A retrospective analysis of medical records of all cases of microbiologically confirmed microsporidial keratoconjunctivitis, who presented between March 2007 and October 2010, was done. In a single-centre, institutional setting, 278 eyes of 277 apparently healthy patients were analysed.

The mean age was 36±14 years (range 6–80). The mean duration of symptoms was 7.7±6.2 days (range 1–60). Keratic precipitates were present in 20.1% patients. A superficial scar was present in 39.2% patients. Majority (26.6%) of the patients reported in the month of August. Microscopic examination of corneal scraping, using potassium hydroxide with calcofluor white and Gram stain, demonstrated microsporidial spores in 98.9% and 89.7% cases, respectively. Patients received either topical 0.02% polyhexamethylene biguanide or lubricants. The mean time for resolution was 6.0±2.9 days (range 2–18). Final visual acuity was ≥20/30 in 75.1% cases.

Microsporidial keratoconjunctivitis is common in India. It is seasonal, can occur in healthy individuals and can be diagnosed using simple microbiological methods. Treatment outcome is generally satisfactory.

All patients with Mooren's ulcer aged <18 years presenting at a single centre from 1987 to 2010 were enrolled. Epidemiological features, symptomatology, clinical signs, disease severity, investigations, treatment, outcomes and complications were studied. Main outcome measures were anatomical and functional outcomes, disease activity and complications.

14 eyes of 11 children (seven males and four females with an average age of 12.45±2.25 years at presentation) with Mooren's ulcer were included. Eight cases were unilateral and three bilateral. Symptoms at presentation were more severe than in adults. Trauma was the commonest predisposing factor. Eight eyes had severe corneal involvement. Medical management included intensive topical steroid therapy, oral steroid therapy and immunosuppressant agents. Surgical therapy included tissue adhesive and bandage contact lens application, amniotic membrane transplantation, optical penetrating keratoplasty and limbal stem cell transplantation and was performed in most eyes as part of primary management or later during the disease course. Patients were followed up for a mean of 69.1 weeks. Ten eyes healed successfully and one developed descemetocele. Three eyes developed secondary infections, one of which ultimately became phthisical. In most eyes, final vision either remained stable or was better than at presentation.

Our data suggest demographic and clinical features of Mooren's ulcer in children differ from those in adults. Good anatomical results and stable visual results can be achieved with appropriate medical and surgical therapies. Systemic steroids and immunosuppression should be used judiciously with close monitoring.

599 people aged 16–85 years in the cross-sectional Norfolk Island Eye Study were included in the validation study. 196 UVAF individual photographs (49 people) and 60 UVAF photographs (15 people) of Norfolk Island Eye Study participants were used for intra- and inter-observer reliability assessment, respectively. Conjunctival UVAF was measured using UV photography. UVAF area was calculated using computerised methods by one grader on two occasions (intra-observer analysis) or two graders (inter-observer analysis). Outdoor activity category, during summer and winter separately, was determined with a UV questionnaire. Total UVAF equalled the area measured in four conjunctival areas (nasal/temporal conjunctiva of right and left eyes).

Intra-observer (_c=0.988, 95% CI 0.967 to 0.996, p<0.001), and inter-observer concordance correlation coefficients (_c=0.924, 95% CI 0.870 to 0.956, p<0.001) of total UVAF exceeded 0.900. When grouped according to 10 mm² total UVAF increments, intra- and inter-observer reliability was very good (=0.81) and good (=0.71), respectively. Increasing time outdoors was strongly with increasing total UVAF in summer and winter (p_trend <0.001).

Intra- and inter-observer reliability of conjunctival UVAF is high. In this population, UVAF correlates strongly with the authors' survey-based assessment of time spent outdoors.

