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Papers by Stephanie West

Research paper thumbnail of Use of MultiColor imaging in the assessment of suspected papilledema in 20 consecutive children

Journal of American Association for Pediatric Ophthalmology and Strabismus, 2016

PURPOSE To investigate the use of MultiColor Scanning Laser Imaging as an adjunct to traditional ... more PURPOSE To investigate the use of MultiColor Scanning Laser Imaging as an adjunct to traditional imaging modalities in the assessment of children with suspected disk swelling. METHODS In this study of 20 consecutive children with suspected papilledema, MultiColor imaging was performed on all patients in addition to fundus examination, fundus autofluorescence, disk spectral domain optical coherence tomography (SD-OCT), and disk retinal nerve fiber layer thickness (RNFL) measurement. Diagnoses were determined in all 20 patients, and all images were evaluated by a medical retina specialist. RESULTS Of the 20 cases (average age 11 years [range, 5-16 years]; 10 males), papilledema was confirmed in 11 (55%) and pseudopapilledema diagnosed in 9 (45%). Of pseudopapilledema cases, there were 4 cases of optic disk drusen, 2 hypermetropic disks, 2 "crowded disks," and 1 anomalous disk. In patients with true papilledema, on the combined MultiColor image, a green shift in the form of an elevated green ring was seen consistently. This ring was also hyperreflective on the blue and green images and surrounded a central "shadow," which was seen best on near infrared reflectance (NIR). The disk margins and vasculature were obscured on the combined, green, blue, and NIR images. These changes were not present in the pseudopapilledema cases. CONCLUSIONS MultiColor imaging of disks with papilledema shows characteristic changes that may prove useful in differentiating true papilledema from pseudopapilledema. It is therefore a useful adjunct to traditional disk imaging modalities in assessing children with suspected disk swelling.

Research paper thumbnail of Amblyopia

BMJ clinical evidence, Jan 30, 2011

Amblyopia is commonly associated with squint (strabismus) or refractive errors resulting in diffe... more Amblyopia is commonly associated with squint (strabismus) or refractive errors resulting in different visual inputs to each eye during the sensitive period of visual development (<7-8 years of age). The cumulative incidence is estimated at 2% to 4% in children aged up to 15 years. We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of interventions to detect amblyopia early? What are the effects of medical treatments for amblyopia? We searched: Medline, Embase, The Cochrane Library, and other important databases up to May 2010 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations, such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). We found 33 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We perf...

Research paper thumbnail of Multicolor imaging in the assessment and diagnosis of optic disc swelling

Investigative Ophthalmology & Visual Science, 2015

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

British Journal of Ophthalmology, 2015

Research paper thumbnail of Using A Crossover Study Design For Early Hospital-Based Health Technology Assessment: The Cost-Effectiveness Of Clinic-Based Chloral Hydrate Sedation Versus General Anaesthesia For Paediatric Ophthalmological Procedures

