Identification of pathogenic genetic variants in patients with acquired early-onset bilateral cataracts using next-generation sequencing.

BACKGROUND: Acquired early-onset bilateral cataracts can result from systemic etiologies or genetic disorders. METHODS: In this observational study, we analyzed individuals 18 months to 35 years of age with acquired bilateral cataracts via a next-generation sequencing panel of 66 genes to identify disease-causing genetic variants. RESULTS: Of 347 patients enrolled, 313 (90.2%) were <19 years (median, 8 years). We identified 74 pathogenic or likely pathogenic variants in 69 patients. Of the variants, we observed 64 single nucleotide variants (SNV) in 24 genes and 10 copy number variants (CNV) of varying size and genomic location. SNVs in crystallin genes were most common, accounting for 27.0% of all variants (20 of 74). Of those, recurrent variants included known cataract-causing variants CRYBA1 c.215+1G>A, observed in 3 patients, and CRYBA1 c.272_274delGAG, CRYBB2 c.463C>T and c.562C>T, and CRYAA c.62G>A, each observed in 2 patients. In 5 patients, we identified CNV deletions ranging from 1.32-2.41 Mb in size associated with 1q21.1 microdeletion syndrome. Biallelic variants in CYP27A1 were identified in two siblings, one as part of targeted follow-up family testing, who were subsequently diagnosed with cerebrotendinous xanthomatosis, a rare but treatable autosomal recessive disease that often presents with acquired early-onset bilateral cataracts. CONCLUSIONS: This study demonstrates the utility of genetic testing in individuals with acquired early-onset bilateral cataracts to help clarify etiology. Identification of causative genetic variants can inform patient management and facilitate genetic counseling by identifying genetic conditions with risk of recurrence in families.

Timing of Diagnosis and Treatment of Glaucoma following Infantile Cataract Surgery.

PURPOSE: To report timing of diagnosis and treatment of glaucoma following cataract surgery (GFCS) in a large cohort of infants undergoing cataract surgery at a tertiary care center. STUDY DESIGN: Cross-sectional study. PARTICIPANTS: All consecutive infants that underwent cataract surgery over a 30-year period from January 1991 to December 2021 were included if they had at least 1 year follow-up. METHODS: The data collection included age at time of cataract surgery, presence of associated ocular or systemic conditions, age at diagnosis of GFCS, and treatment required to control GFCS. Glaucoma diagnosis required intraocular pressure (IOP) > 21 mmHg on > 2 visits with glaucomatous optic nerve head changes and/or visual field changes, or in young children, other anatomic changes such as corneal enlargement or haze or accelerated axial elongation and myopic shift. MAIN OUTCOME MEASURES: The incidence of GFCS was calculated. Linear regression was performed to assess the effect of age at time of cataract surgery. Analysis of risk factors and treatment modalities was performed using univariate and multivariate analysis. RESULTS: Three hundred eighty-three eyes (260 patients) were analyzed. Median age at surgery was 52 days and median follow-up, 8 years. Glaucoma following cataract surgery was noted in 27% (104/383 eyes; median age at surgery, 45 days; median follow-up, 13 years.) Young age at surgery (< 3 months) was the greatest risk factor (P = 0.001) but the incidence was similar for infants operated in the first, second, or third month of life (25%, 36%, 40%, respectively, P = 0.4). Microcornea (41%, P < 0.0001), poorly dilating pupils (25%, P = 0.001), persistent fetal vasculature (PFV, 13%; P = 0.8), or anterior segment dysgenesis (3%, P = 0.02) were considered as additional risk factors. Surgical intervention was needed for 73% (24/33) eyes with early-onset GFCS compared with 14% (10/71) eyes with later-later onset GFCS (P < 0.0001). Medical treatment was effective in 86% with later-onset GFCS (P = 0.006). CONCLUSIONS: The incidence of GFCS was 27%, and timing of diagnosis occurred in a bimodal fashion. Early-onset GFCS usually requires surgical intervention; medical treatment is effective for later-onset GFCS. Cataract surgery within the first 3 months of life, microcornea, and poorly dilating pupils were major risk factors. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.

