Timing of surgery in essential infantile esotropia - What more do we know since the turn of the century?

This review summarizes the results and interpretations of studies pertaining to the long-standing debate regarding the timing of surgery in infantile esotropia, more recently referred to as essential infantile esotropia. A systematic search of studies from the year 2000 onward pertaining to the timing of surgery in infantile esotropia as listed in PubMed, Google Scholar, and the Cochrane database was performed. Appropriate cross-references from the articles were also included. Data collected included demographics, presentation, time of surgery, complications, and outcomes. Very early surgery, that is, within 6 months of the onset of infantile esotropia, offers significant advantages in terms of the quality of stereopsis and binocular vision as well as promoting the development of cortical visual processing, thereby benefiting cortical development in human infants. However, the postoperative alignment was not found to be significantly different in the very early, early, or late surgery groups. The reduction in the incidence of manifest dissociated vertical deviation postoperatively in the very early surgery group also showed measurable benefits. The results of this recent literature review demonstrated that very early surgery, within 6 months of misalignment, showed demonstrable benefits in essential infantile esotropia.

Reporting on Australian childhood visual impairment: the first 10 years.

BACKGROUND: Visual impairment is rare but has significant impact on the neurobehavioural development and quality of life of children. This paper presents the key findings from the Australian Childhood Vision Impairment Register, which commenced in 2008 to report on children diagnosed with permanent visual impairment. SUBJECTS/METHODS: Families consent to completing a data form related to their child and for contact with the child's ophthalmologist. Ophthalmologists complete and return a comprehensive data form on the child's primary and secondary ocular diagnoses, associated disabilities and health conditions, visual acuity and visual fields. Data is stored on a secure database and anonymised data is available to researchers and for planning purposes. RESULTS: Nine-hundred four children and their families provided informed consent for participation, with 57% males and 43% females. Most children spoke English in their home. Eighty-three percent of children were born full term, with a birth weight of >2500 g (81%). Children were commonly suspected to have visual impairment by a parent, with 68% of families receiving a diagnosis of visual impairment by their child's first birthday. The most common primary diagnoses were retinal dystrophy (17%), CVI (15%) and Albinism (11%). A secondary diagnosis of infantile nystagmus occurred in 33% of children. Additional disabilities and/or developmental delay were reported for 44% of children. Corrected binocular visual acuity was reported for 75% of children, with moderate visual impairment being most common. CONCLUSIONS: These findings contribute to knowledge of rare diseases affecting the eye and visual pathway and represent Australian childhood visual impairment.

Visual Acuity Improves in Children and Adolescents With Idiopathic Infantile Nystagmus.

PURPOSE: Idiopathic infantile nystagmus is associated with reduced visual acuity. Recent work has linked extraocular muscle surgery to improvements in visual acuity through childhood but no work has reported long-term secular trends in visual acuity in infantile nystagmus. Our aim is to describe visual acuity changes for children and adolescents with idiopathic infantile nystagmus to allow comparison for future interventional studies. DESIGN: Retrospective chart review. METHODS: Review of patients attending our center up to the age of 18 with a diagnosis of idiopathic infantile nystagmus and visual acuity measured using Snellen visual acuity. Patients provided informed consent. RESULTS: We observed improvements in best corrected visual acuity in 43 children and adolescents with idiopathic infantile nystagmus. Binocular best corrected visual acuity improved at a rate of -0.16 logarithm of the minimum angle of resolution (logMAR)/log year of age (P < 0.001), an improvement of 0.05 logMAR (half a Snellen line) as the age doubles. Intraclass correlation was 0.95 and interindividual correlation between visual acuity and age was significant (r = -0.24, P < 0.001). CONCLUSIONS: We describe a natural history of gradual improvement in binocular visual acuity in infantile nystagmus and provide a baseline against which future interventional work can be compared.

Photographic assessment of retinal hemorrhages in infant head injury: the Childhood Hemorrhagic Retinopathy Study.

