Electroretinogram assessment of children with sensorineural hearing loss: implications for screening.

Background: The guidelines of the National Deaf Children's Society recommend that children with sensorineural hearing loss (SNHL) be routinely screened for ophthalmological problems and suggest electroretinography (ERG) to exclude Usher syndrome. The present study reports the nature and prevalence of abnormal ERG findings in a cohort of children with SNHL undergoing ERG with the aim of identifying risk factors for the diagnosis of Usher syndrome.

Methods: The medical records of children (<18 years of age) with SNHL referred for ERG at Moorfields Eye Hospital, London, between January 2009 and December 2011 were retrospectively reviewed.

Cost-effectiveness analysis of clinic-based chloral hydrate sedation versus general anaesthesia for paediatric ophthalmological procedures.

Background/aims: The inability of some children to tolerate detailed eye examinations often necessitates general anaesthesia (GA). The objective was to assess the incremental cost effectiveness of paediatric eye examinations carried out in an outpatient sedation unit compared with GA.

Methods: An episode of care cost-effectiveness analysis was conducted from a societal perspective.

Survey of policy for MRSA screening in English cataract surgical units and changes to practice after updated National guidelines.

Background: National guidelines on MRSA (methicillin-resistant Staphylococcus aureus) screening policy in England have changed on a number of occasions, but there is limited data on its influence at a local level. The aim of this study was to determine if changes in National policy influenced preoperative screening of cataract patients for MRSA.

Methods: A structured telephone survey was conducted on all 133 ophthalmology units in England in 2004 and again in 2007 for the initial responders, after a change in national policy.

Utilisation of an outpatient sedation unit in paediatric ophthalmology: safety and effectiveness of chloral hydrate in 1509 sedation episodes.

Objective: To report the largest study on the safety and effectiveness of sedation in paediatric ophthalmology in a nurse-led outpatient sedation unit.

Design: Retrospective cohort study reviewing all children who underwent sedation from January 2006 to December 2010. Patients were sedated with 80 mg/kg of chloral hydrate (CH) given orally with top up dose given at half dose as required.

Leigh's disease associated with a dorsal midbrain syndrome.

Leigh's disease is a rare progressive neurodegenerative condition caused by a mitochondrial cytopathy. The authors present the case of a 9-year-old girl with dorsal midbrain syndrome causing convergence retraction nystagmus. Magnetic resonance imaging, skin biopsy, and genetic testing confirmed the cause to be Leigh's disease due to two SURF1 mutations.

β-Defensin 1 haplotype associated with postoperative endophthalmitis.

Purpose: Endophthalmitis is a rare but sight-threatening complication of intraocular surgery. β-Defensins are antimicrobial peptides that appear to be important components of the ocular immune response. We propose that variation in defensin genes may alter susceptibility to endophthalmitis.

Pseudomonas aeruginosa eyelid necrosis associated with Felty syndrome.

We describe a case of left upper eyelid necrosis due to Pseudomonas aeruginosa in a patient with Felty syndrome. This is a rare but potentially serious condition. In our patient, medical management and reconstructive surgery achieved a good outcome.