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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.

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.

Purpose To determine the ability of blue-on-yellow multifocal visual evoked potentials (BonY mfVEP) to identify functional loss in preperimetric glaucoma. Design Prospective case series. Participants Thirty patients with glaucomatous optic discs and normal standard visual fields. Methods All patients underwent BonY mfVEP, dilated optic disc stereophotography, and optical coherence tomography (Fast RNFL protocol). Optic disc photographs were assessed by 2 independent examiners in a masked fashion. Main Outcome Measures The mfVEP amplitude asymmetry and latency values were analyzed and compared topographically with findings of disc assessment. Average retinal nerve fiber layer (RNFL) thickness, RNFL asymmetry, and sectors with RNFL thinning were compared between patients with and without mfVEP defects. Results Fourteen (46.7%) patients demonstrated significant abnormality on amplitude asymmetry deviation plots of BonY mfVEP. In all 14 cases, the defect was monocular and corresponded to the eye with the worse disc. In 13 of 14 patients, the defect also corresponded to the location of the worst affected rim. Average RNFL thickness of eyes with mfVEP defects was 81.2±9.9 μm, significantly lower than that of patients without defects (90±10.5 μm; P = 0.035). Mean asymmetry of RNFL (better minus worse eye) also was significantly higher for patients with mfVEP defects compared with those without such defects (9.0±6.4 μm vs. 3.0±7 μm; P = 0.03). Average latency of both eyes of glaucomatous patients was delayed compared with that of controls, with no difference in latency between worse and better eyes of glaucoma patients. There was no association of latency delay with either the location of disc changes or mfVEP amplitude defects. Conclusions Amplitude asymmetry of the BonY mfVEP seems to be a promising tool to identify functional loss in preperimetric glaucoma. Financial Disclosure(s) Proprietary or commercial disclosure may be found after the references.

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.

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 twolayered 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. [Ophthalmic Surg Lasers 2002; 33:62-65]

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.

Disorders of eye development such as microphthalmia and anophthalmia (small and absent eyes respectively), anterior segment dysgenesis where there may be pupillary and iris anomalies, and associated cataract and glaucoma, often lead to visual impairment or blindness. Currently treatment options are limited, as much is unknown about the molecular pathways that control normal eye development and induce the aberrant processes that lead to ocular defects. Mutation detection rates in most of the known genes are generally low, emphasizing the genetic heterogeneity of developmental ocular defects. Identification of the disease genes in these conditions improves the clinical information available for affected individuals and families, and provides new insights into the underlying biological processes for facilitation of better treatment options. Investigation of chromosomal rearrangements associated with an ocular phenotype has been especially powerful for disease gene identification. Molecular characterization of such rearrangements, which pinpoints the region by physically disrupting the causative gene or its regulatory sequences, allows for rapid elucidation of underlying genetic factors that contribute to the phenotype. Genes includingPAX6, PITX2, FOXC1, MAF, TMEM114, SOX2, OTX2andBMP4have been identified in this way to be associated with developmental eye disorders. More recently, new methods in chromosomal analysis such as comparative genomic hybridization (CGH) microarray, have also enhanced our ability in disease gene identification.

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.

Purpose To describe a case of Euphorbia lactea sap keratouveitis and to review all reported cases of ocular toxicity caused by Euphorbia species. Methods Case report and review of literature. Results A 79-year-old woman presented 34 hours after she felt some sap of an E. lactea plant spray into her right eye. Visual acuity was counting fingers at 1 m. Examination revealed ciliary injection, 90% corneal epithelial defect, marked stromal edema with Descemet folds, and anterior-chamber flare with a 1-mm hypopyon. There was no vitreitis, and funduscopy was unremarkable. No foreign body was seen on B scan ultrasound or computed tomography scan of the orbits. Corneal scraping excluded bacterial and herpetic keratitis. Intensive topical antibiotic therapy was started with cephalothin 5% and gentamicin 0.9%, and the pupil was dilated with atropine. Topical steroids were started once the epithelial defect had healed. Examination 11 weeks after the injury revealed minimal subepithelial corneal haze and marked improvement in visual acuity. Conclusions To the best of our knowledge, this is only the third reported case of E. lactea sap keratouveitis. The clinical course of E. lactea sap keratouveitis is compared with that reported for other Euphorbia species.

