Atypical Retro Internal Limiting Membrane Haemorrhage in Homozygous Sickle Cell Disease

Background

Sickle cell disease (SCD) is an inherited multisystemic blood disorder known to cause several ocular complications associated with inflammatory proangiogenic chorioretinal vaso-occlusive phenomena and spontaneous retinal haemorrhages [1]. The most significant ophthalmic signs are divided into non-proliferative (Goldberg stages I – II) and proliferative (Goldberg stages III – V) sickle cell retinopathy (SCR) [2]. The early stage of the ocular involvement is usually asymptomatic in children and young adults, and the patients may remain unaware of ocular complications until the disease progresses. Therefore, regular ocular preventive screening is recommended. However, acute symptomatic retinal vascular complications could occur and typically take the appearance of salmon patches, small intra-retinal haemorrhages, possibly due to ischemic vessel wall necrosis. They usually disappear without trace, but may sometimes leave a hyperpigmented lesion, called black sunburst [3]. In addition, the proangiogenic state of patients affected by SCD may also, more rarely, lead to dramatic central retinal artery occlusion or paracentral acute middle maculopathy [4]. We report a case of a 19-year-old woman affected by homozygous SCD (SS type) that presented with an acute unilateral paracentral scotoma and floaters due to a solitary retinal lesion observed on funduscopy.

• References

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