Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 58, Num. 3, 2010, pp. 392-397

Neurology India, Vol. 58, No. 3, May-June, 2010, pp. 392-397

Original Article

Decompressive surgery for severe cerebral venous sinus thrombosis

Rahul Lath, Sudhir Kumar, Rajesh Reddy, Gnana Ratnam Boola, Amitava Ray, Subhashini Prabhakar, Alok Ranjan

Stroke Unit, Department of Neurosciences, Apollo Hospitals, Hyderabad - 500 033, Andhra Pradesh, India
Correspondence Address: Sudhir Kumar, Stroke Unit, Department of Neurosciences, Apollo Hospitals, Hyderabad - 500 033, India, drsudhirkumar@yahoo.com

Date of Acceptance: 21-Sep-2009

Code Number: ni10102

PMID: 20644266
DOI: 10.4103/0028-3886.65923

Abstract

Background : Cerebral venous sinus thrombosis (CVST) is one of the common causes of stroke in young people. Mortality in CVST, in addition to progressive thrombosis, is related to elevated intracranial pressure causing transtentorial herniation. The role of decompressive surgery in CVST is not well established.
Aims : We report our experience with decompressive surgery in CVST and review the literature.
Settings and Design : This is a retrospective study carried out in the Stroke Unit of a multispeciality tertiary care hospital in south India.
Materials and Methods : The medical records of patients admitted with the diagnosis of CVST between December 2003 and July 2009 were reviewed. The clinical presentation, etiology, medical management, indications for surgery and outcomes were assessed for patients undergoing decompressive surgery. The sensorium was assessed using the Glasgow Coma Scale (GCS), while the outcome was assessed using the modified Rankin scale (mRS). Descriptive statistics were used as appropriate.
Results : One hundred and six patients were admitted with the diagnosis of CVST during the study period. Eleven patients (10%) underwent decompressive surgical procedure. Indications for surgery included a low GCS at admission with large infarct on the computed tomography scan, mass effect and midline shift, clinical and radiological signs of transtentorial herniation, deterioration in the sensorium in spite of anti-edema measures and postthrombolysis hematoma. Eight patients (73%) had a good outcome while three patients (27%) died. Of the patients who died, two had a low GCS and bilaterally nonreactive pupils before the surgery while one had thrombosis of the deep venous system.
Conclusion : Decompressive surgery for patients with large cerebral venous infarcts is a life-saving procedure. Patients with CVST who develop clinical and radiological features of transtentorial herniation either at presentation or during the course of medical management may benefit from decompressive surgery.

Keywords: Cerebral venous sinus thrombosis, decompressive craniectomy, functional outcome, mortality

Introduction

Cerebral venous sinus thrombosis (CVST) is a disorder of varied etiology, with an annual incidence of three to four cases per 1 million population. ^[1] CVST accounts for 10-20% of the etiology of young strokes in India. ^[2],[3] The therapeutic options in CVST include antithrombotic therapy with conventional heparin, low molecular weight heparin (LMWH) or oral anticoagulants and intravenous or intrasinus local thrombolysis. ^[1],[3],[4] Anti-epileptic drugs and anti-edema agents are used as per the clinical situation. Poor outcome in the International study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT) was 13.6%. ^[5] The factors predicting an unfavorable outcome included male sex, age >37 years, coma, mental status disorder, intracranial hemorrhage on admission, thrombosis of the deep venous system, central nervous system (CNS) infection and cancer. During the acute phase of CVST, 4.3% of the patients died and 3.4% died within 30 days from symptom onset. ^[6] The main cause of death in the acute phase was neurological deterioration secondary to transtentorial herniation. Severe increase in intracranial pressure (ICP) in the acute phase of CVST is usually treated medically, often without success. There have been a few reports of favorable outcome following decompressive surgery in the acute phase of CVST. ^{[7],[8],[9],[10],[11]} We report our experience with decompressive surgery in CVST.

Materials and Methods

The medical records of patients admitted with the diagnosis of CVST between December 2003 and July 2009 were reviewed.

Diagnosis of CVST

The diagnosis of CVST was made on the basis of presenting clinical features and radiological findings, computerized tomography (CT) scan and/or magnetic resonance (MR) scan with venography.

