Anterior Segment Abnormalities Research Articles

Choroidal Vitiligo Masquerading as Large Choroidal Nevus: A Report of Four Cases

To describe 4 patients with choroidal vitiligo masquerading as large choroidal nevus. Retrospective chart review. Observational case series of 4 patients. Retrospective chart review. Clinical features. Four patients referred with the diagnosis of large choroidal nevus were found to have unilateral (n = 1) or bilateral (n = 3) extensive patchy choroidal depigmentation classified as choroidal vitiligo. There was no evidence of choroidal nevus, and the pigmented "lesion" proved to be normal choroidal pigment surrounded by a region of pigment absence (vitiligo). There was no evidence of ocular inflammation or related retinal or retinal pigment epithelial changes. The choroidal vitiligo was clinically flat and measured 12 to 24 mm diameter, involving the post-equatorial fundus in all cases. There were no related anterior segment abnormalities. Cutaneous vitiligo was present in all cases. There was no documented progression of the choroidal or cutaneous vitiligo over a maximum 2-year follow-up. Choroidal vitiligo is an idiopathic benign process that can involve large segments of the posterior choroid, leaving only patches of residual choroidal pigment, simulating, in reverse, a large choroidal nevus. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Age at the time of cataract surgery and relative risk for aphakic glaucoma in nontraumatic infantile cataract

To report the relative risk for aphakic glaucoma as a function of age at surgery in infants who underwent cataract surgery before 10 months of age for nontraumatic infantile cataract without microcornea. This was an institutional retrospective case series (January 1985 to February 2003) of children who underwent cataract surgery without intraocular lens implantation for nontraumatic infantile cataract before 10 months of age. Patients with less than 5 years' postsurgical follow-up, microcornea, persistent fetal vasculature, and/or other significant anterior segment abnormality were excluded. The surgical procedure in all cases was lens aspiration, posterior capsulotomy, and anterior vitrectomy with anterior small-incision techniques. Of 210 eyes (121 patients), 55 eyes (26.2%; 31 patients [25.6%]) developed aphakic glaucoma. Relative risk for later aphakic glaucoma as a function of age at the time of surgery decreased from 1.85 (95% CI, 0.88-3.86) at 0-1 months of age to a nadir at 3-4 months of age (0.22 [95% CI, 0.05-0.96]) and then increased again to peak at 3.17 (95% CI, 1.1-9.11) at 5-6 months of age. The lowest relative risk for later aphakic glaucoma in our cohort was for surgery performed at 3-4 months of age, for which the 95% CI remained less than 1.0. Further conclusions are limited by overlapping confidence intervals. However, because of amblyopia issues, we do not recommend delaying congenital cataract surgery beyond 4 weeks of life.

Complex Deep Lamellar Endothelial Keratoplasty for Complex Bullous Keratopathy With Severe Vision Loss

