1/91. Surgical removal of a free floating cyst of the iris pigment epithelium causing disturbing visual symptoms.Pigmented cysts in the anterior chamber, fixed or free floating, are considered to be unusual but not very infrequent. However, most of these cases usually do not need any treatment other than a periodic observation. We report the surgical removal of an iris pigment epithelial cyst floating freely in the anterior chamber. The reason for surgical removal was, disturbance in near vision being caused by movement of the cyst across the visual axis. This specific symptom of disturbed near vision, to the best of our knowledge, is a rare indication for surgery that has not been pointed out earlier. Histopathological confirmation of the clinical diagnosis was also obtained.- - - - - - - - - - ranking = 1keywords = pigment (Clic here for more details about this article) |
2/91. Histopathological findings of X-linked retinoschisis with neovascular glaucoma.BACKGROUND: X-linked retinoschisis (XLRS) is rarely complicated by neovascular glaucoma. Only a few reports of XLRS histopathological findings with neovascular glaucoma have been published. methods: A 41-year-old man with XLRS complicated by neovascular glaucoma in his left eye was examined with electroretinography, B-scan, ultrasound biomicroscopy and computed tomography. He was examined by ophthalmoscopy and fluorescein angiography in the other eye. An enucleation was performed in his left eye due to uncontrollable high intraocular pressure and persistent ocular pain. We examined the enucleated eye histopathologically. RESULTS: Examination of the enucleated eye showed nuclear sclerosis of the lens, pigmented retrolental membrane and retinoschisis which separated the inner layer of the retina and made a large space in the vitreous cavity without any apparent detachment of the outer layers of the retina. Sclerotic vessels were present histopathologically in both the inner and outer layers of the retina. There was a peripheral anterior synechia, ectropion uveae and a fibrovascular membrane, which contained many lumina of neovascularization, indicating marked rubeosis iridis. Small cystic spaces were observed in both the schitic retina in the peripheral region and the foveal schisis at the outer layer of the retina. The photoreceptor cells had become markedly atrophied and multiple regions of calcification were observed. The optic nerve showed severe atrophy with gliosis, but the central retinal artery and vein were still open within the nerve. CONCLUSIONS: These histopathological findings suggest that rubeosis iridis may have developed secondarily to retinal ischemia due to occlusion of the retinal blood vessels.- - - - - - - - - - ranking = 0.63586712507511keywords = detachment, pigment (Clic here for more details about this article) |
3/91. Hamartomas of the iris and ciliary epithelium in tuberous sclerosis complex.Astrocytic hamartomas of the retina are the principal ocular manifestation of tuberous sclerosis complex. iris abnormalities are rare in tuberous sclerosis complex and include focal areas of stromal depigmentation and atypical colobomata. We describe 2 patients who were found on histopathological examination to have lesions consistent with hamartomas of the iris pigment epithelium and ciliary body epithelium. iris abnormalities, including pupillary irregularities, were noted on clinical examination prior to the development of iris neovascularization in both patients. These observations suggest that iris abnormalities, including atypical colobomas, may be caused by hamartomas of the iris pigment epithelium and ciliary epithelium in some patients with tuberous sclerosis complex. To our knowledge, hamartomas of tissues derived from the anterior part of the neuroectodermal optic cup have not been reported in cases of tuberous sclerosis complex.- - - - - - - - - - ranking = 0.6keywords = pigment (Clic here for more details about this article) |
