Development and Validation of a Photonumeric Scale to Assess Marionette Lines.

BACKGROUND: A validated scale is needed for objective and reproducible comparisons of marionette lines before and after treatment in clinical studies. OBJECTIVE: To describe the development and validation of a 5-point photonumeric marionette lines scale. METHODS: The scale was developed to include an assessment guide, verbal descriptors, and real and morphed subject images for each scale grade. Intrarater and interrater reliability was evaluated in initial scale validation (web-based review) ( N = 51 ) and live-subject validation ( N = 75 ) studies, each completed during 2 sessions. RESULTS: In the initial scale validation study, intrarater agreement for 2 physician raters was near perfect (weighted kappa = 0.92 and 0.94). Interrater agreement was excellent during sessions 1 and 2 (intraclass correlation coefficients of 0.94 and 0.95, respectively). In the live-subject validation study, intrarater agreement for 3 physician raters showed a strong correlation (mean weighted kappa = 0.77). Interrater agreement was high during live-subject validation sessions 1 and 2 (intraclass correlation coefficients = 0.89 for both sessions). CONCLUSION: This new marionette lines scale is a validated and reliable scale for physician rating of marionette line severity.

Transarterial Embolization and Percutaneous Ablation of Primary and Metastatic Soft Tissue Tumors.

Soft tissue tumors (STTs) include a range of benign and malignant tumors originating from soft tissues. Transarterial and percutaneous therapies are image-guided and minimally invasive approaches for managing primary and metastatic STTs. The objective of this review is to discuss transarterial and percutaneous therapies by examining the current literature, including indications, patient selection, safety, and effectiveness. Transarterial therapies (e.g., transarterial bland embolization and transarterial chemoembolization) involve the delivery of either embolic or chemotherapeutic particles using a catheter into arteries feeding the tumor, resulting in localized tumor destruction. Percutaneous therapies (e.g., radiofrequency ablation, cryoablation, irreversible electroporation, laser ablation, and magnetic resonance-guided high-intensity focused ultrasound) involve the delivery of either hot or cold temperatures, electrical current, laser, or ultrasound to specifically target tumor cells. Both therapies have been shown to be safe and effective for reducing morbidity and local control of STTs, specifically in patients who are surgically inoperable or who are unresponsive to conventional therapies. Accurate diagnosis, staging, and histological subtype identification are crucial for treatment selection. A multidisciplinary approach, a thorough understanding of tissue anatomy and surrounding structures, as well as individualized strategies based on assessment are essential for optimal patient care.

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.

Biomonitoring and Digital Data Technology as an Opportunity for Enhancing Animal Study Translation.

The failure of animal studies to translate to effective clinical therapeutics has driven efforts to identify underlying cause and develop solutions that improve the reproducibility and translatability of preclinical research. Common issues revolve around study design, analysis, and reporting as well as standardization between preclinical and clinical endpoints. To address these needs, recent advancements in digital technology, including biomonitoring of digital biomarkers, development of software systems and database technologies, as well as application of artificial intelligence to preclinical datasets can be used to increase the translational relevance of preclinical animal research. In this review, we will describe how a number of innovative digital technologies are being applied to overcome recurring challenges in study design, execution, and data sharing as well as improving scientific outcome measures. Examples of how these technologies are applied to specific therapeutic areas are provided. Digital technologies can enhance the quality of preclinical research and encourage scientific collaboration, thus accelerating the development of novel therapeutics.

Corollary discharge promotes a sustained motor state in a neural circuit for navigation.

Animals exhibit behavioral and neural responses that persist on longer timescales than transient or fluctuating stimulus inputs. Here, we report that Caenorhabditis elegans uses feedback from the motor circuit to a sensory processing interneuron to sustain its motor state during thermotactic navigation. By imaging circuit activity in behaving animals, we show that a principal postsynaptic partner of the AFD thermosensory neuron, the AIY interneuron, encodes both temperature and motor state information. By optogenetic and genetic manipulation of this circuit, we demonstrate that the motor state representation in AIY is a corollary discharge signal. RIM, an interneuron that is connected with premotor interneurons, is required for this corollary discharge. Ablation of RIM eliminates the motor representation in AIY, allows thermosensory representations to reach downstream premotor interneurons, and reduces the animal's ability to sustain forward movements during thermotaxis. We propose that feedback from the motor circuit to the sensory processing circuit underlies a positive feedback mechanism to generate persistent neural activity and sustained behavioral patterns in a sensorimotor transformation.