Prevalence of cerebrotendinous xanthomatosis among patients diagnosed with early-onset idiopathic bilateral cataracts: final analysis.

Cerebrotendinous xanthomatosis (CTX) is a rare, autosomal recessive bile acid synthesis disorder caused by pathologic variants in CYP27A1, a gene involved in bile acid synthesis. Impaired function in this gene leads to accumulation of plasma cholestanol (PC) in various tissues, often in early childhood, resulting in such clinical signs as infantile diarrhea, early-onset bilateral cataracts, and neurological deterioration. The current study aimed to identify cases of CTX in a population of patients with a greater CTX prevalence than the general population, to facilitate early diagnosis. Patients diagnosed with early-onset, apparently idiopathic, bilateral cataracts between the ages of 2 and 21 years were enrolled. Genetic testing of patients with elevated PC and urinary bile alcohol (UBA) levels was used to confirm CTX diagnosis and determine CTX prevalence. Of 426 patients who completed the study, 26 met genetic testing criteria (PC ≥ 0.4 mg/dL and positive UBA test), and 4 were confirmed to have CTX. Prevalence was found to be 0.9% in enrolled patients, and 15.4% in patients who met the criteria for genetic testing.

Durable recovery from amblyopia with donepezil.

An elevated threshold for neuroplasticity limits visual gains with treatment of residual amblyopia in older children and adults. Acetylcholinesterase inhibitors (AChEI) can enable visual neuroplasticity and promote recovery from amblyopia in adult mice. Motivated by these promising findings, we sought to determine whether donepezil, a commercially available AChEI, can enable recovery in older children and adults with residual amblyopia. In this open-label pilot efficacy study, 16 participants (mean age 16 years; range 9-37 years) with residual anisometropic and/or strabismic amblyopia were treated with daily oral donepezil for 12 weeks. Donepezil dosage was started at 2.5 or 5.0 mg based on age and increased by 2.5 mg if the amblyopic eye visual acuity did not improve by 1 line from the visit 4 weeks prior for a maximum dosage of 7.5 or 10 mg. Participants < 18 years of age further patched the dominant eye. The primary outcome was visual acuity in the amblyopic eye at 22 weeks, 10 weeks after treatment was discontinued. Mean amblyopic eye visual acuity improved 1.2 lines (range 0.0-3.0), and 4/16 (25%) improved by ≥ 2 lines after 12 weeks of treatment. Gains were maintained 10 weeks after cessation of donepezil and were similar for children and adults. Adverse events were mild and self-limited. Residual amblyopia improves in older children and adults treated with donepezil, supporting the concept that the critical window of visual cortical plasticity can be pharmacologically manipulated to treat amblyopia. Placebo-controlled studies are needed.

Comparison of baseline biometry measures in eyes with pediatric cataract to age-matched controls.

PURPOSE: To compare baseline biometry measurements in eyes with pediatric cataract versus age-matched controls METHODS: This is a cross-sectional study conducted at a tertiary care hospital that included two arms-prospective arm to collect data from normal eyes and retrospective arm for eyes with pediatric cataract. In the prospective arm, biometry measurements were obtained in healthy children aged 0 to 10 years. Children under the age of four had measurements under anesthesia for an unrelated procedure, while older children had in-office measurements using optical biometry. For comparison, biometric data was collected in children with pediatric cataract through record review. One eye of each patient was randomly selected. Axial length (AL) and keratometry (K) were compared by age and laterality. The medians were compared using Wilcoxon rank-sum tests and variances using Levene's test. RESULTS: There were 100 eyes in each arm, 10 eyes in each age bin of 1-year interval. There was more variability in baseline biometry in eyes with pediatric cataract and a trend for longer AL and steeper K in cataract eyes than aged-matched controls. The difference in AL means was significant in age group 2-4 years, and variances were significant across all age groups (p=0.018). Unilateral cataracts (n=49) showed a trend toward greater variability in biometry than bilateral cataracts, but this did not reach statistical significance. CONCLUSION: Baseline biometry measures are more variable in eyes with pediatric cataract compared to age-matched controls with a trend toward longer AL and steeper K.

