Effect of severing the check ligaments and intermuscular membranes on medial rectus recessions in infantile esotropia.

BACKGROUND: The effect of severing the check ligaments and intermuscular membranes on the dose-response curve for medial rectus recessions in patients with infantile esotropia has not been previously studied by randomized assignment techniques using contemporary control and experimental groups and masked precautions. METHODS: A prospective study of 101 patients was performed over an 8-year period. Patients were randomly assigned to either augmented or nonaugmented groups. In the former group, the medial check ligaments and intermuscular membranes were severed; in the latter group, no surgery was performed on the medial check ligaments or intermuscular membranes. Patients were postoperatively evaluated by an observer masked to group assignment. RESULTS: No difference in response to surgery was observed between the two groups at the time of evaluation, which took place, on average, 6 to 7 months after surgery. CONCLUSION: Severing the check ligaments and intermuscular membranes on patients with infantile esotropia undergoing medial rectus recessions does not appear to affect the dose-response curve.

Infantile glaucoma in Down's syndrome (trisomy 21).

We examined five patients with Down's syndrome and bilateral infantile glaucoma. In the first few months of life four patients had large cloudy corneas, breaks in Descemet's membrane, increased intraocular pressure, photophobia, and tearing. In one patient the diagnosis was delayed until 3 1/2 years of age because of concomitant nasolacrimal duct obstruction. Two patients developed cataracts and retinal detachment and have undergone multiple surgical procedures. The clinical course in these two older patients suggests that coexistence of congenital glaucoma, severe myopia, and cataracts in patients with trisomy 21 strongly predisposes for the development of retinal detachment and poor visual outcome.

Retinal dystrophy and macular coloboma.

Seven cases of retinal dystrophy associated with bilateral macular colobomata are presented. Two separate entities were found. The first is a congenital onset pigmentary retinopathy similar in electrophysiologic findings and symptoms to typical Leber's congenital amaurosis; the second appears to be a form of pregressive cone-rod dystrophy with pigmentary retinopathy. Review of the pertinant literature and clinical evidence suggest that both conditions are distinct entities inherited in the autosomal recessive manner.

Wegener's granulomatosis presenting as orbital pseudotumor in children.

The case of a nine-year-old white female who presented initially with idiopathic inflammatory pseudotumor and was ultimately diagnosed (22 months later) as having Wegener's granulomatosis is presented. A review of the literature reveals that, to date, there are 21 well-documented cases of Wegener's granulomatosis occurring in children under the age of 16. Of these 21 cases, eight patients (39%) manifested ocular or orbital signs during the course of the disease. Four of these cases (50%) presented initially with idiopathic inflammatory pseudotumor. The diagnosis of Wegener's granulomatosis should be considered in children with orbital pseudotumor. Long-term follow-up may be necessary to exclude this possibility.