Retrospective Analysis of the Safety and Outcomes of Percutaneous Radiologic Gastrostomy in Obese vs Nonobese Patients.

BACKGROUND: The purpose of this study is to compare the safety and outcomes of percutaneous radiologic gastrostomy (PRG) in obese vs nonobese patients. METHODS: This is an institutional review board-approved retrospective study of 109 patients (male:female, 68:41; mean age, 64.7 years; range, 21-94 years) who underwent PRG with or without gastropexy at a single academic medical center between 2015 and 2018. Body mass index (BMI) of ≥30 kg/m2 was defined as obese. Patient demographics, indications, technical success, intraoperative variables, and major/minor complications were analyzed. Major and minor complications occurring within 30 days of the procedure were recorded and categorized based on the Society of Interventional Radiology Quality Improvement guidelines. RESULTS: Of 109 patients included in the study, 22.9% (n = 25) of patients were obese (average BMI, 34.6 kg/m2 ; range, 30-50). Overall technical success in the obese and nonobese groups was 100% each. In the obese vs nonobese group, major and minor complications were not significantly different. There were also no significant differences in procedure time, fluoroscopy time, or contrast volume. However, mean peak skin dose was significantly increased in the obese group vs nonobese group (190.3 ± 224.2 vs 59.1 ± 71.1 mGy; P < .0001). CONCLUSION: PRG is a safe procedure in obese patients with similar technical success, major and minor complication rates, procedure time, fluoroscopy time, and contrast volume. However, PRG is associated with increased radiation dose with obese patients; therefore, optimal methods of radiation protection should be utilized.

Digital Biomarkers Enable Automated, Longitudinal Monitoring in a Mouse Model of Aging.

To understand the growing needs of an aging human population, there is demand for scalable and reproducible approaches to study animal models of aging and to test novel therapeutic interventions. We investigated the sensitivity and utility of a continuous monitoring platform and its digital biomarkers (motion, breathing rate, and wheel running) to evaluate behavioral and physiological differences between "young" (12 weeks) and "old" (23 months) male C57BL/6J mice with or without running wheels in the home cage. Compared to young mice, old mice showed marked reductions in motion and breathing rate, as well as altered circadian rhythms. Mice without running wheels possessed lower breathing rates compared to their counterparts with running wheels. Digital biomarkers showed age-dependent changes in response to routine procedures (cage changes and blood sampling) and alterations in subjects that unexpectedly reached endpoint. Continuous collection of digital biomarkers in the home cage can enhance current approaches by providing unbiased longitudinal monitoring for large-scale aging studies.

Continuous, Automated Breathing Rate and Body Motion Monitoring of Rats With Paraquat-Induced Progressive Lung Injury.

Assessments of respiratory response and animal activity are useful endpoints in drug pharmacology and safety research. We investigated whether continuous, direct monitoring of breathing rate and body motion in animals in the home cage using the Vum Digital Smart House can complement standard measurements in enabling more granular detection of the onset and severity of physiologic events related to lung injury in a well-established rodent model of paraquat (PQ) toxicity. In rats administered PQ, breathing rate was significantly elevated while body motion was significantly reduced following dosing and extending throughout the 14-day study duration for breathing rate and at least 5 days for both nighttime and daytime body motion. Time course differences in these endpoints in response to the potential ameliorative test article bardoxolone were also readily detected. More complete than standard in-life measurements, breathing rate and body motion tracked injury progression continuously over the full study time period and aligned with, and informed on interval changes in clinical pathology. In addition, breathing rates correlated with terminal pathology measurements, such as normalized lung weights and histologic alveolar damage and edema. This study is a preliminary evaluation of the technology; our results demonstrate that continuously measured breathing rate and body motion served as physiologically relevant readouts to assess lung injury progression and drug response in a respiratory injury animal model.

Automated and Continuous Monitoring of Animal Welfare through Digital Alerting.

A primary goal in preclinical animal research is respectful and responsible care aimed toward minimizing stress and discomfort while enhancing collection of accurate and reproducible scientific data. Researchers use hands-on clinical observations and measurements as part of routine husbandry procedures or study protocols to monitor animal welfare. Although frequent assessments ensure the timely identification of animals with declining health, increased handling can result in additional stress on the animal and increased study variability. We investigated whether automated alerting regarding changes in behavior and physiology can complement existing welfare assessments to improve the identification of animals in pain or distress. Using historical data collected from a diverse range of therapeutic models, we developed algorithms that detect changes in motion and breathing rate frequently associated with sick animals but rare in healthy controls. To avoid introducing selec- tion bias, we evaluated the performance of these algorithms by using retrospective analysis of all studies occurring over a 31-d period in our vivarium. Analyses revealed that the majority of the automated alerts occurred prior to or simultaneously with technicians' observations of declining health in animals. Additional analyses performed across the entire duration of 2 studies (animal models of rapid aging and lung metastasis) demonstrated the sensitivity, accuracy, and utility of automated alerting for detecting unhealthy subjects and those eligible for humane endpoints. The percentage of alerts per total subject days ranged between 0% and 24%, depending on the animal model. Automated alerting effectively complements standard clinical observations to enhance animal welfare and promote responsible scientific advancement.

