Familial corneal scarring: a new dystrophy?

A 34-year-old white man with skin manifestations of the epidermal nevus syndrome was found to have anesthetic corneas, dry eyes, and localized opaque nodular elevations of both corneas. Similar corneal lesions, unassociated with corneal anesthesia, were found in four family members. Histology of corneal biopsies from the proband revealed superficial stromal scarring. The possible relationship between this familial condition and Salzmann's nodular corneal dystrophy, Goldenhar syndrome, and epidermal nevus syndrome is discussed.

Peripheral corneal degeneration and occlusive vasculitis in Wegener's granulomatosis.

A 61-year-old white woman, who had progressive arthritis over a two-year period, developed bilateral peripheral corneal degeneration. The superior cornea thinned to a thickness of approximately two thirds of normal. She died suddenly in the hospital and a primary occlusive vasculitis of the anterior ciliary arteries within the superior rectus muscles was found. There was necrotizing arteritis in the lungs, kidneys, adrenal caps, spleen, fallopian tubes, and skeletal muscle. The observation of an occlusive vasculitis of the anterior ciliary arteries in this patient supports the belief that ischemia may play a major role in peripheral marginal degeneration of the cornea.

A non-permanent tonic pupil in rheumatoid arteritis.

A 76-year-old male with a severely deforming rheumatoid arthritis, eosinophilia, polymyositis, and episcleritis developed a transient tonic pupil. The episcleritis, and a muscle biopsy revealing an occlusive arteritis with eosinophilia, suggest that a wide-spread rheumatoid arteritis caused a reversible ischemic insult to the ciliary ganglion and thus created a transient denervation of the pupil.

The impossible meningioma.

Experience in three cases has shown that a small meningioma in the extreme posterior portion of the orbit just anterior to the optic foramen may occur with progressive visual loss in the absence of orbital signs. Such lesions cannot be detected by plain skull roentgenography, polytomography, or selective angiography with subtraction and magnification. In one patient, even computerized axial tomography gave normal results, and the meningioma went undetected until exploratory craniotomy.

The tilted disk syndrome.

We noted occasional diagnostic confusion with papilledema and chiasmal compression when its true congential nature was not recognized in 12 patients with a benign ocular syndrome. Retinoscopy, fluorescein angiography, ocular ultrasonography, and histopathology suggested that associated visual field defects were related to a localized staphylomatous ectasia of the posterior globe.