[Association of spheno-orbital dysplasia with plexiform neuroma in von Recklinghausen's neurofibromatosis]. / Association d'une dysplasie sphéno-orbitaire à un névrome plexiforme au cours d'une neurofibromatose de Von Recklinghausen.

We report a case of plexiform neurofibroma of the upper eyelid associated with spheno-orbital dysplasia in a 18-year-old woman with von Recklinghausen neurofibromatosis. Visual acuity was 20/40 in the right eye and 20/20 in the left. Plexiform neurofibroma involving the right upper eyelid was associated with mild ptosis and ipsilateral facial hypertrophy. Biomicroscopic examination showed lisch nodules. Funduscopic examination, visual field and neurologic examinations were normal. Café au lait spots involved the trunk with neck plexiform neurofibroma. Computed tomography disclosed spheno-orbital dysplasia. The patient's status remained unchanged at 6 months follow-up. Cranial features of von Recklinghausen neurofibromatosis are found in 3 to 7% of patients. In patients with plexiform neurofibroma of the eyelid the ophthalmologist should look for associated spheno-orbital dysplasia.

[Posterior scleritis: six case reports]. / Les sclérites postérieures: à propos de 6 cas.

PURPOSE: To study the clinical features, management guidelines, and the course of the disease in six patients with posterior scleritis. SUBJECTS AND METHODS: Six patients with posterior scleritis were enrolled in this retrospective study. All patients underwent a complete ophthalmic examination, fluorescein angiography, and B-scan ultrasonography. Systemic evaluation included physical examination and laboratory screening investigations. Medical management included systemic indomethacin (two cases), oral steroids (two cases), intravenous pulses of methylprednisolone followed by tapered oral steroids (two cases). One patient underwent immunosuppressive therapy (azathioprine) because of steroid dependence. RESULTS: The main presenting symptoms were pain (six cases) and decreased vision (four cases). Chorioretinal changes included chorioretinal folds (six cases), retinal folds (five cases), optic disc edema (five cases), serous retinal detachment (two cases), and a subretinal mass (one case). Ultrasonography disclosed sclerochoroidal thickening in all cases and retrobulbar edema in three cases. Systemic evaluation was unremarkable in five cases and revealed Behçet's disease in one case. All patients showed a good response to medical treatment. CONCLUSION: Posterior scleritis has protean manifestations and can be easily overlooked. This diagnosis must be considered in all inflammatory and painful ocular disorders with no obvious etiology. B-scan ultrasonography is the most useful diagnostic tool in such patients. The disease usually shows a good response to systemic anti-inflammatory therapy.