BACKGROUND: Retinal hemorrhages (RH) in babies in the absence of severe trauma or a medical cause have been strongly associated with abusive head trauma (AHT). We examined the pattern of RH in accidental head injury and AHT objectively using widefield retinal imaging. METHODS: A total of 118 infants and children 1-36 months of age admitted with head injuries at two centers were included in this prospective, consecutive, comparative cohort study. Dilated fundus examination was performed with indirect ophthalmoscopy and widefield imaging. Designation of AHT was made using predetermined criteria independent of retinal findings. Retinal images were graded by two independent observers. RESULTS: There were 21 cases of AHT. RH were present in 14 cases (66%); macular retinoschisis or retinal folds, in 8 (38%). There were 86 cases of accidental head injuries, with RH present in 2 (2%); there were none with retinal folds or retinoschisis. In cases of head injury with intracranial hemorrhage, the positive likelihood ratio of AHT with RH was 5.7 (95% CI, 2.6-12.00) and negative likelihood ratio was 0.26 (95% CI, 0.11-0.62). A severe, panretinal pattern with multilayered hemorrhages was the most specific for AHT. CONCLUSIONS: Our imaging study confirmed that RH in infants with head injury have a high positive likelihood ratio for AHT. A severe hemorrhagic retinopathy, particularly in association with perimacular folds or macular retinoschisis, has the highest positive predictive value for AHT.

Central retinal vein occlusion in an otherwise healthy child treated successfully with a single injection of bevacizumab.

We describe the case of an otherwise healthy 13-year-old boy who presented with blurred vision and deteriorating visual acuity in his left eye. Fundus examination showed left optic disk swelling, exudates, and hemorrhages. He was found to have an elevated left central retinal venous pressure to the level of arterial diastolic pressure, an elevated left central macular thickness and a prolonged disk-to-disk transit time on fluorescein angiography, which confirmed the diagnosis of unilateral central retinal vein occlusion (CRVO). The child was treated with one injection of bevacizumab. He has maintained visual acuity of 6/6 for 2 years following treatment, despite persistent elevated left central venous pressure and chronic optic disk edema.

Primary congenital glaucoma outcomes: lessons from 23 years of follow-up.

PURPOSE: To determine in primary congenital glaucoma whether age of presentation influences surgical success, the degrees of angle surgery needed to achieve glaucoma control, and whether there are critical ages where glaucoma progresses, requiring further surgical management. DESIGN: Retrospective cohort study. METHODS: The medical records of patients with primary congenital glaucoma over a 23-year period were reviewed: 192 procedures were performed on 117 eyes (70 patients). The number and age of angle procedures and final visual acuity was analyzed. Surgical success was defined as stable intraocular pressure and optic disc appearance. RESULTS: Procedures involving 83 of the 110 eyes (75.5%) undergoing angle surgery were successful, with 2-, 4-, 6-, and 10-year success rates of 92%, 86%, 84%, and 75%, respectively. Subgroup analysis (<3 months; 3-6 months; >6 months) comparing age of diagnosis to visual outcome (<20/200, 20/200-20/40, >20/40) was significant (P = .04). The age at first operation (P = .94), the number of angle operations (P = .43), and their effect on angle surgery success was not significant. Seven of 192 operations were performed after the age of 8 years (3.6%). After the initial angle surgeries within the first year of life, the third procedure occurred at a median age of 2.4 years (interquartile ratio [IQR] 0.6-3.8 years) and the fourth procedure occurred at a median age of 5.3 years (IQR 2.5-6.1 years). CONCLUSIONS: Children diagnosed at <3 months of age had a visual outcome of <20/200 despite successful glaucoma control. Age of presentation did not affect surgical success. A total of 78.9% of cases undergoing primary trabeculotomy were controlled with 1 operation: 4 clock hours of angle (120 degrees). Analysis of glaucoma progression suggests critical ages where further glaucoma surgery is required at around 2 and 5 years of age.

Grading system for retinal hemorrhages in abusive head trauma: clinical description and reliability study.

PURPOSE: There is currently no universally accepted grading system for describing retinal hemorrhages (RH) in abusive head trauma (AHT). The purpose of this study was to devise and evaluate a novel grading system and descriptive nomenclature for RH in AHT for clinical and research purposes. METHODS: A traumatic hemorrhagic retinopathy (THR) grading system was developed for assessing and quantitatively analyzing retinal findings in abusive head trauma. The criteria for the THR grade included the extent, spread, and morphology of RH. Extent was classified as region 1 (posterior pole) or region 2 (peripheral). Spread, based on number of retinal hemorrhages, was classified as mild (10 or fewer RH), moderate (more than 10 RH) and severe (more than half of involved regions covered by RH). Morphology was classified by its intraretinal or extraretinal involvement. Two independent graders calculated the THR grade from RetCam images of 38 eyes of 19 patients <3 years of age with retinal hemorrhages associated with head injury. Grading was performed on two separate occasions. Intra- and interobserver reliability was assessed with Spearman correlation coefficient (r) and intraclass correlation coefficient (ICC). RESULTS: There was a high level of intraobserver agreement across both assessments (97% agreement [Spearman r = 0.997; P < 0.0001] and 100% agreement [Spearman r = 1.0; P < 0.0000]). Intraclass correlation (ICC, 0.995; 95% CI, 0.991-0.997; P < 0.0001) confirmed a very high level of agreement overall. CONCLUSIONS: The traumatic hemorrhagic retinopathy grading system demonstrated excellent intraobserver and interobserver reliability. The nomenclature is easily understood and may be useful in medical records and medicolegal reports.

