Author: Shiaowei Wong (United Kingdom)
Co-authors: Chiara Giuffre, Fiona Mason, Laura R Steeples, Sasa Pockar
Purpose
To present multimodal imaging in a severe and delayed-presentation case of Vogt-Harada-Koyanagi disease (VKH)
Setting/Venue
Manchester Royal Eye Hospital
Methods
A case report describing the clinical presentation and findings of multimodal imaging in a 52-year-old female patient with delayed presentation of bilateral VKH. She reported a 3-week history of reduced visual acuity. She was fit and well, with no prodrome or other neurological features, and had no past ocular history. We describe enhanced depth optical coherence tomography (EDI-OCT, (Spectralis®, Heidelberg Engineering)), ultra-widefield imaging including fundus fluorescein (FFA), and indocyanine angiography (ICGA) and auto-fluorescence (Optos®).
Results
The visual acuity (VA) was hand movements right and 0.56 logMar left eye. She had bilateral multifocal, serous detachments (SD). Macula EDI-OCT showed retinal pigment epithelium (RPE) undulation, disruption of the ellipsoid zone (EZ), and significant choroidal thickening. OCT-angiography (OCTA) demonstrated small focal areas of flow void defects in the choriocapillaris. UWF-FFA showed optic disc leakage and peripheral capillary leakage but no typical pinpoint hyper-fluorescence and pooling. UWF-ICGA demonstrated dilated, fuzzy choroidal vessels, disc hyperfluorescence and multiple hypocyanescent spots. Following high-dose systemic corticosteroid, the SD improved partially. However, a symmetrical pattern of striking choroidal hyperpigmentation in the posterior pole emerged on fundoscopy and UWF imaging, representing the remaining normal pigmentation of the fundus. The hypopigmented areas corresponded partially to hypocyanescent areas on ICGA. EDI-OCT through these changes revealed choriocapillaris thinning with thickened underlying choroidal stroma. Permanent thinning of the RPE and loss of integrity of the EZ were noted. Disease remission was achieved, with resolution of the SD and significant reduction of choroidal thickness. The VA was 0.1 logMAR in both eyes at 4-months.
Conlusions
Multimodal imaging was essential in providing supportive evidence for the diagnosis and guiding therapeutic response in this case. Imaging demonstrated the destructive nature of the disease in the acute phase. We believe the late presentation of this patient was contributory to the aggressive and advanced disease stages observed on multi-modal imaging. During the acute phase, the unusual pattern of choroidal pseudo-hyperpigmentation representing the remaining normal fundal pigmentation was striking. Despite resolution of choroidal inflammation, there was RPE thinning and hyperplasia with persistent disruption of EZ integrity. Prompt treatment with very high dose systemic steroid (1mg/kg day) is necessary in VKH until resolution of choroidal inflammatory signs, including choroidal thickening on EDI-OCT. This strategy followed by maintenance immunosuppression with azathioprine led to excellent visual recovery and limited sight-threatening complications.
Financial Disclosure
No financial interest
Comments
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