Congenital trigeminal anesthesia in two siblings and their long-term follow-up.

PURPOSE: To alert ophthalmologists to congenital trigeminal anesthesia as a cause of corneal scarring and amblyopia and its effective treatment with tarsorrhaphies. METHODS: Case reports. A 2-month-old infant presented with bilateral corneal erosions and complete corneal anesthesia. Her sister presented at age 3 years with a corneal ulcer and corneal hypoesthesia (sensation markedly decreased). The father and paternal grandmother of the siblings also had corneal hypoesthesia. RESULTS: Further investigation of the infant revealed bilateral hearing loss, swallowing difficulties, and decreased sensation in the trigeminal nerve distribution. A diagnosis of congenital trigeminal anesthesia was made. The corneal erosions of the patient resolved with bilateral two-thirds width tarsorrhaphies. The girl continues to do well now at 10 years of age with ocular lubrication and superficial corneal scar removal. Her older sister initially required antibiotic ointment for her corneal ulcer but now requires only ocular lubrication for congenital trigeminal anesthesia. CONCLUSION: This study describes the earliest reported use of tarsorrhaphies in an infant with congenital trigeminal anesthesia. The presence of this condition in her sister and relatives makes it one of the few reports of congenital trigeminal anesthesia in more than two generations. Early recognition of this condition is essential in the preservation of useful vision.

Cyclosporine A for the treatment of necrotizing scleritis and corneal melting in patients with rheumatoid arthritis.

Two of the ocular complications of rheumatoid arthritis (RA), necrotizing scleritis and keratolysis, have been associated with poor response to standard ocular therapy, and thus poor ocular outcome. We describe 6 patients with active ocular disease and active RA who failed to respond to disease modifying agents or immunosuppressive therapy and whose ocular and arthritic disease subsequently responded to cyclosporine A. Drug related complications were consistent with those reported for patients receiving similar doses for RA.

Changing indications for penetrating keratoplasty in Vancouver, 1978-87.

Indications for penetrating keratoplasty (PK) were assessed by clinicopathological review of 659 corneal buttons submitted from 1978 to 1987 to the Ophthalmic Pathology Service in Vancouver. Leading indications for PK were bullous keratopathy (22.2%), keratoconus (17.1%), scarring with or without chronic inflammation (13.5%), graft failure (12.1%), scarring or active keratitis secondary to virus (9.0%) and Fuchs' dystrophy (8.3%). The principal factors responsible for graft failure were also judged by clinicopathological correlation. The authors compare their findings with those in other series.

Corneal posterior crocodile shagreen and polymorphic amyloid degeneration.

Globes were obtained post mortem from a 75-year-old patient who had both corneal posterior crocodile shagreen and polymorphic amyloid degeneration. The crocodile shagreen was typified by grayish, polygonal opacities with indistinct edges and separated by clear lines. The cloudy area involved the central two thirds of the corneas and included the posterior two thirds of the stroma, with increasing posterior density. The polymorphic amyloid degeneration was typical, demonstrating polymorphic punctate and filamentous deposits in the deeper corneal layers. The deposits appeared white in direct illumination and refractile in indirect illumination. Transmission electron microscopy revealed sawtoothlike configurations of the stromal collagen lamellae that corresponded to the central cloudy opacities seen clinically. The minute deposits were found to be amyloid by histochemical staining and electron microscopy.

Diagnosis of early lattice corneal dystrophy.

Seven children from two unrelated families had lattice corneal dystrophy. Their ages ranged from 3 to 13 years at initial examination. The children were observed for an average of 35 months. Three distinct early slitlamp characteristics were found. The first characteristic was subepithelial white opacities that were discrete, round or ovoid, nonrefractile, nonstaining, variably sized (estimated at 0.1 to 0.5 mm), and larger than the previously reported minute refractile dots. The second finding was a diffuse axial anterior stromal haze that was either the initial sign or developed in conjunction with the other two characteristics. The third finding was anterior stromal dots and filamentary lines that were refractile on indirect slitlamp illumination and white on direct illumination. Knowledge of these three slitlamp characteristics in conjunction with examination of older family members wil facilitate the early diagnosis of lattice corneal dystrophy.

Corneal elastosis in lattice corneal dystrophy: a clinicopathologic report.

The corneal button of a patient with lattice corneal dystrophy and the clinical features of elastotic degeneration was subjected to histochemical and electron microscopic examination. The elastotic material possesses distinct clinical and histological characteristics, in particular its ability to autofluorescence. This material stained positive with Verhoeff-van Gieson (elastic stain), negative to alizarin red (calcium stain), and was insensitive to elastase digestion. The characteristic electron microscopic picture of elastotic tissue and amyloid was also found. The clinical implications of elastotic degeneration are discussed.