Which retinal changes may be present in Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Answer

Changes in the overlying retinal pigment epithelium overlying the choroidal hemangioma range from mild atrophy to focal proliferation with drusen formation to severe fibrous transformation and focal ossification. The retina over the hemangioma may be attached and well preserved, attached and degenerated, or detached.

Degenerative changes in the overlying retina include focal chorioretinal adhesions, loss of photoreceptors, severe cystoid degeneration of the outer layers, and marked gliosis. Widespread serous detachment, retinal leakage, and edema may occur. In its early stages, the choroidal thickening and elevation of the retina may produce an increasing ipsilateral hyperopia. With progression of secondary changes, visual loss and visual field defects may develop. Subretinal fibrosis in the macular area and cystoid macular edema are associated with the most severe visual loss.


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