Axial length and corneal curvature of normal eyes in the first decade of life.

BACKGROUND/AIMS: To establish normative curves for axial length and corneal curvature in the first decade of life. METHODS: This is a cross-sectional study from a single institution in the United States. Children from 0- to 10-years of age with no underlying ocular pathology were prospectively enrolled to obtain ultrasound biometry and hand-held keratometry while under anaesthesia for an unrelated procedure. Older cooperative children had optical biometry obtained in-office. Logarithmic quantile regression models were used to determine the change in axial length and average keratometry as a function of age. RESULTS: Single-eye measurements from 100 children were included. 75% of children were White and 49% female. Median axial length ranged from 20.6 mm (IQR, 20.2 to 21.1 mm) at age one year to 23.1 mm (IQR, 22.5 to 23.8 mm) at age ten years. Median average keratometry ranged from 44.1 D (IQR, 42.6 to 45.4 D) at age one year to 43.5 (IQR, 42.2 to 44.0 D) at age ten years. As age increased, there was a significant increase in axial length (0.74 mm per doubling of age; 95% CI, 0.62 to 0.82 mm), and a non-significant trend towards lower average keratometry (-0.21 D per doubling of age; 95% CI, -0.62 to 0.08 D). CONCLUSIONS: We provide a set of normative charts for axial length and corneal curvature which may facilitate the identification of eyes outside the normal range and assist in the management of ocular conditions such as glaucoma or cataract.

Paediatric anterior uveitis management in the USA: a single-centre, 10-year retrospective chart review exploring the efficacy and safety of systemic immunomodulatory therapy.

OBJECTIVE: To evaluate the efficacy of immunomodulatory therapy (IMT) in paediatric anterior uveitis. METHODS: Chart review of all patients ≤ 18 years treated for anterior uveitis using a stepladder approach during a 10-year period. The type and duration of IMT were noted. The data were analysed depending on chronicity, aetiology, and type of IMT using appropriate statistical tests. The outcome measures included ocular complications, the need for surgical intervention, and visual outcomes. RESULTS: One hundred and thirty-four patients (191 eyes) were analyzed. The median age at diagnosis was 7 years (interquartile range (IQR): 7.5 years). The median follow-up was 4 years (IQR: 6 years). The most common causes of anterior uveitis were Juvenile idiopathic arthritis (64 patients, 47.8%) and undifferentiated (33 patients, 24.6%). All patients were started on topical steroids and cycloplegics. 94 (70%) patients required IMT. 92 (68.6%) were started on Methotrexate as the first agent, of which 21 (22%) were switched to a different agent owing to side effects. Biologic agent was added in 55 (41%) patients. 21 (16%) required switch to a second biologic agent, 5 (3.7%) to third, and 1 (0.8%) to fourth biologic agent. At the last exam, 11 (8%) had persistent inflammation. 55 (41%) had ocular complications, and 113 (84%) had a best corrected visual acuity ≥ 20/40. CONCLUSION: Early introduction of IMT and switch to different agents may be required to control anterior uveitis and reduce the complications in children. IMT is safe and effective in treating paediatric anterior uveitis.

Systemic and ocular associations in pediatric patients undergoing cataract surgery.

