The clinical features and real world treatment outcomes in von Hippel Lindau related retinal capillary hemangioblastomas

Abstract Background and objective To present the clinical features and outcomes of treatment in von Hippel-Lindau (VHL) related retinal capillary hemangioblastoma (RCH). Methods A retrospective interventional cohort study that included Patients who were diagnosed with VHL-related RCH from 2014 to 20...

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Main Authors: Abdulrahman Khan, Wael A. Alsakran, Abdullah Barry, Abdulrahman H. Badawi, Moustafa S. Magliyah
Format: Article
Language:English
Published: BMC 2025-08-01
Series:International Journal of Retina and Vitreous
Subjects:
Online Access:https://doi.org/10.1186/s40942-025-00721-1
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author Abdulrahman Khan
Wael A. Alsakran
Abdullah Barry
Abdulrahman H. Badawi
Moustafa S. Magliyah
author_facet Abdulrahman Khan
Wael A. Alsakran
Abdullah Barry
Abdulrahman H. Badawi
Moustafa S. Magliyah
author_sort Abdulrahman Khan
collection DOAJ
description Abstract Background and objective To present the clinical features and outcomes of treatment in von Hippel-Lindau (VHL) related retinal capillary hemangioblastoma (RCH). Methods A retrospective interventional cohort study that included Patients who were diagnosed with VHL-related RCH from 2014 to 2024 at a tertiary referral center. Results Thirty-two eyes of 18 patients with VHL-associated RCH were included. Treatment was indicated in 30 eyes (90.9%), and the indications for treatment were tumor leakage and subretinal fluids (SRF) with or without exudative retinal detachments in 25 eyes (83.3%) and tractional/exudative retinal detachments (T-ERD) in 4 eyes (13.3%). Significantly worse visual outcomes were observed in patients who had lower BCVA on presentation, larger tumor sizes, developed retinal complications, underwent retinal surgery, detached retina on the last follow up and had persistent tumor outcomes. Conclusion The management outcomes in VHL-related RCH depend on the lesionsize and location, severity of the clinical picture on presentation and the development of complications. Clinical trial number Not applicable.
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issn 2056-9920
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publishDate 2025-08-01
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series International Journal of Retina and Vitreous
spelling doaj-art-c846f44e7a1947418dd49554a32fdf442025-08-24T11:42:44ZengBMCInternational Journal of Retina and Vitreous2056-99202025-08-011111810.1186/s40942-025-00721-1The clinical features and real world treatment outcomes in von Hippel Lindau related retinal capillary hemangioblastomasAbdulrahman Khan0Wael A. Alsakran1Abdullah Barry2Abdulrahman H. Badawi3Moustafa S. Magliyah4Vitreoretinal Division, King Khaled Eye Specialist HospitalVitreoretinal Division, King Khaled Eye Specialist HospitalVitreoretinal Division, King Khaled Eye Specialist HospitalVitreoretinal Division, King Khaled Eye Specialist HospitalVitreoretinal Division, King Khaled Eye Specialist HospitalAbstract Background and objective To present the clinical features and outcomes of treatment in von Hippel-Lindau (VHL) related retinal capillary hemangioblastoma (RCH). Methods A retrospective interventional cohort study that included Patients who were diagnosed with VHL-related RCH from 2014 to 2024 at a tertiary referral center. Results Thirty-two eyes of 18 patients with VHL-associated RCH were included. Treatment was indicated in 30 eyes (90.9%), and the indications for treatment were tumor leakage and subretinal fluids (SRF) with or without exudative retinal detachments in 25 eyes (83.3%) and tractional/exudative retinal detachments (T-ERD) in 4 eyes (13.3%). Significantly worse visual outcomes were observed in patients who had lower BCVA on presentation, larger tumor sizes, developed retinal complications, underwent retinal surgery, detached retina on the last follow up and had persistent tumor outcomes. Conclusion The management outcomes in VHL-related RCH depend on the lesionsize and location, severity of the clinical picture on presentation and the development of complications. Clinical trial number Not applicable.https://doi.org/10.1186/s40942-025-00721-1Von Hippel-LindauRetinal capillary hemangioblastomaExudative retinal detachmentTractional retinal detachmentPars plana vitrectomy
spellingShingle Abdulrahman Khan
Wael A. Alsakran
Abdullah Barry
Abdulrahman H. Badawi
Moustafa S. Magliyah
The clinical features and real world treatment outcomes in von Hippel Lindau related retinal capillary hemangioblastomas
International Journal of Retina and Vitreous
Von Hippel-Lindau
Retinal capillary hemangioblastoma
Exudative retinal detachment
Tractional retinal detachment
Pars plana vitrectomy
title The clinical features and real world treatment outcomes in von Hippel Lindau related retinal capillary hemangioblastomas
title_full The clinical features and real world treatment outcomes in von Hippel Lindau related retinal capillary hemangioblastomas
title_fullStr The clinical features and real world treatment outcomes in von Hippel Lindau related retinal capillary hemangioblastomas
title_full_unstemmed The clinical features and real world treatment outcomes in von Hippel Lindau related retinal capillary hemangioblastomas
title_short The clinical features and real world treatment outcomes in von Hippel Lindau related retinal capillary hemangioblastomas
title_sort clinical features and real world treatment outcomes in von hippel lindau related retinal capillary hemangioblastomas
topic Von Hippel-Lindau
Retinal capillary hemangioblastoma
Exudative retinal detachment
Tractional retinal detachment
Pars plana vitrectomy
url https://doi.org/10.1186/s40942-025-00721-1
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