Sickle cell retinopathy
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"sickle cell retinopathy" AND management
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Here is a comprehensive overview of sickle cell retinopathy based on authoritative ophthalmology textbooks.
Sickle Cell Retinopathy
Pathophysiology
Sickling haemoglobinopathies cause red blood cells to adopt an anomalous shape under physiological stress (hypoxia, acidosis), leading to vascular occlusion. The relevant genotypes are:
| Genotype | Notes |
|---|---|
| HbSC | Most likely to develop severe proliferative retinopathy |
| HbSS (sickle cell disease) | Proliferative retinopathy less common than SC |
| HbS-thalassaemia | Significant risk |
| HbSA (sickle cell trait) | Rare retinopathy; only if co-existing diabetes or inflammation |
⚠️ Carbonic anhydrase inhibitors (CAIs) are contraindicated in all sickling disorders — they precipitate sickling and vascular occlusion. This applies to IOP management in hyphaema as well.
Anterior Segment Findings
- Conjunctiva: Dark red corkscrew- or comma-shaped vessels (typically transient)
- Iris: Patches of ischaemic atrophy from pupillary edge to collarette; occasional rubeosis
- Hyphaema: May be spontaneous or post-traumatic; requires careful IOP control (avoid CAIs)
Non-Proliferative Retinopathy
| Feature | Description |
|---|---|
| Venous tortuosity | Very common; due to peripheral arteriovenous shunting |
| Optic disc sign of sickling | Dark red blots on the disc surface from small vessel occlusion |
| Salmon patches | Orange-red mid-peripheral superficial intraretinal haemorrhages; initiating event is vascular occlusion |
| Black sunbursts | Peripheral RPE hyperplasia and chorioretinal atrophy evolving from salmon patches; variable pigmentation with a pale outer band |
| Arteriolar occlusions | Branch, central, or macular vessels; "silver wiring" of peripheral arterioles = previously occluded; corkscrew vessels |
| Macular depression sign | Oval temporal macular depression from retinal thinning after arteriolar occlusion |
| Iridescent/refractile deposits | Residual deposits after haemorrhage resorption |
| Angioid streaks | Present in up to 6% |
Proliferative Retinopathy — Goldberg Staging
| Stage | Finding |
|---|---|
| 1 | Peripheral arteriolar occlusion |
| 2 | Peripheral arteriovenous anastomoses proximal to non-perfused areas |
| 3 | "Sea fan" neovascularization — at the edge of perfused retina, typically with one feeding arteriole and one draining venule |
| 4 | Vitreous haemorrhage from the NV |
| 5 | Rhegmatogenous or tractional retinal detachment |
The development of proliferative retinopathy is usually insidious and asymptomatic until stage 4 or 5.
Fundus angiography (FA) in stage 3: sea fan filling and peripheral capillary non-perfusion in early phase → leakage from NV in late phase. Wide-field imaging is particularly well-suited to evaluation.
Fundus Images
Fig. 1 — Sickle cell retinopathy: sea fan neovascularization with vitreous haemorrhage (Wills Eye Manual)
Fig. 2 — Non-proliferative sickle cell retinopathy: salmon patch haemorrhage (arrow) and black sunburst lesion (Kanski's Clinical Ophthalmology)
Differential Diagnosis of Peripheral Retinal Neovascularization
- Sarcoidosis — sea fan NV with uveitis; common in young patients of African descent
- Diabetic retinopathy — posterior pathology predominates; dot-blot haemorrhages
- Eales disease — peripheral vascular occlusion of unknown aetiology (diagnosis of exclusion)
- ROP, FEVR, pars planitis, radiation retinopathy, OIS, collagen vascular disease
- Talc retinopathy — IV drug use history; refractile particles in macular arterioles
Workup
- Full medical and family history (sickle cell disease, diabetes, IV drug use)
- Dilated fundus examination with indirect ophthalmoscopy
- Sickledex, sickle cell preparation, hemoglobin electrophoresis
- Note: HbSC and HbC disease may give a negative Sickledex — electrophoresis is needed
- Widefield IVFA — aids diagnosis and guides treatment planning
- OCT-A — emerging role in monitoring (see recent evidence below)
Treatment
| Situation | Management |
|---|---|
| Stage 3 NV, no VH | Observation acceptable — many sea fans undergo spontaneous auto-infarction; especially in middle-aged/older patients |
| NV with VH | PRP (panretinal photocoagulation) to the avascular area anterior to NV |
| Tractional/persistent VH | Vitrectomy (caution: scleral explant carries high risk of anterior segment ischaemia) |
| Anti-VEGF | May be beneficial but use caution with significant traction |
| Acute arteriolar occlusion | 100% oxygen + exchange transfusion may be considered |
Laser/cryotherapy: Ablation of peripheral non-perfused retina is the optimal approach. Ablation of feeder vessels is now rarely performed due to high rates of subsequent macular NV.
Follow-Up
| Status | Interval |
|---|---|
| No retinopathy | Annual dilated fundus exam |
| Retinopathy present | Every 3–6 months depending on severity |
Recent Evidence
A 2025 systematic review and meta-analysis (PMID: 40404974) found that OCT-angiography (OCTA) is a valuable non-invasive tool for diagnosing and monitoring sickle cell-related eye disease, supporting its use as a complement or alternative to conventional IVFA.
Sources: Kanski's Clinical Ophthalmology: A Systematic Approach, 10th Ed. | Wills Eye Manual, 8th Ed.
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