PURPOSE: Evaluation for systemic diagnosis is an important part of pediatric cataract management. While there are reports on associated systemic and ocular associations in children with infantile cataracts, reports specifying associations in large cohorts of children undergoing cataract surgery are lacking. METHODS: Retrospective chart review of consecutive patients undergoing cataract surgery at a pediatric tertiary referral center during 30-year period was performed. Associated systemic and ocular associations were recorded. The etiologies were analyzed depending on laterality, age, and gender. RESULTS: Seven-hundred twenty-seven patients (1135 eyes) were included for analysis: 408 (56%) with bilateral and 319 (44%) with unilateral cataract. An identifiable cause for cataract was identified in 66% (270/408) bilateral and 55% (176/319) unilateral cataract patients. Hereditary cataract accounted for 22% of bilateral cataracts. An underlying syndrome or genetic diagnosis was found in 24% bilateral (97/408, 86 genetic/syndromic, 11 metabolic) but only in 2% of unilateral cases (5/319). Cataracts were the result of treatment for cancer, or other systemic conditions requiring steroids, in 60/408 bilateral (15%) and 15/319 (5%) unilateral cataract patients. In contrast, unilateral cataracts had higher ocular associations (49%, 156/319) than bilateral cataracts (6%, 23/408) primarily ocular trauma (20%, 64/319) and persistent fetal vasculature (20%, 62/319). CONCLUSION: Clinicians should be aware of potential systemic and ocular associations among children with visually significant cataracts. Those with no family history of juvenile cataract should be evaluated for systemic associations, and referral to genetics may be warranted in select cases.

Deviations From Age-Adjusted Normative Biometry Measures in Children Undergoing Cataract Surgery: Implications for Postoperative Target Refraction and IOL Power Selection.

PURPOSE: To evaluate whether pediatric eyes that deviate from age-adjusted normative biometry parameters predict variation in myopic shift after cataract surgery. METHODS: This is a single institution longitudinal cohort study combining prospectively collected biometry data from normal eyes of children <10 years old with biometry data from eyes undergoing cataract surgery. Refractive data from patients with a minimum of 5 visits over ≥5 years of follow-up were used to calculate myopic shift and rate of refractive growth. Cataractous eyes that deviated from the middle quartiles of the age-adjusted normative values for axial length and keratometry were studied for variation in myopic shift and rate of refractive growth to 5 years and last follow-up visit. Multivariable analysis was performed to determine the association between myopic shift and rate of refractive growth and factors of age, sex, laterality, keratometry, axial length, intraocular lens power, and follow-up length. RESULTS: Normative values were derived from 100 eyes; there were 162 eyes in the cataract group with a median follow-up of 9.6 years (interquartile range: 7.3-12.2 years). The mean myopic shift ranged from 5.5 D (interquartile range: 6.3-3.5 D) for 0- to 2-year-olds to 1.0 D (interquartile range: 1.5-0.6 D) for 8- to 10-year-olds. Multivariable analysis showed that more myopic shift was associated with younger age (P < .001), lower keratometry (P = .01), and male gender (P = .027); greater rate of refractive growth was only associated with lower keratometry measures (P = .001). CONCLUSIONS: Age-based tables for intraocular lens power selection are useful, and modest adjustments can be considered for eyes with lower keratometry values than expected for age.

Long-term outcomes of secondary intraocular lens implantation in children.

PURPOSE: This study aims to report long-term outcomes of secondary intraocular lens (IOL) implantation after early cataract surgery in children. METHODS: This is a retrospective case series that included children undergoing secondary IOL implantation. The patients had either in-the-bag (ITB) or sulcus implantation; alternative methods of IOL fixation were excluded. Single-piece acrylic IOL was used for ITB and 3-piece acrylic or PMMA IOL for sulcus implantation. The visual acuity outcomes and rate of complications at the last follow-up visit were evaluated. RESULTS: One hundred six eyes (70 patients) were analyzed. The mean follow-up was 5.5 ± 3.8 years. Sixty-two eyes (58.5%) had ITB; 44 eyes (41.5%) had sulcus IOL. All but 3 eyes (97.2%) showed stable or improvement in visual acuity. Early inflammation > grade 2 + was noted with sulcus IOL (84% vs 34%, p = 0.01); late inflammation requiring vitrectomy occurred in one eye with sulcus IOL. Mild decentration was seen in 2 eyes with sulcus IOL; one additional subluxed sulcus IOL was exchanged. Sixteen out of 106 eyes (16%) had glaucoma. Eyes that developed glaucoma had early primary surgery (mean, 0.2 years, p < 0.001, significant); there was no difference in glaucoma rates based on implantation site. CONCLUSION: Early postoperative inflammation is higher in eyes with sulcus implantation, but good visual acuity outcomes are noted after secondary IOL implantation in children. Glaucoma is the main complication that requires close monitoring and is associated with early age at primary surgery.