Value in Health, 2014

Objectives: evaluate the cost-effectiveness of the use of toric intraocular lenses (IOLs) in surg... more Objectives: evaluate the cost-effectiveness of the use of toric intraocular lenses (IOLs) in surgery of patients with cataract and preexisting corneal astigmatism (> 0.75 D), versus the use of traditional monofocal intraocular lenses (IOLs) from the perspective of third party payer, as a way to define which technology could considered in the health care reimbursement. MethOds: in a decision tree type model was simulated the cataracts surgery intervention in a time horizon of five years. The outcome measure was the cumulative visual acuity of 20/32, which was expressed in normal vision time without additional support. The drug costs were taken from SISMED (2013), the costs from multi-core procedures of the health ministry (SISPRO), the cost of glasses and contact lenses from commercial channel, and the cost of IOLs were taken as the selling price of Alcon Laboratories. Finally a Montecarlo type sensitivity analysis was performed. Results: as primary outcome, time normal distance vision without additional support was higher with toric IOLs, which it were more effective at 3,64 years versus 2,97 years achieved with traditional monofocal IOLs. Intervention with toric IOLs showed in the time horizon of five years be more expensive with (USD) 1.093,55versuscostsoftraditionalmonofocalIOLs(USD)1.093,55 versus costs of traditional monofocal IOLs (USD) 1.093,55versuscostsoftraditionalmonofocalIOLs(USD)732,71. In this regard cataract surgery is more effective and more costly with toric IOLs than with traditional monofocal IOLs with an incremental cost-effectiveness ratio of (USD) 534,83pereachyearofnormalvisionwithoutadditionalsupport.ItwasobservedthattheICERimprovesifthereisagreatervisualimpairmentduetoastigmatismbeforesurgery,aswell,todiopters<1.50,>1.50<2.00and>2.00theICERwas(USD)534,83 per each year of normal vision without additional support. It was observed that the ICER improves if there is a greater visual impairment due to astigmatism before surgery, as well, to diopters < 1.50, > 1.50 < 2.00 and > 2.00 the ICER was (USD) 534,83pereachyearofnormalvisionwithoutadditionalsupport.ItwasobservedthattheICERimprovesifthereisagreatervisualimpairmentduetoastigmatismbeforesurgery,aswell,todiopters<1.50,>1.50<2.00and>2.00theICERwas(USD) 712.40, (USD) $ 416.75 and (USD) $ 382.16 respectively. cOnclusiOns: toric IOLs proved to be cost-effective for the treatment of patients with cataract and preexisting astigmatism. PSS20 COSt-EffECtivEnESS Of rAnibizUmAb vErSUS lASEr in thE trEAtmEnt Of viSUAl imPAirmEnt dUE tO diAbEtiC mACUlAr OEdEmA (dmE) frOm thE COlOmbiAn hEAlth CArE SyStEm PErSPECtivE

Research paper thumbnail of Electroretinogram assessment of children with sensorineural hearing loss: implications for screening

Journal of American Association for Pediatric Ophthalmology and Strabismus, 2014

In the UK the National Deaf Children's Society guidelines recommend that all children with Sensor... more In the UK the National Deaf Children's Society guidelines recommend that all children with Sensorineural hearing loss (SNHL) undergo electroretinography (ERG) to exclude a retinal dystrophy. The aim is to determine the nature and prevalence of abnormal ERG findings in the cohort of children with SNHL undergoing ERG. Methods: This study was a retrospective review of the medical records of children (\18) with SNHL referred from the paediatric retinal clinics for ERG between January 2009 and December 2011. Results: Data was collected for 84 patients (53.5% male), median age 87 months (range, 9-215). Fourteen (16.67%) had abnormal ERGs; all had bilateral hearing impairment. Thirteen were diagnosed as Usher syndrome, and 1 an undefined cone dysfunction syndrome. Twelve of the patients with Usher syndrome had clinical findings and/or symptoms; 11 had or had been referred for cochlear implants. Sixty-eight of 70 patients with normal ERGs had normal examinations; two had rubella retinopathy. One had a genetic diagnosis of Usher Syndrome but normal examination and normal ERGs. Univariant analysis found the presence of, or referral for cochlear implants (P # 0.0001), having relevant symptoms and/or clinical signs of a retinal dystrophy (P # 0.0001) and profound hearing loss (P 5 0.000) were all highly significant. Discussion: ERG is a useful diagnostic tool but every patient with SNHL need not be investigated. Conclusion: ERG should be performed in children with SNHL who have been referred for cochlear implants and those who have signs/symptoms consistent with a retinal dystrophy.

Research paper thumbnail of Pseudomonas aeruginosa Eyelid Necrosis Associated With Felty Syndrome

