Central Foveal Bouquet Abnormalities

Diagnosis of CB abnormalities is made based on OCT findings.

• In stage 2 (foveolar detachment), there is a central subfoveal hyporeflective pocket corresponding to subretinal fluid accumulation under the interdigitation zone.

• In stage 3 (acquired vitelliform lesion), there is the presence of thick dome-shaped subfoveal hyperreflective material between the ellipsoid zone and retinal pigment epithelium.
• Additionally, OCT can also demonstrate findings related to vitreomacular interface disorders. In eyes having ERM, OCT will show ERM as a hyperreflective line above ILM along with intraretinal findings specific to the stages of ERM. These may include foveal herniation and ectopic inner foveal layers (EIFL).
• EIFL has been observed in stage 3 and 4 ERM and was found to be associated with a lower incidence of CB abnormalities. This has been attributed to EIFL causing reduced displacement and stretching of the outer retina at the fovea.

Symptoms

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• Symptoms are related to the underlying macular pathologies and may include blurred vision and metamorphopsia.
• Previous studies have observed a correlation between stages of CB abnormalities and visual acuity.
• Govetto et al. studied CB abnormalities in eyes with ERM and observed that visual acuity was better in the presence of cotton ball signs and worsened with progression to subsequent stages. This may be related to EZ disruption seen in advanced stages of CB abnormalities.
• In eyes with CME, the presence of CB abnormalities was found to be associated with increased macular thickness, horizontal extent of edema, and worse visual acuity at baseline.

Differential diagnosis

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CB abnormalities need to be differentiated from conditions such as:

• Acute retinal pigment epitheliitis (Krill disease) – It has an acute onset with painless loss of vision or central scotoma. OCT shows a dome-shaped hyperreflective lesion at the level of the photoreceptors layer disrupting the ellipsoid zone and interdigitation zone. There may be initial involvement of the outer nuclear layer. The disease is known to have rapid resolution in 6 to 12 weeks.
• Central serous chorioretinopathy – The patient usually presents with complaints of unilateral central vision loss with or without distortion or a possible central scotoma. However, the presentation can be bilateral. CSCR is usually self-limiting and resolves spontaneously within 2 to 3 months. However, recurrent episodes are known to occur. OCT may show serous PED, hyper-reflective, fibrinous sub-retinal fluid, RPE defect corresponding to potential leakage site, and choroidal thickening.
• Best Disease and Bestrophinopathies – Best vitelliform macular dystrophy generally presents the first decade of life and is usually bilateral. Early stages are associated with minimal visual symptoms. Progression of stages is associated with a gradual decrease in visual acuity. Adult-onset foveomacular vitelliform dystrophy is typically seen between 30-50 years of age with minimal visual symptoms. OCT may show nonhomogenous hyperreflectivity at the level of RPE and thickening of the cone’s outer segments. OCT can also pick up the development of CNV in the later stage.
• Solar retinopathy – The patient has a history of direct sun-gazing or eclipse viewing. Usually, there is bilateral foveal involvement with OCT showing disruption of the outer retinal layers.
• Acute idiopathic maculopathy – The patient presents with acute unilateral visual loss. OCT shows subfoveal hyperreflective thickening at the level of the outer retina and RPE and hyporeflective fluid in subretinal space. It is generally a self-limiting condition.

Management

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• Management includes treatment of underlying associations. These include treating ERM and CME due to various etiologies.
• While CME can be treated with topical non-steroidal anti-inflammatory (NSAID) eyedrops / intravitreal injections / oral therapy, ERM needs pars plana vitrectomy (PPV) for removal.

References

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