In this prospective, cross-sectional and comparative study, 27 eyes of 27 healthy individuals (control group) and 27 eyes of 27 patients with nanophthalmos (study group) were enrolled. Corneal hysteresis (CH), corneal resistance factor (CRF), corneal compensated intraocular pressure (IOPcc) and Goldmann correlated intraocular pressure (IOPg) were recorded for the right eye of each participant using Reichert Ocular Response Analyser measurements. Also, all participants in this study underwent a standardised ocular examination including IOP measurement with Goldmann applanation tonometry (IOP_GAT), central corneal thickness and axial length (AL) assessments.

Mean CH in the nanophthalmic eyes and in the control eyes were 13.3±2.4 mm Hg and 11.6±1.7 mm Hg, respectively (p=0.003); mean CRF values in the nanophthalmic and the control eyes were 13.2±1.8 mm Hg and 11.4±1.9 mm Hg, respectively (p=0.001). Mean IOP_GAT was 15.2±3.3 mm Hg in the nanophthalmic eyes and 13.4±2.7 mm Hg in the control group (p=0.031); mean IOPg values for the nanophthalmic and the control groups were 17.1±5.3 mm Hg and 14.7±3.5 mm Hg, respectively (p=0.042). Mean IOPcc values in the nanophthalmic and the control group were 13.6±6.1 mm Hg and 14.8±3.2 mm Hg, respectively (p=0.365).

The CH, CRF, IOPg and IOP_GAT were significantly higher in the nanophthalmic eyes, whereas no significant differences in IOPcc were observed. These findings may be taken into account when measuring IOP values in patients with nanophthalmos.

The population-based Beijing Eye Study, which included 4439 subjects with an age of 40+ years in the year 2001, was repeated in 2006. Optic disc photographs of the baseline examination versus follow-up examination were compared.

Out of 111 open-angle glaucoma patients examined in 2001, 77 (69%) subjects participated in the follow-up examination and 16 (21%) eyes showed glaucoma progression. Glaucoma progression was associated with smaller rim area (p=0.001), larger β zone (p=0.037), higher frequency of β zone increase during follow-up (p=0.01), higher prevalence of disc haemorrhages (p=0.01) and higher single intraocular pressure (p=0.04). In multiple regression analysis, only smaller rim area remained significantly associated with glaucoma progression. Glaucoma progression was not associated with optic disc size (p=0.70), mean blood pressure (p=0.43), ocular perfusion pressure (p=0.96), retinal vessel diameter and retinal microvascular abnormalities (all p>0.10), prevalence of diabetes mellitus (p=0.75) and arterial hypertension (p=0.26), prevalence of dyslipidaemia (p=0.28), refractive error (p=0.69), and central corneal thickness (p=0.97).

In a population-based setting in adult Chinese, factors showing an association with open-angle glaucoma progression were an advanced stage of the disease (ie, small rim), presence of disc haemorrhages, larger area of β zone and higher frequency of β zone increase, and higher intraocular pressure. Glaucoma progression was not significantly associated with optic disc size, central corneal thickness, retinal vessel diameter and retinal microvascular abnormalities, and systemic diseases such as diabetes mellitus and arterial hypertension.

One hundred consecutive patients certified SI/SSI from the Glaucoma Service at Moorfields Eye Hospital, London, from January 2007 were identified from the England and Wales certification of visual impairment database. Clinical and demographic data were collected from hospital case records.

The median (IQR) age of patients at presentation was 66.3 (55.6 to 75.3) years; median (IQR) interval to certification was 62.2 (22.5 to 129.3) months. Fifty-seven patients presented with bilateral SSI (median (IQR) age 70.4 (59.0 to 76.9) years); interval to certification was 35.4 (5.6 to 78.1) months. Seventeen patients presented with a bilateral SI (median (IQR) age 62.1 (58.7 to 68.4) years; median (IQR) interval to certification: 137.4 (64.4 to 190.4) months). Twenty-eight patients showed disease progression while under National Health Service hospital eye service care, five of whom had no certifiable visual impairment in either eye at presentation. This was attributed to inadequate intraocular pressure control; five of these patients (18%) were deemed poorly compliant to topical hypotensive medication.

