Long-term follow-up of occlusion of the central retinal vein in young adults.

We did a long-term follow-up study of 42 patients aged 40 years and younger who had occlusion of the central retinal vein in order to learn its course and determine the frequency of related systemic disorders. Two groups of venous occlusion (complete and incomplete) were delineated by initial ocular findings. Final visual prognosis could not be predicted by the severity of the venous occlusion at the time of diagnosis.

Ocular manifestations of intracranial chordomas.

Sixty-three patients with confirmed intracranial chordoma were studied retrospectively to determine the incidence of various presenting symptoms and signs. Most of the ocular signs were due to cranial nerve involvement. Sixth cranial nerve palsy occurred as the sole presenting sign in 29% of patients, whereas extraocular muscle palsies of various combinations were present in 62%.

Radiation retinopathy after irradiation of intracranial lesions.

Six patients developed a mild retinopathy after radiation therapy for intracranial lesions. The retinopathy, consisting of capillary microaneurysms, cotton-wool patches, and telangiectasis, developed after a latent period that varied between nine months and three years. The visual acuity remained within normal limits.

Visual prognosis of optic nerve sheath meningiomas producing shunt vessels on the optic disk.

Nine patients--seven women and two men--had meningiomas of the optic nerve sheath. The defect was bilateral in two, in the right eye in six, and in the left eye in one. Most had edema of the disk, and all had progressive loww of vision in the affected eye owing to optic nerve atrophy.

The ophthalmologic manifestations of Wilson's disease.

Fifty-three patients with Wilson's disease were studied with regard to ophthalmologic abnormalities. Of the 35 symptomatic patients initially seen and treated at the Mayo Clinic, 34 (97%) had Kayser-Fleischer rings and 6 (17%) had sunflower cataracts at the time of diagnosis. In patients followed for a year or more, penicillamine therapy resulted in improvement of the Kayser-Fleischer rings in 18 of 20 (90%) patients and total clearing of the sunflower cataracts in 4 of 5 patients.

Transsphenoidal decompression of the optic nerve and chiasm. Visual results in 62 patients.

A review of recent experience with transsphenoidal for lesion in and about the sella turcica establishes the value of this approach for the management of patients which visual loss. The lesions encountered consisted of pituitary adenoma in 45 cases, craniopharyngioma in 10 cases, and miscellaneous tumours involving the sella in the remaining seven cases. Sixty of the 62 patients in this series had quantitative determination of preoperative and postoperative visual status; after surgery, vision was improved in 81%, unchanged in 11%, and worse in 5%.

Pigmented corneal rings in non-Wilsonian liver disease.

Kayser-Fleischer rings are pigmented corneal rings at the limbus of the cornea in Descemet's membrane that have been deemed pathognomonic of Wilson's disease. However, we have observed four exceptions in patients with non-Wilsonian liver disease. Three patients had primary biliary cirrhosis and one patient had chronic aggressive hepatitis with cirrhosis.

Visual prognosis of optic nerve sheath meningiomas producing shunt vessels on the optic disk: the Hoyt-Spencer syndrome.

Nine patients, including seven women and two men, had meningiomas of the optic nerve sheath. The defect was bilateral in two, in the right eye in six, and in the left eye in one. Most had edema of the disk, and all had progressive loss of vision to blindness in the affected eye owing to optic nerve atrophy.

Pigmented corneal rings in a patient with primary biliary cirrhosis.

A patient with primary biliary cirrhosis who had high hepatic, serum, and urine copper levels was found by slit-lamp biomicroscopy to have pigmented corneal rings similar to Kayser-Fleischer rings. Wilson's disease was excluded on the basis of the elevated serum ceruloplasmin and copper levels, the lack of neurological disease despite advanced liver disease, a negative family history, and the typical clinical features of primary biliary cirrhosis. Although the exact chemical nature of the corneal rings is unknown, the cornea may be another tissue associated with elevated tissue copper levels in primary biliary cirrhosis.