Sickle Cell and the Eye Adrienne W. Scott, MD Wilmer Eye Institute - - PowerPoint PPT Presentation

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Sickle Cell and the Eye Adrienne W. Scott, MD Wilmer Eye Institute - - PowerPoint PPT Presentation

Jan 23, 2023 399 likes •674 views

Sickle Cell and the Eye Adrienne W. Scott, MD Wilmer Eye Institute Johns Hopkins University School of Medicine Disclosures Allergan, Inc. (Consultant) No financial interest in this subject matter Sickle Cell and the Eye- Take Home

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Sickle Cell and the Eye

Adrienne W. Scott, MD Wilmer Eye Institute Johns Hopkins University School of Medicine

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Disclosures

  • Allergan, Inc. (Consultant)
  • No financial interest in this subject matter
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Sickle Cell and the Eye- Take Home Points

  • Sickle cell disease can affect any part of the eye
  • Retinopathy risk variable, depends on genotype
  • Male sex, older age, visual symptoms and

floaters associated with retinopathy

  • Dilated fundus exam required at least annually

beginning at age 10, younger if possible

  • Treatment can prevent vision loss
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Orbital Involvement

  • Retro-orbital and Orbital Involvement

– Orbital bone infarction – Orbital compression syndrome

  • Fever, lid edema, facial pain

Schundeln et.al, Journal of Pediatrics, 2014.

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Anterior Segment

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  • Goldberg staging (AJO

1971)

– Stage I: peripheral arteriolar

  • cclusions

– Stage II: peripheral arteriolar-venular anastomoses – Stage III: neovascular and fibrous proliferation – Stage IV: vitreous hemorrhage – Stage V: retinal detachment

Sickle Cell Retinopathy

Top photo by Andrew R. Marks, M.D.

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Non-Proliferative Sickle Cell Retinopathy

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Stage 1: Peripheral arteriolar occlusions

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Stage 2: Arteriolar-venular anastomoses

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Stage 3: Neovascularization

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Stage IV: vitreous hemorrhage

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Stage IV: vitreous hemorrhage Seafans visible despite vitreous heme

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Stage V: vitreous hemorrhage Combined TRD/RRD

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Image Courtesy of the American Society of Retina Specialists (ASRS) image Bank

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Sickle Cell Maculopathy

  • Numerous macular abnormalities

possible

  • Hairpin venular loops
  • Microaneurysmal dots
  • Foveal avascular zone irregularities
  • Macular ischemia does not

develop neovascularization

  • Many patients with abnormalities

but no visual consequence

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Multimodal Imaging in SCR

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Multimodal Imaging in SCR

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Treatments for Sickle Cell Retinopathy

  • Mostly observation, unless….

Stage 3 Stage 4 Stage 5

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Treatments

  • Laser photocoagulation
  • Intravitreal injection of anti-vascular endothelial growth factor

agents (anti-VEGF)

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Pre-treatment Pre-treatment Post-treatment Post-treatment

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Practical Questions

  • What about glasses?

– Refractive error separate

  • Is all this retinal imaging testing necessary?

– Not sure, a thorough dilated exam will pick up serious retinal pathology

  • So do all with SCD have retinopathy?

– Probably, and will increase as they age

  • Is vision loss in SCD certain?

– Probably not, risk is low, but subtle vision loss may be underdiagnosed

  • When are sickle cell retinopathy screening exams necessary?

– At least annually, age 5 and older, NHLBI consensus suggests age 10 – Floaters, or vision loss, HbSC/Beta thal

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Acknowledgements

  • Ian Han, M.D.
  • Sally Ong, M.D.
  • Marguerite Linz, B.A.
  • T.Y. Alvin Liu, M.D.
  • Morton F. Goldberg, M.D.
  • Hematology Department at JHU-SOM