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.

Naevus of Ota (naevus fusculocoeruleus ophthalmomaxillaris) was described by the Japanese dermatologist, Ota, in 1939 as a dermal melanocytic hamartoma that presents as bluish hyperpigmentation along the ophthalmic, maxillary, and mandibular branches of the trigeminal nerve. It is bilateral in less than 5% cases, occurring frequently in Orientals (0.2%–1%) and darker races and rarely in white people (0.04%). Open angle glaucomas and choroidal melanoma are the rare ocular involvements. Ota’s naevus is more common in Asians than white people but uveal melanoma occurs predominantly in white populations.1,2 Dark skinned patients represent only 1% of all cases of orbital melanomas.3 The risk of developing uveal melanoma in a patient with naevus of Ota is one in 400 patients in their lifetime.1,2 We report a rare case of bilateral naevus of Ota with a right (RE) …

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

Clinical indocyanine green (ICG) angiography has gained an established role in the diagnosis of choroidal neovascularisation (CNV) in age related macular degeneration (AMD).1,2 It is potentially able to localise CNV with greater accuracy compared with fluorescein angiography because it fluoresces in the infrared range, allowing imaging of choroidal pathology through blood and pigment.3 Additionally ICG angiography may better define CNV because of its high protein binding affinity in serum,4 resulting in reduced leak from the CNV. This allows ICG angiography to clearly delineate the feeding vascular supply to the CNV allowing direct ablative thermal laser to precisely target this supply.5 Treatments for CNV, including thermal laser, photodynamic therapy, and macula translocation are all reliant on the ability of the clinician to diagnose accurately the location and nature of CNV. Few clinicopathological data exist to correlate the ICG angiographic appearance of CNV with its histology. The presence of CNV was shown pathologically in an eye with a plaque like hyperfluorescence on ICG angiography shown years previously.6 Lafaut reported the pathology of surgically removed recurrent CNV in two eyes that were preoperatively imaged with ICG angiography.7 The hyperfluorescence of drusen on ICG angiography in the monkey model has been correlated with ICG dye that has been found histologically on infrared fluorescence microscopy.8 In this case report we hope to correlate ICG angiographic findings in a patient with exudative AMD with the histological localisation of ICG to the surgically excised CNV. This will verify that ICG angiographic findings are an accurate representation of the location and nature of the CNV. A 71 year old patient with classic subfoveal CNV was …

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.

Ouvrier and Billson (1988) were apparently the first to describe this entity. In the four original cases, the clinical features were as follows: (1) onset usually under 1 year of age, (2) episodes of variably sustained conjugate upward deviation of the eyes, with neck flexion (chin down) apparently compensating for the abnormal eye position, (3) downbeating saccades in attempted downgaze, (4) normal horizontal eye movements, (5) diurnal fluctuation of symptoms, (6) frequent relief by sleep, (7) exacerbation with febrile illnesses, (8) varying degrees of ataxia, (9) neurological examination usually otherwise normal, (10) absence of deterioration during long-term follow-up, (11) eventual improvement, (12) usually negative investigations, including imaging, EEG and CSF neurotransmitters. As of 2002, 49 cases have been reported. Aetiological factors have included autosomal dominant inheritance in four families, foetal exposure to sodium valproate in three cases, and structural lesions in five (hypomyelination x 2, periventricular leukomalacia, Vein of Galen malformation, pinealoma). Only a few cases have responded to L-dopa. The pathophysiology is still not understood. The outcome appears to be good in about half the cases. Ataxia, borderline cognitive abilities and residual minor oculomotor disorders are seen in the remainder.

