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Monitoring optic nerve sheath meningiomas (ONSM) in Neurofibromatosis type 2 (NF2) patients remains difficult. Other ocular manifestations of NF2 may obscure ophthalmic assessment of optic nerve function in these patients. Serial magnetic resonance imaging (MRI) used to assess the optic nerve is not without limitations, being expensive and often requiring general anaesthetic in children, with associated risks. This study was undertaken to describe the use of multifocal visual evoked potentials (multifocal VEP, mfVEP) in the regular monitoring of NF2 patients with ONSM. This study involved three NF2 patients with ONSM who undertook mfVEP testing at an academic ophthalmic centre. Same day mfVEP and routine ophthalmic testing were undertaken. Topographical function of the optic nerve was assessed, utilising tools such as asymmetry deviation and accumap severity index. Results were assessed alongside MRI and visual acuity (VA). From the three patients, five eyes had ONSMs, of which two caused unilateral blindness. The remaining three affected eyes had initial VAs 6/6, 6/24, and 6/18. Over follow up, ranging from 5 to 12 years, all tumours progressed, and VA declined for all patients. Multifocal VEP detected optic nerve functional loss corresponding with visual decline. This case series suggests mfVEP is effective in the objective topographic monitoring of optic nerve function in NF2 patients with ONSM. Due also to its safety in a paediatric population, the test may be considered in the routine monitoring of these patients, to be used to assist regular ophthalmic review and MRI scans.

Congenital cataracts are a significant cause of lifelong visual loss. They may be isolated or associated with microcornea, microphthalmia, anterior segment dysgenesis (ASD) and glaucoma, and there can be syndromic associations. Genetic diagnosis is challenging due to marked genetic heterogeneity. In this study, next‐generation sequencing (NGS) of 32 cataract‐associated genes was undertaken in 46 apparently nonsyndromic congenital cataract probands, around half sporadic and half familial cases. We identified pathogenic variants in 70% of cases, and over 68% of these were novel. In almost two‐thirds (20/33) of these cases, this resulted in new information about the diagnosis and/or inheritance pattern. This included identification of: new syndromic diagnoses due to NHS or BCOR mutations; complex ocular phenotypes due to PAX6 mutations; de novo autosomal‐dominant or X‐linked mutations in sporadic cases; and mutations in two separate cataract genes in one family. Variants were found in the crystallin and gap junction genes, including the first report of severe microphthalmia and sclerocornea associated with a novel GJA8 mutation. Mutations were also found in rarely reported genes including MAF, VIM, MIP, and BFSP1. Targeted NGS in presumed nonsyndromic congenital cataract patients provided significant diagnostic information in both familial and sporadic cases.

Aims: To elucidate if topically applied atorvastatin safely decreases corneal fluorescein staining in dry eyes associated with blepharitis. Methods: Ten dry eye and blepharitis (DEB) patients were enroled in a prospective pilot study. All patients were treated with topical atorvastatin (50 μM) 8 times a day for 4 weeks and allowed to continue with their existing dry eye treatment. The patients were examined weekly for 4 weeks. The primary outcome measure was corneal fluorescein staining. Secondary outcome measures were tear film break-up time (BUT), Schirmer I testing, blepharitis score and bulbar conjunctival injection. The subjective efficacy was evaluated with global symptom and facial analogue scores. Results: An improvement in corneal fluorescein staining in the treated eye by >1 point from baseline to completion of the trial at week 4 was found in 9 of 10 patients (p < 0.01). Topical atorvastatin significantly improved the tear film BUT (p < 0.01), blepharitis score (p < 0.05) and bulbar conjunctival injection (p < 0.05). The global symptom score and facial analogue score also improved (p < 0.05). There were no side effects. Conclusion: Topical atorvastatin is a potential therapy for DEB patients. Larger comparative clinical studies are required to establish the efficacy and safety of topical atorvastatin.

