Blepharokeratoconjunctivitis in Cockayne syndrome.

Cockayne syndrome is a multisystemic, autosomal recessive disease resulting from abnormalities of DNA repair. Ocular manifestations are common, particularly congenital cataract and retinal dystrophy. This study describes a previously unreported association of blepharokeratoconjunctivitis (BKC) in Cockayne syndrome. The authors conducted a retrospective case review of patients with Cockayne syndrome between 1997 and 2006. The ocular manifestations were documented. All cases were bilaterally aphakic from congenital cataract surgery. Four cases of BKC with resultant corneal changes were identified. Two other cases of BKC without corneal changes were also noted. There were no cases of corneal ulceration or visually significant scarring. These findings are clinically important because many patients with Cockayne syndrome wear contact lenses for the refractive correction of aphakia with a resultant risk of corneal ulceration.

Presumed early-onset sarcoidosis: a case of devastating ocular inflammation in an infant.

Involvement of the orbit and posterior segment of the eye in early-onset sarcoidosis may necessitate prompt and aggressive immunosuppressive treatment to prevent a poor visual outcome.

A 10-year follow up of ocular hypertensive patients within the Bolton Corneal Thickness Study. Can measured factors predict prognostic outcomes?

OBJECTIVE: This is a case note review of a cohort of patients examining the effects of central corneal thickness (CCT), presenting intraocular pressure (IOP), age and gender on the risk of progression of ocular hypertension (OHT) to primary glaucoma (POAG) over a 10-year period. DESIGN: Cohort study with retrospective case note review. PARTICIPANTS AND METHODS: 58 case notes from a cohort at the Bolton Royal Infirmary initially assessed 10 years ago were reviewed again. Presenting CCT, IOP, age and gender were recorded. Development of POAG was assessed by visual field and/or optic disc changes being present. MAIN OUTCOME MEASURES: The effects of CCT, IOP, age and gender on the risk of progression of OHT to POAG were analysed in a multivariate logistic regression model following a preliminary univariate analysis. RESULTS: 50 out of 116 eyes developed primary open angle glaucoma over the 10-year period. Thinner CCT (odds ratio 0.985 associated with each 1mum increase in CCT), higher presenting IOP (odds ratio 1.131 associated with each 1mmHg increase in IOP) and increasing age (odds ratio 1.062 associated with each 1 year increase in age) were found to be associated with progression to POAG. CONCLUSION: Patients with a CCT of 579microm or more, a presenting intraocular pressure of 26mmHg or less and age 75 years or less had a lower risk of developing POAG within this cohort of patients.

Assessment of the biomechanical properties of the cornea with the ocular response analyzer in normal and keratoconic eyes.

PURPOSE: To compare hysteresis, a novel measure of ocular rigidity (viscoelasticity) in normal and keratoconic eyes. METHODS: The study consisted of 207 normal and 93 keratoconic eyes. Eyes were diagnosed as keratoconic based on clinical examination and corneal topography. The hysteresis was measured by the Ocular Response Analyzer (ORA; Reichert Ophthalmic Instruments, Buffalo, NY). The data were recorded by Generation 3 software for the ORA. Central corneal thickness (CCT) was measured with a handheld ultrasonic pachymeter in the midpupillary axis. RESULTS: The mean hysteresis was 10.7 +/- 2.0 (SD) mm Hg (range, 6.1-17.6) in normal eyes compared with 9.6 +/- 2.2 mm Hg (range, 4.7-16.7) in keratoconic eyes. The difference was statistically significant (P < 0.0001, unpaired t-test). Mean CCT in the normal and keratoconic eyes was 545.0 +/- 36.4 microm (range, 471-650) and 491.8 +/- 54.7 microm (range, 341-611), respectively; the difference was significant (P < 0.0001, unpaired t-test). CONCLUSIONS: Hysteresis was significantly higher in normal than in keratoconic eyes. It may be a useful measurement in addition to CCT, when assessing ocular rigidity, and may be of particular importance when trying to correct intraocular measurements for increased or decreased ocular rigidity. Long-term studies of change in hysteresis may provide information on the progression of keratoconus.

Standards in consent for cataract surgery.

Consent forms for cataract surgery performed at Burnley General Hospital (BGH) and Blackburn Royal Infirmary (BRI) from October 4 to December 7, 2004, were prospectively reviewed to ensure that the East Lancashire Hospital's National Health Service (NHS) Trust Policy on consent to treatment and Department of Health (DoH) guidelines were being followed when seeking consent for cataract surgery. A set of 22 criteria derived as standards were formulated from the reference guide published by the DoH and from the East Lancashire trust policy document for consent to treatment. Each consent form was measured against these standards. Cases were randomly selected between BRI and BGH prospectively. All consent forms completed by physicians involved in formulating the standards were excluded. The review showed the NHS Trust Policy and DoH guidelines were largely followed when seeking consent for cataract surgery. However, certain areas were found to be deficient. If a health professional fails to obtain proper consent and the patient suffers harm as a result of treatment, it may be a factor in a claim of negligence against that health professional. Subsequent recommendations may include simple solutions that can be implemented to improve clinical practice when obtaining informed consent.

Sequestered viscoelastic after deep lamellar keratoplasty using viscodissection.