Over 80% patients on the certification of visual impairment register from Moorfields Eye Hospital with glaucoma as the primary cause had a significant visual disability at presentation, with almost two-thirds of patients presenting bilaterally ‘blind’. There appear to be delays to certification. Despite being under the hospital eye service, a number of glaucoma patients still progress to certifiable visual impairment.

Anxiety and depression in 408 patients with glaucoma were evaluated using the hospital anxiety and depression scale (HADS) questionnaire, which consists of two subscales, representing HADS-anxiety (HADS-A) and HADS-depression (HADS-D). To identify the risk factors for anxiety and depression, the stepwise and multiple linear regression analyses were carried out with the HADS-A and HADS-D subscores as dependent variables and demographic and clinical features as independent variables.

A stepwise linear regression analysis revealed the significantly related factors to be age for HADS-A (β=–0.046, p=0.0007) and HADS-D (β=0.035, p=0.011) and the mean deviation of the Humphrey Visual Field Analyzer 30-2 (HFA30-2) in the better eye for HADS-D (β=–0.095, p=0.0026). Based on multiple linear regression analyses, significant relationships were confirmed between age and the HADS-A subscore (β=–0.046, p=0.0008). Significant relationships were also confirmed between age (β=0.037, p=0.0077) or the mean deviation of HFA30-2 in the better eye (β=–0.094, p=0.0036) and the HADS-D subscore.

A younger age was thus found to be a risk factor for anxiety, while an older age and increasing glaucoma severity were risk factors for depression in patients with glaucoma.

Prospective non-interventional, multicentre, observational study on 134 patients, naive or on previous treatment with another prostaglandin analogue. In each visit (V1 baseline visit, V2 at 1 month and V3 at 3 months), patients evaluated five ocular surface symptoms as: absent, mild, moderate and severe. Parallelly, the ophthalmologist assessed the tear break-up time, keratitis, conjunctival hyperaemia, blepharitis, Schirmer test and tear meniscus.

Patients with OHT (n=71, 53%) experienced a statistically significant improvement of all symptoms: stinging/burning/irritation, itching, foreign body sensation, tearing and dryness sensation at V3, while glaucoma patients improved all symptoms at both V2 and V3. In patients with OHT, all signs except Schirmer test improved and the decrease in hyperaemia was statistically significant. Eyes with glaucoma ameliorated the keratitis, hyperaemia and tear meniscus at V2 and V3 and the break-up time and blepharitis at V3. In the subset of patients with previous treatment (n=79, 58.9%), patients with OHT presented significant improvement of hyperaemia, yet the rest of signs did not decrease significantly or remained unchanged, while in patients with glaucoma all signs improved significantly at both visits. The intraocular pressure (IOP) drop in naive eyes was 22.2% (24.7–19.7 mm Hg) in OHT and 29.5% (33.7–25.3 mm Hg) in glaucoma eyes. In previously treated eyes, no statistically significant change in IOP was found.

Preservative-free tafluprost is a well tolerated hypotensive agent that can be used in eyes with surface problems and in naive eyes.

Two hundred and three glaucomatous eyes and 88 normal eyes were imaged by Cirrus, RTVue and 3D OCT. The average and the four quadrant RNFL thicknesses were evaluated. Agreement among RNFL measurements was evaluated using Bland–Altman analysis and linear regression analysis. The percentage of each quadrant in the average RNFL thickness value was compared among the three instruments.

Cirrus showed significantly smaller thickness values than RTVue (difference=8.8 μm, p<0.0001) and 3D OCT (difference=8.1 μm, p<0.0001). Although RNFL measurements among the instruments were highly correlated, the Bland–Altman analysis revealed proportional biases for most of the pair-wise agreements. Additionally, 3D OCT showed strong proportional biases with RTVue and 3D OCT. RTVue had a smaller occupied proportion of nasal quadrants (30.2%) and a larger proportion of inferior quadrants (32.4%) compared with Cirrus and 3D OCT.

