1/41. retinitis pigmentosa, nanophthalmos, and optic disc drusen: a case report.OBJECTIVE: Although the associations of retinitis pigmentosa (RP) with nanophthalmos/microphthalmos and RP with optic disc drusen have previously been recognized, the concurrence of all three features, as far as the authors are aware, has not previously been reported. DESIGN: Case report. RESULTS: The authors report a sporadic case of nanophthalmos, RP, and optic nerve drusen with the additional complication of chronic angle closure glaucoma. CONCLUSIONS: Visual loss may be secondary to the complications of nanophthalmos, RP, or optic nerve drusen. Chronic angle closure may be caused by choroidal effusion with serous retinal detachment, which may, in turn, cause a pseudo-RP picture. It is therefore important to recognize the possible association of true RP with nanophthalmos as a cause for visual deterioration.- - - - - - - - - - ranking = 1keywords = retinal detachment, detachment, pigment (Clic here for more details about this article) |
2/41. Familial exudative vitreoretinopathy mimicking persistent hyperplastic primary vitreous.PURPOSE: To report an unusual case of familial exudative vitreoretinopathy in an infant. methods: Case report. A 6-day-old girl had unilateral microphthalmia in the right eye, with a retrolental plaque initially diagnosed as persistent hyperplastic primary vitreous. Three months later, peripheral retinal vascular changes and a fibrovascular ridge were noted in the left eye, suggesting familial exudative vitreoretinopathy as the cause in both eyes. RESULTS: The microphthalmic right eye was unsalvageable. The left eye developed an exudative retinal detachment despite photocoagulation of the peripheral avascular retina. Additional cryotherapy resulted in resolution of the detachment and regression of the vascular changes. CONCLUSIONS: With highly asymmetric involvement, neonatal familial exudative vitreoretinopathy can mimic persistent hyperplastic primary vitreous. Fellow eye involvement can progress rapidly.- - - - - - - - - - ranking = 1.4498879483262keywords = retinal detachment, detachment (Clic here for more details about this article) |
3/41. Sclerotomy in uveal effusion syndrome.PURPOSE: To report visual and anatomic outcomes after modified scleral surgery in patients with uveal effusion syndrome with retinal and choroidal detachment. methods: In five eyes of four patients with retinal and choroidal detachment and uveal effusion syndrome due to nanophthalmos, we performed pars plana full-thickness unsutured sclerotomies without sclerectomy. RESULTS: In all cases, uveal effusion with choroidal and retinal detachment resolved within 3 weeks, and all patients showed improved vision. The functional and anatomic results remained stable for over 2 years. CONCLUSIONS: Uveal effusion syndrome that is refractory to medical treatment (high-dose systemic corticosteroids) can be managed effectively by pars plana full-thickness unsutured sclerotomy without sclerectomy.- - - - - - - - - - ranking = 1.914846705993keywords = retinal detachment, detachment (Clic here for more details about this article) |
4/41. Nanophthalmic uveal effusion syndrome after prophylactic laser treatment.PURPOSE: We report a case of nanophthalmic uveal effusion syndrome (NUES) with total exudative retinal detachment (RD) after prophylactic argon laser (AL) treatment. The RD subsided and eventually resolved with i.v. steroid therapy. methods: A 45-year-old woman was referred to us with NUES and total exudative RD after prophylactic AL treatment for retinoschisis performed elsewhere. The patient had been scheduled for surgical intervention and was given i.v. prednisolone. RESULTS: Two days after starting i.v. prednisolone, the subretinal fluid partially resolved. Intervention had therefore been postponed. After 15 days the RD almost disappeared and vision improved to 20/400. Ten days later the macula was flat and vision was 20/200. At the six-month follow-up visit vision was 20/40. CONCLUSIONS: To our knowledge this is the first report of NUES and total exudative RD following AL treatment that resolved with i.v. steroid therapy alone. Since the AL treatment clearly seemed to play a role in the pathogenesis of the NUES and associated RD we strongly advise careful assessment of the risk/benefit ratio of prophylactic laser treatment in nanophthalmic patients. Although sclerectomy and vortex vein decompression are well-established techniques, we believe steroid therapy might be tried before proceeding to surgery.- - - - - - - - - - ranking = 0.98492919065933keywords = retinal detachment, detachment (Clic here for more details about this article) |
5/41. Nanophthalmos with uveal effusion. A new clinical entity.Five patients exhibited the association of nanophthalmos and uveal effusion, apparently on a familial basis. glaucoma, occurring in the fourth to sixth decades of life, required surgical intervention that was followed by the development of secondary retinal and choroidal detachment. Recognition of this syndrome is important because: (1) surgical procedures for glaucoma should be avoided, if possible, in order to prevent the development of uveal effusion; (2) retinal detachment surgical procedures are ineffective in uveal effusion and should be avoided, and (3) choroidal elevation occurring in the uveal effusion phase may be erroneously diagnosed as an intraocular tumor and unnecessary enucleation may follow.- - - - - - - - - - ranking = 1.4498879483262keywords = retinal detachment, detachment (Clic here for more details about this article) |
