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. The presence of anomalous disk vessels closely correlated with a favorable prognosis in patients who had complete occlusion of the central retinal vein. None of the patients with incomplete central vein occlusion developed neovascular glucoma; three (14%) of the 21 patients with complete venous occlusion developed neovascular glucoma, which resulted in enucleation. Significant associated systemic maladies included cardiovascular disease and diabetes mellitus. An apparent correlation exists between occlusion of the central retinal vein and early death.

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%. Visual field defects were demonstrated in 24% of patients, but only 19% had papilledema or optic atrophy. The chordomas arose from the clivus in 92% of patients, but different patterns of cranial nerve involvement occurred which correlated with the different sites of extension of the tumor.

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. The severity of the retinopathy is related to the retinal or optic nerve dose of radiation.

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. In 10 of the 11 affected eyes, opticocilliary shunt veins developed on the optic disk. Exophthalmos was mild and did not measure more than 3 mm in any patient. Ocular movement was mildly impaired in three patients. Indentation and flattening of the posterior pole of the eye were documented in five eyes. Nine of the 11 eyes progressed to blindness; 1 was worse after operation, and the outcome of the 11th is unknown because the patient was lost to follow-up. Surgical treatment has not been effective in these cases or in those reported in the literature.

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. The specific pattern of copper deposition in Kayser-Fleischer rings and the improvement with treatment occurred along four reproducible stages. None of five asymptomatic siblings of patients with known Wilson's disease had Kayer-Fleischer rings at the time of initial study. In one (untreated) of the five, Kayser-Fleischer rings developed 20 months after the initial normal slit-lamp examination. The presence of Kayser-Fleischer rings, and the absence of other ophthalmologic signs (such as nystagmus, cranial nerve palsies, and other movement disorders), can be of great assistance in the diagnosis of Wilson's disease. Once the condition has been diagnosed, specific medical therapy with penicillamine and low-copper diet dramatically improves what would otherwise be an inevitably fatal course.

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%. Two patients (3%) died during the immediate postoperative period before their visual status could be evaluated.

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. Pigmented corneal rings were seen only by slit-lamp examination. Hepatic, serum, and urinary copper and serum ceruloplasmin levels were significantly elevated in the patients with primary biliary cirrhosis. Radiocopper (64Cu or 67Cu) studies in patients with primary biliary cirrhosis showed plasma disappearance curves which allowed a clear distinction from Wilson's disease in that all three patients with primary biliary cirrhosis showed a secondary rise in radiocopper that presumably represented copper incorporation into ceruloplasmin. In one patient, in whom 64Cu in ceruloplasmin was studied specifically, incorporation was found to be normal.

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. In 10 of the 11 affected eyes, opticociliary shunt veins developed on the optic disk. Exophthalmos was mild and not more than 3 mm in any patient. Ocular movement was mildly impaired in three patients. Indentation and flattening of the posterior pole of the eye was documented in five eyes. Nine of the eleven eyes went on to blindness; one was worse after operation, and the eleventh was lost to follow-up. Surgical treatment has not been effective in these cases or in those reported in the literature.

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.