Vision screening in preschoolers: the New South Wales Statewide Eyesight Preschooler Screening program.

OBJECTIVE: To evaluate screening and diagnostic outcomes of the New South Wales Statewide Eyesight Preschooler Screening (StEPS) program, a state-funded, universal vision screening program for 4-year-old children. DESIGN, SETTING AND PARTICIPANTS: A cross-sectional evaluation of the StEPS program, in which eligible 4-year-old children were offered a vision screen in local health districts in NSW, between 1 July 2010 and 30 June 2011. MAIN OUTCOME MEASURES: Number and proportion of eligible children who were offered screening; accepted screening; were screened and scored a pass or were referred (routinely or urgently) for further vision assessment; and were referred for further assessment and required intervention. RESULTS: Of 91 324 eligible 4-year-olds in NSW, 80 328 (88.0%) were offered screening, and 65 834 (72.1% of the eligible population) were screened. Of the children who were screened, 3867 (5.9%) scored less than 6/9-2 but better than 6/18 in one or both eyes and were referred to their general practitioner or eye health professional for further vision assessment. A further 1425 children (2.2%) scored 6/18 or less in one or both eyes and were referred for high-priority assessment. In the two local health districts with the most complete follow-up data, 704 of 779 children (90.4%) with routine referrals and 278 of 285 (97.5%) with high-priority referrals required treatment or review at a later date. CONCLUSIONS: The StEPS program has achieved a high screening participation rate in NSW. Many children have been diagnosed and received treatment for previously undetected serious vision disorders that may otherwise have been diagnosed too late for effective intervention.

Unilateral inferior rectus hypoplasia in a child with Axenfeld-Rieger syndrome.

A 6-year-old boy with Axenfeld-Rieger syndrome presented with exotropia and a right hypertropia with limited depression. Computed tomography revealed hypoplasia of the right inferior rectus muscle. Forced duction testing was notable for a tight right superior rectus muscle. A superior rectus muscle recession reduced the primary position hypertropia but did not improve the patient's downgaze. To our knowledge, unilateral inferior rectus muscle hypoplasia has not been described previously in a patient with Axenfeld-Rieger syndrome.

APAO's Commitment to Ophthalmic Education.

The Asia-Pacific Academy of Ophthalmology (APAO) is a supranational organization set up in 1960 with the aims to advance the science and art of ophthalmology and eliminate preventable blindness in the Asia-Pacific region through teaching, research, and service. Being the major driving force of ophthalmologic development in the region, the APAO is dedicated to providing education opportunities to ophthalmologists and visual scientists in the region and beyond. Since its inception in 1960, ophthalmic education has topped the APAO's priority list. Accordingly, the APAO organizes an annual congress, organizes scientific meetings and conferences, publishes its official journal and newspaper, recognizes ophthalmologists' achievements, and offers training and learning opportunities to young and aspiring ophthalmologists. This perspective reviews efforts the APAO has made in advancing ophthalmic education and its future plans to further expand the scopes of its service to help push back the frontiers of knowledge.

Successful treatment of infantile haemangiomas of the orbit with propranolol.

BACKGROUND: Propranolol is a novel therapeutic agent in the treatment of cutaneous infantile haemangiomas. We assessed the effect of propranolol therapy in infantile haemangiomas of the orbit. METHODS: A case series of four patients with orbital infantile haemangiomas were referred for management in our tertiary referral hospitals. Two of the patients had inadequate responses to prior corticosteroid therapy. One of the patients was commenced on propranolol at 2.5 years of age when the lesion was not in the proliferative phase. This represented the first case report of propranolol treatment for infantile haemangioma outside infancy. The other three children were in their infancy when propranolol was commenced. The patients were treated with oral propranolol. RESULTS: All patients had significant improvement in their physical appearance, ocular examination findings and size of their lesions on radiological evaluation. No side-effects of propranolol treatment were observed. CONCLUSIONS: Propranolol is a promising treatment against infantile haemangiomas in the orbit, not only in infants but also in an older child with a stable lesion.

A systematic review of the diagnostic accuracy of ocular signs in pediatric abusive head trauma.