Pseudo-Duane retraction syndrome after orbital myositis.

Orbital myositis is a rare, commonly idiopathic, inflammatory condition that affects one or more extraocular muscles. We present a case of unilateral orbital myositis affecting the lateral rectus muscle presenting with gaze-evoked amaurosis, pain, and diplopia, with restrictive limitation of adduction. With improvement in adduction after initiating treatment, we noted narrowing of the palpebral fissure on attempted adduction, mimicking Duane retraction syndrome (DRS). Reported cases of "pseudo-DRS" are associated with multiple etiologies and are characterized by retraction on attempted abduction rather than adduction, as occurs in true DRS. In this case, pseudo-DRS occurred in the setting of idiopathic orbital inflammatory syndrome (orbital myositis) with a motility pattern more consistent with true DRS.

Goal-determined metrics to assess outcomes of esotropia surgery.

PURPOSE: To report outcomes of esotropia surgery with a goal-determined tool. METHODS: A goal-determined outcomes analysis tool was devised to address a quality improvement initiative at Boston Children's Hospital. Surgeons preoperatively ranked four possible goals for intervention: enhancement of binocular potential, restoration of eye contact (reconstructive), management of diplopia, and resolution of torticollis. Criteria for success were goal specific; the primary outcome measure was surgical success at 2-4 months. Secondary outcomes included appraisal of risk factors and a comparison of outcomes with this methodology versus traditional criteria for success based on motor alignment. No patients were excluded based on diagnosis, systemic and ocular risk factors, or intervention performed. RESULTS: A total of 824 patients underwent esotropia surgery from 2006 to 2012 and returned for evaluation at 2-4 months' follow-up. Of these, 777 had sufficient documentation for inclusion: 372 procedures were performed primarily to improve binocular potential; 238, to restore eye contact; 124, to resolve diplopia; and 43, to remediate torticollis. Excellent (71%) or good (13.7%) results were obtained in 84% of cases. Without associated risk factors, 75% had excellent and 14% had good outcomes. Risk factors were present in 444 (57%). Success diminished with prior strabismus surgery (P = 0.004), preoperative angle ≥50(Δ) (P = 0.002), and surgery before 12 months of age (P = 0.003). Patients having surgery to remediate diplopia had the best outcomes (excellent, 79%; good, 8%). Preoperative ranking of goals allowed demonstration of better results than would have been reported with requirement of "traditional" motor alignment criteria (P = 0.009). CONCLUSIONS: Goal-determined methodology can be useful for monitoring outcomes of esotropia surgery in diverse populations.

Pediatric genetic disorders of lens.

Pediatric genetic disorders of lens include various cataractous and non-cataractous anomalies. The purpose of this review is to help determine the genetic cause based on the lens appearance, ocular and systemic associations. Children with bilateral cataracts require a comprehensive history, ophthalmic and systemic examination to guide further genetic evaluation. With advancements in genetics, it is possible to determine the genetic mutations and assess phenotype genotype correlation in different lens disorders. The genetic diagnosis helps the families to better understand the disorder and develop realistic expectations as to the course of their child's disorder.

Uncorrected visual acuity in children with monofocal pseudophakia.

