Choroidal alterations of Sturge-Weber syndrome secondary glaucoma and non-glaucoma port-wine stain patients distinguishe

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RESEARCH ARTICLE

Open Access

Choroidal alterations of Sturge-Weber syndrome secondary glaucoma and nonglaucoma port-wine stain patients distinguished by enhanced depth imaging optical coherence tomography Yue Wu1,2†, Lulu Huang1,2†, Yixin Liu1,2, Li Xu1,2 and Wenyi Guo1,2*

Abstract Background: To evaluate the choroidal changes in Sturge-Weber syndrome (SWS) secondary glaucoma and nonglaucoma port-wine stain (PWS) patients by enhanced depth imaging optical coherence tomography (EDI-OCT). Methods: SWS and PWS patients who were over 3 years old and treated or screened at our ophthalmology department were included in the study. Baseline demographics, EDI-OCT and fundus photography data were collected from all patients. Results: Overall, 46 non-glaucoma PWS (NGPWS) patients and 35 SWS secondary glaucoma (SG) patients were included, with mean ages of 16.52 ± 13.63 and 13.94 ± 8.27 years, respectively (p > 0.05). Among these patients 2 exhibited bilateral PWS and unilateral glaucoma. Thus, the two eyes of each patient were divided into NGPWS and SG group, respectively. Twenty-one eyes had choroidal hemangiomas and 7 eyes had excessive thickening of the choroid without choroidal hemangiomas. Choroidal hemangiomas were only observed in ipsilateral eyes of SG patients. The choroidal thicknesses of the ipsilateral and fellow eyes of NGPWS patients were 358.10 ± 117.40 μm (45 eyes) and 288.20 ± 79.04 μm (41 eyes), respectively (p < 0.05). The choroidal thicknesses of the ipsilateral and fellow eyes of SG patients were 511.40 ± 242.10 μm (15 eyes) and 283.90 ± 92.27 μm (29 eyes), respectively (p < 0.05). Significant differences were found between the ipsilateral eyes of SWS and PWS patients (p < 0.05). Six of 13 eyes (46%) with choroidal hemangiomas exhibited post-operative posterior segment complications. Conclusions: NGPWS and SG patients had a thicker choroid in the ipsilateral eye. The trend was even more pronounced in SG patients. Choroidal hemangiomas were only found in the ipsilateral eyes of SG. In addition, choroidal hemangioma was a risk factor for post-operative posterior segment complications in SWS patients. Keywords: Sturge-weber syndrome, Port-wine stain, Glaucoma, Choroidal Hemangiomas, Optical coherence tomography, Complications * Correspondence: [email protected] † Yue Wu and Lulu Huang contributed equally to this work. 1 Department of Ophthalmology, Ninth People’s Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, No. 639 Zhizaoju Road, Huangpu District, Shanghai 200011, China 2 Shanghai Key Laboratory of Orbital Diseases and Ocular Oncology, Shanghai 200011, China © The Author(s). 2020 Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in thi