Optical Coherence Tomography Angiography of Early Stage 1a Retinal Hemangioblastoma in Von-Hippel-Lindau

Main Article Content

Ananya Goswami
Abhidnya Surve
Pradeep Venkatesh

Keywords

hemangioblastoma, OCTA, retinal capillary hemangioblastoma, VHL

Abstract

Von-Hippel-Lindau (VHL) syndrome is characterized by focal vasoproliferative tumors of retinal capillaries called retinal capillary hemangioblastomas (RCH). These tumors are initially small and can be easily missed if not looked for carefully. As they grow, these tumors are more demanding to treat and hence the importance of detecting them early and treating them. Herein, we describe and review the optical coherence tomography angiography (OCTA) of the early-stage lesion, which suggested the involvement of superficial and a deeper retinal capillary plexus. In addition, to helping us detect these lesions earlier, OCTA may also help to understand the in vivo changes occurring at an earlier phase.

Abstract 631 | PDF Downloads 481 HTML Downloads 172 XML Downloads 49

References

1. Singh AD, Shields CL, Shields JA. von Hippel-Lindau Disease. Surv Ophthalmol. 2001;46(2):117–142. 10.1016/s0039-6257(01)00245-4

2. Venkatesh P, Takkar B. Proposed classification system for retinal capillary angiomatosis. Ophthalmic Res. 2019;61(2):115–119. 10.1159/000494498

3. Wittebol-Post D, Hes FJ, Lips CJ. The eye in von Hippel-Lindau disease. Long-term follow-up of screening and treatment: Recommendations. J Intern Med. 1998;243(6):555–561. 10.1046/j.1365-2796.1998.00338.x

4. Shields CL, Shields JA, De Potter P. Patterns of indocyanine green video angiography of choroidal tumours. Br J Ophthalmol. 1995;79(3):237–245. 10.1136/bjo.79.3.237

5. Apple DJ, Rabb MF. Ocular pathology: Clinical applications and self-assessment. St. Louis: Mosby; 1998.

6. Spencer WH. Ophthalmic pathology: An atlas and textbook. Philadelphia; W.B. Saunders; 1996.

7. Nesper PL, Fawzi AA. Human parafoveal capillary vascular anatomy and connectivity revealed by optical coherence tomography angiography. Invest Ophthalmol Vis Sci. 2018;59(10):3858–3867. 10.1167/iovs.18-24710

8. Lang SJ, Cakir B, Evers C, Ludwig F, Lange CAK, Agostini HT. Value of optical coherence tomography angiography imaging in diagnosis and treatment of hemangioblastomas in von Hippel-Lindau disease. Ophthalmic Surg Lasers Imaging Retina. 2016;47(10):935–946. 10.3928/23258160-20161004-07

9. Chou BW, Nesper PL, Jampol LM, Mirza RG. Solitary retinal hemangioblastoma findings in OCTA pre-and post-laser therapy. Am J Ophthalmol Case Rep. 2018;10:59–61. 10.1016/j.ajoc.2018.01.036

10. Sagar P, Rajesh R, Shanmugam M, Konana VK, Mishra D. Comparison of optical coherence tomography angiography and fundus fluorescein angiography features of retinal capillary hemangioblastoma. Indian J Ophthalmol. 2018;66(6):872. 10.4103/ijo.IJO_1199_17

11. Sagar P, Shanmugam PM, Konana VK, Ramanjulu R, Mishra KCD, Simakurthy S. Optical coherence tomography angiography in assessment of response to therapy in retinal capillary hemangioblastoma and diffuse choroidal hemangioma. Indian J Ophthalmol. 2019;67(5):701–703. 10.4103/ijo.IJO_1429_18

12. Chun LY, Massamba N, Silas MR, Blair MP, Hariprasad SM, Skondra D. Use of optical coherence tomography angiography in the diagnosis of small retina lesions in Von Hippel–Lindau disease. Eye. 2020;34(12):2345–2346. 10.1038/s41433-020-0792-5