Ophthalmic Plastic & Reconstructive Surgery, 2008

A 53-year-old Chinese man presented with a painless left upper eyelid lump of 2 weeks' duration. ... more A 53-year-old Chinese man presented with a painless left upper eyelid lump of 2 weeks' duration. Histopathology was consistent with angiolymphoid hyperplasia with eosinophilia, and also revealed neutrophilic infiltrate secondary to ulceration. After incisional biopsy, the residual lesion, which was not treated, developed surface keratinization over the next 2 weeks and sloughed off, leaving intact epidermis and no scar. This case illustrates that observation awaiting spontaneous regression may be considered prior to surgical and other therapeutic interventions. The authors also briefly review the literature on angiolymphoid hyperplasia with eosinophilia and Kimura disease, with emphasis on ocular involvement. A 53-year-old Chinese man presented with a left upper eyelid lump of 2 weeks' duration. There were no ocular symptoms or fever, and no preceding trauma or insect bite injury. The mass increased in size despite oral amoxicillin and topical gentamycin. An earlier attempt at incision and drainage yielded no purulent material. Examination showed a firm, erythematous nodule measuring approximately 1.5 cm in diameter. There was surface ulceration and hemorrhagic areas (Fig. 1A). There was no madarosis, and there were no similar lesions at other sites. An incisional biopsy was performed under local anesthesia. Histopathology showed an ulcerated lesion (Fig. 2A), which was composed of a dense inflammatory infiltrate, predominantly of eosinophils and mixed with neutrophils. There was proliferation of blood vessels lined by large endothelial cells with eosinophilic cytoplasm and oval vesicular nuclei (Fig. 2B, C). There were no granulomas, and stains for acid-fast bacilli, fungal elements, and bacteria were negative. These histopathologic features were interpreted as angiolymphoid hyperplasia with eosinophilia (ALHE). The presence of neutrophils was likely due to secondary surface ulceration from attempted incision and drainage. Evaluation for peripheral eosinophilia was not performed. Following the biopsy, the residual lesion developed surface keratinization over the next 2 weeks and sloughed off, leaving intact epidermis without scarring (Fig. 1B, C). No recurrence was noted during 3 years' follow-up. DISCUSSION ALHE should not be confused with Kimura disease (KD), an entity previously thought to be related to ALHE. 1,2 KD is likely

Research paper thumbnail of Leigh’s Disease Associated With a Dorsal Midbrain Syndrome

Journal of Pediatric Ophthalmology & Strabismus, 2009

Leigh&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp... more Leigh&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;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&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s disease due to two SURF1 mutations. To the authors&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; knowledge, this is the first reported case of dorsal midbrain syndrome caused by a mitochondrial cytopathy.

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

BMC Ophthalmology, 2013

Background: National guidelines on MRSA (methicillin-resistant Staphylococcus aureus) screening p... more 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. Results: A total of 74 units (56%) responded in 2004 and 71 units (96% of initial respondents) in 2007. In 2004, 57% of units screened for MRSA. They screened groups at high risk of carriage, including patients with previous MRSA (93%) and patients from Nursing homes (21%). Swab sites included the nose (100%), eyes (31%) and perineum (62%). In 2007, there was no significant change in the number of units that screened for MRSA (57% vs 66%; p = 0.118; McNemar test). However, more units screened for MRSA in patients from nursing/residential homes (21% vs 51%; p = 0.004, McNemar test), and in patients who had recent admission to hospital (12% vs 36%; p = 0.003). In the second survey, 3 units (6%) now screened patients who were close relatives of MRSA carriers. Conclusion: This survey has highlighted inconsistences in MRSA screening practice of day-case cataract surgery patients across England after 2 major national policy changes. A change in DoH policy only led to more units screening patients for MRSA from high risk groups.

Research paper thumbnail of Amblyopia in children (aged 7 years or less)

BMJ clinical evidence, Jan 5, 2016

Amblyopia is reduced visual acuity not immediately correctable by glasses, in the absence of ocul... more Amblyopia is reduced visual acuity not immediately correctable by glasses, in the absence of ocular pathology. It is commonly associated with squint (strabismus) or refractive errors resulting in different visual inputs to each eye during the sensitive period of visual development (aged <7-8 years). The cumulative incidence is estimated at 2% to 4% in children aged up to 7 years. We conducted a systematic overview, aiming to answer the following clinical question: What are the effects of medical treatments for amblyopia in children aged 7 years or less? We searched: Medline, Embase, The Cochrane Library, and other important databases up to January 2014 (Clinical Evidence overviews are updated periodically; please check our website for the most up-to-date version of this overview). At this update, searching of electronic databases retrieved 70 studies. After deduplication and removal of conference abstracts, 51 records were screened for inclusion in the overview. Appraisal of titl...