To determine the efficacy and postoperative complications of complex deep lamellar endothelial keratoplasty (DLEK) when used for the management of complex bullous keratopathy with severe vision loss. Twelve consecutive eyes with severe bullous keratopathy and other associated intraocular abnormalities underwent complex DLEK which was combined with other intraocular surgeries such as vitrectomy, intraocular lens removal, and secondary intraocular lens implantation. An air bubble was used in 9 eyes and an air and C3F8 gas bubble in 3 eyes for tissue support. Prospective data of best spectacle-corrected visual acuity, corneal astigmatism, and curvature were collected on all 12 eyes preoperatively and at 6, 9, and 12 months postoperatively. Average donor endothelial cell density was recorded preoperatively and at 9 months postoperatively. Preoperatively, severe visual loss was recorded at hand motions (n = 3), count fingers (n = 7), and 20/400 (n = 2). Best spectacle-corrected visual acuity improved in 83% of eyes with vision of 20/67 or better in 42% (5/12) and 20/100 or better in 66% (8/12) of eyes at 12 months postoperatively. Vision was not improved in three eyes due to coexistent retinal disease. Four aphakic eyes underwent secondary intraocular lens implantation later at 4-6 months after DLEK, and best spectacle-corrected visual acuity was increased from count fingers to 20/200 or better. At 12 months postoperatively, average corneal astigmatism and curvature were 2.3 +/- 0.5 diopters and 44.0 +/- 0.9 diopters, respectively. Average central corneal thickness at 12 months postoperatively (541.3 +/- 26.7 microm) was significantly thinner than that before surgery (673.8 +/- 77.5 microm, P < 0.01). Preoperative average donor endothelial cell density was 2685.1 +/- 193.9 cells/mm. At 9 months postoperatively, average endothelial cell density was 1920.1 +/- 94.0 cells/mm (27.3% of endothelial cell loss) in eyes with primary surgery and 1866.3 +/- 92.8 cells/mm (33.9% of endothelial cell loss) in eyes with secondary surgery. Postoperative complications usually occurred within 3 months of surgery. After surgery, 3 eyes showed narrow gaps between the border of the graft and the host. Epithelial bullae in these areas recurred in 2 eyes but disappeared within 3 months. There was one graft dislocation in this series, no pupillary block cases, and no primary graft failures. In cases of complex bullous keratopathy with severe vision loss, DLEK combined with other intraocular surgeries is a feasible and effective procedure to significantly improve visual acuity to a functional level in most patients. DLEK can be used successfully in eyes with aphakia and other anterior segment abnormalities without a high risk of graft dislocation or failure. Mild postoperative complications usually occurred early after DLEK and did not affect visual outcomes and graft survival if managed appropriately.

Heterozygous deletion at 14q22.1–q22.3 including the BMP4 gene in a patient with psychomotor retardation, congenital corneal opacity and feet polysyndactyly

Here we report on a 1-year-old Japanese girl with psychomotor retardation, bilateral congenital corneal opacity and bilateral postaxial polysyndactyly of the feet. Although she had a normal female karyotype, our in-house bacterial artificial chromosome (BAC)-based array-CGH analysis successfully detected at least a 2.7-Mb heterozygous deletion at 14q22.1-q22.3 harboring 18 protein-coding genes. Among the genes, BMP4 was a candidate for the gene causing the abnormalities of both the eye and digits. It was previously reported that the BMP family was correlated with the morphogenesis of digits and ocular development, and Bmp4 heterozygous null mice revealed skeletal abnormalities including polydactyly and ocular anterior segment abnormalities. Patients with a deletion including BMP4 also hadabnormalities of the eye and digits. These previous reports support that a haplo-insufficiency of the BMP4 gene likely caused the congenital ocular and digit abnormalities. Moreover, among the other genes contained in the deletion, GMFB is a candidate for the gene responsible for the psychomotor retardation.

Indocyanine Green-assisted Phacoemulsification in Cases of Complicated or Simple Advanced Cataracts

During phacoemulsification for advanced cataracts, particularly when complicated by anterior segment abnormalities, capsulorhexis is very difficult and carries a high risk of complications. This study investigated the efficacy and safety of indocyanine green (ICG)-assisted phacoemulsification in complicated or simple advanced cataracts. Thirty-two patients (35 eyes) underwent phacoemulsification for complicated advanced cataracts (group 1) or simple advanced (mature/hypermature) cataracts (group 2). Anterior segment abnormalities (corneal opacity, small pupil, or glaucoma) in group 1 complicated phacoemulsification. In both groups, 0.5% ICG was used for capsulorhexis, and subsequent procedures were performed in the same routine manner. Group 1 included 15 patients (17 eyes) with a mean age of 60.0 years. Group 2 included 17 patients (18 eyes) with a mean age of 69.4 years (p<0.05). Continuous curvilinear capsulorhexis was completed in all eyes in group 2, but radial tears occurred in four (23.5%) eyes in group 1 (p<0.05). Phacoemulsification was performed uneventfully in all eyes in both groups. Postoperative complications (corneal edema, vitreous prolapse, posterior capsule opacity, elevated intraocular pressure) were seen in five (27.8%) eyes in group 1 and four (23.5%) eyes in group 2 (p>0.05). None of these were attributed to the use of ICG. Visual acuity improved in all eyes in group 2, but in only 11 (64.7%) in group 1 (p<0.01). ICG-assisted phacoemulsification is safe and helpful for complicated or simple advanced cataracts. Differences between the two groups in patient age, intraoperative complications, and visual outcome could be explained by differences in the cause(s) of advanced cataracts.