PURPOSE: To report uncorrected distance and near visual acuity in pediatric eyes treated with primary monofocal intraocular lens (IOL) implantation. SETTING: Boston Children's Hospital, Boston, Massachusetts, USA. DESIGN: Retrospective chart review. METHODS: Records of children older than 5 years who had uneventful monofocal IOL implantation targeted for emmetropia within ± 1.00 diopter (D) spherical equivalent were reviewed. Eyes with secondary IOL placement and sulcus-fixated IOLs were excluded. Vision was tested using the Mentor BVAT or M&S system for distance and the standard Jaeger near card for near visual acuity. Keratometry, axial length, type of IOL, and preoperative and postoperative refractions were recorded. The main outcome measure was uncorrected visual acuity at distance and near. Good visual acuity was defined as 20/40 or better. RESULTS: Forty-one eyes of 25 children had uncorrected distance and near visual acuity recorded in the early postoperative period. The mean age was 11.2 years ± 3.6 (SD). Twenty eyes (49%) had good visual acuity at distance and near, 11 had good visual acuity at distance only, 6 had good visual acuity at near only, and 4 had worse than 20/40 at distance and near. Thirty eyes (70%) had with-the-rule astigmatism. All but 2 eyes (95%) had 20/70 or better uncorrected acuity at distance and near. In children with bilateral pseudophakia, 12 (75%) of 16 had 20/40 or better uncorrected acuity at distance and near. CONCLUSION: Monofocal IOL placement resulted in good uncorrected distance and near visual acuity in almost 50% of pediatric eyes and in 75% of patients when targeted within ± 1.00 D of emmetropia.

Secondary intraocular lens implantation after pediatric aphakia.

PURPOSE: To describe our technique for secondary intraocular lens (IOL) implantation in aphakic children and report patient outcomes. PATIENTS AND METHODS: In our technique for in-the-bag IOL implantion, we used a microvitreoretinal (MVR) blade to separate the capsular leaflets for aspiration of reproliferated lens material within Soemmering's ring. When sufficient capsular opening was not possible or capsular support deemed inadequate, an IOL was implanted in the sulcus. Records of patients with secondary IOL implantation between 1999 and 2009 were retrospectively reviewed with regard to intra- and postoperative complications and visual and refractive outcomes. RESULTS: A total of 50 consecutive eyes of 32 patients were evaluated. Mean age at secondary IOL implantation was 9.1 years. Of these, 26 eyes had in-the-bag IOLs and 24 had sulcus IOLs. Forty-four eyes (88%) had an AcrySof IOL (Alcon Inc, Ft. Worth, TX). The prevalence of any corneal edema (54% vs 19%, P = 0.04) and uveal inflammation >2+ (92% vs 31%, P < 0.05) in immediate postoperative period was greater with sulcus IOL than in-the-bag placement. Late inflammation at 6 months developed in 1 eye, and glaucoma developed in 2 eyes with sulcus IOLs. All eyes maintained or gained lines of best-corrected visual acuity. Mean absolute prediction error was 0.9 ± 0.9 with no significant difference between in-the-bag and sulcus placement. CONCLUSIONS: Eyes achieving secondary in-the-bag IOL showed less inflammation and corneal edema in the early postoperative period partly as the result of greater surgical and anatomic challenges in some eyes receiving sulcus IOL. Visual and refractive outcomes were satisfactory in eyes with secondary IOL implantation. Mean prediction error was similar for in-the-bag and sulcus IOLs.

Crystalline cataract caused by a heterozygous missense mutation in γD-crystallin (CRYGD).

PURPOSE: To describe phenotypic characteristics of two pedigrees manifesting early onset crystalline cataract with mutations in the γD-crystallin gene (CRYGD). METHODS: A detailed medical history was obtained from two Caucasian pedigrees manifesting autosomal dominant congenital cataracts. Genomic DNA was extracted from saliva (DNA Genotek). Single Nucleotide Polymorphism (SNP) based genome analysis of the larger pedigree revealed linkage to an 8.2 MB region on chromosome 2q33-q35 which encompassed the crystallin-gamma gene cluster (CRYG). Exons and flanking introns of CRYGA, CRYGB, CRYGC and CRYGD were amplified and sequenced to identify disease-causing mutations. RESULTS: A morphologically unique cataract with extensive refractile "crystals" scattered throughout the nucleus and perinuclear cortex was found in the probands from both pedigrees. A heterozygous C→A mutation was identified at position 109 of the coding sequence (R36S of the processed protein) in exon 2 of CRYGD and this missense mutation was found to cosegregate with the disease in the larger family; this mutation was then identified in affected individuals of pedigree 2 as well. CONCLUSIONS: The heterozygous 109C→A CRYGD missense mutation is associated with a distinct crystalline cataract in two US Caucasian pedigrees. This confirms crystalline cataract formation with this mutation, as previously reported in sporadic childhood case from the Czech Republic and in members of a Chinese family.