TOPIC: To review systematically the diagnostic accuracy of various ocular signs for pediatric abusive head trauma (AHT). CLINICAL RELEVANCE: Intraocular hemorrhages (IOH), perimacular retinal folds, traumatic retinoschisis and optic nerve sheath hemorrhages have been reported as cardinal signs of AHT. The evidence base supporting the accuracy of this interpretation, however, has not been systematically reviewed. METHODS: A systematic keyword search of MEDLINE, EMBASE, and Evidence-Based Medicine Reviews was conducted for original studies reporting ocular findings in AHT. Articles were graded using a checklist for systematic reviews of diagnostic accuracy. RESULTS: The initial search yielded 971 articles, of which 55 relevant studies were graded, and 20 studies met inclusion criteria and were included in the review. The overall sensitivity of IOH for AHT was 75% and their specificity was 94%. Intraretinal hemorrhage at the posterior pole was the most common finding, although extensive, bilateral, and multilayered IOH were the most specific for AHT. Optic nerve sheath hemorrhages had a sensitivity and specificity for AHT of 72% and 71%, respectively. Traumatic retinoschisis and perimacular retinal folds were reported in 8% and 14% of AHT, respectively, but were not reported in other conditions. CONCLUSIONS: Prospective, consecutive studies confirm that IOH in infants-particularly bilateral, extensive, and multilayered-are highly specific for AHT. Optic nerve sheath hemorrhages are significantly more common in AHT than in other conditions, in autopsy studies. Traumatic retinoschisis and perimacular folds are present in a minority of AHT, but rarely seen in other conditions.

Outcomes in retinoblastoma, 1974-2005: the Children's Hospital, Westmead.

BACKGROUND: To report the 31-year experience of outcomes in retinoblastoma from a single centre. METHODS: A retrospective analysis of consecutive cases of retinoblastoma diagnosed and treated at the Westmead Children's Hospital, Sydney between 1974 and 2005 was performed. The subjects were analysed as two groups: those diagnosed between 1974 and 1989 (series alpha) and those diagnosed between 1990 and 2005 (series beta). RESULTS: There were a total of 142 patients included in the study, with a median follow up of 72 months. There were 84 patients with unilateral disease and 58 patients (116 eyes) with bilateral disease. The total enucleation rate remained high throughout both series for those with unilateral disease: 89% (series alpha) and 95% (series beta). There was a reduction in enucleations performed for those with bilateral disease from 68.4% (series alpha) to 43.6% (series beta) (P < 0.025). There were no bilateral enucleations performed after 1995. Actuarial Kaplan-Meier curves showed that 56% of all preserved eyes had not recurred at a median follow up of 95 months and 78.1% had avoided enucleation. Overall 43% of preserved eyes attained a visual acuity better than or equal to 6/12 and 55% achieved a visual acuity better than 6/60. There were four deaths due to retinoblastoma. Five patients were diagnosed with a second non-ocular malignancy. The most common treatment-related complications were cataracts, facial deformity, sepsis and febrile neutropaenia. CONCLUSIONS: The introduction of newer globe-preserving treatments for retinoblastoma was associated with equivalent visual outcomes, stable mortality rate and a greater number of short-term complications but avoided the late side-effects associated with external beam radiotherapy.

Ocular adnexal Langerhans cell histiocytosis clinical features and management.

PURPOSE: To review the clinical features, investigations, management, and outcomes of Langerhans cell histiocytosis (LCH) with ocular adnexal involvement. MATERIALS AND METHODS: Retrospective, non-comparative, chart review of 30 patients with LCH involving the ocular adnexa treated at 6 major Australian hospitals. Clinical features, imaging findings, treatment, local and distant recurrence and outcome were evaluated. RESULTS: Twenty-four patients (80%) were male with a mean age of presentation of 9.5 years. Females presented at an earlier age (mean age 4.1 years) with more severe involvement. Eighty percent of the cases presented with periorbital swelling. Computed tomography usually showed destructive osteolytic lesions centred on the frontal bone. Twenty-four patients (80%) had unifocal, unisystem disease. From this group, none of 13 treated with excision alone had recurrence whereas 2 of 6 treated with excision followed by chemotherapy recurred. CONCLUSIONS: Unifocal, unisystem LCH often presents to the ophthalmologist and can usually be diagnosed on clinical and imaging grounds. Computed tomography and magnetic resonance imaging are complementary imaging studies. Biopsy is essential to confirm diagnosis. Unifocal, unisystem disease can be treated with local excision and curettage. Incomplete excision, recurrent disease or multifocal disease may require systemic chemotherapy.

Comparison of silastic and banked fascia lata in pediatric frontalis suspension.

