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Updated: Jun 27, 2025
Retinal Vascular Reactivity as Assessed by Optical Coherence Tomography Angiography
Published on: March 26, 2020
Optical coherence tomography angiography in pediatric ocular cutis marmorata telangiectatica congenita: A case series
Serena Shah1, Natasha Ferreira Santos da Cruz1, Francisco Lopez-Font1
1Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, 900 NW 17 Street, Miami, FL, 33136, USA.
Insights
Optical coherence angiography (OCTA) revealed an enlarged foveal avascular zone (FAZ) in two pediatric patients with cutis marmorata telangiectatica congenita (CMTC). This highlights the importance of OCTA for comprehensive retinal evaluation in CMTC.
Area of Science:
- Ophthalmology
- Medical Imaging
- Genetics
Background:
- Cutis marmorata telangiectatica congenita (CMTC) is a rare congenital vascular disorder.
- Ocular manifestations in CMTC can include peripheral retinal avascularity.
Purpose:
To report 2 cases of enlarged foveal avascular zone (FAZ) on optical coherence angiography (OCTA) imaging in pediatric patients with cutis marmorata telangiectatica congenita (CMTC).
Observations:
A 10-week-old female and a 3-year-old male diagnosed with CMTC presented for retinal examination. Both had peripheral avascularity on fluorescein angiography (FA) and enlargement of the FAZ on OCTA in both eyes.
Conclusions And Importance:
Pediatric patients with CMTC should undergo ocular evaluation with not only FA, but also OCTA to more robustly evaluate the affected retina in this rare disease.
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