Case Report/Case Series
September 2013

Increased Choroidal Thickness in Patients With Sturge-Weber Syndrome

Author Affiliations
  • 1Glaucoma Center of Excellence, Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine, Baltimore, Maryland
  • 2Neurology and Developmental Medicine, Kennedy Krieger Institute, Baltimore, Maryland
  • 3Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland
  • 4Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland

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JAMA Ophthalmol. 2013;131(9):1216-1219. doi:10.1001/jamaophthalmol.2013.4044

Importance  With the recent development of enhanced depth imaging spectral-domain optical coherence tomography (SD-OCT), it is now possible to measure choroidal thickness in patients with Sturge-Weber syndrome and detect abnormalities that are not visible as part of the fundus examination.

Observations  We were successful in imaging at least 1 eye in 12 individuals with Sturge-Weber syndrome using enhanced depth imaging SD-OCT. Eyes were defined as affected if they manifested at least one of the following: darkened choroid, glaucomatous optic nerve damage, or conjunctival hyperemia. None of the participants had a clinically visible choroidal hemangioma. The affected eyes had over twice the choroidal thickness of the unaffected eyes (mean [SD], 697 [337] μm vs 331 [94] μm; P = .004, determined by use of an unpaired t test). For the 6 unilaterally affected participants who had both eyes imaged, the choroidal thickness was greater in the affected eyes than in the unaffected eyes of 5 participants (mean [SD], 672 [311] μm vs 329 [88] μm; P = .01, determined by use of a paired t test).

Conclusions and Relevance  The advent of enhanced depth imaging SD-OCT has allowed us to quantify choroidal thickness in the posterior pole, even in eyes with a markedly thickened choroid, such as those found in individuals with Sturge-Weber syndrome. Spectral-domain OCT has a much higher resolution (5-10 μm) than B-scan ultrasonography (150 μm) and can be used to distinguish between the retina and the choroid. Furthermore, enhanced depth imaging SD-OCT can detect choroidal thickness in eyes without clinically apparent choroidal abnormalities.