Multimodal imaging in Behçet disease uveitis manifesting with sequential bilateral prepapillary inflammatory vitreous exudate

Abstract Objective: To describe a case of Behçet disease (BD) uveitis manifesting with sequential bilateral prepapillary inflammatory vitreous exudate (PIVE) documented with multimodal imaging, including swept source optical coherence tomography (SS OCT) and OCT angiography. Material and Methods: A single case report.Results: A 37-year-old man developed sequential bilateral PIVE, initially misdiagnosed as toxoplasmic neuroretinitis, before extraocular features of BD became evident and the patient treated accordingly. SS OCT showed at the acute phase in both eyes a typical “mushroom-shaped” prepapillary hyperreflectivity of the PIVE. SS OCTA demonstrated a corresponding prepapillary hypointense area due to shadowing effect, decreasing in size while scanning deeper layers. It also detected peripapillary retinal hypervascularity in both eyes and a sectoral area of flow signal loss in the first involved left eye. Visual acuity improved following the resolution of the PIVE and associated acute inflammatory changes in both eyes. The left eye showed residual optic disc pallor and retinal nerve fiber layer defects.Conclusion: Sequential bilateral PIVE may occur in the absence of other clinical features of BD. SS OCT and OCTA can provide useful information for the diagnosis and management of PIVE and underlying optic nerve inflammation.

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Sequential bilateral Behçet’s neuroretinitis associated with prepapillary vitreous exudate: case report

Journal of Ophthalmic Inflammation and Infection ◽

10.1186/s12348-020-00226-y ◽

2020 ◽

Vol 10 (1) ◽

Author(s):

Imen Ksiaa ◽

Safa Ben Aoun ◽

Sourour Zina ◽

Dhouha Nefzi ◽

Sana Khochtali ◽

...

Keyword(s):

Case Report ◽

Single Case ◽

Signal Loss ◽

Fiber Layer ◽

Swept Source ◽

Single Case Report ◽

Shadowing Effect ◽

Eye Involvement ◽

The Right ◽

Inflammatory Changes

Abstract Objective To describe a case of Behçet disease (BD) uveitis manifesting with sequential bilateral neuroretinitis associated with prepapillary inflammatory vitreous exudate (PIVE). Material and methods A single case report documented with multimodal imaging. Results A 37-year-old man developed neuroretinitis with associated PIVE in the left eye. He was diagnosed with ocular toxoplasmosis and treated accordingly based on positive serologic testing and negative work-up for other entities, including BD. The disease course was favorable, but 1 year later a similar neuroretinitis developed in the right eye. Extraocular features of BD became evident only at the time of the second eye involvement, and the patient received corticosteroid and immunosuppressive therapy. Swept source (SS) OCT showed at the acute phase in both eyes a typical “mushroom-shaped” prepapillary hyperreflectivity of the PIVE. SS OCT angiography (OCTA) demonstrated a corresponding prepapillary hypointense area due to shadowing effect, decreasing in size while scanning deeper layers. It also detected peripapillary retinal hypervascularity in both eyes and a sectoral area of flow signal loss in the first involved left eye. Visual acuity improved following the resolution of the PIVE and associated acute inflammatory changes in both eyes. The left eye showed residual optic disc pallor and retinal nerve fiber layer defects. Conclusion Sequential bilateral neuroretinitis associated with PIVE may occur before other clinical features of BD become evident. SS OCT and OCTA can provide useful information for the diagnosis and management of this rare, but typical, ocular manifestation of BD uveitis.

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Sequential bilateral Behçet’s neuroretinitis associated with prepapillary vitreous exudate: case report

10.21203/rs.3.rs-44040/v3 ◽

2021 ◽

Author(s):

Imen Ksiaa ◽

Safa Ben Aoun ◽

Sourour Zina ◽

Dhouha Nefzi ◽

Sana Khochtali ◽

...

Keyword(s):

Case Report ◽

Single Case ◽

Signal Loss ◽

Fiber Layer ◽

Single Case Report ◽

Shadowing Effect ◽

Eye Involvement ◽

One Year ◽

The Right ◽

Inflammatory Changes

Abstract Objective: To describe a case of Behçet disease (BD) uveitis manifesting with sequential bilateral neuroretinitis associated with prepapillary inflammatory vitreous exudate (PIVE). Material and Methods: A single case report documented with multimodal imaging.Results: A 37-year-old man developed neuroretinitis with associated PIVE in the left eye. He was diagnosed with ocular toxoplasmosis and treated accordingly based on positive serologic testing and negative work-up for other entities, including BD. The disease course was favorable, but one year later a similar neuroretinitis developed in the right eye. Extraocular features of BD became evident only at the time of the second eye involvement, and the patient received corticosteroid and immunosuppressive therapy. SS OCT showed at the acute phase in both eyes a typical “mushroom-shaped” prepapillary hyperreflectivity of the PIVE. SS OCTA demonstrated a corresponding prepapillary hypointense area due to shadowing effect, decreasing in size while scanning deeper layers. It also detected peripapillary retinal hypervascularity in both eyes and a sectoral area of flow signal loss in the first involved left eye. Visual acuity improved following the resolution of the PIVE and associated acute inflammatory changes in both eyes. The left eye showed residual optic disc pallor and retinal nerve fiber layer defects.Conclusion: Sequential bilateral neuroretinitis associated with PIVE may occur before other clinical features of BD become evident. SS OCT and OCTA can provide useful information for the diagnosis and management of this rare, but typical, ocular manifestation of BD uveitis.

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Multimodal Imaging Characteristics and Functional Test Findings in a Case of Acute Macular Neuroretinopathy Accompanied by Behçet Disease

Ocular Immunology and Inflammation ◽

10.1080/09273948.2020.1751857 ◽

2020 ◽

pp. 1-7

Author(s):

Figen Batıoğlu ◽

Özge Yanık ◽

Sibel Demirel ◽

Emin Özmert

Keyword(s):

Multimodal Imaging ◽

Functional Test ◽

Behçet Disease ◽

Behcet Disease ◽

Acute Macular Neuroretinopathy ◽

Imaging Characteristics

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Unusual Late Onset of Parenchymal Neuro-Behçet Disease

Case Reports in Neurological Medicine ◽

10.1155/2016/5720319 ◽

2016 ◽

Vol 2016 ◽

pp. 1-3

Author(s):

Wai Wai Miller ◽

Demetrios Konstas ◽

Chetan Gandhy ◽

Derrick Robertson

Keyword(s):

Late Onset ◽

Neurological Deficits ◽

Behçet Disease ◽

Inflammatory Disorder ◽

Oral Lesions ◽

Behcet Disease ◽

Mild Disease ◽

Multisystem Involvement ◽

Proper Diagnosis ◽

Inflammatory Changes

Neuro-Behçet disease (NBD) is a multisystem inflammatory disorder characterized by oral lesions, genital lesions, uveitis, and neurological deficits. If left untreated, it may lead to worsening neurological function and can be fatal. Here we present a case of a 52-year-old woman who was diagnosed with Behçet disease (BD) as a teenager and had a relatively mild disease course. Decades later after her initial DB diagnosis, she presented to our hospital with a chief complaint of headache. She did not have focal neurological deficits or any active mucosal lesions. Upon further investigation, the patient was found to have multiple inflammatory changes on neuroimaging and abnormal cerebrospinal fluid (CSF), consistent with the diagnosis of NBD. She was treated with intravenous corticosteroid therapy and her symptoms resolved. Although our patient presented with minimal symptoms decades after her initial diagnosis, any neurological complaint warranted a thorough investigation for a proper diagnosis and treatment given the multisystem involvement of BD.

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