Research paper thumbnail of Cerebral visual impairment‐related vision problems in primary school children: a cross‐sectional survey

Developmental Medicine & Child Neurology, 2021

ABBREVIATIONS CVI Cerebral visual impairment SDQ Strengths and Difficulties Questionnaire AIM To ... more ABBREVIATIONS CVI Cerebral visual impairment SDQ Strengths and Difficulties Questionnaire AIM To estimate how many children in mainstream primary schools have cerebral visual impairment (CVI)-related vision problems and to investigate whether some indicators might be useful as red flags, if they were associated with increased risk for these problems. METHOD We conducted a survey of primary school children aged 5 to 11 years, using whether they were getting extra educational help and/or teacher-and parent-reported behaviour questionnaires to identify children at risk for CVI. These and a random 5% sample were assessed for CVI-related vision problems. We compared the usefulness of potential red flags using likelihood ratios. RESULTS We received questionnaires on 2298 mainstream-educated children and examined 248 children (152 [61%] males, 96 females [39%]; mean age 8y 1mo, SD 20mo, range 5y 6mo-11y 8mo). We identified 78 out of 248 children (31.5% of those examined, 3.4% of the total sample), who had at least one CVI-related vision problem. The majority (88%) were identified by one or more red flag but none were strongly predictive. Fewer than one in five children with any CVI-related vision problem had reduced visual acuity. INTERPRETATION Children with CVI-related vision problems were more prevalent than has been appreciated. Assessment of at-risk children may be useful so that opportunities to improve outcomes for children with CVI-related vision problems are not missed.

Research paper thumbnail of A demonstration of the Medisoft strabismus electronic patient record

Journal of American Association for Pediatric Ophthalmology and Strabismus, 2010

following PPC. The incidence of PCO and SM formation is similar regardless of IOL type. However, ... more following PPC. The incidence of PCO and SM formation is similar regardless of IOL type. However, the average time of onset to the development of PCO is slightly longer in the MA60AC group. The recurrence of PCO after Yag capsulotomy is low. Given these results, parents can be better informed regarding the incidence of visual axis opacification depending IOL design that has been placed. 115 Characteristics of retinal hemorrhages in preterm infants screened for retinopathy of prematurity by the Retcam II-A pilot study.

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

British Journal of Ophthalmology, 2013

To report the largest study on the safety and effectiveness of sedation in paediatric ophthalmolo... more To report the largest study on the safety and effectiveness of sedation in paediatric ophthalmology in a nurse-led outpatient sedation unit. 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. All demographic data, sedation and procedure duration, sedation success and adverse events were recorded. Univariate and multiple regression analyses were performed to assess factors associated with success and complications. Data was collected for 1509 sedation episodes. More boys were sedated compared with girls (56.3% vs 43.7% p=0.0003) with an average age of 23.86 months and weight of 11.76 kg. A higher proportion of patients had an American Society of Anaesthesiologists status of II than I (58.5%:41.5%, p=0.0001). Successful sedation was obtained in 96.69% of children with 4.77% requiring a top up dose to achieve this. The average sedation duration was 53.4 min (SD=21.5) with an average of 1.7 procedures performed; the most common being a detailed examination (93.5%) and electroretinogram (45.1%). Adverse events included paradoxical reaction (1.33%), oxygen desaturation (0.99%) and vomiting (0.53%). There were no serious complications or hospital admission. Multiple logistic regression analysis found weight greater than 15 kg and needing a top up dose to be significant risk factors for Failure (OR=2.49 and 8.69, respectively) and Adverse events (OR=2.1 and 3.97, respectively). Sex and American Society of Anesthesiologists Physical Status score did not significantly affect outcomes. CH sedation allows detailed examination and investigations in the majority of children with few side effects. Patients over 15 kg and need for a top up dose are risk factors for failure and adverse events. This is the largest study in the current literature looking at the use of CH sedation in ophthalmology and confirms its safety and effectiveness.

