Iris cysts in children: classification, incidence, and management. The 1998 Torrence A Makley Jr Lecture.
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BACKGROUND: Iris cysts in children are uncommon and there is relatively little information on their classification, incidence, and management. METHODS: The records of all children under age 20 years who were diagnosed with iris cyst were reviewed and the types and incidence of iris cysts of childhood determined. Based on these observations recommendations were made regarding management of iris cysts in children. RESULTS: Of 57 iris cysts in children, 53 were primary and four were secondary. There were 44 primary cysts of the iris pigment epithelium, 34 of which were of the peripheral or iridociliary type, accounting for 59% of all childhood iris cysts. It was most commonly diagnosed in the teenage years, more common in girls (68%), was not recognised in infancy, remained stationary or regressed, and required no treatment. The five mid-zonal pigment epithelial cysts were diagnosed at a mean age of 14 years, were more common in boys (83%), remained stationary, and required no treatment. The pupillary type of pigment epithelial cyst was generally recognised in infancy and, despite involvement of the pupillary aperture, also required no treatment. There were nine cases of primary iris stromal cysts, accounting for 16% of all childhood iris cysts. This cyst was usually diagnosed in infancy, was generally progressive, and required treatment in eight of the nine cases, usually by aspiration and cryotherapy or surgical resection. Among the secondary iris cysts, two were post-traumatic epithelial ingrowth cysts and two were tumour induced cysts, one arising from an intraocular lacrimal gland choristoma and one adjacent to a peripheral iris naevus. CONCLUSIONS: Most iris cysts of childhood are primary pigment epithelial cysts and require no treatment. However, the iris stromal cyst, usually recognised in infancy, is generally an aggressive lesion that requires treatment by aspiration or surgical excision. (+info)
Increase in orthotopic murine corneal transplantation rejection rate with anterior synechiae.
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PURPOSE: To evaluate the immunologic effect of anterior synechiae (AS) in a murine model of corneal transplantation. METHODS: Orthotopic penetrating keratoplasty with 12 interrupted sutures was performed on C57BL/6 donor mice and BALB/c recipient mice without AS (AS- group). In contrast to suturing in the AS- group, 3 of the 12 sutures were placed to create AS (AS+ group). The average graft opacity scores and rejection rates of both groups were compared. Cytotoxic T-lymphocyte (CTL) reactions and delayed hypersensitivity (DH) were evaluated 3 weeks after transplantation. Corneal cytokine expression was evaluated. RESULTS: The opacity scores of the AS+ group were consistently greater than those of the AS- group, and the rejection rate of the AS+ group was significantly greater than that of the AS- group (86% versus 54%, P = 0.03). The AS+ group had significantly higher CTL activity compared with the AS- group. There was no significant difference in DH between the two groups. The cytokine expression pattern in the AS+ group became similar to that of the AS- group in which the grafts were rejected. CONCLUSIONS: These findings indicate that AS impairs ocular immune privilege by mediating CTL activity, but without intensifying the DH response. Therefore, AS is a critical risk factor in allograft rejection in a murine model of corneal transplantation. (+info)
Intraocular gnathostomiasis.
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We report a rare case of intraocular Gnathostomiasis, where a live worm, intracameral in location, was successfully removed. Its identity was confirmed by microscopy. (+info)
Meta-analysis of randomised controlled trials comparing latanoprost with timolol in the treatment of patients with open angle glaucoma or ocular hypertension.
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AIM: To evaluate the comparative efficacy and tolerance of latanoprost versus timolol through a meta-analysis of randomised controlled trials (RCTs). METHODS: Systematic retrieval of RCTs of latanoprost versus timolol to allow pooling of results from head to head comparison studies. Quality of trials was assessed based on randomisation, masking, and withdrawal. Sensitivity analyses were used to estimate the effects of quality of study on outcomes. The data sources were Medline, Embase, Scientific Citation Index, Merck Glaucoma, and Pharmacia and Upjohn ophthalmology databases. There were 1256 patients with open angle glaucoma or ocular hypertension reported in 11 trials of latanoprost versus timolol. The main outcome measures were (i) percentage intraocular pressure (IOP) reduction for efficacy; (ii) relative risk, risk difference, and number needed to harm for side effects such as hyperaemia, conjunctivitis, increased pigmentation, hypotension, and bradycardia expressed as dichotomous outcomes; and (iii) reduction in systemic blood pressure and heart rate as side effects. RESULTS: Both 0.005% latanoprost once daily and 0.5% timolol twice daily reduced IOP. The percentage reductions in IOP from baseline (mean (SE)) produced by latanoprost and timolol were 30.2 (2.3) and 26.9 (3.4) at 3 months. The difference in IOP reduction between the two treatments were 5.0 (95% confidence intervals 2.8, 7.3). However, latanoprost caused iris pigmentation in more patients than timolol (relative risk = 8.01, 95% confidence intervals 1.87, 34.30). The 2 year risk with latanoprost reached 18% (51/277). Hyperaemia was also more often observed with latanoprost (relative risk =2.20, 95% confidence intervals 1.33, 3.64). Timolol caused a significant reduction in heart rate of 4 beats/minute (95% confidence interval 2, 6). CONCLUSION: This meta-analysis suggests that latanoprost is more effective than timolol in lowering IOP. However, it often causes iris pigmentation. While current evidence suggests that this pigmentation is benign, careful lifetime evaluation of patients is still justified. (+info)
Deletion in the OA1 gene in a family with congenital X linked nystagmus.