The Virtual Ophthalmology Clinic (VOC) is an interactive web-based teaching module, with special emphasis on history taking and clinical reasoning skills. The purpose of this study was to determine the impact of VOC on medical students’ learning. A randomised controlled trial (RCT) was conducted with medical students from the University of Sydney (n=188) who were randomly assigned into either an experimental (n=93) or a control group (n=95). A pre- and post-test and student satisfaction questionnaire were administered. Twelve months later a follow-up test was conducted to determine the long-term retention rate of graduates. There was a statistically significant (P

Although willingness, attitudes and beliefs surrounding solid-organ donation have been extensively investigated, much less is known about corneal donation. Despite evidence that a substantial number of families who agree to multiorgan donation also specifically refuse corneal donation, it is unclear why this occurs and what can be done to increase rates of corneal donation. We conducted a survey of 371 Australian adults regarding their views on corneal donation. Although willingness to donate corneas generally reflected a person's willingness to donate all of one's organs, unwillingness to donate corneas appeared to be due to other factors. Specifically, decisions not to donate appear to be driven by a range of concerns surrounding disfigurement. The survey also provides eye banks with reassurance about the acceptability of whole globe procurement, and recognition that research into blindness is a highly valued part of corneal donation. Finally, the survey identifies that many individuals see benefit in having their family engaged in the decision-making process, suggesting that decisions about donation are more complex than a simple appeal to the autonomy of the deceased.

Aim: To study the maturation of multifocal visual evoked potentials (multifocal VEP) in normal children between the ages of 5 and 16 years and to apply the results clinically in selected cases to the diagnosis of optic pathway diseases. Method: 70 normal children were recruited from the community and multifocal VEP (Accumap ObjectiVision, Sydney, Australia) was recorded. The waveform of the evoked responses, the latency and amplitude were analysed. Using these data, an age matched comparison was made with three children with advanced optic nerve disease; two had optic nerve glioma and one had congenital glaucoma. Results: The full field amplitude did not correlate with age and varied greatly within each age group (coefficient of variability 28%). When scaled with respect to the background electroencephalogram the intra-age group variability decreased to 15% and a sigmoid relation was found between amplitude and age. The scaled amplitude remained largely unchanged till 11 years, between 11 and 13 years there was a rapid increase (40%), and remained stable thereafter. This relation was seen at all eccentricities tested. The latency decreased gradually with age and plateaued at 13 years. In the three children with vision abnormalities this test was able to detect scotomas consistent with their condition. Conclusion: Multifocal VEP perimetry shows an age related maturation in the visual pathway, characterised by distinctive timeframe of development for amplitude and latency. It can be performed by children as young as 5 years of age and holds promise as a diagnostic test capable of documenting children’s visual fields objectively, even before they are able to perform subjective field tests.

We examined the records of 53 patients treated for choroidal melanoma between 1985 and 1989. The aim of this study was to assess the safety and short-term results of iodine-125 episcleral plaque therapy. There were 28 males and 25 females, aged 20 to 77 years (median 61 years), treated for single tumours with a median diameter of 9 mm (range 5 to 15 mm) and with a median thickness of 4 mm (range 2 to 10 mm). The plaques containing iodine-125 seeds were chosen according to tumour size: 10 mm (16 patients); 15 mm (36 patients); 20 mm (one patient). All patients are alive at last follow-up (median 1.3 years, range 4 months to 3.3 years). Four patients underwent enucleation for melanoma progression. Thirty patients have developed some type of complication (more than one complication occurred in the same eye in 12 patients): retinitis (19), optic neuropathy (7); cataract (4), rubeosis iridis (2). Overall, visual acuity deteriorated in 32 patients, remained stable in 12 patients and improved in 9 patients. Iodine-125 plaque therapy appears to offer patients good prospects of tumour control and preservation of useful vision.

PURPOSE: To examine the natural history of multifocal visual evoked potentials (mfVEPs) within 12 months of the first episode of optic neuritis (ON) in patients with possible multiple sclerosis (MS). METHODS: Twenty-seven patients with a first episode of ON, no previous demyelinating events, and MRI lesions consistent with demyelination were examined with mfVEP. Changes in amplitude and latency of mfVEP were analyzed at 1, 3, 6, and 12 months after an acute attack. RESULTS: Five of 27 patients had persistent loss of amplitude after 12 months of follow-up. This loss was most marked centrally. Amplitude recovered in the remaining 22 patients at 1 month, but delayed latency, which was also most marked centrally, persisted. Of these, two distinct subgroups were identified: six patients with no improvement in latency and 16 patients with significant latency recovery over the 12 months of follow-up, suggesting remyelination. Conversion to MS was highest in the group with severe amplitude loss, followed by the group with no latency recovery. The conversion rate was lowest in the group of patients with latency improvement. CONCLUSIONS: Distinct patterns of disease evolution were identified using mfVEP in patients with first episode of optic neuritis and at high risk for MS, supporting the concept of heterogeneity of early lesions in MS.