Medical management

Patients were admitted to the intensive care unit (ICU). All patients had a detailed neurological evaluation and assessment of sensorium by Glasgow Coma Scale (GCS). All patients were initially treated with low molecular-weight heparin (LMWH) or conventional heparin infusion. Partial thromboplastin time (PTT) monitoring was done to adjust the dose of conventional heparin. Anti-epileptic drugs and anti-edema agents were used as deemed necessary. Etiological work-up included pretreatment thombophilia screening (Protein S, Protein C, Antithrombin III), serum homocysteine and antiphospholipid antibodies.

Indications for surgery

Indications for surgery included: a low GCS (8 or less) at admission with large infarct on the CT/MRI scan; significant mass effect and midline shift on CT/MRI scan; clinical and radiological signs of transtentorial herniation; deterioration in the sensorium despite anti-edema measures; and post intra-sinus thrombolysis hematoma.

Surgical procedure and postoperative management

The site and size of decompressive craniectomy was based on the location and size of the venous infarct [Figure - 1] and [Figure - 2]. A bone flap larger than the venous infarct was made and the dura was widely opened and patched with the pericranium. The wound was closed in layers over a drain. In three patients, the bone flap was replaced after evacuation of the hemorrhagic infarct in the noneloquent regions [Figure - 3] and [Figure - 4]. One patient with postthrombolysis hematoma had removal of the temporal hematoma [Figure - 5] and [Figure - 6].

Postoperatively, patients were monitored in the ICU and were mechanically ventilated in most cases. Twelve hours after surgery, anticoagulation with either heparin infusion or LMWH was resumed and subsequently changed to oral anticoagulation with warfarin. Oral anticoagulation was continued for at least 6 months. Patients with a craniectomy had a cranioplasty performed 3-6 months following the decompressive surgery.

Assessment of outcome

Patients were followed-up for at least 6 months. Functional outcome among survivors was assessed using the modified Rankin scale (mRS). ^[12] Mortality rate was also calculated.

Results

Patient characteristics

One hundred and six patients were admitted with the diagnosis of CVST during the study period. Eleven patients (10%) underwent decompressive surgery, while three patients (2.8%) underwent a lumbar-peritoneal shunt in the acute phase of CVST. The clinical details of these 11 patients are summarized in [Table - 1] and [Table - 2]. The mean was 30.4 years (range 18-46 years) and the female (73%) was predominant gender.

Indications for surgery included: a low GCS at admission with large infarct on the CT scan in two; mass effect and midline shift in two; clinical and radiological signs of transtentorial herniation in two; deterioration in the sensorium despite best medical management in four; and postthrombolysis haematoma in one. Six patients (55%) underwent decompressive surgery within 24 h of admission. Three patients who underwent a shunt surgery had normal sensorium but developed progressive deterioration in visual acuity with severe papilledema, despite anticoagulation and anti-edema treatment.

Outcome

Three patients (27%) died following the decompressive surgery (one each on the first, second and sixth postoperative days). Of the three patients who died, two had a low GCS and bilaterally nonreactive pupils at admission, while one patient had a normal sensorium but continued to deteriorate in spite of anticoagulation and anti-edema measures. This patient continued to deteriorate even after a decompressive craniectomy. He had features of deep venous system involvement. Survivors were followed for a mean period of 7.4 months (range 6-10 months). At follow-up, eight patients (73%) had a good functional outcome (mRS 0-2). No surgery-related complications were noted.

Discussion

Decompressive hemicraniectomy for malignant arterial infarctions is now well accepted. It reduces mortality and results in better functional outcome. ^{[13],[14],[15]} The mortality from CVST has been variously reported and range from 0 to 36%, it was 4.3% in the the ISCVT. ^[6] The cause of death in the majority of these patients was neurological deterioration secondary to transtentorial herniation. The investigators involved in this study have suggested that decompressive craniectomy should be considered in patients with progressive transtentorial herniation in order to decrease the mortality rates. ^[5],[6]

