History of Present Illness
A 33-year-old male, born in Sri Lanka, who has lived in Switzerland since 2013, presents himself at the eye clinic complaining of vision loss for 4 months in his left eye (OS). He just returned from a holiday in Sri Lanka. When the symptoms started, he noticed blurred vision, followed by floaters and a feeling of pressure in his OS. At the time he was using a steroid cream due to psoriasis-like lesions on his extremities. He denied other health issues and had no systemic medications. In the past few weeks he was seen by two other ophthalmologists—the first prescribed a new pair of glasses, which did not help; the second diagnosed a vitreous hemorrhage in the OS.
| OD | OS | |
|---|---|---|
| Visual acuity | 20/20 | 20/30 |
| Intraocular pressure (IOP) | 14 | 15 |
| Sclera/conjunctiva | White and quiet | White and quiet |
| Cornea | Arcus superior | Arcus superior |
| Anterior chamber (AC) | Deep, cells 0.5+ | Deep, cells 0.5+ |
| Iris | Unremarkable | Unremarkable |
| Lens | Clear | Clear |
The vitreous showed 1+ vitreous cells right eye (OD) and vitreous hemorrhage OS. Funduscopy OD revealed intraretinal dot hemorrhages and pronounced neovascularization elsewhere (NVE). OS showed vitreous hemorrhage, intraretinal hemorrhage, and NVE ( Fig. 26.1A and B , Fig. 26.2A and B , and Fig. 26.3A and B ).
Questions to Ask
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Have you ever had aphthous ulcers, joint pain, or thrombosis?
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Is there any history of eye illnesses in the family?
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Were you born preterm?
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Have you had radiation in the past?
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Do you have diabetes?
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Were you ever diagnosed with sickle cell disease or anemia?
The patient denies all of these questions.
Assessment
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Occlusive vasculitis with neovascularization and vitreous hemorrhage OS > OD of unknown origin
Differential Diagnosis
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Proliferative diabetic retinopathy both eyes (OU) with vitreous hemorrhage OS
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Retinal vascular occlusion
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Hyperviscosity sydrome
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Sarcoidosis
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Syphilis
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Tuberculosis-associated retinal vasculitis
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Eales disease
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Retinopathy of prematurity
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Familial exudative retinopathy
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Behçet disease
Working Diagnosis
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Occlusive vasculitis with neovascularization and vitreous hemorrhage OS > OD of unknown origin
Testing
Occlusive vasculitis, neovascularization, and extensive retinal ischemia need further diagnostic workup. Diabetes, sickle cell disease, hyperviscosity syndrome, and autoimmune diseases such as sarcoidosis and Behçet must be ruled out. Furthermore, infectious diseases as an underlying cause, such as tuberculosis and syphilis, must be excluded.
Laboratory and Imaging Workup
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Blood test:
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Fluorescent treponemal antibody absorption (FTA-ABS), rapid plasma reagin (RPR): Negative
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HbA1c: 5.6%
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Angiotensin-converting enzyme (ACE), lysozyme, interleukin-2 (IL-2) receptor: within normal limits (WNL)
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HLA B51: Negative
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QuantiFERON: Positive
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Coagulation tests: WNL
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Differential blood count: WNL
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Blood smear: Unremarkable
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Chest x-ray: Unremarkable
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Sputum: Negative for Mycobacteria
Management
Due to lack of evidence of any other underlying disease and a positive QuantiFERON test, the patient was diagnosed with Eales disease.
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OD: Panretinal laser photocoagulation (PRP)
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OS: Vitreoretinal surgery, endolaser photocoagulation, and intravitreal bevacizumab and triamcinolone
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Antituberculostatic drugs
Follow-up
During follow-up the patient developed high intraocular pressure (IOP) OS due to corticosteroid responsiveness; however, IOP was well controlled with medical treatment.
Four months after the first consultation, neovascularization OS fully regressed, symptoms resolved, and visual acuity was 20/20 OU. Due to persistent active NVE OD, the patient will receive additional PRP. Figs. 26.4 (A and B), 26.5 (A and B), and 26.6 (A and B) .
