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Purpose To compare rates and severity of complications between infants undergoing cataract surgery with and without intraocular lens (IOL) implantation. Design Prospective randomized clinical trial. Methods A total of 114 infants were enrolled in the Infant Aphakia Treatment Study, a randomized, multi-center (12) clinical trial comparing the treatment of unilateral aphakia in patients under 7 months of age with a primary IOL implant or contact lens. The rate, character, and severity of intraoperative complications, adverse events, and additional intraocular surgeries during the first 5 postoperative years in the 2 groups were examined. Results There were more patients with intraoperative complications (28% vs 11%, P = .031), adverse events (81% vs 56%, P = .008), and more additional intraocular surgeries (72% vs 16%, P Conclusion The increased rate of complications, adverse events, and additional intraocular surgeries associated with IOL implantation in infants

Objective To test the hypothesis that the more severe the acute phase retinopathy of prematurity (ROP) was in the preterm weeks, the more severely compromised is rod photoreceptor function after the ROP has resolved. Methods Electroretinographic (ERG) responses were recorded from 25 dark-adapted children (ages 2.5 months' postterm to 14 years) categorized by maximum, acute phase ROP (None to Very Severe). From the ERG a-wave "S," a sensitivity parameter for the rod photoreceptor response, and R(mp3), the saturated amplitude of the rod photoreceptor response were calculated using a model of the activation of rod phototransduction. The patients' results were compared with those of healthy controls (n = 71). Results Among those in the None, Mild, Moderate, and Severe categories, both S and R(mp3) varied significantly with severity of acute phase ROP. In the Very Severe category, ERG responses were too attenuated to calculate S and R(mp3). Conclusions The rod photoreceptors must be involved in ROP. The more severe the acute phase ROP, the more severe is the compromise of the processes involved in the activation of phototransduction in the rods.

Cataracts are a significant public health problem. Here, we describe the genetic alteration responsible for a progressive form of cataract, segregating as an autosomal dominant trait in a three-generation pedigree. Unlike most autosomal dominant cataracts, these are not clinically apparent at birth but are initially observed in the first year or two of life. The opacification evolves relatively slowly, generally necessitating removal of the lens in childhood or early adolescence. A genome-wide search in our kindred revealed linkage at 2q33–35 where the γ-crystallin gene cluster resides. A single base alteration resulting in an Arg- 14 → Cys (R14C) substitution in γD-crystallin was subsequently identified. Protein modeling suggests that the effect of this mutation is a subtle one, affecting the surface properties of the crystallin molecule rather than its tertiary structure, consistent with the fact that the patients’ lenses are normal at birth. This is the first gene defect shown to be responsible for a noncongenital progressive cataract, and studying the defective protein should teach us more about the mechanisms underlying cataract formation.

Purpose To compare accuracy of intraocular lens (IOL) power calculation formulae in infantile eyes with primary IOL implantation. Design Comparative case series. Methods The Hoffer Q, Holladay 1, Holladay 2, Sanders-Retzlaff-Kraff (SRK) II, and Sanders-Retzlaff-Kraff theoretic (SRK/T) formulae were used to calculate predicted postoperative refraction for eyes that received primary IOL implantation in the Infant Aphakia Treatment Study. The protocol targeted postoperative hyperopia of +6.0 or +8.0 diopters (D). Eyes were excluded for invalid biometry, lack of refractive data at the specified postoperative visit, diagnosis of glaucoma or suspected glaucoma, or sulcus IOL placement. Actual refraction 1 month after surgery was converted to spherical equivalent and prediction error (predicted refraction − actual refraction) was calculated. Baseline characteristics were analyzed for effect on prediction error for each formula. The main outcome measure was absolute prediction error. Results Forty-three eyes were studied; mean axial length was 18.1 ± 1.1 mm (in 23 eyes, it was Conclusions The Holladay 1 and SRK/T formulae gave equally good results and had the best predictive value for infant eyes.