Optical Coherence Tomography Normative Peripapillary Retinal Nerve Fiber Layer and Macular Data in Children 0-5 Years of Age.

PURPOSE: To determine reference values for the peripapillary retinal nerve fiber layer (pRNFL) and macula in children 0-5 years of age. DESIGN: Prospective cross-sectional study. METHODS: This study was set in a single large academic pediatric ophthalmology practice. Healthy, full-term children 0 to <6 years of age presenting for surgery under general anesthesia were prospectively recruited for participation. Excluded were children with systemic neurologic disease, optic nerve or retinal disease (even if unilateral) or any bilateral ocular disease process, and eyes with amblyopia, ocular disease, or spherical equivalent refractive error outside of -3.00 to +8.00 diopters. Following general anesthesia, OCT scans of the optic nerve and retina were acquired using an HRA+OCT Spectralis with Flex module (Heidelberg Engineering). Automated segmentation of the pRNFL and retinal layers was followed by manual correction. RESULTS: Data were obtained from normal eyes of 57 participants (mean age 2.28 ± 1.50 years). Mean global pRNFL thickness was 107.6 ± 10.3 µm. Mean global pRNFL thickness was not dependent on age but showed a negative relationship with axial length (P = .01). The mean total macular volume was 8.56 ± 0.259 mm3 (n = 38). No relationship was found between total macular volume and age. Ganglion cell layer, ganglion cell complex, and inner nuclear layer volumes showed an inverse relationship with age while the photoreceptor layers showed a logarithmic increase with age. CONCLUSIONS: Global pRNFL thickness measurements remain stable over time. Macular volume and thickness values of segmented retinal layers reflect the development of the macula with age.

Handheld Optical Coherence Tomography Normative Inner Retinal Layer Measurements for Children <5 Years of Age.

PURPOSE: Measurements of the ganglion cell complex (GCC), comprising the retinal nerve fiber (RNFL), ganglion cell, and inner plexiform layers, can be correlated with vision loss caused by optic nerve disease. Handheld optical coherence tomography (HH-OCT) can be used with sedation in children who are not amenable to traditional imaging. We report GCC and RNFL measurements in normal children using HH-OCT. DESIGN: Prospective observational study of normal children ≤5 years of age. METHODS: Healthy, full-term children ≤5 years of age undergoing sedation or anesthesia were enrolled. Exclusion criteria included prematurity and pre-existing neurologic, genetic, metabolic, or intraocular pathology. Demographic data, axial length (Master-Vu Sonomed Escalon, Lake Success, New York, USA), and HH-OCT macular and optic nerve volume scans at 0° (Bioptigen, Inc., Morrisville, North Carolina, USA) were obtained. Retinal segmentation was completed with DOCTRAP software, creating average volume thickness maps. RESULTS: Sixty-seven children (67 eyes, 31 males ranging in age from 3.4-70.9 months) were enrolled. Average axial length was 21.2 ± 1.0 mm with mean spherical equivalent +1.49 ± 1.34 diopters (range -2.25 to 4.25). Average GCC volume for the total retina was 0.28 ± 0.04 mm3. Forty-seven of these eyes had RNFL analysis. Average RNFL thickness of the papillomacular bundle was 38.2 ± 9.5 µm. There was no correlation between GCC volume, RNFL thickness, patient age, or axial length. CONCLUSION: Average GCC volume and RNFL thickness was stable from 6 months to 5 years of age. This study provides normative data for GCC and RNFL obtained by HH-OCT in healthy eyes of young children, to serve in evaluating those with optic neuropathies.

Retrospective Analysis of the Effects of Identification Procedures and Cage Changing by Using Data from Automated, Continuous Monitoring.