We have employed proteomics to establish a proteome map of the normal rat retina. This baseline map was then used for comparison with the early diabetic rat retinal proteome. Diabetic rat retinae were obtained from Dark Agouti rats after 10 wk of streptozotocin-induced hyperglycaemia. Extracted proteins from normal and diabetic rat retinae were separated and compared using 2-DE. A total of 145 protein spots were identified in the normal rat retina using MALDI-MS and database matching. LC-coupled ESI-MS increased the repertoire of identified proteins by 23 from 145 to 168. Comparison with early diabetic rat retinae revealed 24 proteins unique to the diabetic gels, and 37 proteins absent from diabetic gels. Uniquely expressed proteins identified included the HSPs 70.1A and 8, and platelet activating factor. There were eight spots with increased expression and 27 with decreased expression on diabetic gels. Beta catenin, phosducin and aldehyde reductase were increased in expression in diabetes whilst succinyl coA ligase and dihydropyrimidase-related protein were decreased. Identification of such changes in protein expression has given new insights and a more comprehensive understanding of the pathogenesis of diabetic retinopathy, widening the scope of potential avenues for new therapies for this common cause of blindness.

To determine the prevalence and risk factors associated with secondary glaucoma postcongenital cataract surgery.All children diagnosed as having congenital cataracts in a major children's hospital between 1985 and 2005 were included in a retrospective case series. Medical records of 423 eyes among 283 patients who underwent cataract surgery with or without intraocular lens implantation at age/=16 for congenital cataract were reviewed. The main outcome measure was presence or absence of secondary glaucoma and time to glaucoma postsurgery. The following risk factors were evaluated: age at cataract surgery, presence of systemic anomalies, microcornea, persistent hyperplastic primary vitreous (PHPV), primary capsulotomy/anterior vitrectomy, primary intraocular lens implantation, secondary membrane surgery and duration of postoperative observation.The statistical methods were the use of Kaplan-Meier survival analysis and Multivariate Cox hazards regression analysis. The mean follow-up was 6.3 (SD 5.0) years (median 4.6 years; range 0.5 to 20.3 years). Glaucoma developed in 36 of 234 patients (15.4%). Multivariate Cox proportional hazards regression analysis identified age less than 9 months at time of surgery (RR 2.9, 95% CI 1.3 to 7.7; p = 0.03), microcornea (RR 3.7, 95% CI 2.0 to 7.0; p0.001), and follow-up time as important predictors of glaucoma. PHPV (RR 1.4, 95% CI 0.7 to 2.7; p = 0.41) and primary posterior capsulotomy/anterior vitrectomy (RR 2.2, 95% CI 0.9 to 5.5; p = 0.17) were not significantly associated with secondary glaucoma in the multivariate model. The mean time to glaucoma after congenital cataract surgery was 4.9 years (range 2 weeks to 16.8 years).Secondary glaucoma is an important sequela in patients who undergo surgery for congenital cataracts. It is imperative that these patients get lifelong surveillance, as glaucoma can occur years after the initial operation.

Anophthalmia and pituitary gland hypoplasia are both debilitating conditions where the underlying genetic defect is unknown in the majority of cases. We identified a patient with bilateral anophthalmia and absence of the optic nerves, chiasm and tracts, as well as pituitary gland hypoplasia and ear anomalies with a de novo apparently balanced chromosomal translocation, 46,XY,t(3;14)(q28;q23.2). Translocation breakpoint analysis using FISH and high-resolution microarray comparative genomic hybridization (CGH) has identified a 9.66 Mb deleted region on the long arm of chromosome 14 which includes the genes BMP4, OTX2, RTN1, SIX6, SIX1, and SIX4. Three other patients with interstitial deletions involving 14q22-23 have been described, all with bilateral anophthalmia, pituitary abnormalities, ear anomalies, and a facial phenotype similar to our patient. OTX2 is involved in ocular developmental defects, and the severity of the ocular phenotype in our patient and the other 14q22-23 deletion patients, suggests this genomic region harbors other gene/s involved in ocular development. BMP4 haploinsufficiency is predicted to contribute to the ocular phenotype on the basis of its expression pattern and observed murine mutant phenotypes. In addition, deletion of BMP4 and SIX6 is likely to contribute to the abnormal pituitary development, and SIX1 deletion may contribute to the ear and other craniofacial features. This indicates that contiguous gene deletion may contribute to the phenotypic features in the 14q22-23 deletion patients.

