Nilüfer HACIFAZLIOĞLU ELDEŞ, Günsel KUTLUK, Yüksel YILMAZ, Deniz ERTEM, Hilal HOROZOĞLU

Çocukluk çağı ülseratif kolit hastalığına bağlı beyin ven trombozu

Beyin ven trombozu çocuklarda enflamatuar bağırsak hastalığının; özellikle ülseratif kolitin seyri sırasında gelişebilen nadir ancak önemli bir komplikasyondur. Dokuz yaşında kanlı, mukuslu dışkılama ve beraberinde gelişen şiddetli başağrısı yakınması ile gelen ülseratif kolit ile aynı anda beyin ven trombozu tanısı alan bir olgu sunuldu. Dokuz yaşında erkek hasta üç haftadır süren kanlı ve mukuslu dışkılama nedeni ile başvurdu. Tetkiklerinde anemi, lökositoz, trombositoz ve yüksek eritrosit çökme hızı saptanan hastanın kolonoskopik incelemesinde yaygın ülserler ile seyreden pankolit görüldü ve tanı histopatolojik inceleme ile ülseratif kolit olarak kesinleştirildi. Kolonoskopiden bir gün sonra sürekli, zonklar tarzda, frontal ve sol oksipital bölgeye yerleşik başağrısı yakınması gelişen hastanın nörolojik değerlendirilmesinde sol göz kapağında pupil seviyesinde pitoz, her iki göz dibinde birinci derece papil ödem saptandı. Kraniyal manyetik rezonans görüntüleme ve manyetik rezonans venografi incelemesinde sol transvers ve sigmoid sinüsde tromboz saptanan hastaya antikoagülan tedavi ile ülseratif kolit için mesalamin ve steroid başlandı. Trombofili açısından da değerlendirilen hastanın protein C, protein S, antitrombin III, faktör VIII, homosistein, protrombin II and fibrinojen düzeyleri normal sınırlarda; metilen tetrahidrofolat redüktaz gen mutasyonu ise heterozigot pozitif saptandı. Tedavinin ikinci haftasında kanlı dışkılaması kontrol altına alınan hastanın başağrısı azaldı, papilödem ve pitozu kayboldu. Mesalamin, prednisolon, azatiyopürin ve düşük molekül ağırlıklı heparin ile tedavisi devam ettirilen olgumuzda tedavinin birinci yılında manyetik rezonans venografi incelemesinde trombozda radyolojik açıdan artma yada düzelme izlenmedi.

Cerebral venous thrombosis associated with childhood ulcerative colitis

Ulcerative colitis (UC) can be complicated by various extra–intestinal manifestations such as thromboembolic disease. Most thrombotic events occure in lower extremities, whereas the incidence of cerebral venous involvement is very rare and serious in children. We present a case of a nine years old boy with UC and cerebral venous thrombosis diagnosed at the same time. A nine years old boy with a 3- week history of bloody diarrhea and abdominal pain was admitted to our hospital. On admission his laboratory data showed anemia, leukocytosis , thrombocytosis and elevated erythrocyte sedimentation rate. Colonoscopic evaluation revealed severe active pancolitis with diffuse ulceration. The day after colonoscopy he had complained of severe headache. Neurolological examination revealed pitosis of the left palpebra and bilateral papilledema. Magnetic resonance (MR) imaging and MR venography revealed thrombosis in the left transvers sinus and sigmoid sinus. The patient was treated immediatly with heparin. At the same time he was diagnosed with severe active UC based upon histopathological findings and managed initially with mesalamin and prednisolon. He also had been screened forthrombophilia. Protein C, protein S,antithrombin III, factor VIII, homocystein, prothrombin II and fibrinogen levels were in the normal range but he was heterozygous for the methylene tetrahydrofolate reductase gene mutation. After two weeks of therapy his bloody diarrhea was controlled, headache and neurolological findings were improved. His treatment was continued with mesalamin, prednisolon, azathioprin and low molecular weight heparin, but repeated MRI and MRI venography showed neither extension nor improvement of the thrombosis.