OBJECTIVES To understand retinopathy of prematurity (ROP) follow-up care for preterm very low-birth-weight infants (VLBW;lt;1500 g) in the context of the chronic care model and identify opportunities for improvement under accountable care organizations. METHODS We conducted focus groups and interviews with parents (N = 47) of VLBW infants and interviews with neonatal intensive care unit and ophthalmologic providers (N = 28) at 6 sites in Massachusetts and South Carolina. Themes are reported according to consolidated criteria for reporting qualitative research guidelines. RESULTS Respondents perceived that legal liability and low reimbursement contributed to shortages of ROP providers. Some neonatal intensive care units offered subsidies to attract ophthalmologic providers or delayed transfers to institutions that could not provide ROP examinations and/or treatment. Sites used variable practices for coordinating ROP care. Even at sites with a tracking database and a dedicated ROP coordinator, significant time was required to ensure that examinations and treatment occurred as scheduled. Parents' ability to manage their children's health care was limited by parental understanding of ROP, feeling overwhelmed by the infant's care, and unmet needs for resources to address social stressors. CONCLUSIONS Under accountable care organizations, hospitals and ophthalmology practices should share responsibility for ensuring coordinated ROP care to mitigate liability concerns. To promote integrated care, reimbursement for ROP care should be bundled to include screening, diagnosis, treatment, and appropriate follow-up. Clinical information systems should be enhanced to increase efficiency and limit lapses in care. Self-management tools and connections to community resources could help promote families' attendance of follow-up appointments.

Objective To report the accuracy of intraocular lens (IOL) power calculations and the early refractive status in pseudophakic eyes of infants in the Infant Aphakia Treatment Study. Methods Eyes randomized to receive primary IOL implantation were targeted for a postoperative refraction of +8.0 diopters (D) for infants 28 to 48 days old at surgery and +6.0 D for those 49 days or older to younger than 7 months at surgery using the Holladay 1 formula. Refraction 1 month after surgery was converted to spherical equivalent, and prediction error (PE; defined as the calculated refraction minus the actual refraction) and absolute PE were calculated. Baseline eye and surgery characteristics and A-scan quality were analyzed to compare their effect on PE. Main Outcome Measures Prediction error. Results Fifty-six eyes underwent primary IOL implantation; 7 were excluded for lack of postoperative refraction (n = 5) or incorrect technique in refraction (n = 1) or biometry (n = 1). Overall mean (SD) absolute PE was 1.8 (1.3) D and mean (SD) PE was +1.0 (2.0) D. Absolute PE was less than 1 D in 41% of eyes but greater than 2 D in 41% of eyes. Mean IOL power implanted was 29.9 D (range, 11.5-40.0 D); most eyes (88%) implanted with an IOL of 30.0 D or greater had less postoperative hyperopia than planned. Multivariate analysis revealed that only short axial length ( Conclusions Short axial length correlates with higher PE after IOL placement in infants. Less hyperopia than anticipated occurs with axial lengths of less than 18 mm or high-power IOLs. Application to Clinical Practice Quality A-scans are essential and higher PE is common, with a tendency for less hyperopia than expected. Trial Registration clinicaltrials.gov Identifier: NCT00212134

Objective To validate longitudinal postnatal weight gain as a method for predicting severe retinopathy of prematurity (ROP) in a US cohort. Methods Both ROP evaluations and weekly weight measurements from birth to postmenstrual week 36 for 318 infants were entered into a computer-based surveillance system, WINROP. This system signaled an alarm when the rate of weight gain decreased compared with control subjects. Infants were classified into 3 groups: (1) no alarm, (2) low-risk alarm, or (3) high-risk alarm. Maximum ROP for each infant was categorized as (1) no ROP (immature or mature vascularization), (2) mild ROP (stage 1 or 2 ROP in zone II or III, without plus disease), or (3) severe ROP (any prethreshold, any stage 3, or threshold ROP). A high-risk alarm identified infants at risk for developing severe ROP. Results A high-risk alarm occurred in 81 infants (25.5%) and detected all infants who developed severe ROP a median of 9 weeks before diagnosis. The remaining infants received no alarm or a low-risk alarm. None of these infants developed more than mild ROP. Conclusions Longitudinal postnatal weight gain may help predict ROP. In a US cohort, the WINROP system had a sensitivity of 100% and identified infants early who developed severe ROP. With further validation, WINROP has the potential to safely reduce the number of ROP examinations.

To present strabismus data for premature infants with prethreshold retinopathy of prematurity (ROP) enrolled in the Early Treatment for Retinopathy of Prematurity study.The prevalence of strabismus was tabulated for all of the infants with high-risk prethreshold disease who participated in the randomized trial of the Early Treatment for Retinopathy of Prematurity study and were examined at 6 and/or 9 months' corrected age as well as for all of the infants with low-risk prethreshold disease who were examined at 6 months' corrected age.Presence or absence of strabismus at 6 and 9 months' corrected age.The prevalence of strabismus at 6 months was higher for infants with high-risk prethreshold ROP than for those with low-risk prethreshold ROP (20.3% vs 9.6%, respectively; P.001). Risk factors associated with the development of strabismus at 9 months include abnormal fixation behavior, presence of amblyopia, and outborn birth status (ie, born outside of a study-affiliated hospital). At 9 months, 30% of infants with high-risk prethreshold ROP had strabismus, although only 42% showed strabismus at 6 months. Thirty percent of infants with strabismus at 6 months showed normal alignment at 9 months.Infants with high-risk prethreshold ROP show significant variability in the presence vs absence of strabismus in the first year of life; thus, conservative management is recommended.Ophthalmologists managing strabismus in infants who have high-risk prethreshold ROP should be aware of the significant variability in ocular alignment during the first year of life.