4/91. iris mammillations as the only sign of ocular melanocytosis in a child with choroidal melanoma.An 8-year-old girl had visual loss in her left eye over 2 months. Ocular examination showed that visual acuity was counting fingers in the left eye. The left iris was moderately pigmented and thickened with numerous confluent, dome-shaped elevations on its surface, consistent with iris mammillations arising from ocular melanocytosis. There was total retinal detachment and an inferiorly located large amelanotic choroidal mass compressing the optic nerve. A specimen from a fine-needle aspiration biopsy showed spindle and epithelioid melanoma cells. The eye was enucleated. Pathologic examination showed that the bland melanocytes comprising the anterior border layer of iris formed focal aggregates, corresponding to the iris mammillations observed clinically. The uvea was diffusely thickened. Arising from the posterior choroid and obscuring the optic nerve head was a moderately pigmented spindle and epithelioid cell choroidal melanoma with diffuse lymphocytic infiltration and high mitotic activity. This case demonstrates that iris mammillations can be the initial manifestation of ocular melanocytosis in the absence of scleral pigmentation.- - - - - - - - - - ranking = 1.4790367867941keywords = retinal detachment, detachment, pigment (Clic here for more details about this article) |
5/91. Management of atypical epicapsular star.An 18-year-old woman had brown pigment deposits on the central anterior capsule of the lens associated with poor visual acuity and significant anisometropia. Surgical removal of the deposits, phacoemulsification, and intraocular lens implantation resulted in visual improvement. Epicapsular stars are formed by the confluence of multiple fine pigmentary deposits. Although they cause amblyopia, this does not appear to be dense. Surgical removal of these central deposits may improve vision, even if the patient presents as an adult.- - - - - - - - - - ranking = 0.4keywords = pigment (Clic here for more details about this article) |
6/91. Deletion in the OA1 gene in a family with congenital X linked nystagmus.AIMS: To elucidate the molecular genetic defect of X linked congenital nystagmus associated with macular hypoplasia in three white males of a three generation family with clear features of ocular albinism in only one of them. methods: A three generation family with congenital nystagmus following X linked inheritance, and associated with macular hypoplasia was clinically examined (three males and two obligate carriers). Flash VEP was performed to look for albino misrouting. dna samples were subjected to PCR and subsequent analysis using SSCP for all exons of the OA1 gene. RT-PCR was performed on a mRNA preparation from a naevus from one patient. PCR products presenting divergent banding patterns in SSCP and from the RT-PCR were sequenced directly using cycle sequencing with fluorescent chain termination nucleotides and electrophoresis in a capillary sequencer. RESULTS: The index case (patient 1, IV.1) was diagnosed with X linked OA1 at the age of 3 months because of typical clinical features: congenital nystagmus, iris translucency, macular hypoplasia, fundus hypopigmentation, normal pigmentation of skin and hair, and typical carrier signs of OA1 in his mother and maternal grandmother. Pigmentation of the iris and fundus had increased at the last examination at age 4 years. Albino misrouting was present at this age. In the maternal uncle (III.3, 51 years) who also suffered from congenital nystagmus there was clear macular hypoplasia and stromal focal hypopigmentation of the iris but no iris translucency or fundus hypopigmentation. Patient 3 (II.3, 79 years, maternal uncle of patient III.3) had congenital nystagmus and was highly myopic. The fundus appearance was typical for excessive myopia including macular changes. The iris did not show any translucency. Molecular genetic analysis revealed a novel 14 bp deletion of the OA1 gene at nt816 in exon 6. The mutation abolishes four amino acids (Leu 253-Ile-Ile-Cys) and covers the splice site. nucleotides 814/815 are used as a new splice donor thus producing a frame shift in codon 252 and a new stop codon at codon 259. CONCLUSIONS: Macular hypoplasia without clinically detectable hypopigmentation as the only sign of X linked OA1 has been reported occasionally in African-American, Japanese, and white patients. The present family shows absent hypopigmentation in two patients of a white family with a deletion in the OA1 gene. We propose a model of OA1 that allows increase of pigmentation with age. We hypothesise that macular hypoplasia in all forms of albinism depends on the extracellular DOPA level during embryogenesis, and that in OA1 postnatal normalisation of the extracellular DOPA level due to delayed distribution and membrane budding/fusion of melanosomes in melanocytes results in increasing pigmentation.- - - - - - - - - - ranking = 1.6keywords = pigment (Clic here for more details about this article) |