PDF

___

1. Umit H, Asil T, Celik Y, et al. Cerebral sinus thrombosis in patients with inflammatory bowel disease: a case report. World J Gastroenterol 2005; 11: 5404-7.
2. Stange EF Travis SP Vermeire S, et al. European evidence-based consensus on the diagnosis and management of ulcerative colitis: Definitions and diagnosis. J Crohns Colitis 2008; 2(1): 1-23.
3. Dejaco C, Oesterreicher C, Angelberger S, et al. Diagnosing colitis: a prospective study on essential parameters for reaching a diagnosis. Endoscopy 2003; 35(12): 1004-8.
4. Miehsler W, Reinisch W, Valic E, et al. Is inflammatory bowel disease an independent and disease specific risk factor for thromboembolism? Gut 2004; 53(4): 542-8.
5. Hudson M, Chitolie A, Hutton RA, et al. Thrombotic vascular risk factors in inflammatory bowel disease. Gut 1996; 38(5): 733-7.
6. Kao A, Dlugos D, Hunter JV, Mamula P Thorarensen O. Anticoagulation therapy in cerebral sinovenous thrombosis and ulcerative colitis in children. J Child Neurol 2002; 17(7): 479-82.
7. Al-Malik H, Green MR. Cerebral venous thrombosis as a complication of Crohn disease: a case report. J Pediatr Gastroenterol Nutr 2001; 32(2): 209-11.
8. Guerra Montero LJ, Ingver A, Casañas A, Sosa C, Iade B. Clinical characteristics of patients with inflammatory bowel disease and thromboembolic events. Acta Gastroenterol Latinoam 2010; 40(2): 134-41.
9. Sébire G, Tabarki B, Saunders DE, et al. Cerebral venous sinus thrombosis in children: risk factors, presentation, diagnosis and outcome. Brain 2005; 128(3): 477-89.
10. Barclay AR, Keightley JM, Horrocks I, et al. Cerebral thromboembolic events in pediatric patients with inflammatory bowel disease. Inflamm Bowel Dis 2010; 16: 677-83.
11. Huber W, Herrmann G, Schuster T, et al. Life-threatening complications of Crohn’s disease and ulcerative colitis: a systematic analysis of admissions to an ICU during 18 years. Dtsch Med Wochenschr 2010; 135(14): 668-74.
12. Scaldaferri F Lancellotti S, Pizzoferrato M, De Cristofaro R. Haemostatic system in inflammatory bowel diseases: new players in gut inflammation. World J Gastroenterol 2011; 17(5): 594-608.
13. Saibeni S, Saladino V, Chantarangkul V, et al. Increased thrombin generation in inflammatory bowel diseases. Thromb Res 2010; 125(3): 278-82.
14. Bernhard H, Deutschmann A, Leschnik B, et al. Thrombin generation in pediatric patients with Crohn’s disease. Inflamm Bowel Dis 2011; 17(11): 2333-9.
15. Murch SH, Braegger CP Walker-Smith JA, MacDonald TT. Location of tumour necrosis factor alpha by immunohistochemistry in chronic inflammatory bowel disease. Gut 1993; 34(12): 1705-9.
16. Murch SH. Sulphation of proteoglycans and intestinal function. J Gastroenterol Hepatol 1995; 10(2): 210-2.
17. Urgesi R, Zampaletta C, Masini A, et al. Spontaneous right ventricular thrombus in a patient with active ulcerative colitis and protein C deficiency: a review with a case report. Eur Rev Med Pharmacol Sci 2010; 14(5): 455-63.
18. Twig G, Zandman-Goddard G, Szyper-Kravitz M, Shoenfeld Y. Systemic thromboembolism in inflammatory bowel disease: mechanisms and clinical applications. Ann N Y Acad Sci 2005; 1051: 166-73.
19. Danese S, Papa A, Saibeni S, Repici A, Malesci A, Vecchi M. Inflammation and coagulation in inflammatory bowel disease: The clot thickens. Am J Gastroenterol 2007; 102(1): 174-86.
20. Akbulut S, Altiparmak E, Topal F Ozaslan E, Kucukazman M, Yonem O. Increased levels of homocysteine in patients with ulcerative colitis. World J Gastroenterol 2010; 16(19): 2411-6.
21. Turri D, Rosselli M, Simioni P et al. Factor V Leiden and prothrombin gene mutation in inflammatory bowel disease in a Mediterranean area. Digest Liver Dis 2001; 33(7): 559-62.
22. Papa A, Danese S, Grillo A, Gasbarrini G, Gasbarrini A. Review article: inherited thrombophilia in inflammatory bowel disease. Am J Gastroenterol 2003; 98(6): 1247-51.
23. Wakefield A, Cohen Z, Levy G. Procoagulant activity in gastroenterology. Gut 1990; 31(2): 239-41.
24. Ertem D, Ozguven E, Acar Y, Alper G, Pehlivanoglu E. Thromboembolic complications in children with Crohn’s disease. J Pediatr Gastroenterol Nutr 1999; 28(5): 540-1.
25. Yakaryilmaz F Guliter S, Degertekin B, Tuncer C, Unal S. Cerebral sinus thrombosis in a patient with active ulcerative colitis and double heterozygosity for Factor V Leiden and prothrombin gene mutations. Neurol India 2009; 57: 188-90.
26. Kupfer SS, Rubin DT. Inflammatory bowel disease and cerebral venous sinus thrombosis. Gastroenterology and Hepatology 2006; 2: 917.
27. Koutroubakis IE. Therapy insight: vascular complications in patients with inflammatory bowel disease. Nat Clin Pract Gastroenterol Hepatol 2005; 2(6): 266-72.
28. deVeber G, Chan A, Monagle P al. Therapy in pediatric patients et with sinovenous thrombosis: a cohort study. Arch Neurol 1998; 55(12): 1533-7.
29. Roach ES, Golomb MR, Adams R, et al. Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke 2008; 39(9): 2644-91.
30. deVeber GA, MacGregor D, Curtis R, Mayank S. Neurologic outcome in survivors of childhood arterial ischemic stroke and sinovenous thrombosis. J Child Neurol 2000; 15(5): 316-24.
31. De Veber G, Andrew M, and the Canadian Pediatric Ischemic Stroke Study Group. The epidemiology and outcome of sinovenous thrombosis in pediatric patients. N England J Med 2001; 345: 417-23.
32. Murata S, Ishikawa N, Oshikawa S, et al. Cerebral sinus thrombosis associated with severe active ulcerative colitis. Intern Med 2004; 43(5): 400-3.