Ophthalmic Manifestations in Patients With Neurofibromatosis

PURPOSE: To report the ophthalmic manifestations of neurofibromatosis in Korea. METHODS: Ophthalmologic examinations were performed from November 2001 to January 2008 for 153 consecutive patients who were diagnosed with neurofibromatosis according to the diagnostic criteria for neurofibromatosis. A retrospective analysis was performed according to the medical records of these 153 patients. RESULTS: Seventy seven out of the 153 patients were men, 76 were women and the mean age was 20.44 +/- 14.34 years old. One hundred twelve were neurofibromatosis type 1 and six were neurofibromatosis type 2. Remained thirty five were segmental neurofibromatosis type 1. Ophthalmic manifestations of the neurofibromatosis type 1 were Lisch nodule (52.68%), high myopia (14.29%), plexiform neurofibroma in the orbit (4.46%), cafe au lait spots (4.46%) and optic glioma (3.58%). In the neurofibromatosis type 2, epiretinal membrane (33.33%) showed highest incidence and posterior subcapsular opacity (16.67%), Lisch nodule (16.67%), optic disc edema (16.67%), and optic nerve glioma (16.67%) were also noted. Lisch nodule (25.71%) was the most common ophthalmic finding in segmental neurofibromatosis type 1. CONCLUSIONS: Lisch nodule, which was the most common manifestation of the neurofibromatosis type 1, was less manifested in our cases compared to the previous reports of western countries. In the neurofibromatosis type 2, epiretinal membrane and posterior subcapsular cataract showed higher incidence than those of other types of neurofibromatosis.

The Effect of Enucleation in Childhood on Orbital Volumetric Growth

PURPOSE: To evaluate the effect on the growth rate of orbital volume of those who received enucleation or evisceration with or without implants, according to different sizes and operation periods. METHODS: The subjects comprised 47 patients who had previously undergone evisceration or enucleation. The volumes of both anophthalmic and normal orbit were measured from CT scans of patients and compared. The relationship between the orbital volume growth rate and the operation at young age with different size implants or operation without implants was evaluated. RESULTS: Five groups, divided by age at operation, were evaluated. Four groups comprising under 12 years of age, had a significant delay in orbital volume growth development (p<0.05). Three groups, divided by size of implants, were evaluated and 17 mm or smaller sized orbital implants showed significant orbital volume growth retardation (p<0.05) CONCLUSIONS: The effect of early enucleation on orbital volume growth should be considered in patients younger than 12 years old. Furthermore, during the operation, implants with sizes larger than 18mm should be used to prevent the retardation of orbital volume growth.