The Eye Siren - A Case of VHL

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File number: 133868


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    • By surabhi gupta
      centre for sight new delhi
      Co-author(s): Dr Surabhi Gupta, Dr Manisha Agarwal
    • Uploaded on Dec 3, 2025.
    • Last modified by Joshua Friedman on Dec 4, 2025.
    Rating
    Appears in
    Miscellaneous
    Condition/keywords
    retinal capillary hemangioma
    Photographer
    Mr Brajesh Kumar
    Imaging device
    zeiss visucam 500
    Description
    A 25 year old man presented with chief complaints of dimuntion of vision in right eye for past 2 weeks. Best corrected visual acuity in right eye was 6/12, N8 and left eye was 6/6, N6. Montage color fundus photograph shows bilateral multiple capillary hemangioma with epiretinal membrane causing traction over macula in right eye. On PET-CT multiple metabolically inactive cystic lesion were noted in the pancreas and seminal vesicle with low grade metabolically active cystic lesion with enhancing septations in kidneys which were suspicious of malignant etiology. MRI brain showed presence of a small cystic lesion anterior to celebellar vermis suggestive of CNS hemangioblastoma. A diagnosis of Von Hippel- Lindau syndrome was made the patient is under oncologist care for suspected RCC.

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