Author: Ritu Chaturvedi
Co-authors: Guillermo de la Mata, Eftychia Lazari, Marta Ugarte
Abstract
PurposeTo describe the diverse phenotype and clinical outcome of polypoidal choroidal vasculopathy with peripheral and macular lesions.
Setting/Venue
The study was undertaken at a Manchester Royal Eye Hospital, a tertiary referral centre in the North of England.
Methods:
A retrospective observational case series was conducted. Seven eyes of four patients diagnosed with peripheral exudative haemorrhagic chorioretinopathy (PEHCR) were identified. The presence of PCV was confirmed with indocyanine green angiography (ICG) in 5 eyes and clinical examination in 2 eyes. All patients underwent optical coherence tomography. The clinical presentation and course (with or without intervention) were analysed.
Results:
All patients were Caucasian with no gender preponderance and a mean age of 82 years (range, 76-87 years). Three patients had bilateral involvement. Temporal periphery was the most common location.
One patient presented with unilateral peripheral exudative haemorrhagic chorioretinopathy with bilateral macular polypoidal changes.
Another patient had unilateral peripheral raised haemorrhagic changes and was referred to ocular oncology services with suspected melanoma. Clinical examination along with imaging investigations confirmed a diagnosis of PEHCR.
A third patient was treated for suspected acute retinal necrosis. There was no clinical improvement with antiviral treatment and all the investigations, including vitreous tap and blood tests, were normal. Presence of peripheral polypoidal changes on widefield ICG enabled a correct diagnosis and appropriate management.
The last patient developed subretinal haemorrhage while she was receiving anti VEGF therapy for unilateral wet age-related macular degeneration. PCV was suspected and peripheral retina examination showed presence of bilateral inferotemporal chorioretinal scars consistent with PEHCR-PCV related.
Conclusion:
We describe 4 cases of PEHCR where diverse phenotype presented a diagnostic challenge. Widefield imaging was the key to diagnosis in the majority of the patients.
PEHCR is an uncommon condition but should be included in the differential in patients with peripheral
exudative-haemorrhagic retinal lesions. It can mimic uveal melanoma or posterior uveitis as shown in two of the cases. Awareness and appropriate imaging can avoid unnecessary patient anxiety and a correct management strategy.
Although most eyes with PEHCR experience a benign course with spontaneous resolution of peripheral lesions, anti-VEGF therapy is often needed for associated macular involvement as was observed in our study. This can also aid with resolution of peripheral lesions.