6/41. autopsy case of multiple anomalies with hypoplastic cerebrum, eyes, and endocrine organs, mimicking Micro syndrome.We report an autopsy case of multiple anomalies with severe micrencephaly, bilateral microphthalmos, and hypoplastic endocrine organs. We examined expressions of calcium-binding proteins and hypothalamic and pituitary hormones. A female proband presented with microcephaly, microphthalmia, and psychomotor development delay. At the age of 23 years, she died of cardiorespiratory failure. The endocrine organs demonstrated severe underdevelopment, and the hypoplastic eyeballs had remnant lens, vitreous hemorrhage, and retinal detachment. The brain weighed 260 g; the cerebrum, cerebellum, and brain stem were extremely small; and the tertiary sulci were absent in the cerebral surface. The cross-sectional area of cerebral cortex was reduced to about one third of those in the control, although six-layered lamination, density of pyramidal neurons, and expressions of calcium-binding proteins were comparatively preserved in the cerebral cortex. The third ventricle was hypoplastic, and the bilateral thalami appeared to be fused and the hippocampus was unrolled, whereas the corpus callosum was preserved. In the hypothalamus, the paraventricular nucleus was only identified, and the adenohypophysial somatotrophs were reduced. This may be the first autopsy report of Micro syndrome, which is characterized by microcephaly, brain malformations, optic atrophy, and hypogenitalism, although the case lacked agenesis of the corpus callosum.- - - - - - - - - - ranking = 0.98492919065933keywords = retinal detachment, detachment (Clic here for more details about this article) |
7/41. Angle closure glaucoma in nanophthalmos and pigmentary retinal dystrophy: a rare syndrome.The simultaneous occurrence of nanophthalmos, angle closure glaucoma and pigmentary retinal dystrophy documented in the present case represents a rare syndrome.- - - - - - - - - - ranking = 0.01507080934067keywords = pigment (Clic here for more details about this article) |
8/41. Nanophthalmic cataract extraction.Nanophthalmos is a rare ophthalmic condition with potential sight-threatening complications. In addition, nanophthalmic eyes have a high incidence of intraoperative and postoperative complications, including uveal effusion and retinal detachment. Prophylactic measures to prevent these complications may be used. The case is presented where cataract extraction for a nanophthalmic eye was performed from a posterior approach in combination with a pars plana vitrectomy and gas procedure. To the authors' knowledge, this method of cataract extraction has not been reported before for this condition.- - - - - - - - - - ranking = 0.98492919065933keywords = retinal detachment, detachment (Clic here for more details about this article) |
9/41. Massive gliosis of the retina: report of a case investigated by immunohistochemistry and clonality assays.We report a rare case of massive retinal gliosis that developed in a 32-year-old woman who had been born with bilateral microphthalmia. The patient had recently noticed left ophthalmos and underwent total resection of the affected eyeball. Histologically, the vitreous body had been totally replaced by massively proliferated spindle cells, which had delicate fibrillary cytoplasm without nuclear atypia. Because the attenuated retinal pigment epithelium and intact sclera were preserved at the periphery of the tumor, the tumor was thought to be retinal in origin. Immunohistochemically, the spindle cells were strongly positive for glial fibrillary acidic protein and neuron-specific enolase and partly positive for S-100 protein. These findings led to a diagnosis of massive gliosis of the retina. Clonality analysis of the tumor using a human androgen receptor assay revealed the polyclonal nature of the proliferating spindle cells. This is the first documentation of the polyclonality of this disease.- - - - - - - - - - ranking = 0.037294543623737keywords = retinal pigment, pigment (Clic here for more details about this article) |
10/41. Choroidal effusion following laser peripheral iridotomy for the treatment of angle closure glaucoma in a patient with nanophthalmos.We report a case of Nanophthalmos with choroidal effusion and retinal detachment following a peripheral laser iridotomy for the treatment of angle closure glaucoma. Angle closure glaucoma was diagnosed in the right eye of the patient after finding an elevated intraocular pressure and narrow angles on gonioscopy. The glaucoma was treated by lowering the intraocular pressure medically followed by laser peripheral iridotomy. The first day after a laser peripheral iridotomy, choroidal effusion was noticed that was followed by an exudative retinal detachment within a week. The medical and laser therapies for the lowering of intraocular pressure for the treatment of angle closure glaucoma in patients with nanophthalmos can be associated with choroidal effusion, retinal detachment and loss of vision.- - - - - - - - - - ranking = 2.954787571978keywords = retinal detachment, detachment (Clic here for more details about this article) |
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