Marcel Frenkel

Cavernous hemangioma of the retina: Genetic and central nervous system involvement

A 1967 report described retinal “telangiectasia” in a brother and sister. The brother also had concurrent immune deficiency with hypogammaglobulinemia. Several reports have since been published on similar retinal vascular lesions, which have been termed “cavernous hemangioma of the retina.” In retrospect, it is likely that this sibling pair also had cavernous hemangioma of the retina and brain, and that the hypogammaglobulinemia was an independent problem. Our report provides a 19-year follow-up on three generations of this family. The affected generations support the diagnosis of a phakomatosis having autosomal dominant inheritance. Cavernous hemangioma of the retina is usually a stable disease, but there is a potential risk of intracranial hemorrhage from cavernous hemangioma of the brain. Family members, with and without fundus findings, should therefore undergo computed tomography or magnetic resonance imaging of the brain, because neurosurgical prophylaxis or treatment may be indicated. RETINA 5:215-220, 1985

Diagnosing Acute Retrobulbar Neuritis by Vitreous Fluorophotometry

We studied six patients with acute retrobulbar neuritis (including two with definite multiple sclerosis) during acute episodes and in convalescence, clinically and with fluorescein angiography and vitreous fluorophotometry. We compared vitreous fluorophotometric readings with visual-evoked responses. In seven of ten eyes with abnormal visual-evoked responses, there was an acute increase in posterior vitreous fluorophotometric readings. Vitreous fluorophotometry may be useful as objective evidence of (1) acute retrobulbar neuritis, (2) involvement of the asymptomatic eye in acute retrobulbar neuritis, (3) the duration of neuritic inflammation in acute retrobulbar neuritis, (4) a recurrence of acute retrobulbar neuritis, (5) alterations in the blood-ocular barrier in patients with acute retrobulbar neuritis in multiple sclerosis, and (6) the effects of corticosteroids and other therapeutic agents in the treatment of acute retrobulbar neuritis.

Vitreous fluorophotometry in optic nerve disease.

Posterior vitreous fluorophotometry measurements have been shown to be increased in patients with acute retrobulbar neuritis. We studied 12 patients who had optic neuropathy by posterior vitreous fluorophotometry, with and without correction for blood fluorescein concentration. Both uncorrected and corrected posterior vitreous measurements were increased in patients with inflammatory, obstructive, and infiltrative optic neuropathies. Concurrent values from anterior chamber fluorophotometry were not increased in any of the 8 patients studied.