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Sickle Cell Retinopathy and Hemoglobinopathies

  • Chapter
Retinal Vascular Disease

Abstract

Sickle cell hemoglobinopathies all share the common feature of an abnormal globin chain, which leads to sickling of erythrocytes and obstruction of the microcirculation. Sickle vaso-occlusive events are insidious and affect virtually every vascular bed in the eye, often with visually devastating consequences. Vaso-occlusion most profoundly affects the retina, the light-sensitive tissue that lines the inside wall of the posterior aspect of the eye, because it is exquisitely sensitive to deprivation of oxygen. Even temporary vaso-occlusion, if longer than about 1.5–2 h, can result in permanent infarction of the retina. Most, if not all, of the complications of sickle cell disease in retina originate from the vaso-occlusive processes. The pathological changes can be divided into nonproliferative and proliferative events.

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Authors and Affiliations

  1. The Wilmer Eye Institute, Johns Hopkins Hospital, 600 N. Wolfe Street, Baltimore, MD, 21287-9115, USA

    Gerard A. Lutty PhD

  2. The Rotterdam Eye Hospital, Schiedamsevest 180, 3011, BH Rotterdam, The Netherlands

    Jan van Meurs MD, PhD

  3. Moorfields Eye Hospital London, City Road, London, EC1V 2PD, UK

    Alan C. Bird FRCS, FRCOphth

  4. Oxford Eye Hospital, Radcliffe Infirmary, Oxford, OX2 6HE, UK

    Susan M. Downes FRCOphth

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  1. Gerard A. Lutty PhD
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  2. Jan van Meurs MD, PhD
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  3. Alan C. Bird FRCS, FRCOphth
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  4. Susan M. Downes FRCOphth
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Editors and Affiliations

  1. Department of Ophthalmology, University of Düsseldorf, Moorenstraße 5, 40225, Düsseldorf, Germany

    Antonia M. Joussen MD, PhD (Professor of Ophthalmology) (Professor of Ophthalmology)

  2. Department of Ophthalmology, Penn State College of Medicine, 500 University, Drive Box 850, Hershey, PA, 17033, USA

    Thomas W. Gardner MD, MS (Professor of Ophthalmology) (Professor of Ophthalmology)

  3. Department of Vitreoretinal Surgery, Center for Ophthalmology, University of Cologne, Joseph-Stelzmann-Straße 9, 50931, Cologne, Germany

    Bernd Kirchhof MD (Professor of Ophthalmology) (Professor of Ophthalmology)

  4. Doheny Eye Institute, University of Southern California, 1450 San Pablo Street, Los Angeles, CA, 90033, USA

    Stephen J. Ryan MD (Professor of Ophthalmology) (Professor of Ophthalmology)

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Lutty, G.A., van Meurs, J., Bird, A.C., Downes, S.M. (2007). Sickle Cell Retinopathy and Hemoglobinopathies. In: Joussen, A.M., Gardner, T.W., Kirchhof, B., Ryan, S.J. (eds) Retinal Vascular Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-29542-6_27

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