Surgical management of macular edema associated with von Hippel-Lindau disease: a case report

Authors

DOI:

https://doi.org/10.31288/oftalmolzh202555861

Keywords:

high-frequency electric welding of biological tissues, von Hippel-Lindau disease, vitrectomy, retinal hemangioblastoma, macular edema, retina

Abstract

We report a case of surgical management of macular edema associated with von Hippel-Lindau (VHL) disease. The patient received vitrectomy with angioma endoresection using high-frequency welding of biological tissues. Follow-up findings included complete resorption of hard exudates, improvement in the macular profile and partial restoration of vision. A review of the treatment outcome for this case confirms the efficacy of vitrectomy with endoresection of retinal hemangioblastoma in the management of macular edema associated with VHL disease. Timely diagnostic evaluation and adequate postoperative follow-up are essential for the best treatment outcome.

Author Biographies

K. S Kantser, SI «The Filatov Institute of Eye Diseases and Tissue Therapy of the NAMS of Ukraine»

Kantser K. S., Intern Physician, SI “The Filatov Institute of Eye Diseases and Tissue Therapy of the National Academy of Medical Sciences of Ukraine”, Odesa (Ukraine)

M. M. Umatets, SI «The Filatov Institute of Eye Diseases and Tissue Therapy of the NAMS of Ukraine»

Umanets M. M., Dr Sc (Med), Professor, SI “The Filatov Institute of Eye Diseases and Tissue Therapy of the National Academy of Medical Sciences of Ukraine”, Odesa (Ukraine)

References

Vaganovs P, Bokums K, Miklaševics E, Plonis J, Zarina L, Geldners I, et al. Von hippel-lindau syndrome: diagnosis and management of hemangioblastoma and pheochromocytoma. Case Rep Urol. 2013;2013:624096. https://doi.org/10.1155/2013/624096

Binderup MLM, Stendell AS, Galanakis M, Møller HU, Kiilgaard JF, Bisgaard ML. Retinal hemangioblastoma: prevalence, incidence and frequency of underlying von Hippel-Lindau disease. Br J Ophthalmol. 2018 Jul;102(7):942-947. https://doi.org/10.1136/bjophthalmol-2017-310884

Webster AR, Maher ER, Moore AT. Clinical characteristics of ocular angiomatosis in von Hippel-Lindau disease and correlation with germline mutation. Arch Ophthalmol. 1999 Mar;117(3):371-8.https://doi.org/10.1001/archopht.117.3.371

Khan HA, Shahzad MA, Iqbal F, Awan MA, Khan QA, Saatci AO, et al. Ophthalmological Aspects of von-Hippel-Lindau Syndrome. Semin Ophthalmol. 2021 Oct 3;36(7):531-540. https://doi.org/10.1080/08820538.2021.1897851

Parmar DN, Mireskandari K, McHugh D. Transpupillary thermotherapy for retinal capillary hemangioma in von Hippel-Lindau disease. Ophthalmic Surg Lasers. 2000 Jul-Aug;31(4):334-6.https://doi.org/10.3928/1542-8877-20000701-14

Di Nicola M, Williams BK Jr, Hua J, Bekerman VP, Mashayekhi A, Shields JA, Shields CL. Photodynamic Therapy for Retinal Hemangioblastoma: Treatment Outcomes of 17 Consecutive Patients. Ophthalmol Retina. 2022 Jan;6(1):80-88.https://doi.org/10.1016/j.oret.2021.04.007

Wiley HE, Krivosic V, Gaudric A, Gorin MB, Shields C, Shields J, et al. Management of retinal hemangioblastoma in von Hippel-Lindau disease. Retina. 2019 Dec;39(12):2254-2263. https://doi.org/10.1097/IAE.0000000000002572

Feletti A, Anglani M, Scarpa B, Schiavi F, Boaretto F, Zovato S, et al. Von Hippel-Lindau disease: an evaluation of natural history and functional disability. Neuro Oncol. 2016 Jul;18(7):1011-20. https://doi.org/10.1093/neuonc/nov313

Dollfus H, Massin P, Taupin P, Nemeth C, Amara S, Giraud S, et al. Retinal Hemangioblastoma in von Hippel-Lindau disease: A Clinical and Molecular Study. Invest Ophthalmol Vis Sci. 2002;43:3067-74.