Medical management of raised ICP consists of mannitol, steroids and hyperventilation. Mannitol has a rapid onset of action and is very effective in reducing ICP. Thus, mannitol is often the treatment of choice in a patient with CVST with features of elevated ICP. However, mannitol has several limitations. Its use is limited in the setting of hypotension, renal failure and cardiac failure. Further, there is the risk of rebound increase in ICP following discontinuing mannitol. Osmotic agents might be harmful in patients with venous outflow obstruction because these agents may not be quickly eliminated from intracerebral circulation as in the case of other disease states. ^[4] Corticosteroids efficacy is unproven and are harmful as they promote the thrombotic process. ^[4] A recent study had shown that steroids in the acute phase of CVST were not useful and were detrimental in patients without parenchymal cerebral lesions. ^[16] In patients with massive space-occupying venous infarct, these management strategies may not be effective. There are no randomized trials comparing decompressive surgery with best medical management in CVST. However, it is evident that in severe cases with life-threatening unilateral large hemorrhagic infarct, decompressive surgery may be the only life saving option. ^[4] In view of the relatively low incidence of CVST and fewer number of patients requiring surgery (1.4% in ISCVT), a randomized trial would be difficult. A prospective multicenter study, ISCVT-2, is underway and the data following decompressive surgery in CVST are being collected. ^[17]

In the pre-CT/MRI scan era, the largest number (34 patients) of decompressive surgeries in CVST was reported by Nagpal in 1983. ^[18] The preoperative neurological status and outcome assessment have not been mentioned. The author has stated that 56% of the patients undergoing surgery survived while 44% died. The outcomes of decompressive surgery in CVST in the post-CT/MRI scan era are summarized in [Table - 3]. It is evident from these reports that decompressive surgery in CVST has survival benefit and this is in spite of a poor preoperative neurological status. This survival benefit was also seen in patients with bilateral nonreactive pupils. ^[8] However, two of our patients with bilateral nonreactive pupils did not survive. One them was a case of leukemia. We feel that decompressive craniectomy should be offered before pupillary changes. Decompressive craniectomy may be beneficial in patients who develop hematoma following thrombolysis therapy. Characteristics of patients who may benefit or who may not benefit from decompressive surgery are given in [Table - 4].

Of the 106 patients with CVST, three patients had lumbar-peritoneal shunt (2.8%) surgery in the acute phase of illness, the indications were deterioration in vision and severe papilledema, despite adequte anticoagulation. None of them had significant venous infarct and altered mental status. Vision improved in all the three patients following the shunt procedure. Shunt surgery was the treatment in 1.6% of the patients in the ISCVT study. ^[5],[6] A lumbar-peritoneal shunt would be indicated in patients with visual deterioration in the acute phase. ^[19] Shunts may be required in the chronic phase of illness in those patients who develop features of elevated intracranial hypertension. Surgical procedures like open thrombectomy and venous bypass may not be of much significance in the treatment of CVST in view of the endovascular modalities currently available. ^[20],[21]

