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.
PurposeTo compare clinical and multimodal imaging findings in patients with PHOMS and correlate them with the etiology.MethodsA prospective descriptive comparative study carried out at the Ophthalmology Department of Habib Thameur University Hospital of Tunis between March 1st 2020 and March 30th 2021. Inclusion criteria were presence of blurred optic disc margins. Patients with optic disc drusen were excluded. All patients underwent complete ophthalmic examination, 24.2 Humphrey visual field (VF), fluorescein angiography (FA) and Swept‐Source Optical Coherence Tomography (SS‐OCT). Comparative Mann Whitney and chi‐squared tests were performed.ResultsSixteen eyes of 8 patients were eligible for the study. Mean age was 41.63 years +/‐ 10.49 years. Mean Best Corrected Visual Acuity was 0.23+‐0.27 log MAR. Main etiologies were sequelae of idiopathic intracranial hypertension (IIH) in 8 eyes (50%) and tilted disc syndrome (TDS) in 6 eyes (37.5%). VF defects were found in 12 eyes (75%). They included non‐specific scotomas (35.7%), blind spot enlargement (21.4%) and peripheral arcuate defect (12.5%). Peripapillary Retinal Nerve Fiber Layer (pRNFL) were thickened in 62.5% of eyes, altered in 31.3% and preserved in 6.3%. Inferior and nasal pRNFL showed no alterations in all eyes. SS‐OCT revealed hyperreflective lesions in all eyes, associated to posterior shadowing in 12 eyes (75%), hyperreflective dots in 10 eyes (62.5%) and peripapillary pigment epithelium detachment in 9 eyes (56.3%). There were no statistically significant differences in VF alterations between TDS and IIH group. Patients with IIH presented thicker pRNFL (p = 0.023) and were more likely to present annular leakage in FA (p = 0.012).ConclusionsPHOMS is a recently described entity that can be related to several etiologies such as IIH, TDS or ischemic optic nerve neuropathy. Both TDS and IIH can lead to visual field defects. IIH is more likely to give annular retention in FA and thickening of pRNFL.
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