0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Case Report/Case Series |

Increased Choroidal Thickness in Patients With Sturge-Weber Syndrome

Karun S. Arora, BA1; Harry A. Quigley, MD1; Anne M. Comi, MD2,3,4; Rhonda B. Miller, COA1; Henry D. Jampel, MD, MHS1
[+] 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
JAMA Ophthalmol. 2013;131(9):1216-1219. doi:10.1001/jamaophthalmol.2013.4044.
Text Size: A A A
Published online

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.

Figures in this Article

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

First Page Preview

View Large
First page PDF preview

Figures

Place holder to copy figure label and caption
Figure 1.
Right and Left Eye Fundus Images and Cross-sectional Enhanced Depth Imaging Scans of a 16-Year-Old White Girl

Right (A) and left (B) eye fundus images and cross-sectional enhanced depth imaging scans of a 16-year-old white girl (case 8). Her average choroidal thickness was 1189 μm OD and 478 μm OS. The yellow circular spots near the center of the fundus image assist the person acquiring the image in bringing the image into focus. The blue arrows indicate the interface of the choroid with the retinal pigment epithelium, and the white arrows indicate the choroidal-scleral interface. Choroidal abnormalities were not observed clinically or in the fundus photographs, but were observed in the cross-sectional scan of the right eye.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.
Right and Left Eye Fundus Images and Cross-sectional Enhanced Depth Imaging Scans of a 35-Year-Old White Man

Right (A) and left (B) eye fundus images and cross-sectional enhanced depth imaging scans of a 35-year-old white man (case 11). His average choroidal thickness was 1218 μm OD and 832 μm OS. The yellow circular spots near the center of the fundus image assist the person acquiring the image in bringing the image into focus. The blue arrows indicate the interface of the choroid with the retinal pigment epithelium, and the white arrows indicate the choroidal-scleral interface. Choroidal abnormalities were not observed clinically or in the fundus photographs, but were observed in the cross-sectional scans in both eyes.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3.
Right and Left Eye Fundus Images and Cross-sectional Enhanced Depth Imaging Scans of an 11-Year-Old White Girl

Right (A) and left (B) eye fundus images and cross-sectional enhanced depth imaging scans of an 11-year-old white girl (case 6). Her average choroidal thickness was 326 μm OD and 520 μm OS. The yellow circular spots near the center of the fundus image assist the person acquiring the image in bringing the image into focus. The blue arrows indicate the interface of the choroid with the retinal pigment epithelium, and the white arrows indicate the choroidal-scleral interface. Choroidal abnormalities were not observed clinically or in the fundus photographs, but were observed in the cross-sectional scan of the left eye.

Graphic Jump Location

Tables

References

Correspondence

CME
Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Web of Science® Times Cited: 1

Sign in

Create a free personal account to sign up for alerts, share articles, and more.

Purchase Options

• Buy this article
• Subscribe to the journal

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections
PubMed Articles
Jobs
brightcove.createExperiences();