Transconjunctival XEN45 implantation for secondary open-angle glaucoma management in a pediatric patient with WAGR syndrome.

Purpose: To report a case of XEN45 gel stent implantation in a pediatric patient with WAGR syndrome as a successful surgical intervention in the management of multifactorial secondary open-angle glaucoma. Observations: A 6-year-old female with a history of WAGR syndrome, bilateral congenital aniridia, pseudophakia OD and glaucoma OD, was referred for a XEN45 gel stent OD. IOP was persistently elevated at 24 mm Hg despite two glaucoma medications. Implantation of the XEN45 gel stent was performed using a transconjunctival ab externo approach. There were no significant intra-or-postoperative adverse events associated with the stent. The patient achieved good IOP-lowering control without glaucoma medications across the 18-month follow-up period. Conclusions: A XEN45 stent through a transconjunctival ab externo approach may be an effective surgical intervention in pediatric patients with secondary open-angle glaucoma associated with aniridia and aphakia.

Screening for Retinopathy of Prematurity Through Utilization a Pediatric Retinal Camera at Jim Pattison Children's Hospital: A Vision for Improved Care.

Retinopathy of Prematurity (ROP) is a vascular proliferative disorder of preterm infants, with increased disease severity and incidence occurring with lower gestational age and birth weight. An alternate approach to ROP screening with wide-field digital retinal imaging helps with the early detection of ROP, especially during the pandemic.

Ocular manifestations of xeroderma pigmentosum.

Xeroderma pigmentosum (XP) is an autosomal recessive (AR) condition characterized by photosensitivity and inability to repair ultra-violet (UV) induced DNA damage. Patients diagnosed with XP, presenting to the Paediatric ophthalmology department of Al-Shifa Trust Eye Hospital, Rawalpindi, were evaluated and followed-up over a period of one year, for the effects of the disease process on vision and for the development of ocular tumours. Excision of the tumours, if present, was performed under general anaesthesia. Counselling of the patients was done. Referral to oncologist and dermatologist was made, if so warranted, after histopathology of excision biopsy.

A Clinical Randomized Trial Comparing the Cycloplegic Effect of Cyclopentolate Drops Applied to Closed Eyelids Versus Open Eyelids.

BACKGROUND AND PURPOSE: Adequate cycloplegia and dilation are required for refraction and fundus exam in children. Standard practice is to instill cycloplegic drops in the inferior cul-de-sac, and this is often traumatic for children. Our study assesses the use of cyclopentolate on closed lids as a method of instillation for ensuring complete cycloplegia. PATIENTS AND METHOD: Ninety children presenting for annual refraction were enrolled. Three were excluded as they did not finish the testing. One drop of Alcaine® and one drop of cyclopentolate HCL 1% were used in each eye. Cyclopentolate drops were placed on the inner canthus near the lid margin on the closed eye and directly onto the conjunctiva of the fellow eye. RESULTS: Overall, 145/174 eyes (83%) were fully cyclopleged with one drop. The methods of instillation were equally successful (seventy-two indirect vs. seventy-three direct). Age, eye color, spherical refractive error, astigmatic refractive error, and presence of amblyopia on the study visit played no role in the success of either method. Dark irises where the pupil margin was clinically indistinguishable had the largest number of failures (n = 17/44) in comparison to light irises (12/130), but had an equal amount of failures for both direct and indirect methods. CONCLUSION: Placing one drop of cyclopentolate HCL 1% on a closed eyelid had a success rate for complete cycloplegia that was equivalent to placing one drop directly on the cornea.

Color visual evoked potentials in children with type 1 diabetes: relationship to metabolic control.

PURPOSE: To examine the association between metabolic control (HbA(1c)) and the chromatic mechanisms of children with type 1 diabetes (T1D), by using the color visual evoked potential (VEP). METHODS: Fifty children with T1D (age range, 6-12.9 years) and 33 age-matched control subjects were tested. VEPs were recorded by placing five electrodes on the scalp according to the International 10/20 System of Electrode Placement. Active electrodes O1, O2, and Oz were placed over the visual cortex. Short-wavelength (S), and long- and medium-wavelength (LM) color stimuli consisted of vertical, photometric isoluminant (1 cyc/deg) gratings presented in a pattern onset (100 ms)-offset (400 ms) mode. Achromatic vertical gratings were presented at 3 cyc/deg. Primary outcome measure was VEP latency. The relationship between S, LM, and achromatic VEP latency, and HbA(1c) was determined by ANCOVA regression. RESULTS: S-, LM-, achromatic VEP latencies were not associated significantly with HbA(1c). Pubertal status, however, was associated significantly (P = 0.0114) and selectively with S-VEP latency. Pubertal children with T1D had delayed (mean delay, 9.5 ms) S-VEP latencies when compared with the prepubertal children with T1D. However, there was no statistically significant difference (P = 0.1573) in the effect of pubertal status on S-VEP latency between the T1D and control groups. CONCLUSIONS: Pubertal status rather than HbA(1c) appears to affect selectively the S-VEP latency of preteen children with T1D. Further study is warranted to determine whether the delay in S-VEP latency in pubertal children with T1D changes over time and whether this change could be a predictive marker for future development of background diabetic retinopathy.