Elliptical anterior iris stromal defects associated with PAX6 gene sequence changes

PAX6 gene mutations have been observed in aniridia and other anterior segment abnormalities. We report a novel PAX6 genotype and phenotype with an autosomal-dominant mode of inheritance in two unrelated pedigrees. Two unrelated pedigrees were identified: one involving four generations; the other involving three generations. Full ocular examination was performed on all available members. Total genomic DNA from peripheral blood was used for genetic analysis. A novel phenotype was identified in both families, with variable expression of elliptical anterior stromal iris defects. Presenile nuclear sclerosis, corectopia, corneal pannus, optic nerve hypoplasia, nystagmus, and macular hypoplasia were also seen in different combinations in different members of both families. One child had classic aniridia. Molecular genetic testing of affected members in Family 1 showed a deletion of a guanine in exon 5 at position 468, which has been previously reported. Affected members of Family 2 have a missense mutation in exon 5 (G469A). This is a novel sequence change. PAX6 sequence changes in both families segregated with the anterior segment phenotype and were not observed in controls. Both mutations occur in the paired domain of the PAX6 gene. The crystal structure of DNA-bound PAX6 indicates that residue G36 does not have a role in DNA binding. Therefore the mutation would likely not affect the stability of the paired domain. The importance of the phenotypes reported herein lies in the fact that recognition will allow for appropriate genetic testing and counseling.

352 La tomographie par cohérence optique du segment antérieur comme outil diagnostique d’une cyclodialyse traumatique

Introduction To evaluate the ability of the Optical Coherence Tomography to provide anterior segment imaging to diagnose traumatic cyclodialysis and to correlate it with the clinical examination and ultrasound biomicroscopy. Material and Methods A patient with post-traumatic hypotonus maculopathy, reduced vision and suspected cyclodialysis cleft was examined with slit-lamp biomicroscopy, gonioscopy, ultrasound biomicroscopy and anterior segment Optical Coherence Tomography. Observation Anterior segment Optical Coherence Tomography provided high-resolution images of the disinsertion of the ciliary body from the scleral spur and associated ciliary body detachment that served to confirm the diagnosis and helped substantially in the descision making process. Discussion Anterior segment Optical Coherence Tomography is a non-invasive tool that does not require any contact with the patient's eye. It provides accurate and reproducible images of the anterior segment. This technique has a good correlation to ultrasound biomicroscopy, allows observing iridocorneal angle abnormalities in the presence of turbid media and is more comfortable for the patient. Conclusion Anterior segment Optical Coherence Tomography is a non-invasive method that can diagnose abnormal anterior segment anatomy, in our case traumatic cyclodialysis.

Telemedical diagnosis of anterior segment eye diseases: validation of digital slit-lamp still images

This study compared digital images from a portable slit-lamp camera with 35 mm slit-lamp photographs and traditional ophthalmic assessments in anterior segment disorder's detection. A total of 196 patients (392 eyes) were recruited from an anterior segment ophthalmology clinic. Each patient underwent an examination by an anterior segment ophthalmologist. Two to three standardized views of 640 x 480 pixels digital images (portable digital slit-lamp camera) and 35 mm photographic slides (Zeiss slit-lamp camera) were taken after the examination. The same ophthalmologist reviewed these images in a masked fashion. Two other masked graders also assessed the digital images. The presence or absence of 33 specific findings was noted at each examination. Digital images showed moderate to excellent agreement to clinical findings (kappa 0.45-0.82) in areas other than lid pathologies. Lens findings from digital images had moderate to good agreement with the clinical gold standard (unweighted kappa 0.43-0.65, sensitivity 59-77%, specificity 86-94%). Gross cornea signs were well detected with digital images, (kappa 0.72-0.85, sensitivity 67-100%, specificity 98-99). More subtle corneal, conjunctival and lid abnormalities were not identified well. The statistical figures were very similar to the above-mentioned figures when the 35-mm film results were compared to clinical diagnoses. The two image formats showed better agreement when compared to each other than when either is compared with clinical findings. Diagnoses using digital slit-lamp images were comparable to diagnosis using 35 mm photographic slides for some anterior segment abnormalities.