RNFL measurements among the instruments were well correlated but had different values for thickness. The measurement circle of RTVue might be more superior-temporally located compared with the other instruments. Differences in the measurement protocols might be affected by the disagreements. These instruments should not be used interchangeably.

65 patients who had undergone over 25 injections of botulinum toxin A for long-term control of their deviation were identified and asked to fill in and return the Adult Strabismus questionnaire (AS-20) to assess their QoL.

46 questionnaires were available for analysis. The mean AS-20 score in our patients compared favourably with that reported for normal controls and was much higher than that reported for patients with strabismus.

Long-term injections with botulinum toxin A is a good treatment for maintaining ocular alignment if squint surgery is not indicated and those patients receiving treatment score near the level of normal controls in QoL terms.

31 patients with OPMD satisfied the following inclusion criteria: LF ≥10 mm; no previous eyelid surgery; and pre-operative measurements, silcone frontalis suspension and post-operative measurements performed by a single individual. The following data were collected: age; gender; pre-operative margin reflex distance (MRD), palpebral fissure height (PF), and LF; post-operative MRD, PF and lagophthalmos; follow-up; and complications.

Mean age at surgery was 61.5±5.8 years. Pre-operative measurements for MRD, PF and LF were –0.05±0.82 mm (OD), –0.13±0.91 mm (OS); 5.2±1.2 mm (OD), 5.2±1.3 mm (OS); 11.6±1.3 mm (OD), and 11.7±1.3 mm (OS), respectively. Post-operative measurements for MRD and PF were 2.23±0.97 mm (OD), 2.10±1.09 mm (OS), 7.9±1.4 mm (OD), and 7.7±1.6 mm (OS), respectively (all p<0.0001). The mean follow-up period was 22.8±22.4 months. There was no sling (infection or extrusion) or ophthalmic (significant corneal compromise) complication after the surgery. Six patients (19%) underwent early (within 3 months) tightening of their slings for under correction. Three patients (10%) underwent late (>39 months) tightening of their frontalis slings for recurrent ptosis after their initial surgery.

Primary bilateral silicone frontalis suspension for good LF ptosis secondary to OPMD appears to be an effective, safe treatment which gives symmetrical upper lid elevation. Early sling adjustment may be required to attain optimal eyelid height and late tightening for expectant loosening of the sling is safe and effective.

The Singapore Malay Eye Study is a population-based study of 3280 Singapore Malay patients aged 40–80 years, of which 3225 had data available for inclusion. Cataracts were graded from digital lens photographs according to the Wisconsin scale. Study subjects were categorised as having: bilateral cataract surgery performed; unilateral cataract surgery performed with minimal cataract in the fellow eye; unilateral cataract surgery performed with significant cataract in the fellow eye; and bilateral cataract. Visual functioning was assessed using the modified VF-9 scale culturally adapted for Singaporean individuals, validated by Rasch analysis. The overall Rasch-modified vision-specific functioning score was compared across the four groups after adjusting for confounders such as age, gender, ocular and systemic comorbidities.

Persons with bilateral cataract had poorer visual functioning than those who had bilateral cataract surgery (mean visual functioning scores 3.38 vs 3.11, respectively, p=0.029). When compared with bilateral surgery, visual functioning improvements among patients with unilateral cataract surgery depended on the status of the fellow eye, with improvements only seen if the fellow eye had significant cataract (mean visual functioning scores 2.81 vs 3.25, p=0.019) or poor visual acuity (mean visual functioning scores 2.78 vs 3.25, p=0.018) after adjusting for confounders.

Bilateral cataract surgery was associated with greater visual functioning over unilateral cataract surgery when the fellow eye had a significant cataract or poor presenting visual acuity, supporting the current practice of second eye surgery depending on the fellow eye's cataract status and visual acuity.

