Sir, A 24-year-old black male with sickle cell disease (SCD) ‘SS’ presented to the emergency room (ER) in sickle cell crisis with acute painless loss of vision OD, chest, and leg pains since

one day. His visual acuity was HM OD and J1 OS with 3+ afferent pupillary defect OD. Anterior segment examination was unremarkable. Ophthalmoscopy OD revealed retinal pallor with dilated

tortuous vessels. OS fundus was normal. Systemic evaluation revealed icterus with normal pulmonary, cardiovascular, or neurological examination with no history of comorbid conditions. The

haemoglobin was 9.6 gm/dl and haematocrit was 27.9%. Exchange transfusion was performed subsequent to hydration. At 1 week the visual acuity (VA) was HM OD and 20/25 OS. Ophthalmoscopy OD

(Figure 1b) in the late phases (staining). FA OS was normal. At 3 months VA was CF 3 feet OD, 20/20 OS. Ophthalmoscopy OD revealed pale disc with marked arterial attenuation and pigmentary

changes in the macula (Figure 2a). Macular perimetry (Nidek Technologies, Vigonza, Italy) revealed unstable fixation with absolute macular scotoma OD with normal findings OS (Figure 2b-d).

His haemoglobin was 9.5 gm/dl and haematocrit was 26.0%. COMMENTS Occlusive disease of the peri-foveal arterioles is known to occur in sickle cell disease.1, 2 We present a dramatic

occlusive event of the macula surrounding the foveal avascular zone, resulting in severe and permanent loss of vision. We are unaware of any previous report of FA showing possible

microemboli in the retinal vessels with macular infarction in SCD. Direct sickling may cause occlusion in arterioles or in capillary beds creating a ‘log-jam’ in the arterioles.3 In this

case, the occlusions surrounded the macula from multiple directions, suggesting perifoveal arteriolar occlusion due to microemboli and/or slugging of sickle cells with slowed circulation in

the venules. Our patient presented with an acute visual loss resulting in irreversible macular infarction. His visual recovery was minimal because of delay in presentation. REFERENCES *

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Department of Ophthalmology, University of Florida, College of Medicine, 580 W 8th street, Jacksonville, 32209, FL, USA K V Chalam & V A Shah Authors * K V Chalam View author

publications You can also search for this author inPubMed Google Scholar * V A Shah View author publications You can also search for this author inPubMed Google Scholar CORRESPONDING AUTHOR

Correspondence to K V Chalam. ADDITIONAL INFORMATION Proprietary Interest: None. Presented as a poster at the 21st Annual Meeting of the American Society of Retina Specialists, New York, NY,

August 2003. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Chalam, K., Shah, V. Macular infarction a presentation of sickle cell crisis. _Eye_ 18,

1277–1278 (2004). https://doi.org/10.1038/sj.eye.6701409 Download citation * Published: 23 April 2004 * Issue Date: 01 December 2004 * DOI: https://doi.org/10.1038/sj.eye.6701409 SHARE THIS

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