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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. (+info)
Fibrous congenital iris membranes with pupillary distortion.
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BACKGROUND: In 1986 Cibis and associates described 2 children with a new type of congenital pupillary-iris-lens membrane with goniodysgenesis that was unilateral, sporadic, and progressive. These membranes were different from the common congenital pupillary strands that extend from 1 portion of the iris collarette to another or from the iris collarette to a focal opacity on the anterior lens surface. They also differed from the stationary congenital hypertrophic pupillary membranes that partially occlude the pupil, originating from multiple sites on the iris collarette, but not attaching directly to the lens. CASE MATERIAL: The present report is an account of 7 additional infants with congenital iris membranes, similar to those reported by Cibis and associates, which caused pupillary distortion and were variably associated with adhesions to the lens, goniodysgenesis, and progressive occlusion or seclusion of the pupil. Six of the 7 patients required surgery to open their pupils for visual purposes or to abort angle closure glaucoma. A remarkable finding was that the lenses in the area of the newly created pupils were clear, allowing an unobstructed view of normal fundi. CONCLUSION: This type of fibrous congenital iris membrane is important to recognize because of its impact on vision and its tendency to progress toward pupillary occlusion. Timely surgical intervention can abort this progressive course and allow vision to be preserved. (+info)
Ocular manifestations of congenital rubella syndrome in a developing country.
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PURPOSE: To describe the ocular manifestations of congenital rubella syndrome (CRS), a common cause of congenital cataracts in developing countries. METHODS: Retrospective analysis of case records of 46 sero-positive infants under 12 months of age who presented at Aravind Eye Hospital, Madurai between July 1993 and February 2001. The ocular and systemic examination details were recorded. RESULTS: Both eyes were affected in 41 (89%) patients. Cataract was present in 81 (93.1%) eyes; most of them were nuclear cataract (79, 97.5%). Other common ocular presentations included microphthalmos in 74 (85.1%) eyes, iris abnormalities in 51 (58.6%) eyes, and pigmentary retinopathy in 33 (37.9%) eyes. Cataract, microphthalmos and iris hypoplasia was a common combination present in 49 (56.3%) eyes. Systemic manifestations included cardiac anomalies in 23 (50%) and neurological anomalies in 16 (34%) children. Multi-system involvement was present in 32 (70%) children. Low birth weight (below 2 kg) was seen in 30% infants. CONCLUSION: CRS may present with a wide spectrum of ocular and systemic findings and requires a high index of suspicion for diagnosis. Any sick infant with unilateral or bilateral congenital cataract should be investigated thoroughly for CRS. (+info)
Surgical approaches to the management of epithelial cysts.
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PURPOSE: The purpose of this study was to review management strategies for treatment of epithelial cysts. STUDY DESIGN: Retrospective consecutive interventional case series. METHODS: Charts of patients treated for epithelial ingrowth over a 10-year period by a single surgeon (J.A.H.) were reviewed. Cases of epithelial cysts were identified and the following data were recorded: details of ocular history, preoperative and postoperative visual acuity, intraocular pressure (IOP), ocular examination findings, type of surgical intervention, and details of subsequent procedures performed. RESULTS: Seven eyes with epithelial cysts were identified. Patients ranged in age from 1 1/2 years to 53 years at presentation. Three patients were children. Four cysts were due to trauma, one was presumably congenital, one developed after corneal perforation in an eye with Terrien's marginal degeneration, and one developed after penetrating keratoplasty. Three patients were treated with vitrectomy, en bloc resection of the cyst and associated tissue, fluid-air exchange, and cryotherapy. Four patients were treated with conservative strategy consisting of cyst aspiration (three cases) or local excision (one "keratin pearl" cyst) and endolaser photocoagulation of the collapsed cyst wall or base. In all cases, the epithelial tissue was successfully eradicated; one case required a second excision (follow-up, 9 months to 78 months; mean, 45 months). Two eyes required subsequent surgery for elevated IOP, two for cataract extraction, and one for a second penetrating keratoplasty. Final visual acuity ranged from 20/20 to hand motions, depending on associated ocular damage. Best visual results were obtained in the more conservatively managed eyes. CONCLUSION: Epithelial cysts can be managed conservatively in selected patients with good results. This strategy may be particularly useful in children, in whom preservation of the lens, iris, and other structures may facilitate amblyopia management. (+info)