A Melanoma Masquerader

A 23 year old hispanic presented for a comprehensive examination. Her health and ocular history was unremarkable and she was on no medications.

Her uncorrected VA was 20/20 OU. Pupils, confrontation visual field and EOM were all normal.

Refraction:

+0.25-0.50×180 OU BCVA 20/20

Anterior segment was WNL OU

Posterior pole was WNL OU

Peripheral evaluation of the right eye showed a 2-3DD elevated lesion in the mid periphery by the vortex veins. The lesion was mildly pigmented and there was no overlying lipofuscin or drusen or visible subretinal fluid.

The differential diagnosis for these choroidal tumors includes several life-threatening conditions, such as choroidal metastasis, lymphoma, and melanoma (Melanotic or Amelanotic). However, there are benign choroidal lesions including choroidal nevus and, rarely, vortex vein varix (VVV) that should be included in the differential.

Discussion:

A Melanoma Masquerader: “Choroidal Vortex Vein Varix”

Varix of the vortex vein ampulla is a bulging of the vein and overlying retina into the vitreous. It is of uncertain etiology. The lesion is lightly pigmented and ranges in size from one to three disc diameters. It has a dynamic nature and can be flattened by applying pressure to the globe. This finding carries little to no risk for thrombosis but can simulate other choroidal tumors with serious clinical implications, including choroidal metastasis and melanoma. Therefore, it is important to understand the imaging features of VVV to clearly differentiate this condition from potentially malignant lesions.

Below is the imaging of a 55-year-old man with VVV. Fundus image shows a red-brown lesion superonasally (A). Fluorescein angiography showed minimal hyperfluorescence superonasally corresponding with area of lesion (B). ICGA demonstrated good choroidal flow and a clear outline of the dilated vortex vein ampulla corresponding to the mass (C).

OCT through the lesion showed internal hyporeflectivity with smooth surface, consistent with VVV.

B-scan ultrasonography showed inflated varix upon gaze toward the lesion (C) and deflation on primary gaze (D) consistent with VVV.

 

Conclusion:

Vortex Vein Varix is a benign condition that may be mistaken with serious life threatening conditions such as choroidal melanoma or choroidal metastasis for instance. Imaging is key in these patients and should include B-scan ultrasound, OCT and ICGA. Our patient was sent for further evaluation which will include the above mentioned imaging modalities to confirm this (likely) diagnosis of VVV.

I hope you enjoyed this case, see you next month for another episode of Interesting Retinal Cases!