Research paper thumbnail of β-Defensin 1 haplotype associated with postoperative endophthalmitis

Acta Ophthalmologica, 2010

Research paper thumbnail of Use of MultiColor imaging in the assessment of suspected papilledema in 20 consecutive children

Journal of American Association for Pediatric Ophthalmology and Strabismus, 2016

PURPOSE To investigate the use of MultiColor Scanning Laser Imaging as an adjunct to traditional ... more PURPOSE To investigate the use of MultiColor Scanning Laser Imaging as an adjunct to traditional imaging modalities in the assessment of children with suspected disk swelling. METHODS In this study of 20 consecutive children with suspected papilledema, MultiColor imaging was performed on all patients in addition to fundus examination, fundus autofluorescence, disk spectral domain optical coherence tomography (SD-OCT), and disk retinal nerve fiber layer thickness (RNFL) measurement. Diagnoses were determined in all 20 patients, and all images were evaluated by a medical retina specialist. RESULTS Of the 20 cases (average age 11 years [range, 5-16 years]; 10 males), papilledema was confirmed in 11 (55%) and pseudopapilledema diagnosed in 9 (45%). Of pseudopapilledema cases, there were 4 cases of optic disk drusen, 2 hypermetropic disks, 2 "crowded disks," and 1 anomalous disk. In patients with true papilledema, on the combined MultiColor image, a green shift in the form of an elevated green ring was seen consistently. This ring was also hyperreflective on the blue and green images and surrounded a central "shadow," which was seen best on near infrared reflectance (NIR). The disk margins and vasculature were obscured on the combined, green, blue, and NIR images. These changes were not present in the pseudopapilledema cases. CONCLUSIONS MultiColor imaging of disks with papilledema shows characteristic changes that may prove useful in differentiating true papilledema from pseudopapilledema. It is therefore a useful adjunct to traditional disk imaging modalities in assessing children with suspected disk swelling.

Research paper thumbnail of Amblyopia

BMJ clinical evidence, Jan 30, 2011

Amblyopia is commonly associated with squint (strabismus) or refractive errors resulting in diffe... more Amblyopia is commonly associated with squint (strabismus) or refractive errors resulting in different visual inputs to each eye during the sensitive period of visual development (<7-8 years of age). The cumulative incidence is estimated at 2% to 4% in children aged up to 15 years. We conducted a systematic review and aimed to answer the following clinical questions: What are the effects of interventions to detect amblyopia early? What are the effects of medical treatments for amblyopia? We searched: Medline, Embase, The Cochrane Library, and other important databases up to May 2010 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations, such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). We found 33 systematic reviews, RCTs, or observational studies that met our inclusion criteria. We perf...

Research paper thumbnail of Multicolor imaging in the assessment and diagnosis of optic disc swelling

Investigative Ophthalmology & Visual Science, 2015

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

British Journal of Ophthalmology, 2015

Research paper thumbnail of Using A Crossover Study Design For Early Hospital-Based Health Technology Assessment: The Cost-Effectiveness Of Clinic-Based Chloral Hydrate Sedation Versus General Anaesthesia For Paediatric Ophthalmological Procedures

