ORIGINAL RESEARCH article
Front. Med.
Sec. Ophthalmology
Volume 12 - 2025 | doi: 10.3389/fmed.2025.1575006
This article is part of the Research TopicImaging in the Diagnosis and Treatment of Eye DiseasesView all 22 articles
En face swept source optical coherence tomography (SS-OCT) and SS-OCT angiography findings of retinal astrocytic hamartomas in patients with tuberous sclerosis complex
Provisionally accepted
- Peking Union Medical College Hospital (CAMS), Beijing, China
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Introduction: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by multi-system hamartomas, including retinal astrocytic hamartomas (RAHs), a key diagnostic criterion.This study evaluates en face swept-source optical coherence tomography (SS-OCT) and SS-OCT angiography (SS-OCTA) features of TSC-associated RAHs.A retrospective analysis of ten patients with TSC-associated RAHs was conducted using en face SS-OCT, SS-OCTA, and fundus photography. Structural and vascular features were assessed.Results: Of the ten TSC patients, 21 RAH lesions (18 type 1, one type 2, two type 3 ) were fully scanned. En face SS-OCT revealed vitreous changes in 17 of 21 RAH lesions, with clear visualization of vitreoretinal traction in 6 lesions. Type 1 RAH exhibited isoreflective or mildly hyporeflective masses with disarrangement of retinal nerve fibers. Calcified components of type 2 or type 3 RAHs appeared differently at the en face choroid slab, with type 2 RAH featured closely arranged isoreflective vesicles, while type 3 RAH presented as sharply-defined dark area. On SS-OCTA, a dense vascular network with disorganization of radial papillary capillaries was revealed in almost all the type 1 RAHs, with half of them exhibiting congestive intrinsic microvasculature. Feeder vessels were identified in only two type 1 lesions. Nonflow moth-eaten cavities were the characteristics for the calcified components of type 2 or type 3 RAHs. The tumor vascular density was positively correlated with tumor maximal thickness in type 1 RAH (p=0.037).Conclusions: En face SS-OCT had a good display of RAH-related vitreoretinal traction and tumor calcification, while SS-OCTA, clearly visualizing the intratumoral vascularity, may assist in searching for signs of progressive tumor growth in TSC-associated RAH.
Keywords: En face, Swept source, optical coherence tomography angiography, Retinal astrocytic hamartoma, tuberous sclerosis complex
Received: 04 Mar 2025; Accepted: 21 Apr 2025.
Copyright: © 2025 Zhang, Xu, Long, Zhang, Yang, Dai and Zhang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Zhi-Qiao Zhang, Peking Union Medical College Hospital (CAMS), Beijing, China
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