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LETTER TO EDITOR
Year : 2013  |  Volume : 61  |  Issue : 2  |  Page : 173-175

Isolated cortical vein thrombosis in a patient with sickle cell disease: Treatment with decompressive craniotomy and anticoagulation and literature review


Department of Neurology, Psychology, and Psychiatry, Botucatu Medical School, São Paulo State University, Brazil

Date of Submission21-Dec-2012
Date of Decision23-Jan-2013
Date of Acceptance17-Mar-2013
Date of Web Publication29-Apr-2013

Correspondence Address:
Pedro Tadao Hamamoto Filho
Department of Neurology, Psychology, and Psychiatry, Botucatu Medical School, São Paulo State University
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.111132

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How to cite this article:
Hamamoto Filho PT, Gabarra RC, Braga GP, Ruiz e Resende LS, Bazan R, Zanini MA. Isolated cortical vein thrombosis in a patient with sickle cell disease: Treatment with decompressive craniotomy and anticoagulation and literature review. Neurol India 2013;61:173-5

How to cite this URL:
Hamamoto Filho PT, Gabarra RC, Braga GP, Ruiz e Resende LS, Bazan R, Zanini MA. Isolated cortical vein thrombosis in a patient with sickle cell disease: Treatment with decompressive craniotomy and anticoagulation and literature review. Neurol India [serial online] 2013 [cited 2020 Feb 22];61:173-5. Available from: http://www.neurologyindia.com/text.asp?2013/61/2/173/111132


Sir,

Sickle cell disease (SCD) is reported to be an important cause of stroke. However, in sickle cell disease cerebral sinus venous thrombosis (CSVT) is uncommon. Only a few cases have been reported in literature[1],[2],[3],[4],[5],[6],[7],[8] None of the reports include isolated cortical vein thrombosis. We present a case of a young man with sickle cell disease, who presented with cortical vein thrombosis and underwent decompressive craniotomy.

An 18 year old young man was referred to our service with a seven hour history of progressive dysphasia and right sided hemiparesis. The previous day the patient had mild alcohol intake. He had prior diagnosis of homozygous sickle cell disease with recurrent crisis of sickling. He was neither on hydroxyurea nor had multiple transfusions. Initial hemoglobin and hematocrit were 10.7 g/dl and 22.8%, respectively. Thrombophilia studies were negative. The initial brain computerized tomography (CT) scan showed hemorrhagic infarction in the left temporal lobe [Figure 1]. Digital subtraction angiography (DSA) showed the artery shift caused by the mass effect of the hematoma, and an avascular area within the typical topography of the Labbé's complex [Figure 2]. Twelve hours later the patient showed progressive drowsiness and a precocious decompressive craniotomy was proposed, as the literature suggested survival benefit and improved outcomes with precocious decompressive craniotomy in patients with progressive neurological impairment, under risk of herniation. Intraoperatively, there was an evident thrombosis in the Labbé's vein [Figure 3]. Surgery was uneventful and a gentle puncture of the hematoma was performed to alleviate the mass effect. The patient had good postoperative evolution. He was put on anticoagulants for six months. The patient showed a good recovery at the six month follow up, with a modified Rankin score of 1 (mild dysphasia) and underwent cranioplasty.
Figure 1: CT shows venous infarction with hemorrhage transformation within the temporal lobe

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Figure 2: Digital subtraction angiography shows an arterial shift from the mass effect (*) and a deep venous recruitment (arrow)

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Figure 3: Intraoperatively it was visualized a thrombosis in the Labbé's vein

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Sickle Cell Disease may present with a wide variety of neurological syndromes, including ischemic and hemorrhagic stroke, transient ischemic attacks, seizures, headache, and cognitive difficulties. Above all, stroke is the most devastating complication of SCD. Thrombosis of the venous system is uncommon and most reported cases involve the sinuses or deep venous system. Through a literature review, we found eight cases [Table 1]: Three in the superior sagittal sinus, three in the transverse sinuses, one in the straight sinus and vein of Galen, and one in the internal cerebral veins. To our knowledge, our case is the first with isolated thrombosis of a cortical vein.The pathogenesis of venous thrombosis in SCD patients involves blood stasis, caused by hyperviscosity during hypoxic episodes or dehydration. We hypothesize that our patient suffered from dehydration after alcohol intake, and the hyperviscous state predisposed the thrombosis. Treatment for CVST includes identification and elimination of the underlying cause, anticoagulation, and management of the intracranial hypertension. The gold standard treatment for CVST is anticoagulation. Decompressive craniotomy was for many years controversial. Of late it is accepted that decompressive craniotomy can be helpful when there is progressive neurological impairment and large parenchymal lesions, increasing the risk of herniation, as described in our patient.
Table 1: Summary of the cerebral venous thrombosis cases in patients with SCD cited in the literature

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Ischemic stroke is a major complication of SCD, and CSVT is very rare. Our patient is unique in two ways: (1) Isolated cortical vein thrombosis without sinus evolvement and (2) a need for decompressive craniotomy.

 
 » References Top

1.Schenk EA. Sickle cell trait and superior longitudinal sinus thrombosis. Ann Intern Med 1964;60:465-70.  Back to cited text no. 1
    
2.Feldenzer JA, Bueche MJ, Venes JL, Gebarski SS. Superior sagittal sinus thrombosis with infarction in sickle cell trait. Stroke 1987;18:656-60.  Back to cited text no. 2
    
3.Oguz M, Aksungur EH, Soyupak SK, Yildrim AU. Vein of Galen and sinus thrombosis with bilateral thalamic infarcts in sickle cell anaemia: CT follow-up and angiographic demonstration. Neuroradiology 1994;36:155-6.  Back to cited text no. 3
    
4.Di Roio C, Jourdan C, Terrier A, Artru F. Sickle cell disease and internal cerebral vein thrombosis. Ann Fr Anesth Réanim 1997;16:967-9.  Back to cited text no. 4
    
5.van Mierlo TD, van den Berg HM, Nievelstrein RA, Braun KP. An unconscious girl with sickle-cell disease. Lancet 2003;361:136.  Back to cited text no. 5
    
6.Ciurea SO, Thulborn KR, Gowhari M. Dural venous sinus thrombosis in a patient with sickle cell disease: Case report and literature review. Am J Hematol 2006;81:290-3.  Back to cited text no. 6
    
7.Sidani CA, Ballourah W, El Dassouki M, Muwakkit S, Dabbous I, Dahoui H, et al. Venous sinus thrombosis leading to stroke in a patient with sickle cell disease on hydroxiurea and high hemoglobin levels: Treatment with trhombolysis. Am J Hematol 2008;83:818-20.  Back to cited text no. 7
    
8.Çelikbliek A, Çelikbliek M, Bozkurt A, Göksel BK, Tan M, Özdogu H. Woman with sickle cell disease with current sigmoid sinus thrombosis and history of inadequate warfarin use during a past thrombotic event. Case Rep Neurol 2009;1:15-9.  Back to cited text no. 8
    


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