7/91. Acquired anterior ocular melanocytosis following cataract extraction.The right eye of a 59-year-old man was the subject of extracapsular cataract extraction and posterior chamber lens implantation. He gradually developed superior sectoral scleral and diffuse iris hyperpigmentation within 7 years postoperatively. The iris was also uniformly thickened. He sustained 20/20 visual acuity in the pseudophakic eye. Despite marked pigmentation of the angle, his right intraocular pressure remained within normal limits. We use the term acquired anterior ocular melanocytosis because the pigmentary changes were confined to the anterior segment and the choroid was not involved. We conclude that surgical injury in the form of cataract extraction can induce episcleral/scleral hyperpigmentation and iris hyperchromia.- - - - - - - - - - ranking = 0.8keywords = pigment (Clic here for more details about this article) |
8/91. Recurring transitory blindness caused by primary marginal pigment epithelial iris cysts.PURPOSE: To report four cases of primary pupillary pigment epithelial iris cysts, all members of one family, in which two of the patients had recurring transitory visual impairment. methods: Observational case series. history was taken, the patients were examined with slit-lamp and ultrasound biomicroscopy, and surgically removed cyst tissue was examined with transmission electron microscopy. RESULTS: Pupillary pigment epithelial cysts of the iris generally show an autosomal dominant heredity pattern, with occasional lack of penetrance. In two of our cases, the size and location of the cysts caused visual symptoms, necessitating surgical removal. The cyst wall consists entirely of pigment epithelial cells. CONCLUSION: The origin of pupillary pigment epithelial cysts is unclear, and a hereditary background is very likely. Their clinical significance is in their similarity to pigmented tumors of the iris. They may also be indicative of coexisting systemic disease. In symptomatic cases, treatment is indicated.- - - - - - - - - - ranking = 1.8keywords = pigment (Clic here for more details about this article) |
9/91. iris synechia after laser goniopuncture in a patient having deep sclerectomy with a collagen implant.neodymium:YAG (Nd:YAG) goniopuncture is an efficient and safe treatment for low filtration through the trabeculo-Descemet's membrane after deep sclerectomy with a collagen implant (DSCI). The only reported complication of this procedure is choroidal detachment. However, we found an iris synechia in a patient whose intraocular pressure (IOP) was elevated again 1 month after Nd:YAG goniopuncture. Synechiolysis and peripheral iridectomy with Nd:YAG and argon lasers effectively removed the iris synechia, and IOP immediately dropped to the normal range. We believe that iris synechia is a potential complication that may cause elevated IOP after laser goniopuncture in patients having DSCI.- - - - - - - - - - ranking = 0.43586712507511keywords = detachment (Clic here for more details about this article) |
10/91. Clinical findings in Japanese patients with waardenburg syndrome type 2.PURPOSE: To determine the visual characteristics of Japanese subjects with the waardenburg syndrome type 2. methods: The visual functions of 11 albino patients who were identified from the screening of 240 children attending a school for children with a hearing deficit were studied. The ophthalmological examinations included eye position, visual acuity, biomicroscopy, ophthalmoscopy, visual field by confrontation or Goldmann's perimetry, stereoacuity by the Titmus test, and color vision by the Ishihara pseudoisochromatic plates. RESULTS: A combination of congenital sensory deafness and partial ocular albinism without lateral displacement of the lacrimal puncta was observed in 11 (4.6%) of the students with hearing deficit. All these children had sectorial heterochromia irides with local retinal hypopigmentation. Lid deformities were not present. The retinal vasculature was normal, and macular hypoplasty was not found. Other than 1 eye with hyperopic amblyopia, no serious visual disturbance was found in these patients. CONCLUSIONS: The 11 students were classified as having waardenburg syndrome type 2. None had a critical visual deficit, and all had partial heterochromia irides and retinal hypopigmentation.- - - - - - - - - - ranking = 0.4keywords = pigment (Clic here for more details about this article) |
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