Retrospective study of the clinical data of 62 JIA patients with uveitis. The main outcome measurements consisted of uveitis activity measured as mean cell grade in the anterior chamber, topical and systemic medication and ocular complications related to disease activity. All data were scored and evaluated per year of age.

Uveitis activity took a biphasic course with a quiet phase around the age of 9 years and showed increased activity during early teenage years. The biphasic course was significantly related to age (p=0.048) but not to uveitis duration. More patients were treated with systemic immunosuppressive medication in estimated puberty years (63% in boys, 53% in girls) compared with prepuberty years (46% and 28%, respectively), although the difference was only significant in girls (p<0.001). The presence of cystoid macular oedema and papillitis was not significantly related to estimated puberty, but the development of an hypotonous eye was more frequently observed in boys in estimated puberty years (p=0.026).

JIA-associated uveitis appears to take a biphasic course with the second phase of activity during early teenage years and more treatment with systemic immunosuppressive medication occurred during estimated puberty compared with prepuberty years.

Small intestinal biopsy reports performed between July 1969 and February 2008 were collected from all (n=28) pathology departments in Sweden. From these reports, 29 044 patients with CD (equals villous atrophy, Marsh 3) were identified. Uveitis was defined according to relevant International Classification of Disease codes in the Swedish National Patient Register. Cox regression was used to estimate HR for uveitis in individuals with CD compared with those in reference individuals matched for age, sex, county and calendar year.

During follow-up, 148 patients with CD developed uveitis (expected count 112), corresponding to a HR of 1.32 (95% CI 1.10 to 1.58). The absolute risk of uveitis was 50/100 000 person-years in CD. The risk estimate did not change more than marginally when adjusted for type 1 diabetes, rheumatoid arthritis and autoimmune thyroid disease (HR 1.30; 95% CI 1.08 to 1.56). The risk of uveitis remained significantly increased even 5 years after CD diagnosis (HR 1.31; 95% CI 1.04 to 1.64).

A moderately increased risk of uveitis was found in patients with biopsy-verified CD. CD might be considered in patients with uveitis of unknown aetiology.

Patient population and setting: A retrospective case series of patients with acute endophthalmitis following either cataract surgery or IVI presenting to a tertiary referral centre—Sydney Eye Hospital—between 2007 and 2010. Main outcome measures: (1) identification of the causative organism; (2) time to presentation; (3) odds of improvement in visual acuity (VA) following treatment; (4) odds of final VA of counting fingers (CF) or less and (5) odds of enucleation.

Of the 101 patients in our study, 48 had preceding cataract surgery and 53 had preceding IVI. There was an increased incidence of Streptococcus spp. endophthalmitis in post-IVI cases (24.53% vs 6.25%; OR 5.85; p=0.022). Endophthalmitis following IVI had increased likelihood of a final VA of CF or less (OR=6.0; p<0.01), decreased likelihood of any improvement in acuity following treatment (OR=0.13; p<0.01) and an increased likelihood of presenting within a week of the procedure (OR=3.93; p<0.01). Endophthalmitis caused by Streptococcus spp. was associated with increased likelihood of a final VA of CF or less (OR=10.2; p<0.01), decreased likelihood of any improvement in acuity following treatment (OR=0.06; p<0.01) and increased likelihood of enucleation (OR=17.11; p<0.01).

Endophthalmitis following IVI is associated with an increased incidence of Streptococcus spp. infection, earlier presentation and poorer visual outcomes when compared with endophthalmitis following cataract surgery.

15 eyes of 15 patients with iERM who underwent vitrectomy with indocyanine green-assisted membrane peeling were included in this study. The authors analysed the intraoperative fundus images and preoperative Cirrus HD-OCT to detect the shape and the size of the iERM as well as the shape and the size of each thickness-indicating colour (white, red, orange and yellow) on the RTM, respectively. The correlation of areas and morphologic characteristics between both groups was explored.