Value in Health, 2014

Objectives: evaluate the cost-effectiveness of the use of toric intraocular lenses (IOLs) in surg... more Objectives: evaluate the cost-effectiveness of the use of toric intraocular lenses (IOLs) in surgery of patients with cataract and preexisting corneal astigmatism (> 0.75 D), versus the use of traditional monofocal intraocular lenses (IOLs) from the perspective of third party payer, as a way to define which technology could considered in the health care reimbursement. MethOds: in a decision tree type model was simulated the cataracts surgery intervention in a time horizon of five years. The outcome measure was the cumulative visual acuity of 20/32, which was expressed in normal vision time without additional support. The drug costs were taken from SISMED (2013), the costs from multi-core procedures of the health ministry (SISPRO), the cost of glasses and contact lenses from commercial channel, and the cost of IOLs were taken as the selling price of Alcon Laboratories. Finally a Montecarlo type sensitivity analysis was performed. Results: as primary outcome, time normal distance vision without additional support was higher with toric IOLs, which it were more effective at 3,64 years versus 2,97 years achieved with traditional monofocal IOLs. Intervention with toric IOLs showed in the time horizon of five years be more expensive with (USD) 1.093,55versuscostsoftraditionalmonofocalIOLs(USD)1.093,55 versus costs of traditional monofocal IOLs (USD) 1.093,55versuscostsoftraditionalmonofocalIOLs(USD)732,71. In this regard cataract surgery is more effective and more costly with toric IOLs than with traditional monofocal IOLs with an incremental cost-effectiveness ratio of (USD) 534,83pereachyearofnormalvisionwithoutadditionalsupport.ItwasobservedthattheICERimprovesifthereisagreatervisualimpairmentduetoastigmatismbeforesurgery,aswell,todiopters<1.50,>1.50<2.00and>2.00theICERwas(USD)534,83 per each year of normal vision without additional support. It was observed that the ICER improves if there is a greater visual impairment due to astigmatism before surgery, as well, to diopters < 1.50, > 1.50 < 2.00 and > 2.00 the ICER was (USD) 534,83pereachyearofnormalvisionwithoutadditionalsupport.ItwasobservedthattheICERimprovesifthereisagreatervisualimpairmentduetoastigmatismbeforesurgery,aswell,todiopters<1.50,>1.50<2.00and>2.00theICERwas(USD) 712.40, (USD) $ 416.75 and (USD) $ 382.16 respectively. cOnclusiOns: toric IOLs proved to be cost-effective for the treatment of patients with cataract and preexisting astigmatism. PSS20 COSt-EffECtivEnESS Of rAnibizUmAb vErSUS lASEr in thE trEAtmEnt Of viSUAl imPAirmEnt dUE tO diAbEtiC mACUlAr OEdEmA (dmE) frOm thE COlOmbiAn hEAlth CArE SyStEm PErSPECtivE

Research paper thumbnail of Electroretinogram assessment of children with sensorineural hearing loss: implications for screening

Journal of American Association for Pediatric Ophthalmology and Strabismus, 2014

In the UK the National Deaf Children's Society guidelines recommend that all children with Sensor... more In the UK the National Deaf Children's Society guidelines recommend that all children with Sensorineural hearing loss (SNHL) undergo electroretinography (ERG) to exclude a retinal dystrophy. The aim is to determine the nature and prevalence of abnormal ERG findings in the cohort of children with SNHL undergoing ERG. Methods: This study was a retrospective review of the medical records of children (\18) with SNHL referred from the paediatric retinal clinics for ERG between January 2009 and December 2011. Results: Data was collected for 84 patients (53.5% male), median age 87 months (range, 9-215). Fourteen (16.67%) had abnormal ERGs; all had bilateral hearing impairment. Thirteen were diagnosed as Usher syndrome, and 1 an undefined cone dysfunction syndrome. Twelve of the patients with Usher syndrome had clinical findings and/or symptoms; 11 had or had been referred for cochlear implants. Sixty-eight of 70 patients with normal ERGs had normal examinations; two had rubella retinopathy. One had a genetic diagnosis of Usher Syndrome but normal examination and normal ERGs. Univariant analysis found the presence of, or referral for cochlear implants (P # 0.0001), having relevant symptoms and/or clinical signs of a retinal dystrophy (P # 0.0001) and profound hearing loss (P 5 0.000) were all highly significant. Discussion: ERG is a useful diagnostic tool but every patient with SNHL need not be investigated. Conclusion: ERG should be performed in children with SNHL who have been referred for cochlear implants and those who have signs/symptoms consistent with a retinal dystrophy.

Research paper thumbnail of Pseudomonas aeruginosa Eyelid Necrosis Associated With Felty Syndrome