Central Corneal Thickness: Congenital Cataracts and Aphakia

To evaluate central corneal thickness (CCT) in normal children (controls) and in those with cataracts, pseudophakia, and aphakia. Prospective, observational case series. CCT was measured in 369 eyes of 223 children. Subjects with glaucoma, anterior segment abnormalities, or intraocular pressure of more than 30 mm Hg were excluded. Group means were compared for controls and for eyes with pediatric cataracts, pseudophakia, and aphakia. The mean CCT of eyes with cataracts was more than that of controls (574 +/- 54 microm [n = 46] and 552 +/- 38 microm [n = 230], respectively; P = .001). After excluding from the cataract group those eyes with aniridia, Down syndrome, Marfan syndrome, or glaucoma surgery, the mean CCT (564 +/- 34 microm [n = 36]) was no longer greater than that of controls (P = .07). The mean CCT of pseudophakic eyes (598 +/- 56 microm [n = 29]) was greater than the mean CCT of controls (P < .001) and was similar to the mean CCT of eyes with cataracts (P = .06). The mean CCT of aphakic eyes (642 +/- 88 microm [n = 64]) was greater than the mean CCT of controls (P < .001), eyes with cataracts (P < .001), and eyes with pseudophakia (P = .003). In the absence of factors known to affect CCT (Down syndrome, Marfan syndrome, and aniridia), CCT is similar in eyes with pediatric cataracts and normal controls and increases after cataract surgery.

A case of mycotic endophthalmitis after triamcinolone acetonide-assisted pars plana vitrectomy

Only two cases of bacterial endophthalmitis after triamcinolone acetonide (TA)-assisted pars plana vitrectomy (PPV) have been reported. As far as we are aware there has been no report of fungal endophthalmitis occurring after TA-assisted PPV. We report a case of endophthalmitis due to Fusarium after TA-assisted PPV. An otherwise healthy 61-year-old woman suffered from branch retinal vein occlusion with macular edema, which led to reduced visual acuity and metamorphopsia. Because she complained of severe discomfort, TA-assisted PPV was performed to reduce the macular edema. At the end of the surgery 4 mg TA was injected into the vitreous cavity. Two weeks after the surgery her visual acuity had improved to 20/20. Six weeks after the surgery her visual acuity decreased to 20/200 but without pain. Slit lamp examination showed no conjunctival hyperemia and no inflammatory cell infiltration in the anterior chamber. Fundus examination showed several small, creamy-white, circumscribed retinal lesions, epiretinal membranes, vitreoretinal traction, and rhegmatogenous retinal detachment. PPV was performed again. Intra-operative light microscopy revealed filamentous fungi in the epiretinal membrane, which was removed during the second PPV. Five days after the second surgery, retinal detachment recurred. A third PPV with encircling procedures and silicone oil tamponade was performed. Fungal cultures from the epiretinal membrane were identified as Fusarium sp. Seven months after the third surgery the silicone oil was removed. The retina remained attached and visual acuity was 20/200. Any patient who has undergone TA-assisted PPV should be carefully followed up for possible post-operative endophthalmitis, even if the anterior segment abnormality is minor.

Central Corneal Thickness in Children: Racial Differences (Black vs. White) and Correlation With Measured Intraocular Pressure

To test the hypotheses that the mean central corneal thickness (CCT) of healthy black children is thinner than that of healthy white children and to confirm the relationship between increased CCT and measured intraocular pressure (IOP) in children. This prospective observational case series included 178 eyes of 92 children aged 9 months to 17 years without anterior segment abnormalities or a clinical diagnosis of glaucoma. CCT was measured by ultrasonic pachymetry and IOP was measured by Goldmann or Tono-pen technique. The mean CCT for the 102 eyes of 52 white children was 562+/-35 microm versus 543+/-37 microm for the 66 eyes of 35 black children (P = 0.02). There was a positive relationship between CCT and IOP (P = 0.0002). For every 100 mum increase in CCT, the IOP increased by 2.2+/-0.6 mm Hg. The mean CCT of black children is thinner than that of white children. There is a positive relationship between increasing measured IOP and CCT among children with normal corneas and anterior segments. The interpretation of elevated IOP in eyes with abnormal anterior segment anatomy and thickened corneas awaits further study.

