Geoffrey Lam

Fluorescein angiographic observations of peripheral retinal vessel growth in infants after intravitreal injection of bevacizumab as sole therapy for zone I and posterior zone II retinopathy of prematurity.

Aim To evaluate vascularisation of the peripheral retina using fluorescein angiography (FA) digital recordings of infants who had been treated with intravitreal bevacizumab (IVB) as sole therapy for zone I and posterior zone II retinopathy of prematurity (ROP). Methods A retrospective evaluation was performed of medical records, RetCam fundus images and RetCam fluorescein angiogram videos of 10 neonates (20 eyes) who received intravitreal bevacizumab injections as the only treatment for zone I and posterior zone II ROP between August 2007 and November 2012. Results All eyes had initial resolution of posterior disease after IVB injection as documented by RetCam colour fundus photographs. Using a distance of 2 disc diameters from the ora serrata to vascular termini as the upper limit of allowable avascular retina in children, the FA of these infants demonstrated that 11 of 20 eyes had not achieved normal retinal vascularisation. Conclusions Although bevacizumab appears effective in bringing resolution of zone I and posterior zone II ROP and allowing growth of peripheral retinal vessels, in our series of 20 eyes, complete normal peripheral retinal vascularisation was not achieved in half of the patients.

Management of congenital cataract in children younger than 1 year using a 25-gauge vitrectomy system

PURPOSE: To evaluate the safety and efficacy of a 25‐gauge vitrectomy system for the management of congenital cataract in children younger than 1 year. SETTING: Princess Margaret Hospital for Children, Western Australia, Australia. METHODS: Children in a tertiary pediatric hospital and private practice had cataract extraction using a 25‐gauge vitrectomy system between January 2005 and June 2008. Each eye had anterior vitrectorhexis, lens aspiration, posterior vitrectorhexis, and anterior vitrectomy through two 25‐gauge limbal side ports. The ports were created with a 25‐gauge trocar or a 0.6 mm paracentesis knife. The eyes were left aphakic. RESULTS: Nineteen (95%) of the 20 eyes (14 children) had a successful surgical outcome with the 25‐gauge vitrectomy system. One eye was converted to the 20‐gauge system because of an unusually tough and fibrous lens capsule. The limbal side ports in 16 of 17 eyes created with the 25‐gauge trocar required suturing to seal the ports; none of the 3 eyes with side ports created with the paracentesis knife required suturing. There were no significant intraoperative or postoperative complications other than ocular hypertension in 1 eye. CONCLUSIONS: The 25‐gauge vitrectomy system appears safe and effective for the management of infantile cataract. Advantages include more precise manipulations with smaller instruments in infant eyes, a more stable anterior chamber, and less postoperative astigmatism.

Screening for retinopathy of prematurity (ROP) using wide-angle digital retinal photography by non-ophthalmologists: a systematic review

Retinopathy of prematurity (ROP) is one of the leading and preventable causes of blindness. The investigation of choice for diagnosing ROP is binocular indirect ophthalmoscope (BIO) done by ophthalmologists. Since the number of ophthalmologists available to do BIO examination is limited, especially in developing countries, there is a need for an alternate, cheap, reliable and feasible test. Telemedicine imaging with Digital Retinal Photography (DRP) is one such alternate diagnostic test which can be performed easily by non-ophthalmologists, with adequate training. Our objective was to conduct a systematic review to evaluate the accuracy of DRP performed by trained personnel (non-ophthalmologists) in diagnosing clinically significant ROP. Medline, EMBASE, CINAHL and Cochrane databases were searched independently by two authors. Eligible studies were assessed using the Quality Assessment of Diagnostic Accuracy Studies (QUADAS)-2, an evidence-based tool for the assessment of quality in systematic reviews of diagnostic accuracy studies. Six were included in the review (three prospective; N=120, three retrospective; N=579). Studies had methodological limitations on QUADAS-2. Because of the heterogeneity of studies, data could not be pooled to derive single-effect size estimates for sensitivity and specificity. The included studies reported sensitivity of 45.5–100% with the majority being more than 90%; specificity 61.7–99.8% with the majority being more than 90%, positive predictive value 61.5–96.6% and negative predictive value of 76.9–100% for diagnosing clinically significant ROP. We conclude that diagnostic accuracy of DRP must be established in prospective studies with adequate sample size where DRP is compared against the simultaneously performed BIO examination.