Ophthalmic Plastic & Reconstructive Surgery, 2008

A 53-year-old Chinese man presented with a painless left upper eyelid lump of 2 weeks' duration. ... more A 53-year-old Chinese man presented with a painless left upper eyelid lump of 2 weeks' duration. Histopathology was consistent with angiolymphoid hyperplasia with eosinophilia, and also revealed neutrophilic infiltrate secondary to ulceration. After incisional biopsy, the residual lesion, which was not treated, developed surface keratinization over the next 2 weeks and sloughed off, leaving intact epidermis and no scar. This case illustrates that observation awaiting spontaneous regression may be considered prior to surgical and other therapeutic interventions. The authors also briefly review the literature on angiolymphoid hyperplasia with eosinophilia and Kimura disease, with emphasis on ocular involvement. A 53-year-old Chinese man presented with a left upper eyelid lump of 2 weeks' duration. There were no ocular symptoms or fever, and no preceding trauma or insect bite injury. The mass increased in size despite oral amoxicillin and topical gentamycin. An earlier attempt at incision and drainage yielded no purulent material. Examination showed a firm, erythematous nodule measuring approximately 1.5 cm in diameter. There was surface ulceration and hemorrhagic areas (Fig. 1A). There was no madarosis, and there were no similar lesions at other sites. An incisional biopsy was performed under local anesthesia. Histopathology showed an ulcerated lesion (Fig. 2A), which was composed of a dense inflammatory infiltrate, predominantly of eosinophils and mixed with neutrophils. There was proliferation of blood vessels lined by large endothelial cells with eosinophilic cytoplasm and oval vesicular nuclei (Fig. 2B, C). There were no granulomas, and stains for acid-fast bacilli, fungal elements, and bacteria were negative. These histopathologic features were interpreted as angiolymphoid hyperplasia with eosinophilia (ALHE). The presence of neutrophils was likely due to secondary surface ulceration from attempted incision and drainage. Evaluation for peripheral eosinophilia was not performed. Following the biopsy, the residual lesion developed surface keratinization over the next 2 weeks and sloughed off, leaving intact epidermis without scarring (Fig. 1B, C). No recurrence was noted during 3 years' follow-up. DISCUSSION ALHE should not be confused with Kimura disease (KD), an entity previously thought to be related to ALHE. 1,2 KD is likely

Research paper thumbnail of Leigh’s Disease Associated With a Dorsal Midbrain Syndrome

Journal of Pediatric Ophthalmology & Strabismus, 2009

Leigh&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp... more Leigh&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;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&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s disease due to two SURF1 mutations. To the authors&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; knowledge, this is the first reported case of dorsal midbrain syndrome caused by a mitochondrial cytopathy.

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

BMC Ophthalmology, 2013

Background: National guidelines on MRSA (methicillin-resistant Staphylococcus aureus) screening p... more 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. Results: A total of 74 units (56%) responded in 2004 and 71 units (96% of initial respondents) in 2007. In 2004, 57% of units screened for MRSA. They screened groups at high risk of carriage, including patients with previous MRSA (93%) and patients from Nursing homes (21%). Swab sites included the nose (100%), eyes (31%) and perineum (62%). In 2007, there was no significant change in the number of units that screened for MRSA (57% vs 66%; p = 0.118; McNemar test). However, more units screened for MRSA in patients from nursing/residential homes (21% vs 51%; p = 0.004, McNemar test), and in patients who had recent admission to hospital (12% vs 36%; p = 0.003). In the second survey, 3 units (6%) now screened patients who were close relatives of MRSA carriers. Conclusion: This survey has highlighted inconsistences in MRSA screening practice of day-case cataract surgery patients across England after 2 major national policy changes. A change in DoH policy only led to more units screening patients for MRSA from high risk groups.

Research paper thumbnail of Amblyopia in children (aged 7 years or less)

BMJ clinical evidence, Jan 5, 2016

Amblyopia is reduced visual acuity not immediately correctable by glasses, in the absence of ocul... more Amblyopia is reduced visual acuity not immediately correctable by glasses, in the absence of ocular pathology. It is commonly associated with squint (strabismus) or refractive errors resulting in different visual inputs to each eye during the sensitive period of visual development (aged <7-8 years). The cumulative incidence is estimated at 2% to 4% in children aged up to 7 years. We conducted a systematic overview, aiming to answer the following clinical question: What are the effects of medical treatments for amblyopia in children aged 7 years or less? We searched: Medline, Embase, The Cochrane Library, and other important databases up to January 2014 (Clinical Evidence overviews are updated periodically; please check our website for the most up-to-date version of this overview). At this update, searching of electronic databases retrieved 70 studies. After deduplication and removal of conference abstracts, 51 records were screened for inclusion in the overview. Appraisal of titl...