PURPOSE: To compare the use of Silastic and banked fascia lata in pediatric frontalis suspension surgery for functional success, ptosis recurrence, and infection and granuloma rates. METHODS: This retrospective study analyzed the medical records of 72 patients who underwent 131 frontalis suspension operations using either Silastic or banked fascia lata during the past 12 years at Children's Hospital at Westmead, Sydney, Australia. RESULTS: Functional success rates for primary frontalis suspension procedures were not significantly different for banked fascia lata and Silastic (60% versus 67.2%, respectively; P = .4666). Infection and granuloma rates also were not significantly different (7.1% for banked fascia lata versus 15.2% for Silastic; P = .1381). There was, however, a statistically significant difference between the two materials in ptosis recurrence (35.3% for banked fascia lata versus 13% for Silastic; P = .0062). CONCLUSIONS: Silastic was significantly better than banked fascia lata in terms of ptosis recurrence. Both materials were comparable in terms of functional success after one procedure and in infection and granuloma rates. Given the conflicting evidence presented in the literature, large prospective studies are needed to compare the use of the most common synthetic materials with banked fascia lata in pediatric frontalis suspension.

Causes and associations of amblyopia in a population-based sample of 6-year-old Australian children.

OBJECTIVES: To describe the prevalence of amblyopia and associated factors in a representative sample of 6-year-old Australian children. METHODS: Logarithm of minimum angle of resolution visual acuity (VA) was measured in both eyes before and after pinhole correction, correcting cylindrical refractive components greater than 0.50 diopter (D), and with spectacles (if worn) in a population-based sample of 1741 schoolchildren. Retinal pathological abnormalities were excluded based on photographs. Amblyopia was defined using various best-available corrected VA measures in the absence of significant organic pathological abnormalities. RESULTS: Using the criteria of corrected VA less than 20/40 and at least a 2-line difference between eyes, amblyopia was diagnosed in 13 children (0.7%). The inclusion of children with amblyopia who had been successfully treated (n = 19) increased the amblyopia prevalence to 1.8%. Strabismus or strabismus surgery history was present in 37.5% of the children with amblyopia, anisometropia in 34.4%, both conditions in 18.8%, and isoametropia in 6.3%. Mean corrected VA in amblyopic eyes was 37.7 logarithm of minimum angle of resolution letters (Snellen VA equivalent < 20/40), ranging from 0 to 48 logarithm of minimum angle of resolution letters (Snellen VA equivalent < 20/200-20/25). Most amblyopic eyes (58.7%) were significantly hyperopic (spherical equivalent > or = +3.00 D); 8.7% were myopic. CONCLUSIONS: A relatively low prevalence of amblyopia in a sample of 6-year-old children is documented. The majority of these children had already been diagnosed and treated for this condition.

Distribution of ocular biometric parameters and refraction in a population-based study of Australian children.

PURPOSE: To study the distribution of spherical equivalent refraction and ocular biometric parameters in a young Australian population. METHODS: Noncontact methods were used to examine ocular dimensions and cycloplegic autorefraction in a stratified random cluster sample of year 1 Sydney school students (n = 1765), mean age 6.7 years (range, 5.5-8.4 years). Repeated measures of axial length, anterior chamber depth, and greatest and least corneal radius of curvature (CR1, CR2, respectively) were taken in each eye. Refraction was measured as the spherical equivalent. RESULTS: Mean spherical equivalent refraction in right eyes was +1.26 +/- 0.03 D (SEM; range, -4.88 to +8.58). The distribution was peaked (kurtosis 14.4) and slightly skewed to the right (skewness, 1.7). Prevalence of myopia, defined as spherical equivalent refraction < or = -0.5 D, was 1.43% (95% CI, 0.94-2.18) in the overall population. Axial length, anterior chamber depth, and corneal radii of curvature were normally distributed. The mean axial length in right eyes was 22.61 +/- 0.02 mm (SEM; range, 19.64-25.35). The mean anterior chamber depth was 3.34 +/- 0.01 mm (SEM; range, 2.14-4.06). Mean CR1 was 7.85 +/- 0.01 mm (SEM) and mean CR2 was 7.71 +/- 0.01 mm (SEM). The distribution of axial length/mean corneal radius ratio was peaked (leptokurtic) with a mean of 2.906. Mean axial length was longer, anterior chambers were deeper, and corneas were flatter in the boys. CONCLUSIONS: A peaked (leptokurtic) distribution of spherical equivalent refraction was present in this predominantly hyperopic 6-year-old population. The results also showed that ocular biometric measures were normally distributed, with statistically significant gender differences found in measurements.