Objective To monitor the difference in conversion rates to multiple sclerosis (MS) in 46 patients with optic neuritis between patients with multifocal visual evoked potential latency delay and those with normal latency. Design Prospective case series. Setting Metropolitan neuro-ophthalmology clinic. Participants Forty-six patients with optic neuritis who did not have a diagnosis of MS on enrollment in the study. Main Outcome Measures Conversion to MS according to the McDonald criteria. Results Analysis revealed that only 22 subjects had multifocal visual evoked potential latency delay. Over 1 year, 36.4% of patients with optic neuritis with latency delays progressed clinically to MS compared with 0% of those with normal latencies ( P = .03, χ 2 ). Conclusion This may indicate that multifocal visual evoked potential latency delay can assist in predicting progression to future MS.

Background/aims: The literature on refractive change in thyroid eye disease (TED) is limited. This study documents the refractive change in patients with TED undergoing orbital decompression. The authors propose possible mechanisms for their acquired refractive error. Methods: This is a retrospective observational case study of five patients with progressive TED. Their detailed eye examinations including refractive state preoperatively and postoperatively are presented. Results: An acquired hypermetropic shift with active TED before orbital decompression of up to 3.75D spherical equivalent refraction (SER) is reported in one patient. Post-orbital decompression, an induced myopic shift of between 1.00–2.50D SER for all patients is observed, noted to range from 1 day following surgery to up to 9 months, dependent on the availability of data. Axial length increased in two cases corresponding to postoperative myopic shift. Magnetic resonance imaging findings of one patient demonstrate flattening of the posterior pole as a cause of the acquired preoperative hypermetropia. Conclusions: TED has a significant effect on the refractive state of patients. The proposed mechanism of acquired hypermetropia relates to increased volume of orbital contents with flattening of the posterior globe. This is reversed with successful orbital decompression. Documentation of refractive error in all cases of progressive TED is recommended. Progressive acquired hypermetropia may be suggestive of TED activity.

Objective: To determine the safety of a single intravitreal injection of triamcinolone acetonide (4 mg) in patients with subfoveal choroidal neovascularization caused by age-related macular degeneration. Methods: A double-masked, placebo-controlled, randomized clinical trial was conducted at a public tertiary referral eye hospital. Patients participating had agerelated macular degeneration with evidence of choroidal neovascularization, any part of which was classic; age older than 59 years; and best-corrected visual acuity of 20/200 or better. Eyes were assigned to active study treatment or to placebo. Intraocular pressure and cataract grading were performed every 6 months for 3 years. Adverse events, from mild to vision-threatening or lifethreatening, were recorded as procedure-related or corticosteroid-related. Results: Seventy-five eyes were assigned to study treatment and 76 eyes to placebo. There were no moderate or severe adverse events related to the surgical procedure in either group. Triamcinolone-treated eyes had a significantly increased risk of developing mild or moderate elevation of the intraocular pressure. Topical glaucoma medication reduced intraocular pressure to acceptable levels in all patients. There was significant progression of cataract in the triamcinolone-treated eyes. Conclusion: Despite a significant adverse event profile, intravitreal triamcinolone is generally well tolerated by the human eye as long as patients are carefully followed up by their surgeon and treated appropriately, when necessary. Arch Ophthalmol. 2004;122:336-340

Aims: To review factors affecting graft survival and determinants of visual acuity after penetrating keratoplasty in children. Methods: All cases of penetrating keratoplasty performed in an ophthalmic unit, in children aged less than 15 years at the time of operation, for the period 1984 to 2002 were included. Results: 19 penetrating keratoplasties were done in 18 eyes of 16 children, age range 2 weeks to 14 years 8 months (mean 9.24 years), with mean follow up 6.6 years. 73.7% of grafts have remained clear for up to 14 years. Postoperative visual acuity among congenital indications for graft was better than 6/60 in only 14.2% of cases, but was better than or equal to 6/12 in all cases of keratoconus. Conclusion: This series shows that prolonged corneal graft survival can be achieved in children, but successful restoration of visual acuity depends upon a period of normal visual development before the onset of corneal opacification.

