Table 1.
Brief summary of the clinical features in three VHL patients
Patient 1 | Patient 2 | Patient 3 | |
---|---|---|---|
Age | 40 | 40 | 35 |
Gender | Male | Female | Female |
Systemic VHL involvement | Bilateral renal cell carcinoma and brainstem hemangioblastomas | Kidney cyst and Spinal cord hemangioblastoma (T4 and T5) | Brainstem hemangioblastoma Spinal cord hemangioblastoma (C6) |
Duration of ocular VHL | 25 years | 9 years | 21 years |
Ocular VHL | One large juxtapapillary RH and several small peripheral RHs | One large juxtapapillary RH and several small peripheral RHs | Multiple small peripheral RHs |
Therapies | Intravitreal anti-VEGF (ranibizumab and bevacizumab), laser photocoagulation and radiation | Intravitreal anti-VEGF (ranibizumab), laser photocoagulation and radiation | Laser photocoagulation and radiation, no anti-VEGF was administered |
Enucleation | Left eye in 2003 Right eye in 2012a |
Right eye in 2009 a | Left eye in 1991 Right eye in 2004 a |
The enucleated eye presented in this study.