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CASE REPORT
Year : 2020  |  Volume : 12  |  Issue : 1  |  Page : 27-29

Superior sagittal sinus thrombosis presenting as diplopia in pregnancy


Department of Ophthalmology, AIIMS, Bhubaneswar, India

Date of Submission23-Mar-2020
Date of Acceptance11-Jul-2020
Date of Web Publication27-Aug-2020

Correspondence Address:
Dr. Sucheta Parija
Additional Professor, Department of Ophthalmology, AIIMS, Bhubaneswar
India
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DOI: 10.4103/sjopthal.sjopthal_6_20

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  Abstract 

Cerebral sagittal sinus thrombosis is a very rare condition and presents as diagnostic challenge due to its varied presentations. Most common signs and symptoms are headache, blurring of vision, diplopia, and papilledema. Pregnancy and puerperium constitute one of its rare etiological factors. A high index of clinical suspicion of the disease is important when a pregnant female presents with stroke like symptoms of headache, hemiparesis, diplopia, and seizures in the early stages of pregnancy because early neuroimaging can clinch the diagnosis. Management with adequate dosage of anticoagulants can be life-saving and can prevent severe visual loss. Here, we report and discuss the presentation and management of a case of cerebral superior sagittal sinus thrombosis presenting as diplopia in a young primigravid in the first trimester of pregnancy.

Keywords: Papilledema, pregnancy, superior sagittal sinus thrombosis


How to cite this article:
Parija S, Banerjee A, Naik S. Superior sagittal sinus thrombosis presenting as diplopia in pregnancy. Sudanese J Ophthalmol 2020;12:27-9

How to cite this URL:
Parija S, Banerjee A, Naik S. Superior sagittal sinus thrombosis presenting as diplopia in pregnancy. Sudanese J Ophthalmol [serial online] 2020 [cited 2020 Oct 28];12:27-9. Available from: https://www.sjopthal.net/text.asp?2020/12/1/27/293636


  Introduction Top


Cerebral venous sinus thrombosis (CVST) is a rare disease in pregnancy and puerperium, the incidence ranging from 1:10,000 to 1:25,000.[1] It is a life-threatening condition with diagnostic challenges, because of its varied clinical presentations.[2] The most frequent symptoms and signs are headache, focal or generalized seizures, unilateral or bilateral paresis, and papilledema. Risk factors for the development of CVST include hypercoagulable states, use of oral contraceptives, malignancy, otitis, mastoiditis, dehydration, low cerebral blood flow, pregnancy, and puerperium.[2],[3]

This case highlights the significance to examine for CVST risk factors when a young pregnant women presents with stroke-like symptoms of headache, hemiparesis, and diplopia in the first trimester of pregnancy.


  Case Report Top


A 30-year-old primigravida presented to the emergency department with complaints of nausea and vomiting along with blurring of vision of 3 days' duration during the first trimester of pregnancy. She was diagnosed as hyperemesis gravidum and admitted to the obstetrics ward. After 2 days, she developed sudden onset of headache and double vision and was referred for ophthalmology consultation.

She had no past history of fever, facial, or sinus infections. No recent history of head trauma or any episodes of seizure attacks. She also had no medical history of hypertension, atrial fibrillation, or hematological disorder, and was not on any medications.

On examination, she was alert and conscious with normal vital signs. Her best-corrected visual acuity (BCVA) was 20/60 in both eyes. Slitlamp biomicroscopic examination of anterior chamber was normal. Pupil showed sluggish reaction to light in both the eyes. Intraocular pressure was 14 mm Hg in both eyes. Ocular movements showed mild limitation of abduction (−1/2) in the left eye. Fundus examination showed elevated and blurred disc margins along with flame shaped hemorrhages in both eyes, suggestive of papilledema [Figure 1]. Neurological examination revealed mild weakness in left-sided upper limb. Humphrey visual fields revealed peripheral field changes. To rule out the suspicion of raised intracranial tension (ICT), magnetic resonance imaging (MRI) scan of brain and orbit was advised. Routine hematological tests and coagulation profile of the patient were within normal limits. Echocardiogram was also normal.
Figure 1: Fundus photograph of (a) right and (b) left eye showing blurred and elevated disc margin, suggestive of papilledema

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MRI, axial T2W MRI showed loss of flow void in middle part of superior sagittal sinus without any parenchymal abnormality [Figure 2]a and [Figure 2]b. She was further advised for magnetic resonance venogram (MRV), which showed nonvisualization of anterior two-third of superior sagittal sinus and left transverse sinus suggesting sinus thrombosis [Figure 2]c.
Figure 2: Axial T2W magnetic resonance imaging (a) and (b) shows loss of flow void in middle part of superior sagittal sinus (arrow in a), without any parenchymal abnormality. (c) Contrast MR venography shows nonvisualization of anterior two-third of superior sagittal sinus and left transverse sinus suggesting sinus thrombosis

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The patient was diagnosed as superior sagittal sinus thrombosis with the left lateral rectus paresis. As per the neurologist advice, the patient was started on low molecular weight (LMW) heparin 0.4 IU subcutaneously in once daily dosing, intravenous antibiotic ceftriaxone 1 g twice daily and oral syrup glycerol 30 ml once daily till the condition stabilized. Gradually, over a period of 3 months, the patient's left-sided lateral rectus paresis resolved and BCVA improved to 20/30 in both eyes. Fundus evaluation showed resolved papilledema [Figure 3]. Optical coherence tomography of optic nerve head showed resolved papilledema [Figure 4]. At 1 year follow-up period, she was orthophoric and had delivered a full-term normal baby.
Figure 3: Fundus photograph of (a) right and (b) left eye showing normal disc and vessels in the same eye after resolution of disc edema