Management and outcomes of cataract in children: the Toronto experience.

PURPOSE: To review the management and outcomes of pediatric patients with cataract who were managed by one of two surgeons at a single institution. METHODS: An observational series of consecutive cases identified from the hospital's outpatient billing records and surgical data program were used. Visual acuity was measured with the Snellen or Allen charts. Glaucoma was defined as IOP >20 mm Hg with clinical signs of glaucoma or visual field loss. Procedures for aphakic/pseudophakic glaucoma were excluded from analysis of additional surgeries performed subsequent to cataract extraction. RESULTS: The search identified 778 patients (1,122 eyes) diagnosed with cataract over 10 years. Of these, 74% of eyes were treated surgically. Those patients with total, nuclear, and lamellar cataracts were significantly more likely than the overall population to undergo surgery. Additional surgeries were required in 12% of surgically treated eyes, with pseudophakic eyes representing more than one-half. Aphakic and pseudophakic glaucoma prevalence were 12% and 1%, respectively. Cataract morphology was not found to be a predisposing factor in the development of glaucoma. Visual outcomes were significantly better for posterior subcapsular (P = 0.0001), nuclear (P = 0.025), lamellar (P = 0.03), and traumatic cataracts (P = 0.005) than for other morphological types at all ages. Visual acuity was 20/30 or better in 63% of children with unilateral pseudophakia, 45% of children with unilateral aphakia, and approximately 75% of children with bilateral aphakia and pseudophakia. CONCLUSIONS: Patients with total, nuclear, and lamellar cataracts were more likely to undergo surgery. Approximately 10% of patients required additional surgeries. No cataract morphology predisposed patients to developing glaucoma. Good visual outcomes were attained in bilaterally pseudophakic/aphakic and unilaterally pseudophakic children.

Macular and peripapillary retinal nerve fibre layer thickness in adults with amblyopia.

OBJECTIVE: To evaluate macular and peripapillary retinal nerve fiber layer (RNFL) thickness in amblyopic eyes compared to the fellow eye. DESIGN: Cross-sectional study. PARTICIPANTS: 30 patients (60 eyes) older than 18 years of age with amblyopia. METHODS: Inclusion criteria included individuals older than 18 years, amblyopia, and best-corrected visual acuity (BCVA) ≤ 20/40. A complete medical history was taken and an eye examination carried out. Optical coherence tomography (OCT) was carried out on both eyes of all patients. Exclusion criteria included intraocular pressure (IOP) >23 mm Hg and eye pathology that may affect OCT measurements. The primary outcome measures were foveal thickness and average peripapillary RNFL thickness, which were compared using a paired t test. Quadrants in peripapillary scans and concentric rings in macular scans were analyzed. RESULTS: The average age was 56 years (range = 33-82 years). Visual acuity ranged from 20/40 to 20/4000 (mean = 20/275). The average peripapillary RNFL thickness was 90.6 µm (SD = 9.6 µm) in the amblyopic eye and 90.1 µm (SD = 12.1 µm) in the fellow eye (p = 0.64). The average macular thickness in amblyopic eyes was 260.1 µm (SD = 32.0 µm), and 254.7 µm (SD = 32.5 µm) in fellow eyes (p = 0.10). No statistical difference existed between peripapillary quadrants or macular concentric rings. These differences were smaller when the strabismic amblyopes were isolated. CONCLUSIONS: There does not seem to be a difference in peripapillary RNFL or macular thickness between the amblyopic eye and fellow eye.

Pediatric cataract: the Toronto experience-etiology.

PURPOSE: To study the etiology and characteristics of pediatric cataracts in a large sample from 1 institution. DESIGN: Observational case series. METHODS: The study population included 778 consecutive cases (1122 eyes) of children presenting with any type of pediatric cataract over a 10-year period. Age at presentation, laterality, cataract morphology and etiology, and presence of strabismus were recorded. Association with systemic diseases or syndromes and coexistent ocular anomalies were noted. Snellen or Allen picture charts were used to record visual acuity, when able. RESULTS: Unilateral cataract accounted for slightly over half of the cases. The etiology was idiopathic in the majority of patients. The most common systemic association was diseases with steroid treatment-induced cataracts. Cataracts, when associated with other isolated ocular anomalies, tended to occur unilaterally. Among syndrome-associated cataracts, Down syndrome represented a third of the cases. Posterior subcapsular cataract was the most common morphologic type. Almost half of the patients presented with Snellen 6/9 or better or "central, steady, maintained" vision where it was not possible to obtain quantitative vision data. CONCLUSIONS: Despite the diverse nature of cases with frequent systemic associations, unilateral cataracts and idiopathic etiology accounted for the majority of cases.