PURPOSE: Deep lamellar keratoplasty (DLKP) is an intricate procedure that preserves the host's endothelium, thus eliminating the possibility of endothelial graft rejection and potentially offering great benefits over penetrating keratoplasty. DLKP may be performed by a variety of techniques including viscodelamination, in which the stroma is separated from Descemet's membrane using viscoelastic. METHODS: Here we present an operative complication of this technique, which was not initially recognized, that caused significant morbidity to our patient and eventually led to the eye requiring a full thickness regraft. We also attempt to reproduce the lesion using nonviable cadaver corneas and illustrate histologically the nature of the corneal stroma and its relationship to Descemet's membrane following viscoelastic delamination.

Treatment of dry eye: an analysis of the British Sjögren's syndrome association comparing substitute tear viscosity and subjective efficacy.

PURPOSE: This study aims to address the lack of independent subjective efficacy data on artificial tear substitutes in the treatment of dry eye due to the anecdotal association of 'thicker' products being more effective. METHODS: This is an independent study of the subjective use and efficacy of topical treatments used by members of the British Sjögren's Syndrome Association (BSSA) related to product viscosity. 2000 members of the BSSA were sent a questionnaire regarding their physical condition and the use of artificial tear substitutes. Viscosity analysis was performed on the most popular preparations. Statistical comparison is made between subjective efficacies related to substitute tear viscosity. RESULTS: 1088 patients responded giving information regarding their condition together with the subjective use and efficacy data of artificial tear substitutes. Visco-analysis was performed on the most popular preparations; these had more than 50 patients using them. In terms of subjective benefits related to viscosity for 'frequency' and 'duration' the data suggests a general trend toward viscous preparations being instilled less frequently and lasting longer; however this was not shown to be significantly correlated and some interesting comparisons are reported. CONCLUSIONS: The results confirm high levels of ocular lubricant use in the BSSA population. Our data investigates the often-anecdotal evidence that thicker preparations are more effective. However, we did not find this correlation to be statistically significant suggesting further study into factors related to subjective product efficacy. These results lay foundations for the development of future products in the treatment of severe dry eye.

Clinical review of periorbital capillary hemangioma of infancy.

PURPOSE: To explore the role of intralesional steroid injections (ILSI) and oral steroids in the management of periocular hemangioma of infancy (HOI). METHODS: In this retrospective study, treatment options studied were observation, ILSI, and oral steroids. All children received adjunctive amblyopia treatment if required. The main indications for treatment were cosmetic, worsening astigmatism, and visual axis obscuration. Success was defined as complete HOI regression before the age of 5 years (cosmetic group), reduction of astigmatism of at least 1 diopter cylinder (DC) (astigmatism group), or no evidence of amblyopia at the last follow-up (visual axis obscuration group). RESULTS: Twenty-four of 41 children (58.5%) had amblyopia at presentation. Eighteen children formed the observation group, 17 children received ILSI, and 6 children received oral steroids. Successful outcome was achieved in all except 2 patients in the cosmetic group and 6 of 7 in the visual axis obscuration group. Mean astigmatic correction of all cases was 1.65 ± 1.34 DC before treatment and 0.91 ± 1.17 DC after treatment, the change being statistically significant (P < .001). CONCLUSION: Observation appears to be a highly effective strategy if coupled with amblyopia therapy, especially for mild cases. Intralesional and oral steroids appear to be equally effective for lesions requiring treatment, but their exact role cannot be clearly determined in the presence of a spontaneously resolving lesion.

Diurnal variation of ocular hysteresis in normal subjects: relevance in clinical context.

BACKGROUND: This study was conducted to assess the diurnal variation in ocular hysteresis, as measured by the Ocular Response Analyser to establish a relationship between diurnal hysteresis variation and diurnal intraocular pressure (IOP) variation. METHODS: Forty-two normal eyes of 21 colleagues and staff in a teaching hospital in Birmingham, UK, were recruited. The IOP and hysteresis were measured by the Ocular Response Analyser. The central corneal thickness (CCT) was measured using a hand-held ultrasonic pachymeter in the mid-pupillary axis. RESULTS: The mean ocular hysteresis at 8 am was 12.7 +/- 2.3 mmHg, at 11 am was 12.2 +/- 2.0 mmHg, at 2 PM was 12.7 +/- 2.1 mmHg and at 5 PM was 12.7 +/- 1.7 mmHg; the difference between the values at any time of measurement was not statistically significant (P > 0.9, repeated measures). IOP as measured by non-contact tonometry was 18.4 +/- 2.8 mmHg, 17.9 +/- 3.3 mmHg, 16.9 +/- 3.1 mmHg and 16.8 +/- 3.2 mmHg, respectively, for the same time period; the difference between the values in the morning and afternoon was statistically significant (P < 0.0001, repeated measures). The CCT was 548.8 +/- 29.5 microm, 547.0 +/- 31.4 microm, 548.2 +/- 29.6 microm and 548.6 +/- 29.4 microm, respectively; the difference between the values was not statistically significant at any time points. Multiple regression analysis showed the relationship between IOP and hysteresis was not statistically significant (P = 0.9). CONCLUSION: The ocular hysteresis reading was almost constant throughout the day, whereas the IOP readings showed highest values in the morning with a reducing trend being lowest in the afternoon. The CCT values were almost stable throughout the day. IOP appears to vary independently of a variation in hysteresis or CCT.