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Figure 4: Optical Coherence Tomography showing (a) increased retinal nerve fiber layer thickness in acute stage of papilledema, (b) resolution of nerve fiber layer thickness after three months

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  Discussion Top


CVST is a potentially life-threatening condition as it causes cerebral infarction. Among the varied etiological risk factors, pregnancy-postpartum state is an important one. The rate of occurrence of CVST is 10–13 times more common during puerperium than in pregnancy with very rare presentation in first trimester.[4] Pathophysiology of CVST in pregnancy is due to hypercoagulable state, which is further aggravated by anemia and dehydration, leading to thrombus formation. This results in venous congestion, cerebral edema, raised intracranial pressure with mass effect and finally cerebral infarction.[2],[4] According to the International Study on Cerebral Vein and Dural Sinus Thrombosis, the most commonly affected sinuses in order of frequency are transverse sinus, superior sagittal sinus and straight sinus.[5]

CVST commonly presents with signs of raised intracranial pressure. Ocular manifestations are headache (the most common symptom), loss of vision, diplopia, and cranial nerve palsies.[6] In the acute stage due to raised ICT, there is limitation of abduction, a false localizing sign. Papilledema is the most common ocular sign seen in 28% of cases.[5] Other systemic signs include focal seizures, hemiparesis, aphasia, and altered sensorium.

CVST is mainly diagnosed by radiological examination. A noncontrast computed tomography may not demonstrate any abnormal findings in patients presenting with no neurological deficits but MRI shows loss of signal void appearance diagnostic of CVST. At present, MRI along with MRV is considered to be the most accurate and safe method to diagnose cerebral venous occlusions.[7]

The current treatment of CVST involves thrombolysis.[8],[9] The European Stroke Organization guideline for the diagnosis and treatment of CVST recommends use of parenteral anticoagulants, preferentially LMW heparins in the acute phase.[8] As per the above guidelines, our patient was started on 0.4 IU subcutaneous LMW heparin as the thrombolytic agent and continued for 6 months. The current protocols recommend use of thrombolytics for 6 months in cases related to pregnancy and puerperium.[9],[10] The aim is always to keep INR between 2 and 3 to prevent further thromboembolism. As per the treatment regimen, our patient recovered within 3 months and delivered a full-term baby at 38 weeks of gestation. The European Society of Neurology suggests that these women should be asked to avoid oral contraceptives containing estrogen in future.[8] It also suggests that future pregnancies are safe, but prophylactic LMW heparin is continued throughout pregnancy and puerperium.[8]

Cantú and Barinagarrementeria[2] reported that obstetric cases if managed properly, have a better prognosis than CVST due to other causes. Overall, the prognosis is around 80% in these cases.[4]


  Conclusion Top


CTVS during pregnancy must be kept as a differential diagnosis along with other intracranial pathologies of raised ICT. Ophthalmologist must be aware of this condition and play a crucial role in early diagnosis and referral. Multidisciplinary management by adequate anticoagulant therapy causing venous recanalization, in pregnant women with CVST cases reduces the mortality of the mother and child as well as ensures good visual recovery.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
McCaulley JA, Pates JA. Postpartum cerebral venous thrombosis. Obstet Gynecol 2011;118:423-5.  Back to cited text no. 1
    
2.
Cantú C, Barinagarrementeria F. Cerebral venous thrombosis associated with pregnancy and puerperium. Review of 67 cases. Stroke 1993;24:1880-4.  Back to cited text no. 2
    
3.
Lemke DM, Hacein-Bey L. Cerebral venous sinus thrombosis. J Neurosci Nurs 2005;37:258-64.  Back to cited text no. 3
    
4.
Munira Y, Sakinah Z, Zunaina E. Cerebral venous sinus thrombosis presenting with diplopia in pregnancy: A case report. J Med Case Rep 2012;6:336.  Back to cited text no. 4
    
5.
Ferro JM, Canhao P, Stam J, Bousser MG, Barinagarrementeria F. for the ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis – Results of International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke 2004;35:664-70.  Back to cited text no. 5
    
6.
Wang D, Fang B, Wei S. Analysis of clinical features of ocular presentation in cranial venous sinus thrombosis. Eur J Med Res 2011;16:324-7.  Back to cited text no. 6
    
7.
El Damarawy EA, El-Nekiedy AE, Fathi AM, Eissa AE, Darweesh RM. Role of magnetic resonance venography in evaluation of cerebral veins and sinuses occlusion. Alex J Med 2012;48:29-34.  Back to cited text no. 7
    
8.
Ferro JM, Bousser MG, Canhão P, Coutinho JM, Crassard I, Dentali F, et al. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis-Endorsed by the European Academy of Neurology. Eur J Neurol 2017;24:1203-13.  Back to cited text no. 8
    
9.
Allroggen H, Abbott RJ. Cerebral venous sinus thrombosis. Postgrad Med J 2000;76:12-5.  Back to cited text no. 9
    
10.
Tanislav C, Siekmann R, Sieweke N, Allendörfer J, Pabst W, Kaps M, et al. Cerebral vein thrombosis: Clinical manifestation and diagnosis. BMC Neurol 2011;11:69.  Back to cited text no. 10
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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