Analysis of iERM morphologic characteristics (shape) showed a similarity between the iERM contour and the corresponding RTM in 13 cases (86.6%). Furthermore, retinal folds were found in six iERMs and in their corresponding RTMs. Analysis of iERM size (area) revealed a positive correlation between the iERM area and each studied coloured area in RTM. The most significant correlation was between iERM and the red area (440–480 μm; r=0.87, p<0.0001).

The iERM-related retinal folds are clearly distinguishable on the HD-OCT. The red area in RTM representing the 440–480 μm retinal thickness can be a reliable predictor of the extent and the shape of the iERM.

Analysis of the Oxford Record Linkage Study (ORLS), 1963–1998, and English linked hospital episode statistics (LHES), 1999–2010; calculation of rate ratios of eye disease in a hypertension cohort and a diabetes cohort, compared with a reference cohort as control.

Risk of cataract following hypertension was marginally elevated (rate ratio ORLS 1.15, 95% CI 1.00 to 1.31; LHES 1.06, 1.01 to 1.10), as was risk of glaucoma (LHES 1.07, 1.00 to 1.14) and age-related macular degeneration (AMD) (LHES 1.14, 1.02 to 1.27). Risk of retinal vein or artery occlusion was elevated three- to fivefold in both populations. Risk of retinal detachment was elevated in LHES at 1.52 (1.43 to 1.73). Risk of cataract in diabetes was high in ORLS and LHES at, respectively, 2.95 (2.75 to 3.16) and 2.30 (2.24 to 2.35), as was risk of glaucoma: 2.47 (2.14 to 2.84) and 2.23 (2.15 to 2.30). Risks were high for AMD (10.3, 8.1 to 13.1, and 3.46, 3.35 to 3.58) and retinal detachment (3.41, 2.71 to 4.25, and 7.96, 7.63 to 8.30), and very high for retinal vein and artery occlusion.

With the exception of retinal vascular occlusion, elevations of risk of the ophthalmic diseases studied in hypertension were modest. By contrast, there were significant and substantial increases of risk for each eye disease in people with diabetes.

Inclusion criteria were patients presenting with retinoblastoma at <3 months of age or <6.0 kg in weight, with large tumours not amenable to local laser treatment, cryotherapy or plaque brachytherapy. Patients received intravenous carboplatin 18.7 mg/kg every 3–5 weeks, contingent on recovery of blood counts, until they had grown sufficiently to receive intra-arterial chemotherapy. ERG was performed during examination under anaesthesia at monthly intervals, using contact lens electrodes and a hand-held ganzfeld stimulator. 30-Hertz flicker responses are reported.

Four patients were treated for bilateral retinoblastoma. All eyes responded well to systemic chemotherapy. 30-Hertz flicker ERGs improved during treatment in all eyes, significantly in six of eight eyes, and at least in one eye of each patient.

Effective systemic chemotherapy for retinoblastoma in children who are too small for intra-arterial chemotherapy is compatible with significant increases in ERG amplitudes, even in eyes presenting with extinguished ERGs. ERG signals may increase independent of resolution of retinal detachment.

102 periocular injections of 2 cc (10 mg of carboplatin/1 cc) were given every 4–6 weeks in 33 eyes of 29 patients. Patient's data were reviewed retrospectively.

Thirty-three eyes were followed for 7–148 months following initiation of periocular injections, and 13 (39%) have avoided enucleation. There were two cases of second malignancy resulting in one death and one survivor, two survivors of metastatic disease, and two survivors of orbital recurrence. Twenty eyes were enucleated for disease progression at a mean time of 15 months postinitiation of periocular carboplatin (POC). The Kaplan–Meier estimate of eye survival at 36 months is 36%. Eleven of the 13 salvaged eyes received concurrent treatment with chemotherapy (n=4, 30%), external beam radiation and chemotherapy in (n=6, 46%), or brachytherapy (n=1, 8%). Two of the salvaged eyes (16%) were treated with POC alone. Orbital swelling occurred in 14/33 (42%) eyes. There were no symptomatic motility disorders. One severe toxicity reaction resulted in acute loss of vision, down to light perception, which failed to recover and one eye had progression of optic nerve pallor.