Uveal melanoma, affecting iris, ciliary body, and choroid, is the most common primary ocular malignancy in adults, with an average incidence of 8 per 1 000 000. Smaller primary lesions may be controlled with local resection, laser photocoagulation, proton therapy, or plaque radiotherapy. However, these treatments are often associated with sight threatening complications including radiation retinopathy, macula oedema, scarring, and haemorrhaging.1 Recurrence of the primary tumour after local resection is also commonly observed, with recurrence rates for iris, ciliary body, and choroidal tumours between 11% and 32%.2–4 Treatment of recurrent disease usually involves enucleation of the eye. Even with effective early treatment of the primary tumour, uveal melanoma displays an unpredictable clinical course during which clinically evident metastases may occur after a prolonged disease free interval.1 Metastatic disease from primary uveal melanoma usually appears in 10–40% of patients within 10 years, mostly in the liver, and with an average survival of 5.4 months following diagnosis.5,6 Development of metastatic disease is currently untreatable. Chemotherapy is generally ineffective because of the restricted period of action during the therapeutic cycle. Immunotherapy is still in the experimental stage and its effects remain elusive. However, α radioimmunotherapy is worth consideration as a potential treatment of primary and metastatic ocular melanoma. For example, the radionucleotide bismuth-213 (213Bi) (t1/2 = 46 minutes), which has been chelated to some monoclonal antibodies, emits an α particle with short range (80 μm) and high linear energy transfer radiation which is about 100 times greater than for β particles.7 mAb 9.2.27 recognises a Mr 250 000 glycoprotein NG2, associated with a chondroitin sulphate proteoglycan8,9 and is expressed on most human cutaneous melanomas and some gliomas.10 The epitope for antibody binding has high in vivo expression and has been detected on >90% of melanoma cells in cutaneous lesions,11 with high cell surface specificity for human cutaneous melanoma cells. The expression of mAb 9.2.27 has not yet been investigated in human ocular melanoma. mAb 9.2.27 has also been used extensively in vitro and in vivo as a tumour marker for therapeutic purposes,12–15 and has also been tested in a limited phase I clinical trial.16 Our group has recently chelated the α emitting radioisotope 213Bi to mAb 9.2.27 to form an α immunoconjugate (AIC), which is highly specific and cytotoxic to cutaneous melanoma cells in vitro17 and can completely regress tumour growth in a xenograft mouse model after local injection.18 Preliminary results from our phase I clinical trial also demonstrate that AIC can target cutaneous melanoma cells after local injection (unpublished data). As a first step in the selection of a possible cell surface target antigen for AIC therapy of uveal melanoma, we examined 26 cases of pathologically confirmed uveal melanomas for NG2 immunoreactivity, using mAb 9.2.27 immunohistochemistry.

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.

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.

Maternal infection with rubella in the first trimester is an important cause of congenital cataract. Any injury affecting the foetus following maternal rubella infection in the phase of organogenesis results in congenital defects collectively termed as congenital rubella syndrome (CRS). Although rubella embryopathy is a less common cause for congenital cataract than in the past, it is still seen. The number of cases reduced to one in 1997 after which there were no new cases till 2002. However, there have been two new cases of CRS in 2003. Herein another one in early 2004 is reported. Outbreaks of CRS will continue until the percentage of susceptible individuals is reduced to a minimum through immunization. The majority of rubella cases in Australia are confined to young female immigrants, many coming for marriage. We must continue to immunize children, identify and immunize vaccine failures and susceptible women before they become pregnant, and to screen pregnant women so they can be vaccinated after delivery.

