Clinicopathologic correlation of argon laser photocoagulation of retinal angiomas in a patient with von Hippel-Lindau disease followed for more than 20 years.

PURPOSE

The authors review the histopathologic findings in the eyes of a patient with multiple retinal angiomas and von Hippel-Lindau disease, who underwent treatment with argon laser photocoagulation with follow-up of more than 20 years.

METHODS

The patient was studied ophthalmoscopically and by fluorescein angiography before and after argon laser photocoagulation of retinal angiomas. The eyes were obtained postmortem, and the central portion of the right eye, including the macula and optic nerve head, was sectioned serially for light microscopy. The pupil-optic nerve segment of the left eye was step-sectioned serially for light microscopy.

RESULTS

Histopathologic study of the right eye disclosed mild cystoid macular edema and focal areas of exudation in the midperiphery possibly secondary to irradiation of the head. A 1.5 x 0.3-mm area of residual angioma was present in the nasal peripapillary retina. Superotemporally, four chorioretinal scars were present in one photocoagulated area. These scars were composed of dense fibrous tissue with vascularization and variable retinal pigment epithelium hyperplasia. Large, nonangiomatous vessels within each of the scars were continuous with other retinal vessels. Inferotemporally, two chorioretinal scars were present in one photocoagulated area. Histopathologically, these scars were similar to the superotemporal scars, except that no patent retinal vessels traversed the inferotemporal scars. Neovascularization of the retina was associated with one superotemporal and one inferotemporal scar. No residual angiomatous tissue was present in the supero- or inferotemporal areas. Histopathologic examination of the left eye disclosed extensive vitreous organization and periretinal fibrovascular proliferation, extensive gliosis of the retina, and a 4.5 x 2-mm schisis cavity filled with fibrinous exudate. Three angiomas with variable fibrosis were present in the left eye.

CONCLUSION

Despite a poor clinical course in one eye treated with xenon arc photocoagulation, trans-scleral diathermy, and argon laser photocoagulation, a patient with von Hippel-Lindau disease and multiple retinal angiomas retained good vision in the other eye after successful treatment with argon laser photocoagulation with follow-up of more than 20 years. The amount of regression of angiomatous tissue after photocoagulation varied from lesion to lesion (complete in some; minimal in others). The authors conclude that argon laser photocoagulation of early lesions is effective in ablating smaller ( < or = 3-disc diameter) retinal angiomas.