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Exudative Vitreoretinopathy

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Inherited Chorioretinal Dystrophies

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Abstract

Familial exudative vitreoretinopathy (FEVR) is an inherited group of conditions that is associated with a failure in the developmental vascularisation of the peripheral retina. A highly variable phenotype, FEVR, may be inherited as autosomal recessive, autosomal dominant and X-linked traits. Avascularity of the peripheral retina and the ensuing ischaemia-related complications are the cause of the associated visual effects that present in childhood and/or early adulthood.

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Acknowledgement

Professor Borja Corcostegui, Barcelona, Spain, kindly provided the following pictures: Figs.

Fig. 44.1
figure 1

Asymmetrical aspect of FEVR in a 13-year-old boy. The posterior pole of the left eye is normal. The right eye presents marked dragging of the retinal vessels

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Fig. 44.2
figure 2

Fluorescein angiography of the temporal periphery of the right eye of Fig. 44.1

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Fig. 44.3
figure 3

Wide-angle ophthalmoscopy of a stage 1 FEVR

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44.3,

Fig. 44.4
figure 4

Fluorescein angiography of the same eye as Fig. 44.3, showing marked peripheral ischaemia

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44.4,

Fig. 44.5
figure 5

Stage 2 FEVR

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44.5,

Fig. 44.6
figure 6

Fluorescein angiography of the eye of Fig. 44.5: dragging of the temporal retinal vessels, peripheral ischaemia and retinal new vessels

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Fig. 44.7
figure 7

Stage 2 FEVR. Dragging of the temporal retinal vessels and retinal neovascularisation with glial proliferation in the temporal periphery

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44.6,

Fig. 44.8 and 44.9
figure 8figure 8

Wide-field fundus picture and fluorescein angiography in a stage 2 FEVR

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44.8, 44.9,

Fig. 44.10
figure 9

Wide-field ophthalmoscopy and fluorescein angiography (stage 2 FEVR)

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Fig. 44.11
figure 10

Stage 4 FEVR with dragging of the retinal vessels, macular detachment and massive exudation (16-year-old male)

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Fig. 44.12
figure 11

Left eye of the patient in Fig. 44.11: normal aspect of the posterior pole but peripheral ischaemia and retinal new vessels

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44.10,

Fig. 44.13
figure 12

Peripheral retinal detachment

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44.13,

Fig. 44.14
figure 13

Laser treatment of the ischaemic retinal periphery

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44.14 and

Fig. 44.15
figure 14

Encircling procedure for retinal detachment in a patient with FEVR

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44.15.

Author information

Authors and Affiliations

  1. Manchester Royal Eye Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Sciences Centre (MAHSC), Oxford Road, Manchester, M13 9WL, UK

    Graeme Charles M. Black

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  1. Graeme Charles M. Black
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Correspondence to Graeme Charles M. Black .

Editor information

Editors and Affiliations

  1. Service d’Exploration de la Vision et Neuro-ophtalmologie, Centre Hospitalier Universitaire de Lille, Hôpital Roger Salengro, Lille Cedex, France

    Bernard Puech

  2. Department of Ophthalmology, Ghent University Hospital, Ghent University, Ghent, Belgium

    Jean-Jacques De Laey

  3. Department of Electrophysiology, Moorfields Eye Hospital, London, United Kingdom

    Graham E. Holder

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© 2014 Springer-Verlag Berlin Heidelberg

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Black, G.C.M. (2014). Exudative Vitreoretinopathy. In: Puech, B., De Laey, JJ., Holder, G. (eds) Inherited Chorioretinal Dystrophies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-69466-3_44

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  • DOI: https://doi.org/10.1007/978-3-540-69466-3_44

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-69464-9

  • Online ISBN: 978-3-540-69466-3

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