|Year : 2021 | Volume
| Issue : 2 | Page : 208-209
A solitary retinal hemangioblastoma
Syed Saifuddin Adeel, Syed Mohideen Abdul Khadar, Vinit Jayendra Shah, Shobita Nair
Department of Retina and Vitreous, Aravind Eye Hospital, Tirunelveli, Tamil Nadu, India
|Date of Submission||03-Oct-2020|
|Date of Acceptance||30-Nov-2020|
|Date of Web Publication||24-Jun-2021|
Dr. Syed Saifuddin Adeel
Department of Retina and Vitreous, Aravind Eye Hospital, Tirunelveli, Tamil Nadu
Source of Support: None, Conflict of Interest: None
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
| Case Summary|| |
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|| |
Retinal hemangioblastoma, also known as RCH is a vascular hamartoma that usually has onset in the first two decades of life. 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.
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.
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.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
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[Figure 1], [Figure 2], [Figure 3]