Technological advances in pediatric cataract surgery.

PURPOSE: Managing pediatric cataracts is often challenging. It is technically difficult to perform surgery in these small complaint eyes and there is higher prevalence of postoperative complications. The outcomes of surgery depend on technique and technology. The purpose of this study is to review the current literature on technological advances in pediatric cataract surgery. METHODS: Review of literature on management of pediatric cataract surgery. RESULTS: The advent of vitrectomy machines and intraocular lenses (IOLs) has revolutionized pediatric cataract surgery. "Vitrectorhexis" has become a good alternative to manual capsulorhexis. Primary management of posterior capsule and limited anterior vitrectomy has reduced the incidence of visual axis opacification. Primary IOL implantation is becoming a standard of care in the youngest children. Single piece Acrysof® is preferred for in-the-bag implantation and 3-piece Acrysof® for sulcus implantation. Newer IOLs are being evaluated in pediatric eyes. Precise measurement of intraocular lens power and predicting refractive change are major challenges in long term care of children after surgery. CONCLUSION: New technology and surgical techniques have refined pediatric cataract surgery. It is critical to focus our efforts on precise biometry measurements, IOL power calculation, and designing IOLs that may address refractive changes in the growing pediatric eye.

Comparison of intraocular lens power calculation formulae in pediatric eyes.

PURPOSE: To evaluate accuracy of intraocular lens (IOL) power calculation formulae (SRK II, SRK/T, Holladay 1, Hoffer Q) in pediatric eyes. DESIGN: Retrospective case series. PARTICIPANTS: One hundred thirty-five eyes of 96 children with congenital, developmental, or acquired cataracts who underwent uncomplicated cataract surgery and IOL implantation by a single surgeon over a 10-year period. METHODS: Axial length (AL), keratometry (K), and manufacturer's A constant were employed in 4 common IOL power calculation formulae to predict the refractive outcome. Retinoscopy was measured at 4 to 8 weeks postoperatively and converted to spherical equivalent. For analysis, eyes were grouped by age at surgery, AL, and mean K. MAIN OUTCOME MEASURES: We determined the prediction error (PE) = predicted refraction - actual refraction and the absolute PE = |predicted refraction - actual refraction|. The formula that gave the best prediction (minimum PE) was determined. RESULTS: The mean age at surgery was 6.4 years. Mean absolute PE was 1.11 for the SRK II, 0.84 for SRK/T, 0.76 for Holladay, and 0.76 for Hoffer Q formulae. There was a trend toward greater PE in eyes of younger children (< or =2 years), shorter AL (AL < or = 22 mm) and steeper corneas (mean K > 43.5 diopters [D]). On comparing absolute PE obtained with 4 formulae in each patient, Hoffer Q gave the minimum PE in 46% of eyes compared with 23% with SRK II, 18.5% with SRK/T, and 12.5% with Holladay 1. The SRK/T, Holladay 1, and Hoffer Q were similar in accurately predicting refractive error within +/-0.5 D in about 43% eyes. When clinically significant deviation in PE occurred (>0.5 D), there was usually an undercorrection (72%), except for Hoffer Q, which was almost as likely to overcorrect as undercorrect (44% vs 56%). The PE was lower with office measurements when compared with anesthesia measurements, owing probably to better fixation in older children with higher ALs. CONCLUSION: The PE was insignificant (PE < or = 0.5 D) in 43% eyes, and similar for all formulae. However, the Hoffer Q was predictable for the highest number of eyes. When the PE was >0.5 D, most formulae gave an undercorrection, except for the Hoffer Q, which the surgeon may want to consider when targeting postoperative refractions.