De novo double mutation in PAX6 and mtDNA tRNA Lys associated with atypical aniridia and mitochondrial disease

We report on the clinical, molecular and biochemical findings of a patient with the rare event (<4.02 x 10(-9) per generation) of coinciding de novo mutations in the nuclear PAX6 (c.1252-1267del16) and the mitochondrial mt.RNA (Lys) (8347A-->G) genes. The boy suffers from exercise intolerance, ptosis, nystagmus, macular hypoplasia and anterior segment abnormalities evocative of Axenfeld-Rieger anomaly. The PAX6 mutation is predicted to cause haploinsufficiency. The novel mt.RNA (Lys) mutation is located close to the classic myoclonic epilepsy with ragged-red-fibers mutation, but the patient exhibits neither myoclonic epilepsy nor ragged-red-fibers. The degree of mutant mtDNA heteroplasmy, as determined by a very accurate pyrosequencing assay, varies between 31% (muscle) and 38% (fibroblasts). We discuss a potential effect of the PAX6 mutation on the mtDNA mutation rate.

Endocyclophotocoagulation in Pediatric Glaucoma with Anterior Segment Abnormalities

Endocyclophotocoagulation in Pediatric Glaucoma with Anterior Segment Abnormalities

Newborn Primary Congenital Glaucoma: 2005 Update

Newborn primary congenital glaucoma is an unusual and important subtype of primary congenital glaucoma. Affected patients typically manifest specific signs that facilitate its recognition at birth and become important in the assessment and determination of appropriate treatment for this severe expression of primary congenital glaucoma. The medical records of 35 patients with newborn primary congenital glaucoma were reviewed to determine its clinical manifestations and response to therapy. The published literature related to newborn primary congenital glaucoma was reviewed and referenced. Newborn primary congenital glaucoma is recognized at birth because of the associated corneal opacification. Ocular examination reveals anterior segment abnormalities of the cornea, iris, and filtration angle as well as related elevated intraocular pressure. Genetic analysis of a subset of patients with newborn primary congenital glaucoma confirmed its relationship with the less severe infantile form of primary congenital glaucoma, which is characterized by favorable results after goniosurgery. In contrast, goniosurgery was found to have unfavorable results and be less successful compared with trabeculectomy and glaucoma drainage tube shunts as initial therapy for newborn primary congenital glaucoma. Newborn primary congenital glaucoma is an important subtype of primary congenital glaucoma. It can be differentiated from the more frequent and familiar infantile type by history and careful anterior segment examinations to enable and support appropriate choices for its successful surgical treatment.

Clinical and Molecular Characterization of a Family With Autosomal Recessive Cornea Plana

Autosomal recessive cornea plana is characterized by a flattened corneal surface associated with hyperopia and various anterior segment abnormalities. Mutations have been detected in the keratocan gene (KERA), a member of the small leucine-rich proteoglycan family. To clinically and molecularly characterize a consanguineous family of Hispanic origin in which 3 individuals are affected with cornea plana. Clinical ophthalmic examination, including corneal topography and axial eye length measurement, was performed on 7 family members. Molecular analysis of KERA was performed on DNA from each family member who had been examined. All 3 affected individuals showed extreme flattening of the cornea (< 36 diopters [D]), normal axial eye lengths, and hyperopia greater than 6.25 D (spherical equivalent). Anterior segment abnormalities included scleralization of the cornea and central iris strands to the corneal endothelium. Affected individuals were homozygous for a novel mutation in KERA. The sequence change was found in exon 2, which results in an asparagine to aspartic acid change at codon 131. This amino acid change occurs within a highly conserved leucine-rich repeat of keratocan. The cause of disease in this family is likely to be a mutation in exon 2 of KERA. Other mutations in KERA known to cause cornea plana also fall within the region encoding the leucine-rich repeat motifs and are predicted to affect the tertiary structure of the protein. This is the first report of the identification of a mutation within KERA in a family of Hispanic origin with autosomal recessive cornea plana. Although the vast majority of cases of cornea plana are in individuals of Finnish descent, this report demonstrates the occurrence of the disease in other populations.