Many variables can influence animal behavior and physiology, potentially affecting scientific study outcomes. Laboratory and husbandry procedures-including handling, cage cleaning, injections, blood collection, and animal identification-may produce a multitude of effects. Previous studies have examined the effects of such procedures by making behavioral and physiologic measurements at specific time points; this approach can be disruptive and limits the frequency or duration of observations. Because these procedures can have both acute and long-term effects, the behavior and physiology of animals should be monitored continuously. We performed a retrospective data analysis on the effects of 2 routine procedures, animal identification and cage changing, on motion and breathing rates of mice continuously monitored in the home cage. Animal identification, specifically tail tattooing and ear tagging, as well as cage changing, produced distinct and reproducible postprocedural changes in spontaneous motion and breathing rate patterns. Behavioral and physiologic changes lasted approximately 2 d after tattooing or ear tagging and 2 to 4 d for cage changing. Furthermore, cage changes showed strain-, sex-, and time-of-day-dependent responses but not age-dependent differences. Finally, by reviewing data from a rodent model of multiple sclerosis as a retrospective case study, we documented that cage changing inadvertently affected experimental outcomes. In summary, we demonstrate how retrospective analysis of data collected continuously can provide high-throughput, meaningful, and longitudinal insights in to how animals respond to routine procedures.

The UBR-1 ubiquitin ligase regulates glutamate metabolism to generate coordinated motor pattern in Caenorhabditis elegans.

UBR1 is an E3 ubiquitin ligase best known for its ability to target protein degradation by the N-end rule. The physiological functions of UBR family proteins, however, remain not fully understood. We found that the functional loss of C. elegans UBR-1 leads to a specific motor deficit: when adult animals generate reversal movements, A-class motor neurons exhibit synchronized activation, preventing body bending. This motor deficit is rescued by removing GOT-1, a transaminase that converts aspartate to glutamate. Both UBR-1 and GOT-1 are expressed and critically required in premotor interneurons of the reversal motor circuit to regulate the motor pattern. ubr-1 and got-1 mutants exhibit elevated and decreased glutamate level, respectively. These results raise an intriguing possibility that UBR proteins regulate glutamate metabolism, which is critical for neuronal development and signaling.

Microphthalmia, Dermal Aplasia, and Sclerocornea Syndrome: Endoscopic Cyclophotocoagulation in the Management of Congenital Glaucoma.

PURPOSE: To report on the use of endoscopic cyclophotocoagulation (ECP) to treat congenital glaucoma in a triple X female with microphthalmia, dermal aplasia, and sclerocornea (MIDAS) syndrome. OBSERVATIONS: The patient demonstrated linear streaks on the face and neck consistent with dermal aplasia. The corneas were scleralized with ectatic areas of corneal thinning, and the eyes were microphthalmic. Ultrasound biomicroscopy demonstrated congenital aphakia and iris stumps. The patient had elevated intraocular pressure (IOP) that responded to topical glaucoma therapy in the right but not the left eye. Intraoperative endoscopy of the posterior segment revealed multiple hypopigmented chorioretinal lacunae surrounding a pale, cupped optic nerve. ECP of the ciliary processes in the left eye led to marked improvement in IOP. CONCLUSIONS AND IMPORTANCE: Patients with MIDAS syndrome can develop congenital glaucoma secondary to angle dysgenesis. This is the first case report to demonstrate the safe and effective use of ECP to treat elevated IOP in a patient with MIDAS.

Development of the Digital Arthritis Index, a Novel Metric to Measure Disease Parameters in a Rat Model of Rheumatoid Arthritis.

Despite a broad spectrum of anti-arthritic drugs currently on the market, there is a constant demand to develop improved therapeutic agents. Efficient compound screening and rapid evaluation of treatment efficacy in animal models of rheumatoid arthritis (RA) can accelerate the development of clinical candidates. Compound screening by evaluation of disease phenotypes in animal models facilitates preclinical research by enhancing understanding of human pathophysiology; however, there is still a continuous need to improve methods for evaluating disease. Current clinical assessment methods are challenged by the subjective nature of scoring-based methods, time-consuming longitudinal experiments, and the requirement for better functional readouts with relevance to human disease. To address these needs, we developed a low-touch, digital platform for phenotyping preclinical rodent models of disease. As a proof-of-concept, we utilized the rat collagen-induced arthritis (CIA) model of RA and developed the Digital Arthritis Index (DAI), an objective and automated behavioral metric that does not require human-animal interaction during the measurement and calculation of disease parameters. The DAI detected the development of arthritis similar to standard in vivo methods, including ankle joint measurements and arthritis scores, as well as demonstrated a positive correlation to ankle joint histopathology. The DAI also determined responses to multiple standard-of-care (SOC) treatments and nine repurposed compounds predicted by the SMarTRTM Engine to have varying degrees of impact on RA. The disease profiles generated by the DAI complemented those generated by standard methods. The DAI is a highly reproducible and automated approach that can be used in-conjunction with standard methods for detecting RA disease progression and conducting phenotypic drug screens.