Purpose: The use of adjunctive antimitotics with conventional filtering surgery appears to have decreased the need for Molteno implants in glaucomas with a poor prognosis. We review the current status, results and complications of Molteno implants. Methods: Review of the literature and practical experience from two university departments of ophthalmology. Results: Improvements in the design and surgical techniques for Molteno implants have decreased the incidence of over filtration, but this and other complications continue to pose problems. Increased success rates may be possible if antimitotics are used with Molteno implants. Conclusion: Molteno implants continue to be an option when conventional filtration with or without antimitotics has failed: they continue to be regarded as a last resort for filtration. The use of antimitotics to increase the success rate of Molteno implants is not proven, but merits further investigation.

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

Aniridia and its variants may cause diagnostic difficulty especially when the iris is not wholly or partially absent.1,2 In congenital iris ectropion (also called ectropion uveae), the posterior pigment epithelium of the iris extends onto the anterior iris surface causing darkening around the pupil. We report a man and his infant son with variant aniridia and a mutation in the PAX6 gene, where the major anterior segment finding was iris ectropion. A 30 year old man with bilateral iris ectropion, vision impairment, and nystagmus attributed to optic nerve hypoplasia, and his infant son also with poor vision, were examined. A search of the man’s previous ophthalmic records indicated that at 6 weeks of age he had poor vision, nystagmus, myopia (−6.0 D), and small, anomalous optic discs. Ectropion uveae was noted in the records at age 9 years. A poorly developed foveal reflex was identified in his late teens. A magnetic resonance imaging (MRI) …

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.

We compared a newly available nonabsorbable monofilament ophthalmic suture, 9/0 Novafil (Davis & Geck), with 10/0 nylon (Alcon), currently the most popular suture for closure of corneoscleral sections. Surgery was performed on nine rats and ten rabbits. In each case a 120° corneoscleral section was made in each eye and closed with interrupted sutures of 9/0 Novafil in one eye and 10/0 nylon in the other. We compared their handling qualities during surgery, as well as their effect on postoperative wound inflammation. In addition we examined the suture material from each eye of the rats by scanning electron microscopy (SEM) after 3 months, and we compared the surgically induced astigmatism in the rabbits in the two suture groups. Both suture materials were easy to handle and well tolerated. Nylon sutures from six of the eight rat eyes studied showed SEM evidence of surface disintegration after 3 months, whereas all the Novafil sutures remained intact.

This paper reports all cases of retinoblastoma occurring in Victoria over a 20-year period between 1956 and 1976. It suggests that the natural history of treated retinoblastoma in an Australian community, is characterized by low mortality (8.3%), but significant morbidity. The most common problem in management of unilateral cases was one of diagnosis in contrast to bilateral cases, in which the problem was one of maintaining useful vision whilst at the same time eradicating a life-threatening tumour. Suggestions are made as to how management of retinoblastoma may be further improved, and the need for an active national register is discussed.

A case of optic nerve hypoplasia in the Conradi-Hunermann form of chondrodysplasia punctata is reported. This is the first published report of such a case. The authors suggest that optic nerve hypoplasia in chondrodysplasia punctata may be the significant lesion of the optic nerve rather than atrophy as has been previously reported.

In recent years, the ophthalmic examination of infants has been of increasing interest to both clinicians and vision researchers. Clinicians have documented a greater risk of retinopathy, strabismus and amblyopia in premature infants, especially those of low birthweight. In addition to the external and retinal examination of the infant eye, a number of clinical tests can help the ophthalmologist to detect visual dysfunction through the evaluation of pupillary responses and ocular motility. Recently, the development of objective techniques (optokinetic nystagmus, forced choice preferential looking, and visually evoked potentials) have not only aided in the detection of ophthalmic disorders in infants; they have contributed to useful definitions of "normal" vision at various ages and to the understanding of factors that influence the pre- and post-gestational development of visual function.

A case of buphthalmos and regional giantism in neurofibromatosis with the characteristics of the Francois syndrome is reported. Difficulties of early diagnosis are emphasized. A discussion of the mechanisms which may produce buphthalmos in neurofibromatosis is presented. We propose that buphthalmos in neurofibromatosis may be primarily an expression of regional giantism rather than a consequence of uncontrolled intraocular pressure.