This paper demonstrates that POC has limited short-term and long-term systemic toxicity and most of the ocular complications were acute, not delayed. Only two select cases showed long-term complete responses to POC alone and the data do not support the use as monotherapy, although where it was used in combination with other modalities, 39% of the eyes were saved.

Medical notes of the 525 patients of the Jules-Gonin Eye Hospital Retinoblastoma Clinic between 1964 and 2008 were reviewed and the charts of 36 patients with retinocytomas and/or phthisis bulbi were selected.

The proportion of patients with retinocytomas and/or phthisis bulbi was 3.2%. The mean age at diagnosis was 28.7±17 years. Five tumours presented a cystic pattern (5.8%). Evidence of aggressive exophytic disease prior to spontaneous regression was documented in two eyes, and of invasive endophytic disease (regressed vitreous seeding or internal limiting membrane disruption) in three eyes. Twenty patients were followed with a mean follow-up of 44±60 months. Tumour growth was observed in 16% cases, benign cystic enlargement in 4% and malignant transformation in 12%.

This large study of retinocytomas substantially expands the published features of retinocytoma by describing the cystic nature of some retinocytomas as well as clinical characteristics of the endophytic and exophytic preregression growth patterns. The authors report two different patterns of reactivation: benign cystic enlargement and malignant transformation with or without cystic growth. Higher than previously reported frequency of growth and possible life-threatening complications impose close lifetime follow-up of retinocytoma patients.

Case series of Saudi children with previously documented homozygous mutations in COL18A1 or ADAMTS18.

All eight children (4–15 years old; five families) had smooth (cryptless) irides, high myopia (–10 to –20 dioptres) and distinctive vitreo-retinal degeneration consisting of diffuse very severe retinal pigment epithelium atrophic changes with prominent choroidal vessel show, macular atrophic lesions with or without a ‘punched out’ appearance and white fibrillar vitreous condensations. In two probands and a sibling, this distinctive retinal appearance was the basis for initial clinical diagnosis. Six children had temporal ectopia lentis and four had posterior perinuclear lens opacity. Additional features included developmental delay (two), epilepsy (one) and heterotopic grey matter in the lateral ventricles (one). Four children had no clinically discernible occipital defect.

Taken together, smooth iridies, ectopia lentis and characteristic vitreo-retinal degeneration seem pathognomonic. Although it is a defining feature of the syndrome, clinically discernible occipital defect is not a sine qua non for the diagnosis. Ophthalmologists are uniquely able to diagnose Knobloch syndrome.

The systematic reviews and meta-analysis in ophthalmology published in peer-reviewed journals were retrieved. The distribution of systematic reviews and meta-analyses in various ophthalmic subspecialties, type of study and country of origin were determined.

A total of 533 records were identified as systematic reviews and meta-analysis in ophthalmology. Overall, retina and glaucoma were the two major subspecialties accounting for 35% and 21% of the published systematic reviews and meta-analyses, respectively. The major topics published in retina were age-related macular degeneration (37%), tumours (14%), and diabetic retinopathy (12%). More than half (56%) the systematic reviews and meta-analyses were interventional. The author affiliations of these studies were largely from the USA (30%) and the UK (22%). About 60% of the systematic reviews and meta-analyses were published in ophthalmology journals, followed by the Cochrane Library (15.75%) and other non-ophthalmic journals (25.14%), respectively. The number of publications increased from 3 per year in 1994 to almost 100 per year in 2010.

The number of published systematic reviews and meta-analyses has been increasing progressively over the past few years. Retina and glaucoma are the two most commonly published topics. Non-ophthalmology journals form a sizeable proportion of avenues for ophthalmic publications.