References

1.	Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005;352:1791-8.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.	Banerjee AK. Pathology of cerebrovascular disease. Neurol India 2000;48:305-7.  Back to cited text no. 2  [PUBMED]
3.	Nagaraja D, Sarma GR. Treatment of cerebral sinus/venous thrombosis. Neurol India 2002;50:114-6.  Back to cited text no. 3  [PUBMED]
4.	Einhdupl K, Bousser MG, de Bruijn SF, Ferro JM, Martinelli I, Masuhr F, et al. EFNS guideline on the treatment of cerebral venous and sinus thrombosis. Eur J Neurol 2006;13:553-9.  Back to cited text no. 4
5.	Ferro JM, Canhao P, Stam J, Bousser MG, Barinagarrementeria F; ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004;35:664-70.   Back to cited text no. 5
6.	Canhao P, Ferro JM, Lindgren AG, Bousser MG, Stam J, Barinagarrementeria F; ISCVT Investigators. Causes and predictors of death in cerebral venous thrombosis. Stroke 2005;36:1720-5.   Back to cited text no. 6
7.	Girot M, Ferro JM, Canhao P, Stam J, Bousser MG, Barinagarrementeria F, et al. Predictors of outcome in patients with cerebral venous thrombosis and intracerebral hemorrhage. Stroke 2007;38:337-42.   Back to cited text no. 7
8.	Stefini R, Latronico N, Cornali C, Rasulo F. Bollati A. Emergent decompressive craniectomy in patients with fixed dilated pupils due to cerebral venous and dural sinus thrombosis: report of three cases. Neurosurgery 1999;45:626-29.   Back to cited text no. 8
9.	Keller E, Pangalu A, Fandino J, Kfn? D, Yonekawa Y. Decompressive craniectomy in severe cerebral venous and dural sinus thrombosis. Acta Neurochir Suppl 2005;94:177-83.   Back to cited text no. 9
10.	Lin HS, Lin JF, Chang CK, Tsai CC, Chen SJ. Cerebral sinus thrombosis with intracerebral hemorrhage in pregnancy: a case report. Acta Neurologica Taiwanica 2008;17:189-93.   Back to cited text no. 10  [PUBMED]
11.	Lanterna LA, Gritti P, Manara O, Grimod G, Bortolotti G, Biroli F. Decompressive surgery in malignant dural sinus thrombosis: report of 3 cases review of literature. Neurosurg Focus 2009;26:E5.  Back to cited text no. 11
12.	van Swieten JC, Koudstaal PJ, Visser MC, Schouten HJ, van Gijn J. Interobserver agreement for the assessment of handicap in stroke patients. Stroke 1988;19:604-7.  Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.	Jattler E, Schwab S, Schmiedek P, Unterberg A, Hennerici M, Woitzik J, et al. DESTINY Study Group. Decompressive Surgery for the Treatment of Malignant Infarction of the Middle Cerebral Artery (DESTINY): a randomized, controlled trial. Stroke 2007;38:2518-25.  Back to cited text no. 13
14.	Vahedi K, Vicaut E, Mateo J, Kurtz A, Orabi M, Guichard JP, et al. Investigators Sequential-design, multicenter, randomized, controlled trial of early decompressive craniectomy in malignant middle cerebral artery infarction (DECIMAL Trial). Stroke 2007;38:2506-17.  Back to cited text no. 14  [PUBMED]  [FULLTEXT]
15.	Hofmeijer J, Kappelle LJ, Algra A, Amelink GJ, van Gijn J, van der Worp HB; HAMLET investigators. Surgical decompression for space-occupying cerebral infarction (the Hemicraniectomy After Middle Cerebral Artery infarction with Life-threatening Edema Trial [HAMLET]): a multicentre, open, randomised trial. Lancet Neurol 2009;8:326-33.   Back to cited text no. 15  [PUBMED]  [FULLTEXT]
16.	Canhao P, Cortesao A, Cabral M, Ferro JM, Stam J, Bousser MG. ISCVT Investigators. Are steroids useful to treat cerebral venous thrombosis? Stroke 2008;39:105-10.  Back to cited text no. 16
17.	Coutinho JM, Majoie CB, Coert BA, Stam J. Decompressive hemicraniectomy in cerebral sinus thrombosis: consecutive case series and review of the literature. Stroke 2009;40:2233-5.  Back to cited text no. 17  [PUBMED]  [FULLTEXT]
18.	Nagpal RD. Dural sinus and cerebral venous thrombosis. Neurosurg Rev 1983;6:155-60.  Back to cited text no. 18  [PUBMED]
19.	Enam SA. Role of surgery in cerebral venous sinus thrombosis. J Pak Med Assoc 2006;56:543-7.  Back to cited text no. 19  [PUBMED]
20.	Stam J, Majoie CB, van Delden OM, van Lienden KP, Reekers JA. Endovascular thrombectomy and thrombolysis for severe cerebral sinus thrombosis: a prospective study. Stroke 2008;39:1487-90.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]
21.	Kumar S, Rajshekher G, Reddy CR, Venkateswarlu J, Prabhakar S. Intrasinus thrombolysis in cerebral venous sinus thrombosis: Single-center experience in 19 patients. Neurol India 2010;58:225-9.  Back to cited text no. 21  [PUBMED]

Copyright 2010 - Neurology India

The following images related to this document are available:

Photo images