Keratitis associated with the multiple endocrine deficiency, autoimmune disease, and candidiasis syndrome.

Optimal management of the ocular and systemic components of the multiple endocrine deficiency, autoimmune disease, candidiasis syndrome requires early identification of affected individuals. This autosomal recessive syndrome is characterized by hypoparathyroidism, Addison's disease, and chronic mucocutaneous candidiasis among other endocrinopathies and immune disorders. We retrospectively reviewed 16 patients, 14 with the full syndrome and two with the syndrome partially manifested. Four of these patients manifested a self-limited, bilateral keratitis in which the age of onset ranged from 2 to 9 years. Keratitis preceded the onset of any endocrinopathy in two of four patients and was among the first signs of the syndrome. The keratitis was not caused by hypoparathyroidism or candidiasis. The anterior stromal vascularization and scarring resulted in a visual acuity of 20/50 or worse in four of eight affected eyes. We recommend medical management of the corneal disease without surgical intervention.

Keratitis with hypoparathyroidism.

Chronic kiratitis developed in two children with nonsurgical hypoparathyroidism, as part of an autosomal recessive syndrome that included adrenal insufficiency and moniliasis in what was postulated to be an autoimmune disease. The corneal changes may also have been caused by autoimmune mechanism. Activity of the keratitis diminished once the hypoparathyroidism had been brought under control. However, these patients were thought to be at risk for adrenal insufficiency: neither had moniliasis or adrenocortical insufficiency at present, but the features of the hypoparathyroidism adrenal insufficiency-moniliasis syndrome appeared at different ages and in differing sequences.

Lingeous conjunctivitis with tracheal obstruction. A case report, with light and electron microscopy findings.

A white male infant of 1 year had unilateral membranous conjunctivitis and severe laryngotracheobronchitis which required tracheostomy. Cultures from eye and throat swabs and of fluid suctioned through the tracheostomy grew many organisms, including H. influenzae, adenovirus type 3, and Candida species, but he had no specific immunologic disturbance. Ligneous conjunctivitis was diagnosed. The infant's general condition responded slowly to intensive therapy but the membrane continued to slough off the regrow. The excised membrane contained massive subepithelial deposits of eosinophilic material and a moderately vascular chronic inflammatory-cell infiltrate with numerous mast cells in the perivascular spaces and the hyaline membrane. The conjunctivitis cleared when treated with topical sodium cromoglycate (Intal), a known inhibitor of mediator release from mast-cell granules. The success of Intal therapy in this case supports the theory that mast cells are involved in the pathogenesis of ligneous conjunctivitis.

Sodium cromoglycate (intal) in the treatment of vernal keratoconjunctivitis and allergic conjunctivitis.

Intal drops (sodium cromoglycate) relieved symptoms of vernal conjunctivitis in all the 19 patients we studied, reducing the need for steroids although the response varied. Intal was also effective in all 11 patients with acute or subacute seasonal allergic conjunctivitis, but in only 4 of 11 patients with mild chronic conjunctivitis associated with atopy. No side-effects were noted other than mild irritation in some cases.

Impression cytology in conjunctivitis.

A technique is described for studying the cellular response in various conjunctival disorders. A round plastic disc (Thermanox) is pressed against the patient's conjunctive, and the material adhering to the disc is stained with a modified Wright's stain (Diff-Quik) for light microscopy. This method compares favourably with the classic conjunctival scraping method. The advantages of the impression technique are that no local anesthetic is needed for abtaining conjunctival samples, it is painless and can readily be used in children, repeated samples can be taken within a short time, the staining procedure can be completed within 15 seconds, and the discs can be processed for electron microscopic studies of the cellular samples.

The Vogt--Koyanagi--Harada syndrome in children.

Two cases of the Vogt--Koyanagi--Harada syndrome in children, a very rare disorder in this age group, are reported; one patient had the chronic diffuse type, and the other had neurologic symptoms and exudative retinal detachments. Various theories of causation of the syndrome have been proposed, including viral infection, sympathetic ophthalmia, and altered immunologic status, but the etiology remains obscure.

Uveitis in children.

Endogenous uveitis in 117 children aged 15 years or under was investigated at The Hospital for Sick Children, Toronto, in a 12-year period from 1953 to 1964. This group included 55 children with anterior uveitis, 59 with posterior uveitis, and three with diffuse uveitis. An etiologic diagnosis could be made or the uveitis recognized as part of a definite clinical syndrome in approximately 47% of the 117 children. The commonest cause of posterior uveitis was toxoplasmosis and the commonest associated finding in anterior uveitis was juvenile rheumatoid arthritis. Chronic cyclitis of unknown etiology was a relatively common disease.