THE RETINAL DEEP CAPILLARY PLEXUS AS A VENOUS OUTFLOW SYSTEM: INSIGHTS FROM STURGE-WEBER SYNDROME.

PURPOSE

To report on the venous abnormalities of a patient with Sturge-Weber syndrome.

METHOD

Case report.

PATIENT

A 29-year-old woman with a history of Sturge-Weber syndrome since infancy was referred for evaluation of possible diffuse choroidal hemangioma. Multimodal imaging, including ultra-widefield fluorescein, indocyanine green, and optical coherence tomography angiography, was performed.

RESULTS

Dilated fundus examination was remarkable for increased cupping of the optic disk in the right eye, venous tortuosity, and marked dilation of the choroidal vessels. Ultra-widefield fluorescein angiography confirmed marked venous tortuosity and dilation, as well as anastomoses of the retinal veins ipsilateral to the port wine stain. Indocyanine green angiography revealed marked engorgement of the vortex veins and choroidal vasculature. Optical coherence tomography angiography revealed dilated vascular channels in the deep capillary plexus that were directly anastomosing to the superficial capillary plexus, but not the intermediate capillary plexus. Engorgement of the ampullae of the deep capillary plexus vortex system was also observed. The normal contralateral eye was used as comparison for all imaging studies.

CONCLUSION

These findings support the notion of generalized venous hypertension state in adult eyes with Sturge-Weber syndrome and corroborate previous evidence that the deep capillary plexus acts as a venous outflow system.