To assess whether an association exists between systemic use of anti-inflammatory medications at baseline and the prevalence or incidence of either late or early age-related maculopathy (ARM) in a population-based cohort.3654 participants of the Blue Mountains Eye Study baseline examination (1992-94) were followed during 1997-99. Use of anti-inflammatory medication was recorded during the baseline interview. After excluding 543 persons who died since baseline, 2334 (75% of the surviving participants) attended 5-year follow-up examinations. Retinal photographs taken during both examinations were graded using the Wisconsin Age-Related Maculopathy Grading System. Prevalence refers to the proportion of participants having ARM at baseline. Incidence refers to the proportion of participants without ARM at baseline who developed it over the 5-year period. Known ARM risk factors were adjusted for when assessing the relationship between use of anti-inflammatory medications and ARM.At baseline, 1010 (27.6%) of 3654 participants were current users of non-steroidal anti-inflammatory drugs (NSAIDs), 514 (14.1%) were past users and 1282 (35.1%) were ever users. The corresponding numbers of subjects reporting current, past or ever use of corticosteroids (including inhaled steroids) were 225 (6.2%), 519 (14.2) and 564 (15.4), respectively. Late ARM was present in 72 participants (2.0%) and early ARM was present in 171 participants (4.9%) at the baseline examination. During the follow-up period, 25 participants (1.1%) developed incident late ARM and 192 (8.7%) developed incident early ARM. After adjusting for age, sex, family history of ARM and smoking, no significant associations were evident for the use of NSAIDs or corticosteroids and the prevalence of either late or early ARM. There were also no associations found between use of these medications at baseline and the 5-year incidence of either late or early ARM.No association was found between use of systemic anti-inflammatory medications and either the cross-sectional prevalence or longitudinal incidence of ARM in this population.

To describe the natural history of optic pathway gliomas (OPGs) in patients with neurofibromatosis type 1 (NF1), and to evaluate the current recommended guidelines for monitoring and follow-up of OPGs in this population.Retrospective case series.Patients with OPGs and NF1 seen in the neurofibromatosis clinic at the Children's Hospital at Westmead in Sydney, Australia.Patients with definite NF1 and confirmed OPGs were identified and their medical records searched to obtain data on demographics, details of the OPG diagnosis, and serial ophthalmic examination findings. Patients were stratified into groups according to age and mode of presentation.Visual acuity was recorded for each eye and grouped into mild (Snellen equivalentor = 6/12), moderate (Snellen equivalent = 6/15-6/60), and severe (Snellen equivalent6/60) visual impairment at time of diagnosis, during follow-up, and at the most recent examination.Data were collected on 54 patients, the majority of whom (78%) were seen from 1990 to 2002, with an average follow-up of 8.6 years. The mean age at the time of OPG diagnosis was 5.2 years, with 32 patients having symptoms or signs at the time of diagnosis. Seventeen patients were diagnosed after the age of 6 years (range, 7-15). Twenty-two patients had tumor progression within 1 year of diagnosis, and a further 6 patients showed progression after 1 year. Most patients' conditions were managed conservatively (68.5%). At follow-up, 17 patients (31.5%) had severe visual impairment (6/60 Snellen equivalent) in their worse eye, and 16.7% had bilateral moderate/severe visual impairment.Contrary to some previous reports, our results show that OPGs in patients with NF1 often present in older children and may progress some time after diagnosis. Given the potential for serious visual consequences, these findings indicate a need for regular ophthalmological monitoring of this population for a long duration.

Aims: To assess the aetiological factors associated with the occurrence of perforating ocular injuries in children in an urban setting and to assess the visual outcomes of such injuries. Methods: All cases of perforating ocular injury presenting to a single paediatric hospital (age less than 16 years) over a 17 year period were identified by a medical record search. All new cases of perforating ocular injury identified were included. All information was obtained retrospectively from the medical records. Results: There were 72 cases identified. The commonest causes of perforating ocular injury were sharp tools (knives/scissors) poked by the child into his/her own eye (17%), or objects thrown at the child (17%). Injuries were most likely to have occurred at home (58%). The age range for injuries was 8 months to 14 years 8 months. Perforating ocular injury was most frequent in the 3–6 year group (32%) followed by the 6–9 year group (25%). Males were more frequently involved than females (48–24). There was no correlation between the laterality of the eye, the time of day of the occurrence, or the day of the week of the occurrence. The final acuity achieved was better or equal to 6/12 in 36% and less than 6/60 in 31%. Injuries occurred more frequently on weekends than on weekdays. There were six enucleations (8%). Follow up was for an average period of 25 months. Conclusions: Penetrating ocular injury occurs most frequently in the home setting and mostly as the result of the use of sharp tools or by thrown objects. Prevention of penetrating ocular injury requires greater education of children and their carers especially on the potential dangers within the home.