The Tennessee Mouse Genome Consortium: Identification of ocular mutants

The Tennessee Mouse Genome Consortium (TMGC) is in its fifth year of a ethylnitrosourea (ENU)-based mutagenesis screen to detect recessive mutations that affect the eye and brain. Each pedigree is tested by various phenotyping domains including the eye, neurohistology, behavior, aging, ethanol, drug, social behavior, auditory, and epilepsy domains. The utilization of a highly efficient breeding protocol and coordination of various universities across Tennessee makes it possible for mice with ENU-induced mutations to be evaluated by nine distinct phenotyping domains within this large-scale project known as the TMGC. Our goal is to create mutant lines that model human diseases and disease syndromes and to make the mutant mice available to the scientific research community. Within the eye domain, mice are screened for anterior and posterior segment abnormalities using slit-lamp biomicroscopy, indirect ophthalmoscopy, fundus photography, eye weight, histology, and immunohistochemistry. As of January 2005, we have screened 958 pedigrees and 4800 mice, excluding those used in mapping studies. We have thus far identified seven pedigrees with primary ocular abnormalities. Six of the mutant pedigrees have retinal or subretinal aberrations, while the remaining pedigree presents with an abnormal eye size. Continued characterization of these mutant mice should in most cases lead to the identification of the mutated gene, as well as provide insight into the function of each gene. Mice from each of these pedigrees of mutant mice are available for distribution to researchers for independent study.

Targeted disruption ofCol8a1andCol8a2genes in mice leads to anterior segment abnormalities in the eye

Collagen VIII is localized in subendothelial and subepithelial extracellular matrices. It is a major component of Descemet's membrane, a thick basement membrane under the corneal endothelium, where it forms a hexagonal lattice structure; a similar structure, albeit less extensive, may be formed in other basement membranes. We have examined the function of collagen VIII in mice by targeted inactivation of the genes encoding the two polypeptide subunits, Col8a1 and Col8a2. Analysis of these mice reveals no major structural defects in most organs, but demonstrates that type VIII collagen is required for normal anterior eye development, particularly the formation of a corneal stroma with the appropriate number of fibroblastic cell layers and Descemet's membrane of appropriate thickness. Complete lack of type VIII collagen leads to dysgenesis of the anterior segment of the eye: a globoid, keratoglobus-like protrusion of the anterior chamber with a thin corneal stroma. Descemet's membrane is markedly thinned. The corneal endothelial cells are enlarged and reduced in number, and show a decreased ability to proliferate in response to different growth factors in vitro. An important function of collagen VIII may therefore be to generate a peri- or subcellular matrix environment that permits or stimulates cell proliferation.

Posterior Polar Cataract: Genetic Analysis of a Large Family

Congenital cataracts are clinically and genetically heterogeneous. Loci for autosomal dominant posterior polar cataracts have been mapped to chromosomes 1p36, 11q22-q22.3, 16q22, and 20p12-q12. We investigated a large four-generation family with 20 individuals affected with congenital posterior polar cataracts. After exclusion of known loci for posterior polar cataracts, a genome-wide screen was conducted. In this family, we mapped dominant congenital posterior polar cataracts to chromosome 10q24. On haplotype analysis, we identified an 11-cM interval between loci D10S1680 and D10S467, which included the PITX3 gene. On sequencing the coding region of PITX3, we found a 17-base-pair duplication in exon 4. Although the same genotype was described in a family with ASMD and cataracts, the common phenotype of this mutation is probably posterior polar cataract; a modifier gene is presumed to cause anterior segment abnormalities in the previously described patients. The same mutation was recently identified in four families with congenital cataracts. This study provides further evidence of genetic heterogeneity of autosomal dominant posterior polar cataract.

