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Bilateral Papilledema and Partially Empty Sella in a Woman in Her 30s

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 30s was referred for evaluation of bilateral papilledema and progressive visual loss in the right eye, which deteriorated to blindness in 3 months. She denied any other neurologic symptoms, such as headache, nausea, vomiting, seizures, and paresis. She had a 4-year history of Behçet disease (BD), and her condition had been stabilized with thalidomide. Her optic neuropathy of BD had been treated with high-dose intravenous methylprednisolone for 4 days followed by oral prednisone for a month without improvement.

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Cerebral venous thrombosis

C. Obtain cerebral magnetic resonance venography or computed tomographic venography

Optic neuropathy of BD usually occurs with systemic flare-up and responds well to steroids.1 This patient showed no signs of relapse of BD, and her vision continued to deteriorate despite the use of corticosteroids. Therefore, it is not proper to use immunosuppressants and corticosteroids (choices A and B) before a definite diagnosis. Although the patient lacked neurological symptoms other than visual loss, the bilateral papilledema and partially empty sella indicated intracranial hypertension, the possible causes of which include arteriovenous malformations, intracranial mass lesions, obstruction to venous drainage, decreased flow through arachnoid granulations, and idiopathic intracranial hypertension.2 Moreover, a negative magnetic resonance imaging result cannot fully rule out cerebral vascular abnormalities, among which cerebral venous thrombosis (CVT) accounts for 9.4% of the presumed idiopathic intracranial hypertension.3 In suspected CVT, magnetic resonance venography or computed tomographic venography (choice C) is recommended when the magnetic resonance imaging result is negative.4 Therefore, a cerebral magnetic resonance venography of the patient was performed, showing thromboses of the superior sagittal sinus and the right transverse and sigmoid sinus (Figure 2). Draining cerebrospinal fluid is suggested when intracranial hypertension results in vision-threatening papilledema.5 Therefore, a lumber puncture was performed to confirm intracranial hypertension (showing opening pressure of 30.5 cm H2O) and to remove cerebrospinal fluid. Acetazolamide was also initiated to lower the intracranial pressure. Anticoagulation therapy of heparin was started to facilitate recanalization and to prevent recurrent CVT and other venous thrombosis. Endovascular thrombolysis was performed to prevent visual loss of the left eye. The catheter cerebral angiography (choice D), which is invasive, is only considered when magnetic resonance venography or computed tomography venography results are inconclusive or an endovascular procedure is performed.4 Therefore, it was performed along with endovascular thrombolysis to visualize thrombosed veins.

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Article Information

Corresponding Author: Lin Lu, MD, PhD, State Key Laboratory of Ophthalmology, Image Reading Center, Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangzhou 510060, China (drlulin@126.com).

Published Online: February 28, 2019. doi:10.1001/jamaophthalmol.2018.6948

Conflict of Interest Disclosures: None reported.

Funding/Support: This work was supported by grant 81770971 from the National Natural Science Foundation of China (Dr Luo).

Role of the Funder/Sponsor: The funder had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Al-Araji  A, Kidd  DP.  Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management.  Lancet Neurol. 2009;8(2):192-204. doi:10.1016/S1474-4422(09)70015-8PubMedGoogle ScholarCrossref
2.
Wall  M.  Idiopathic intracranial hypertension.  Neurol Clin. 2010;28(3):593-617. doi:10.1016/j.ncl.2010.03.003PubMedGoogle ScholarCrossref
3.
Lin  A, Foroozan  R, Danesh-Meyer  HV, De Salvo  G, Savino  PJ, Sergott  RC.  Occurrence of cerebral venous sinus thrombosis in patients with presumed idiopathic intracranial hypertension.  Ophthalmology. 2006;113(12):2281-2284. doi:10.1016/j.ophtha.2006.05.065PubMedGoogle ScholarCrossref
4.
Saposnik  G, Barinagarrementeria  F, Brown  RD  Jr,  et al; American Heart Association Stroke Council and the Council on Epidemiology and Prevention.  Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association.  Stroke. 2011;42(4):1158-1192. doi:10.1161/STR.0b013e31820a8364PubMedGoogle ScholarCrossref
5.
Bousser  MG, Ferro  JM.  Cerebral venous thrombosis: an update.  Lancet Neurol. 2007;6(2):162-170. doi:10.1016/S1474-4422(07)70029-7PubMedGoogle ScholarCrossref
6.
Stam  J.  Thrombosis of the cerebral veins and sinuses.  N Engl J Med. 2005;352(17):1791-1798. doi:10.1056/NEJMra042354PubMedGoogle ScholarCrossref
7.
Subash  M, Parmar  DN.  Papilloedema as the sole presenting feature of postpartum cerebral venous sinus thrombosis.  Can J Ophthalmol. 2009;44(2):e1-e2. doi:10.3129/i09-016PubMedGoogle ScholarCrossref
8.
Ferro  JM, Bousser  MG, Canhão  P,  et al; European Stroke Organization.  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(10):1203-1213. doi:10.1111/ene.13381PubMedGoogle ScholarCrossref
9.
Lenz  RA, Saver  J.  Venous sinus thrombosis in a patient taking thalidomide.  Cerebrovasc Dis. 2004;18(2):175-177. doi:10.1159/000079739PubMedGoogle ScholarCrossref
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