A 26-year-old male presented with a two-year history of decreased visual acuity (VA) in left eye (LE).
He had the causal mutation of VHL disease, father and brother with systemic manifestations of the syndrome.
On examination, the VA of the left eye is 0.4.
The fundus of the left eye showed an endophytic tumor with two papilla diameters in the superior temporal arch with dilated nutrient vessels.
Optical coherence tomography (OCT) showed diffuse cystic edema in the posterior pole and angiography (AFG), early hyperfluorescence of the lesion with leakage in late times.
A systemic study was requested that also showed multiple solid nodules in both kidneys suggestive of bilateral hypernephroma (posteriorly confirmed after performing left radical nephrectomy), a probable hypernephroblastoma and multiple medullary prouberangioblastoma in the right medullary cord, pancreatic.
1.
An argon laser photocoagulation was initiated in two sessions during which subretinal hemorrhage occurred along the vascular arcade and vitreous hemorrhage (VH).
At two months and in the absence of reabsorption of the HV, a decision was made to perform a 23G resection, during which the treatment of the lesion by means of endolaser was completed.
Three months after surgery the VA is 0.6.
OCT showed a good foveal profile, with resolution of macular edema.
The GAF confirms the closure of the nutrient vessels, the lesion presents a centric aspect, and although it captures from early times, there is no leak, observing the vessels of substitution at the periphery of the lesion.