Manifestações destrutivas da córnea e esclera associadas a doenças do tecido conectivo: relato de 9 casos

OBJETIVO: Estudar as caracteristicas clinicas, tratamento e evolucao de pacientes com acometimento da cornea e esclera associados a doencas do tecido conectivo. METODO: Descricao das alteracoes de segmento anterior em nove pacientes com doencas do tecido conectivo, previamente diagnosticada (5 casos) ou no qual o acometimento ocular foi sua primeira manifestacao (4 casos). Todos os pacientes foram atendidos no setor de Doencas Externas Oculares e Cornea da Universidade Federal de Sao Paulo - Escola Paulista de Medicina (UNIFESP-EPM), acompanhados no ambulatorio no periodo de julho/1999-dezembro/2000 e submetidos a exame oftalmologico completo, avaliacao sistemica e investigacao laboratorial. Tratamento clinico e/ou cirurgico foi proposto de acordo com a gravidade e evolucao das manifestacoes oculares. RESULTADOS: Os diagnosticos sistemicos observados nos portadores de doenca inflamatoria do segmento anterior foram de artrite reumatoide em sete pacientes (77,8%), esclerose sistemica e granulomatose de Wegener em um paciente cada (22,2%). As manifestacoes oculares mais frequentes foram esclerite (66,6%), ceratite ulcerativa periferica (55,5%) e ceratoconjuntivite seca (44,4%). Oitenta e nove por cento dos pacientes necessitaram de terapia imunossupressora sistemica para o controle da inflamacao ocular. A remissao da inflamacao observou-se no periodo de tres meses apos o inicio do imunos-supressor (metotrexato, ciclofosfamida e/ou ciclosporina A). Em 55,5% dos pacientes uma abordagem cirurgica (resseccao da conjuntiva do limbo, adesivo tecidual com lente de contato terapeutica, enxerto de esclera, transplante de cornea) se fez necessaria. CONCLUSAO: O envolvimento da cornea e da esclera associado a doenca do tecido conectivo e sinal de atividade da doenca e geralmente necessita de terapia imunossupressora para o seu controle. O oftalmologista deve estar alerta para deteccao precoce e tratamento apropriado da doenca de base.

Refractive error and visual impairment in urban children in southern china.

To assess the prevalence of refractive error and visual impairment in school-age children in a metropolitan area of southern China. Random selection of geographically defined clusters was used to identify children 5 to 15 years of age in Guangzhou. Children in 22 clusters were enumerated through a door-to-door survey and examined in 71 schools and 19 community facilities from October 2002 to January 2003. The examination included visual acuity measurements, ocular motility evaluation, retinoscopy, and autorefraction under cycloplegia and examination of the external eye, anterior segment, media, and fundus. A total of 5053 children living in 4814 households were enumerated, and 4364 (86.4%) were examined. The prevalence of uncorrected, presenting, and best-corrected visual acuity 20/40 or worse in the better eye was 22.3%, 10.3%, and 0.62%, respectively. Refractive error was the cause in 94.9% of the 2335 eyes with reduced vision, amblyopia in 1.9%, other causes in 0.4%, and unexplained causes in the remaining 2.8%. External and anterior segment abnormalities were seen in 1496 (34.3%) children, mainly minor conjunctival abnormalities. Media and fundus abnormalities were observed in 32 (0.73%) children. Myopia (spherical equivalent of at least -0.50 D in either eye) measured with retinoscopy affected 73.1% of children 15 years of age, 78.4% with autorefraction. The prevalence of myopia was 3.3% in 5-year-olds with retinoscopy and 5.7% with autorefraction. Females had a significantly higher risk of myopia. Hyperopia (+2.00 D or more) measured with retinoscopy was present in 16.7% of 5-year-olds, 17.0% with autorefraction. The prevalence of hyperopia was below 1% in 15-year-olds, with both methods. Astigmatism (cylinder of > or = 0.75 D) was present in 33.6% of children with retinoscopy and in 42.7% with autorefraction. The prevalence of reduced vision because of myopia is high in school-age children living in metropolitan Guangzhou, representing an important public health problem. One third of these children do not have the necessary corrective spectacles. Effective strategies are needed to eliminate this easily treated cause of significant visual impairment.