Deborah K. VanderVeen, Dwight T. Jones, Robert A. Petersen; Children’s Hospital of Boston, Boston, MA Introduction: We previously reported our technique and success rates for pediatric endoscopic dacryocystorhinostomy (DCR). Here we review a larger series of patients, including patients with acquired NLDO, and discuss causes of failure. Methods: Endoscopic DCRs performed from 1997 to 2004 on patients. Abnormalities in the area of the lacrimal system, site of the DCR, time until tube removal, and time to success/failure were recorded. Success was defined as complete resolution of tearing by physician evaluation and parental history. Results: Thirty-four patients aged 11 months to 14 years (43 ducts) were identified. Follow-up data were available for 33 patients. Seven patients had congenital syndromes associated with craniofacial abnormalities, and two patients had acquired craniofacial abnormalities. Overall, 64% of patients (60% ducts) showed complete resolution of symptoms. Excluding patients with congenital craniofacial abnormalities, 74% of patients (77% ducts) were cured, and additionally excluding acquired cases, 79% of patients (82% ducts) were cured. Discussion: Endoscopic DCR is effective for relieving persistent NLDO in children with apparently normal craniofacial anatomy, even when complete bony obstruction of the lacrimal duct is present. For patients with abnormal facial anatomy, clinical failure is common (86%). Even if patency of the system is established, symptoms may persist due to chronic nasal congestion and small nasal cavities. Half of the failed craniofacial patients never showed improvement of symptoms; patients with normal anatomy usually failed only after tube removal. Conclusion: Endoscopic DCR is safe and effective for most children, though patients with craniofacial abnormalities or syndromes are extremely difficult to cure.

PURPOSE. To evaluate a semiautomated image analysis software package, Retinal Image multiScale Analysis (RISA), for the diagnosis of plus disease in preterm infants with retinopathy of prematurity (ROP). METHODS. Digital images of the posterior pole showing both disc and macula in preterm infants with ROP were analyzed with an enhanced version of RISA. Venules (N 106) and arterioles (N 44) were identified, and integrated curvature, diameter, and tortuosity of the vessels were calculated. After the RISA calculations were completed, the origins of the vessels were determined to be 32 eyes in 16 infants (12 eyes with plus disease, 20 with no plus disease, as diagnosed by ophthalmic examination). Vessels were sorted into two groups—plus disease and no plus disease—and each RISA parameter was compared using the Mann-Whitney test. For each parameter, sensitivity and specificity were plotted as a function of cutoff criterion, receiver operating characteristic (ROC) curves were constructed, and the areas under the curve (AUC) were calculated. RESULTS. For both arterioles and venules, each of the three parameters was significantly larger for the plus disease group. For instance, the median estimated arteriolar and venular diameters were approximately 12 m greater in plus disease. Sensitivity and specificity plots indicated good accuracy of each parameter for the diagnosis of plus disease. The AUC showed that curvature had the highest diagnostic accuracy (0.911 for arterioles, 0.824 for venules). CONCLUSIONS. The strong performance of RISA parameters in this sample suggests that RISA may be useful for diagnosing plus disease in preterm infants with ROP. (Invest Ophthalmol Vis Sci. 2005;46:4734‐4738) DOI:10.1167/iovs.05-0646

Central retinal vein occlusion (CRVO) is rarely reported in children. A young boy with cyanotic heart disease due to a double-outlet right ventricle, visceral heterotaxia, and asplenia syndrome required a bidirectional Glenn procedure1(palliative cavopulmonary artery anastomosis) at age 5 months for treatment of the cardiac anomaly. Ischemic CRVO was noted at 20 months of age, with subsequent development of neovascular glaucoma. Report of a Case. A 20-month-old boy was initially examined because of mild right-sided weakness after a viral upper respiratory infection. Cranial computed tomography demonstrated multiple bilateral white matter lesions consistent with acute demyelinating encephalomyelitis. The workup included an ophthalmic examination. The right fundus showed engorged retinal veins with hemorrhages in every quadrant, multiple cotton-wool patches in the posterior pole, and macular edema. The disc was edematous. The left fundus was normal except for mild engorgement of the retinal veins. Two weeks after discovery of the CRVO