RETINAL PHAKOMATOSIS AND VON HIPPEL-LINDAU PERIPHERAL CAPILLARY HEMANGIOBLASTOMA: PROPOSAL FOR STAGED SURGERY.

PURPOSE

Retinal capillary hemangioblastoma is present in about half of the carriers of the von Hippel-Lindau gene mutation and can lead to the evolution of blindness. Herewith is a proposal for surgical intraretinal feeder vessel ligature to induce ischemia of the phakoma and to resolve the exudative secondary retinal detachment, with satisfying results at the 6-month follow-up endpoint.

METHODS

The patient underwent a chandelier-assisted 23-gauge pars plana vitrectomy with valved trocars. A bimanual transretinal ligature of the feeder vessel of hemangioblastoma was performed, followed by localized endolaser to ablate the surrounding retina and capillaries, and a polydimethylsiloxane 1,000 final tamponade. After 3 months, exchange of polydimethylsiloxane/air/balanced salt solution, phacoemulsification, and in-the-bag intraocular lens implantation were performed.

RESULTS

At the sixth month, fundus fluorescein angiography showed a significant reduction of blood flow in the phakoma. The final best-corrected visual acuity was 6/6 (9 lines gain obtained compared with the baseline time).

CONCLUSION

This surgical technique seems to be effective and promising for the treatment of von Hippel-Lindau retinal capillary hemangioblastomas and their related retinal complications.