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PHOTO ESSAY
Year : 2021  |  Volume : 59  |  Issue : 2  |  Page : 208-209

A solitary retinal hemangioblastoma


Department of Retina and Vitreous, Aravind Eye Hospital, Tirunelveli, Tamil Nadu, India

Date of Submission03-Oct-2020
Date of Acceptance30-Nov-2020
Date of Web Publication24-Jun-2021

Correspondence Address:
Dr. Syed Saifuddin Adeel
Department of Retina and Vitreous, Aravind Eye Hospital, Tirunelveli, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_150_20

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  Abstract 


Retinal hemangioblastoma is a rare benign retinal vascular tumor that occurs sporadically or in association with von Hippel-Lindau (VHL) disease. If associated with VHL, it can be multiple and/or bilateral. All retinal hemangioblastoma cases should be investigated for the presence of associated tumors such as central nervous system hemangiomas and visceral abdominal tumors. Herewith, we report a case of solitary capillary hemangioma in a young male.

Keywords: Macular scar, retinal capillary hemangioma, retinal hemangioblastoma, von Hippel-Lindau disease


How to cite this article:
Adeel SS, Khadar SM, Shah VJ, Nair S. A solitary retinal hemangioblastoma. TNOA J Ophthalmic Sci Res 2021;59:208-9

How to cite this URL:
Adeel SS, Khadar SM, Shah VJ, Nair S. A solitary retinal hemangioblastoma. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2021 Aug 5];59:208-9. Available from: https://www.tnoajosr.com/text.asp?2021/59/2/208/319272




  Case Summary Top


A 37-year-old male patient with a history of ocular trauma at 12 years of age came for a routine checkup. His best-corrected visual acuity in OD was 6/6 and OS 2/60. The anterior segment was within the normal limits. The left eye fundus showed scarring at the macula due to previous trauma and a solitary, spherical well-circumscribed reddish lesion in the supero-temporal mid periphery about two-disc diameter in size with feeder vessels suggestive of retinal capillary hemangioma (RCH) [Figure 1]. Associated adjacent neurosensory retinal detachment and hard exudates were noted [Figure 2]. Magnetic resonance imaging brain and ultrasonography abdomen were within the normal limits ruling out von Hippel-Lindau (VHL) disease. Optical coherence tomography of the OS macular area showed significant scarring [Figure 3]. As the macula was already scarred, the patient was advised observation alone and asked to have regular follow-ups.
Figure 1: Montage fundus photograph of the left eye: Solitary retinal capillary hemangioma seen in the supero-temporal mid-periphery with the macular scar

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Figure 2: Fundus photograph of the left eye: retinal capillary hemangioma with tortuous feeder vessels, adjacent neurosensory retinal detachment, and hard exudates

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Figure 3: Spectral-domain optical coherence tomography of the left eye showing IS/OS disruption with the macular scar

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  Discussion Top


Retinal hemangioblastoma, also known as RCH is a vascular hamartoma that usually has onset in the first two decades of life.[1] Most of the peripheral RCH tumors reported are seen in the supero-temporal or inferotemporal quadrants with feeder vessels extending from the optic disc. Lipid exudates and serous retinal detachment surrounding the tumor is common.[2]

Treatment is decided on the size of the tumor, location, associated retinal detachment, and visual acuity. Observation alone is advised if it is a small solitary lesion with minimal or no exudation, not threatening the vision. Treatment options include surgical resection, cryotherapy, photodynamic therapy, photocoagulation, anti-vascular endothelial growth factor therapy, or radiation.[3]

Solitary RCH can also be an initial manifestation of VHL, and subsequently, patients can develop other tumors later on in life, emphasizing the need for regular ophthalmic and systemic evaluation.[4]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Maher ER, Yates JR, Harries R, Benjamin C, Harris R, Moore AT, et al. Clinical features and natural history of von Hippel-Lindau disease. Q J Med 1990;77:1151-63.  Back to cited text no. 1
    
2.
Webster AR, Maher ER, Moore AT. Clinical characteristics of ocular angiomatosis in von Hippel-Lindau disease and correlation with germline mutation. Arch Ophthalmol 1999;117:371-8.  Back to cited text no. 2
    
3.
Singh AD, Nouri M, Shields CL, Shields JA, Perez N. Treatment of retinal capillary hemangioma. Ophthalmology 2002;109:1799-806.  Back to cited text no. 3
    
4.
Singh AD, Nouri M, Shields CL, Shields JA, Smith AF. Retinal capillary hemangioma: A comparison of sporadic cases and cases associated with von Hippel-Lindau disease. Ophthalmology 2001;108:1907-11.  Back to cited text no. 4
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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