Optic nerve hypoplasia is a developmental anomaly of the retina and optic nerves in which there is a reduction in the number of ganglion cells in the retina and of their centripetal fibers projecting through the optic nerve to the lateral geniculate body. The condition may be unilateral or bilateral and is frequently misdiagnosed as optic atrophy. In about 25% of cases, bilateral optic nerve hypoplasia is associated with a variety of cerebral malformations of which the commonest single disturbance is absence of the septum pellucidum (septo-optic dysplasia). Cerebral malformations and their endocrine accompaniments are also seen, though less frequently, in unilateral hypoplasia. The endocrine disturbances that may accompany optic nerve hypoplasia include growth hormone deficiency, adrenal insufficiency, hypothyroidism, and disturbances of antidiuretic hormone production. Precocious puberty and hypogonadism have also been observed. The prognosis of optic nerve hypoplasia depends upon the severity of the changes in the optic nerves and especially the degree of associated cerebral malformation. The finding of optic nerve hypoplasia should lead to thorough ophthalmologic, neurologic, and endocrinologic evaluation of the patient. ( J Child Neurol 1986;1:181-188)

We studied nine cases of retrobulbar neuritis with confirmed multiple sclerosis and six cases of optic atrophy from other causes. Pattern and focal electroretinograms (macular ERGs) were recorded with high (400 cd/m2) and low (40 cd/m2) intensity stimuli. Contrast sensitivity was also measured with a simple printed test. Luminance was not markedly important. High spatial frequency contrast sensitivity was significantly correlated with pattern ERG amplitude. Pattern and focal ERG amplitude ratio was usually reduced, but the effect was not correlated with contrast sensitivity or large enough to be useful clinically. In optic atrophy the pattern ERG (PERG) was clearly more severely reduced than the focal ERG (FERG). In retrobulbar neuritis both ERGs were equally and more severely reduced even though the visual losses were less. In unilateral cases the PERG increased then decreased after the initial attack, as previously described (Arden et al., 1982). The results suggest that retinal layers beyond the ganglion cells may be affected in retrobulbar neuritis, but proximally generated, pattern-specific ERG components are selectively lost in optic atrophy.

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.

Optic nerve hypoplasia, having once been regarded as exceedingly rare, is now regarded as one of the major causes of visual loss in infancy. Recognition of subtle forms and segmental forms of optic nerve hypoplasia are important in explaining the evaluation of subnormal vision, and have an added significance in the recognised associations of central nervous system anomalies including abnormalities of the endocrine system. These changing clinical perspectives are reviewed, together with a discussion of the pathogenesis of optic nerve hypoplasia in the light of cell death and nerve fibre degeneration seen in normal development of the neural visual system.

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.

Eighteen patients with early maculopathies of various etiologies were tested with pattern and focal electroretinograms (macular ERGs), with high (400 cd/M2) and moderate (40 cd/M2) stimulus intensities and a four-alternative forced choice (4AFC) contrast sensitivity test in addition to intensive clinical examinations. High spatial frequency contrast sensitivity loss on the 4AFC test was the most striking and consistent feature of all cases. The only eyes not outside normal contrast sensitivity limits were three in which diagnosis was uncertain and the patients had not recognized any problem, including two marginal solar burns. Maculopathy also substantially reduced macular ERG amplitudes. Criterion scores on these tests separated patients from normals more effectively than other noninvasive procedures and only missed one eye detected by contrast sensitivity. Latencies were affected but the delays were of no clinical significance in the individual case. Stimulus intensity was not critical. The results indicate that contrast sensitivity testing and macular ERGs are very reliable indices of central visual dysfunction at a stage when visible macular changes are too subtle for confident diagnosis. Contrast sensitivity has appeal because of its reliability, objectivity, simplicity, and noninvasive nature. It is equally applicable to children and adults. Pattern and focal ERGs can establish that the visual deficit has a retinal origin and can provide the most reliable objective confirmation.