulant in the form of low molecular weight heparin is indicated in CVT, even in cases with intracerebral bleed to dissolve the thrombus and decreases the thrombus spread.
Neurological complications of both venous and arterial types may be observed in IBD being associated with low serum levels of folate, Vitamin B6 and B12 and high levels of homocysteine, high activation of platelets, and microvascular endothelial dysfunction. Thromboembolism is an extraintestinal complication of IBD, more frequently venous thrombosis and pulmonary thromboembolism. Thromboembolic events occur more commonly in ulcerative colitis than Crohn's disease. CVT is a rare complication of IBD. Ulcerative colitis is a type of chronic IBD that affects any age group, although there are peaks at ages 15–30 and then again at 50–70.
| Conclusion|| |
CVT is a rare but devastating complication of IBD. It is important to recognize the clinical signs to start anticoagulation expeditiously and improve neurological outcomes. CVT can be accompanied by neurologic abnormalities caused by rapidly elevating intracranial pressure, and can result in serious adverse events. Therefore, early diagnosis of this rare complication is important for IBD patients complaining of headache and vomiting.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Zezos P, Kouklakis G, Saibil F. Inflammatory bowel disease and thromboembolism. World J Gastroenterol 2014;20:13863-78.
Katsanos AH, Katsanos KH, Kosmidou M, Giannopoulos S, Kyritsis AP, Tsianos EV. Cerebral sinus venous thrombosis in inflammatory bowel diseases. QJM 2013;106:401-13.
Guenther G, Arauz A. Cerebral venous thrombosis: A diagnostic and treatment update. Neurologia 2011;26:488-98.
Frey JL, Muro GJ, McDougall CG, Dean BL, Jahnke HK. Cerebral venous thrombosis: Combined intrathrombus rtPA and intravenous heparin. Stroke 1999;30:489-94.
Umit H, Asil T, Celik Y, Tezel A, Dokmeci G, Tuncbilek N, et al.
Cerebral sinus thrombosis in patients with inflammatory bowel disease: A case report. World J Gastroenterol 2005;11:5404-7.
Einhäupl K, Stam J, Bousser MG, De Bruijn SF, Ferro JM, Martinelli I,et al
. EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients. Eur J Neurol 2010;17:1229-35.
Grainge MJ, West J, Card TR. Venous thromboembolism during active disease and remission in inflammatory bowel disease: A cohort study. Lancet 2010;375:657-63.
Gala NB, Agarwal N, Barrese J, Gandhi CD, Prestigiacomo CJ. Current endovascular treatment options of dural venous sinus thrombosis: A review of the literature. J Neurointerv Surg 2013;5:28-34.
Samia BS, Lamia K, Fatma N, Sawssen BR, Rim E, Mourad Z, et al.
Cerebral venous thrombosis in inflammatory bowel disease: A case series. Int J Colorectal Dis 2011;26:257-8.
Kim I, Min KH, Yeo M, Kim JS, Lee SH, Lee SS, et al.
Unusual case of cerebral venous thrombosis in patient with Crohn's disease. Case Rep Neurol 2015;7:115-20.
[Figure 1], [Figure 2], [Figure 3], [Figure 4]