The purpose of this report is to describe a modified surgical iridocyclectomy technique and lensectomy for the removal of a recurrent iris cyst and a cataract in a child. A 3-year-old boy underwent uncomplicated standard iridocyclectomy for the removal of an enlarging congenital epidermal iris cyst. In the postoperative period, the cyst recurred. A second surgical intervention was performed using a modified iridocyclectomy technique. Sclerocorneal dissection of the involved quadrant was performed. After a lensectomy, an additional deep lamellar dissection of the peripheral cornea was undertaken prior to iris cyst removal and pupil reconstruction. This modified two-layered iridocyclectomy technique permits an elegant access to the iris lesion and allows the construction of a two-layered watertight wound, reducing the risk of hypotony and wound ectasia. We believe it also allows a better control of astigmatism and is a safe procedure in the pediatric population, particularly during the amblyogenic period.

AIMS—To describe features influencing the management of primary iris melanoma and report the outcome of conservative surgical treatment of patients diagnosed with this condition in a tertiary referral academic setting over a 20 year period. METHODS—Retrospective non-comparative case series of consecutive patients diagnosed with iris melanoma from 1980-2000 using medical records from the University of Sydney Department of Ophthalmology and NSW Cancer Registry RESULTS—51 cases were identified. The most common presentation was growth of a previously noted pigmented lesion. Initial management was either observation or local resection (two had enucleations) with iris reconstruction where possible (23.8%). The mean follow up was 8.7 years (range 1-17 years). Vision of 6/12 or better was maintained in the majority (78.6%) treated by local resection. Pupil reconstruction significantly reduced reported postoperative glare symptoms. Four patients had features suggestive of local recurrence and there was no documented metastatic disease or death from iris melanoma in this series. Histologically, the majority were spindle B cell melanomas. Clinical features including prominent tumour vascularity, rapid growth, and heterogeneous pigmentation were each significantly associated with an epithelioid cell component. Involvement of the iridocorneal angle was frequently associated with ciliary body invasion. CONCLUSIONS—Management decisions for iris melanoma will depend on the clinical features. Mixed or epithelioid histology is more likely in the presence of two or more of the features of malignancy and may justify earlier intervention. When treatment is undertaken, local resection achieves long term tumour clearance with an acceptable morbidity. In resecting iris melanoma, careful assessment for iridocorneal angle involvement is important in treatment planning. Iris reconstruction has a useful role in reducing postoperative photophobia.

The authors present a case of optic neuritis in an adult patient who had been self-prescribing extraordinarily large dosages of chloramphenicol for chronic prostatitis over several years. The visual symptoms resolved upon cessation of the drug and prescription of B group vitamins. Chloramphenicol optic neuritis has not been described in the literature for over 20 years and previously predominantly in children with cystic fibrosis.

A number of reports have suggested that oxidative damage in the retina may contribute to the pathogenesis of Age-related macular degeneration (AMD). The present study was designed to investigate the hypothesis that serum levels of the antioxidants, Vitamin E and selenium are related to the pathogenesis of AMD. Fasting bloods were obtained from 80 patients with AMD and 86 controls. Assays for serum levels of Vitamin E, selenium, cholesterol and triglycerides were performed. Assessment of patients and controls was based upon eye examination, fundus photography and medical history. No significant difference was found in serum levels of Vitamin E between subjects and controls, however, there was a borderline association between AMD and both serum selenium levels and current smoking status. The results suggest that if oxidative damage is a factor in the pathogenesis of AMD, it is not reflected in serum levels of Vitamin E; further studies are required to clarify the possible relationship between serum selenium levels, smoking and AMD.

Parry Romberg syndrome is a rare disorder characterised by progressive hemifacial atrophy that is usually unilateral, involving the skin, subcutaneous tissue, and subsequently the muscle, cartilage and bone. Previous cases with various ocular manifestations have been reported. A case of Parry Romberg syndrome with retinal vasculitis is reported.