Aiello LP, George DJ, Cahill MT, Wong JS, Cavallerano J, Hannah AL, et al. Rapid and durable recovery of visual function in a patient with von Hippel-Lindau syndrome after systemic therapy with vascular endothelial growth factor receptor inhibitor su5416. Ophthalmology. 2002 Sep;109(9):1745-51.https://doi.org/10.1016/S0161-6420(02)01159-4

Schlesinger T, Appukuttan B, Hwang T, Atchaneeyakasul LO, Chan CC, Zhuang Z, et al. Internal en bloc resection and genetic analysis of retinal capillary hemangioblastoma. Arch Ophthalmol. 2007 Sep;125(9):1189-93.https://doi.org/10.1001/archopht.125.9.1189

Gaudric A, Krivosic V, Duguid G, Massin P, Giraud S, Richard S. Vitreoretinal surgery for severe retinal capillary hemangiomas in von hippel-lindau disease. Ophthalmology. 2011 Jan;118(1):142-9. https://doi.org/10.1016/j.ophtha.2010.04.031

Umanets MM, Pasyechnikova NV, Vit VV, Naumenko VO. [Vitrectomy using high-frequency welding of biological tissues in resection of retinal hemangioma in patients with von Hippel-Lindau syndrome]. Oftalmol Zh. 2013;6: 51-56. Russian.

Pasyechnikova NV, Umanets MM, Artiomov OV, Vasylchenko VA. [High-frequency welding of posterior segment tissues (modified generator EK-300M1) using proprietary unipolar and bipolar instrumentarium]. Oftalmol Zh. 2012;2: 45-49. Russian.

Pasyechnikova NV, Naumenko VO, Umanets MM, Levytska GV, Nazatrtyan RE, Lakiza GV, inventors: SI "The Filatov Institute of Eye Diseases and Tissue Therapy of the National Academy of Medical Sciences of Ukraine", owner. [Method of surgical treatment for retinal hemangioma associated with von Hippel-Linday disease using high-frequency welding of biological tissues]. Patent of Ukraine No. 133452 issued 25.03.2015. Ukrainian.

Otero-Marquez O, Chui TY, Pinhas A, Castanos Toral MV, Zhou DB, Migacz J, et al. 3-D OCT angiographic evidence of Anti-VEGF therapeutic effects on retinal capillary hemangioma. Am J Ophthalmol Case Rep. 2022 Feb 7;25:101394. https://doi.org/10.1016/j.ajoc.2022.101394

Dahr SS, Cusick M, Rodriguez-Coleman H, Srivastava SK, Thompson DJ, Linehan WM, et al. 3rd, Chew EY. Intravitreal anti-vascular endothelial growth factor therapy with pegaptanib for advanced von Hippel-Lindau disease of the retina. Retina. 2007 Feb;27(2):150-8. https://doi.org/10.1097/IAE.0b013e318030a290

Wong WT, Liang KJ, Hammel K, Coleman HR, Chew EY. Intravitreal ranibizumab therapy for retinal capillary hemangioblastoma related to von Hippel-Lindau disease. Ophthalmology. 2008 Nov;115(11):1957-64. https://doi.org/10.1016/j.ophtha.2008.04.033

Downloads

Published

2025-10-30

How to Cite

[1]
Kantser, K.S. and Umatets, M.M. 2025. Surgical management of macular edema associated with von Hippel-Lindau disease: a case report. Journal of Ophthalmology (Ukraine). 5 (Oct. 2025), 58–61. DOI:https://doi.org/10.31288/oftalmolzh202555861.

Issue

No. 5 (2025): Journal of Ophthalmology (Ukraine)

Section

Case Reports

License

Copyright (c) 2025 Kantser K. S., Umatets M. M.

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

This work is licensed under a Creative Commons Attribution 4.0 International (CC BY 4.0) that allows users to read, download, copy, distribute, print, search, or link to the full texts of the articles, or use them for any other lawful purpose, without asking prior permission from the publisher or the author as long as they cite the source.

COPYRIGHT NOTICE

Authors who publish in this journal agree to the following terms:

  1. Authors hold copyright immediately after publication of their works and retain publishing rights without any restrictions.
  2. The copyright commencement date complies the publication date of the issue, where the article is included in.

DEPOSIT POLICY

  1. Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) during the editorial process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
  2. Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work with an acknowledgement of its initial publication in this journal.
  3. Post-print (post-refereeing manuscript version) and publisher's PDF-version self-archiving is allowed.
  4. Archiving the pre-print (pre-refereeing manuscript version) not allowed.