Research paper thumbnail of Cerebral visual impairment‐related vision problems in primary school children: a cross‐sectional survey

Developmental Medicine & Child Neurology, 2021

ABBREVIATIONS CVI Cerebral visual impairment SDQ Strengths and Difficulties Questionnaire AIM To ... more ABBREVIATIONS CVI Cerebral visual impairment SDQ Strengths and Difficulties Questionnaire AIM To estimate how many children in mainstream primary schools have cerebral visual impairment (CVI)-related vision problems and to investigate whether some indicators might be useful as red flags, if they were associated with increased risk for these problems. METHOD We conducted a survey of primary school children aged 5 to 11 years, using whether they were getting extra educational help and/or teacher-and parent-reported behaviour questionnaires to identify children at risk for CVI. These and a random 5% sample were assessed for CVI-related vision problems. We compared the usefulness of potential red flags using likelihood ratios. RESULTS We received questionnaires on 2298 mainstream-educated children and examined 248 children (152 [61%] males, 96 females [39%]; mean age 8y 1mo, SD 20mo, range 5y 6mo-11y 8mo). We identified 78 out of 248 children (31.5% of those examined, 3.4% of the total sample), who had at least one CVI-related vision problem. The majority (88%) were identified by one or more red flag but none were strongly predictive. Fewer than one in five children with any CVI-related vision problem had reduced visual acuity. INTERPRETATION Children with CVI-related vision problems were more prevalent than has been appreciated. Assessment of at-risk children may be useful so that opportunities to improve outcomes for children with CVI-related vision problems are not missed.

Research paper thumbnail of A demonstration of the Medisoft strabismus electronic patient record

Journal of American Association for Pediatric Ophthalmology and Strabismus, 2010

following PPC. The incidence of PCO and SM formation is similar regardless of IOL type. However, ... more following PPC. The incidence of PCO and SM formation is similar regardless of IOL type. However, the average time of onset to the development of PCO is slightly longer in the MA60AC group. The recurrence of PCO after Yag capsulotomy is low. Given these results, parents can be better informed regarding the incidence of visual axis opacification depending IOL design that has been placed. 115 Characteristics of retinal hemorrhages in preterm infants screened for retinopathy of prematurity by the Retcam II-A pilot study.

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

British Journal of Ophthalmology, 2013

To report the largest study on the safety and effectiveness of sedation in paediatric ophthalmolo... more To report the largest study on the safety and effectiveness of sedation in paediatric ophthalmology in a nurse-led outpatient sedation unit. 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. All demographic data, sedation and procedure duration, sedation success and adverse events were recorded. Univariate and multiple regression analyses were performed to assess factors associated with success and complications. Data was collected for 1509 sedation episodes. More boys were sedated compared with girls (56.3% vs 43.7% p=0.0003) with an average age of 23.86 months and weight of 11.76 kg. A higher proportion of patients had an American Society of Anaesthesiologists status of II than I (58.5%:41.5%, p=0.0001). Successful sedation was obtained in 96.69% of children with 4.77% requiring a top up dose to achieve this. The average sedation duration was 53.4 min (SD=21.5) with an average of 1.7 procedures performed; the most common being a detailed examination (93.5%) and electroretinogram (45.1%). Adverse events included paradoxical reaction (1.33%), oxygen desaturation (0.99%) and vomiting (0.53%). There were no serious complications or hospital admission. Multiple logistic regression analysis found weight greater than 15 kg and needing a top up dose to be significant risk factors for Failure (OR=2.49 and 8.69, respectively) and Adverse events (OR=2.1 and 3.97, respectively). Sex and American Society of Anesthesiologists Physical Status score did not significantly affect outcomes. CH sedation allows detailed examination and investigations in the majority of children with few side effects. Patients over 15 kg and need for a top up dose are risk factors for failure and adverse events. This is the largest study in the current literature looking at the use of CH sedation in ophthalmology and confirms its safety and effectiveness.

Research paper thumbnail of β-Defensin 1 haplotype associated with postoperative endophthalmitis

Acta Ophthalmologica, 2010