Upward extension of an atrophic tract of the retinal pigment epithelium associated with congenital macular toxoplasmosis.

PURPOSE: To report an unusual case of gravitational atrophic tract of the retinal pigment epithelium in a 20-year old woman. METHODS: Case Report. RESULTS: The patient had macular cicatricial congenital toxoplasmic chorioretinitis in both eyes. In the right eye, an atrophic tract of the retinal pigment epithelium originating from the upper margin of the macular scar extended upwards toward the retinal periphery. CONCLUSION: The unusual upward direction of the atrophic tract of retinal pigment epithelium may be explained by the in utero head position during the active phase of the chorioretinal disease.

Retinal venous macroaneurysm associated with premacular hemorrhage.

To report an unusual association of a retinal venous macroaneurysm with premacular hemorrhage in a 50-year-old man, using a case report method. The patient exhibited a dense premacular hemorrhage in the left eye. Fluorescein angiography demonstrated that the source of bleeding was an isolated retinal venous macroaneurysm. The anterior surface of the hematoma was opened with an argon green laser, resulting in rapid clearing of the premacular hemorrhage and improvement in vision. Treatment of the retina surrounding the macroaneurysm to prevent recurrence of bleeding was ineffective to achieve occlusion of the lesion. No recurrent hemorrhage occurred during the observation period. Retinal venous macroaneurysm, a quite rare condition, may be complicated by premacular hemorrhage. Argon green laser may be useful in treating such hemorrhage. Hemorrhagic detachment of the internal limiting membrane or subhyaloid hemorrhage in the macula may occur after retinal vessel rupture with physical exertion (Valsalva retinopathy) or in retinal vascular diseases, such as proliferative diabetic retinopathy, and retinal arterial macroaneurysm. Arterial macroaneurysms are a common, well-described retinal vascular disorder. In contrast to retinal arterial macroaneurysms, retinal venous macroaneurysms are quite rare. In this article we describe a patient who presented with premacular hemorrhage that was caused by a retinal venous macroaneurysm. The hematoma and the macroaneurysm were treated with argon green laser.

[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.

[Compressive optic neuropathy caused by fibrous dysplasia]. / Neuropathie optique compressive secondaire à une dysplasie fibreuse.

We report a case of compressive optic neuropathy caused by fibrous dysplasia in a 28-year-old woman. The patient had no significant medical history. Her best-corrected visual acuity was 20/20 in the right eye and 20/400 in the left eye. There was an afferent pupillary defect in the left eye. Slit-lamp examination was unremarkable. Funduscopy showed a normal optic disc bilaterally and congenital hypertrophy of the retinal pigment epithelium in the right eye. Systemic evaluation disclosed facial asymmetry and mucocutaneous lentiginosis involving the face and the limbs. Goldmann visual field testing showed a cecocentral scotoma in the left eye. Imaging studies demonstrated extensive changes of craniofacial fibrous dysplasia involving the sphenoid bone, with compression of the left optic nerve by a cystic structure. Results of gastrointestinal fibroscopy were unremarkable. The patient was given systemic steroids. After 4 days of treatment, her visual acuity had improved to 20/40, with resolution of the afferent pupillary defect and visual field improvement. Debulking of the tumor was recommended, but the patient declined the procedure. She has remained stable over 13 months of follow-up. Compressive optic neuropathy should be considered as a leading cause of visual loss in patients with craniofacial fibrous dysplasia. Early diagnosis, close follow-up, and appropriate management are mandatory to improve or maintain the visual function in such patients.