Wii eye injury: Self-inflicted globe rupture and vision loss in a 7-year-old boy from a video game accident

The Nintendo Wii is a home video game console released in November 2006. It is distinguished by its Wii Remote, a wireless controller that can be used as a handheld pointing device. We report a self-inflicted penetrating eye injury, late retinal detachment, and vision loss in a 7-year-old boy resulting from the use of a Wii Remote. To our knowledge, this is the first reported case of severe ocular trauma from use of a wireless game controller.

Syndrome of microcephaly, microphthalmia, cataracts, and intracranial calcification.

We present two sisters with microcephaly, developmental delay, marked microphthalmia, congenital cataracts, cerebral and cerebellar hypoplasia, and intracranial calcification. No evidence of intrauterine infection was found. There have been previous reports of microcephaly, intracranial calcification, and an intrauterine infection-like autosomal recessive condition, but the sibs in this report appear to represent a more severe form of such a condition or a previously undescribed entity.

Sports-related eye and adnexal injuries in the Western Australian paediatric population

To identify the causes of sports‐related eye and adnexal injuries in children in Perth, Western Australia, to determine which sporting activities pose the highest risk of eye and adnexal injury to children.

Duration of anaemia during the first week of life is an independent risk factor for retinopathy of prematurity

This study evaluated the correlation between retinopathy of prematurity (ROP), anaemia and blood transfusions in extremely preterm infants.

Retinal sequelae of incontinentia pigmenti.

Correspondence: Chandrakumar Balaratnasingam, MD, Department of Ophthalmology, Princess Margaret Hospital for Children, Roberts Road, Subiaco, WA 6008, Australia. Email: bala@graduate.uwa.edu.au Received 19 December 2005; revised 10 March 2007; accepted 25 April 2007. doi: 10.1111/j.1442-200X.2008.02780.x Incontinentia pigmenti (IP) is a rare, X-linked dominant, multisystem disorder involving ocular, dental, musculoskeletal, central nervous system and dermatologic structures. Mutations in the NEMO / IKKgene result in the IP phenotype, with 80% of cases being due to a single mutation that results in the deletion of exons 4 – 10. 1 Being an X-linked dominant condition, it has a high level of lethality in male embryos, and female subjects account for 97% of all living patients. 2 Occasional survival of affected male subjects is attributed to somatic mosaicism or due to the presence of an extra X chromosome such as that seen in Kleinfelter’s syndrome. Dermatologic fi ndings are most often the fi rst observed sign of IP, and the diagnosis is very frequently made following skin biopsy. These cutaneous manifestations are classically subdivided into four stages: vesicular, verrucous, hyperpigmented, and atrophic, based on the distribution, appearance and period in childhood in which they appear. The skin changes, although distressing to the child and family, very often disappear completely between the 20th and 30th years of life, and the long-term morbidity is determined by the neurological and ophthalmological manifestations of this disease. Nineteen percent of patients with IP are at risk of severe visual loss in one or both eyes. 3 Although the commonest abnormality is strabismus (present in 18.2%), it is the changes in the posterior segment of the eye that pose the greatest threat to vision. 3 We present our experience with three children with retinal sequelae of IP to illustrate the variability in visual prognosis as a result of disease. In keeping with current opinion we also offer guidelines regarding screening of affected children to prevent progression of disease and onset of sight-threatening complications.

SCALP syndrome: What is it and its ophthalmic manifestations

Purpose To present the ophthalmic manifestations of a 3-month old female with SCALP syndrome. Observations The patient presented with multiple ocular anomalies including bilateral limbal dermoids, esotropia and left optic nerve hypoplasia. Conclusions We describe systemic and ocular anomalies in a rare case of SCALP syndrome. This report provides additional information on the ocular anomalies not previously described that may be associated with this clinical entity.

Paediatric ocular and adnexal injuries requiring hospitalisation in Western Australia

The aim was to investigate the characteristics and outcomes of ocular and adnexal injuries requiring hospitalisation in children in Perth, Western Australia.