PURPOSE. To determine whether simultaneous binocular (dichoptic) stimulation for multifocal visual evoked potentials (mfVEP) detects glaucomatous defects and decreases intereye variability. METHODS. Twenty-eight patients with glaucoma and 30 healthy subjects underwent mfVEP on monocular and dichoptic stimulation. Dichoptic stimulation was presented with the use of virtual reality goggles (recording time, 7 minutes). Monocular mfVEPs were recorded sequentially for each eye (recording time, 10 minutes). RESULTS. Comparison of mean relative asymmetry coefficient (RAC; calculated as difference in amplitudes between eyes/ sum of amplitudes of both eyes at each segment) on monocular and dichoptic mfVEP revealed significantly lower RAC on dichoptic (0.003 0.03) compared with monocular testing (0.02 0.04; P 0.002). In all 28 patients, dichoptic mfVEP identified defects with excellent topographic correspondence. Of 56 hemifields (28 eyes), 33 had Humphrey visual field (HFA) scotomas, all of which were detected by dichoptic mfVEP. Among 23 hemifields with normal HFA, two were abnormal on monocular and dichoptic mfVEP. Five hemifields (five patients) normal on HFA and monocular mfVEP were abnormal on dichoptic mfVEP. In all five patients, corresponding rim changes were observed on disc photographs. Mean RAC of glaucomatous eyes was significantly higher on dichoptic (0.283 0.18) compared with monocular (0.199 0.12) tests (P 0.0006). CONCLUSIONS. Dichoptic mfVEP not only detects HFA losses, it may identify early defects in areas unaffected on HFA and monocular mfVEP while reducing testing time by 30%. Asymmetry was tighter among healthy subjects but wider in patients with glaucoma on simultaneous binocular stimulation, which is potentially a new tool in the early detection of glaucoma. (Invest Ophthalmol Vis Sci. 2007;48:4590‐4596) DOI: 10.1167/iovs.07-0318

Objective To determine if a single intravitreal injection of 4 mg of triamcinolone acetonide in patients with classic choroidal neovascularization associated with age-related macular degeneration can safely reduce the risk of severe visual loss. Methods A double-masked, placebo-controlled, randomized clinical trial was performed in patients 60 years or older who had choroidal neovascularization with any classic component, a duration of symptoms of less than 1 year, and a visual acuity of 20/200 or better. Best-corrected visual acuity, intraocular pressure, and cataract grading were performed before the injection and then at 3, 6, and 12 months. Main Outcome Measure The development of severe loss of vision (30 letters) by survival analysis on an intention-to-treat basis. Results One hundred fifty-one eyes were randomized into the study, and follow-up data were obtained for 73 (97%) of the 75 eyes in the treated group and for 70 (92%) of the 76 eyes in the control group. There was no difference between the 2 groups for the development of severe visual loss during the first year of the study (log-rank χ21= 0.03,P= .90). In both groups, the 12-month risk of severe visual loss was 35%, with a hazard ratio of 1.05 (95% confidence interval, 0.59-1.86). The change in size of the neovascular membranes, however, wassignificantly less in eyes receiving triamcinolone thanin those receiving placebo 3 months after treatment (P= .01), although no difference was noted after 12 months. After 12 months, treated eyes had a significantly higher risk of an elevated intraocular pressure (31/75 [41%] vs 3/76 [4%];P Conclusions A single dose of intravitreal triamcinolone had no effect on the risk of loss of visual acuity during the first year of the study in eyes with age-related macular degeneration and classic choroidal neovascularization, despite a significant antiangiogenic effect found 3 months after treatment. This biological effect warrants further study.

Purpose: To evaluate the presence of central nervous system degeneration in patients with low-tension glaucoma using magnetic resonance imaging. Method: Ten patients with low-tension glaucoma and ten age-matched control subjects underwent magnetic resonance imaging. Cortical atrophy and cerebral infarcts were graded from "0" (normal) to "3," which was done subjectively by two neuroradiologists independently in a masked fashion. Midsagittal corpus callosum section was evaluated by measuring the thickness and cross-sectional area. Results: There was a significantly greater extent of cerebral infarcts in the patients with low-tension glaucoma ( P = 0.02). The thickness of the body ( P = 0.03) and genu ( P = 0.04) of the corpus callosum were thinner in the patients with low-tension glaucoma. The corpus callosum cross-sectional area was smaller in the low-tension glaucoma group ( P = 0.04). There were no significant differences in the other parameters in this study. Conclusion: This study suggests a greater extent of cerebral infarcts and corpus callosum atrophy in patients with low-tension glaucoma. This may imply a greater degree of neuronal degeneration, possibly on an ischemic basis in low-tension glaucoma.

Four hundred and forty-eight consecutive corneal grafts are analysed and their survival calculated using the actuarial life-table method. Overall survival at two years is 81 +/- 4% and at five years is 65 +/- 5%. Within diagnostic subgroups keratoconus has the best prognosis. Previous graft failure and recipient corneal vascularization are shown to have a negative effect on graft survival. Sex of patient, urgency of operation and use of combined procedures do not affect survival and second regrafts fare no worse than first regrafts. The use of the actuarial life-table method of analysing graft survival is discussed and its importance emphasized.

Three cases of sympathetic ophthalmia that occurred up to 62 years after an ocular injury are reported. Sympathetic ophthalmia occurred spontaneously in the first case, 62 years after a shot-gun pellet had penetrated one eye. In the other two cases, further surgery had been performed on previously-injured eyes. This was followed by a bilateral granulomatous panuveitis that developed within a week in one case and after seven years in the other case. The risk of elective surgery in eyes that are already at risk of sympathetic ophthalmia and the practical aspects of patient counselling are discussed.

A 21-year-old man who had radial keratotomy performed two years previously presented with a painful, red right eye. Suppurative keratitis was found in one of the eight radial incisions and Staphylococcus epidermidis was isolated by culture. Lack of stromal healing of the infected incision and the presence of epithelial cysts in four other incisions in the same eye suggest that the cause of the corneal infection was loss of the epithelial barrier function because of epithelial breakdown.

This report concerns three patients with human immunodeficiency virus (HIV) infection in whom malignant melanoma developed. One patient had metastatic malignant melanoma, one had iris melanoma, and one had a single skin melanoma. All three had lower absolute numbers of CD4+ cells than a control group, and the severity of their disease was inversely proportional to the absolute number of CD4+ cells. This report suggests an association between the immunodeficiency resulting from HIV infection and the development of malignant melanoma.

The corneas of 50 normal subjects were examined before and after electroretinography performed with gold foil electrodes. Examination included slit-lamp biomicroscopy and staining with sodium fluorescein. All corneas were normal on examination prior to electroretinography. Three types of transient corneal changes were observed--punctate epithelial keratitis, corneal erosions, and stromal thinning. Each cornea was assigned a numerical damage score based on a simple scoring system. Thirty one subjects (62%) had some degree of corneal change, and in three cases (6%) follow-up was required. Multiple regression analysis was performed to discover any risk factors. Both age of the subject and the use of local anaesthetic were strongly associated with corneal changes.

We investigated 107 cases of presumed microbial keratitis amongst patients presenting to the Sydney Eye Hospital between October 1986 and August 1988 to determine the frequency of infection, the common causative organisms and those factors that predispose to corneal infection. We found that 95% of culture-proven cases were caused by bacteria and that Staphylococcus epidermidis and Corynebacterium sp. accounted for 42.5% of these cases. All bacteria (except Staphylococcus epidermidis and Mycobacterium chelonae) were sensitive to gentamicin. In 62.6% of cases, cultures for bacteria and fungi were negative. Retrospective review of the clinical records of these patients showed that herpes simplex virus, contact lens wear, staphylococcal marginal keratitis and recurrent erosion were important causes of keratitis in this group.

• We present the clinical and electrophysiological findings in three cases of osteopetrosis with visual loss. In all cases the findings on electroretinography suggested diffuse retinal degeneration, despite normal ophthalmoscopic appearance of the retinas. We suggest that retinal degeneration may be a common finding in osteopetrosis and a major cause of visual loss. Patients with osteopetrosis and poor vision should be examined using electrophysiological techniques to detect unsuspected retinal dysfunction. (Am J Dis Child133:955-958, 1979)

We studied eight infants who developed unilateral axial myopia associated with neonatal eyelid closure. Clinical findings and ultrasonographic biometric measurements suggested that axial elongation of the eye may occur as a result of neonatal eyelid closure, a finding similar to that for experimental myopia produced in neonatal animals after eyelid fusion. Early treatment of eyelid occlusion in the neonate may prevent axial myopia and subsequent anisometropic amblyopia.