Improved immediate breast reconstruction as a result of oncoplastic multidisciplinary meeting.

INTRODUCTION: The National Institute for Health and Clinical Excellence guidelines recommend that breast reconstruction should be available to all women undergoing mastectomy and discussed at the initial surgical consultation (2002, and updated 2009). The National Mastectomy and Breast Reconstruction Audit (2009) showed that 21% of mastectomy patients underwent immediate breast reconstruction (IBR) and 11% had delayed breast reconstruction (DBR). Breast reconstruction has been shown to have a positive effect on quality of life postmastectomy. This retrospective study investigated the impact of the introduction of a dedicated oncoplastic multidisciplinary meeting (OP MDM) on our unit's breast reconstruction rate. PATIENTS AND METHODS: A retrospective analysis of 229 women who underwent mastectomy, of whom 81 (35%) underwent breast reconstruction between April 2014 and March 2016. Data were analyzed before and after introduction of OP MDM in April 2015. Data on patient age, type of surgery (mastectomy only, mastectomy and reconstruction), timing of reconstruction (IBR, DBR), and type of reconstruction (implant, autologous) were collected. RESULTS: Between April 2015 and March 2016, following establishment of OP multidisciplinary team in April 2015, of the 120 patients who had mastectomy, 50 (42%) underwent breast reconstruction with 78% (39/50) choosing IBR (56% implant reconstruction and 22% autologous). Compared to the period between April 2014 and March 2015 preceding the OP MDM, of 109 patients who underwent mastectomy, only 31 (28%) had breast reconstruction with 64% (20/31) choosing IBR (45% implant reconstruction and 19% autologous). The rate of DBR was lower, 22% (11/50), following OP MDM compared to 35% (11/31) before OP MDM. CONCLUSION: There has been an increased uptake of breast reconstruction surgery from 28% to 42%. The biggest impact was on those opting for the immediate type reconstruction option (78%). The OP MDM has significantly contributed to this increased rate of reconstruction.

Ocular Pathology of Oculocerebrorenal Syndrome of Lowe: Novel Mutations and Genotype-Phenotype Analysis.

Mutations in the OCRL1 gene result in the oculocerebrorenal syndrome of Lowe, with symptoms including congenital bilateral cataracts, glaucoma, renal failure, and neurological impairments. OCRL1 encodes an inositol polyphosphate 5-phosphatase which preferentially dephosphorylates phosphatidylinositide 4,5 bisphosphate (PI(4,5)P2). We have identified two novel mutations in two unrelated Lowe syndrome patients with congenital glaucoma. Novel deletion mutations are detected at c.739-742delAAAG in Lowe patient 1 and c.1595-1631del in Lowe patient 2. End stage glaucoma in patient 2 resulted in the enucleation of the eye, which on histology demonstrated corneal keloid, fibrous infiltration of the angle, ectropion uvea, retinal gliosis, and retinal ganglion cell loss. We measured OCRL protein levels in patient keratinocytes and found that Lowe 1 patient cells had significantly reduced OCRL protein as compared to the control keratinocytes. Genotype-phenotype correlation of OCRL1 mutations associated with congenital glaucoma revealed clustering of missense and deletion mutations in the 5-phosphatase domain and the RhoGAP-like domain. In conclusion, we report novel OCRL1 mutations in Lowe syndrome patients and the corresponding histopathologic analysis of one patient's ocular pathology.

360-Degree Trabeculotomy for Medically Refractory Glaucoma Following Cataract Surgery and Juvenile Open-Angle Glaucoma.

PURPOSE: Although angle surgeries show good success in primary congenital glaucoma, reported success in glaucoma following cataract surgery (GFCS) and juvenile open-angle glaucoma (JOAG) is variable and with relatively short follow-up. We evaluated longer-term outcomes of 360-degree trabeculotomy for medically refractory GFCS and JOAG. DESIGN: Retrospective case series. METHODS: First operated eyes of consecutive patients with medically refractory GFCS and JOAG in a single-surgeon pediatric glaucoma practice who underwent illuminated microcatheter-assisted 360-degree trabeculotomy from February 2008 to June 2015 were reviewed. Baseline characteristics, time to failure or last visit, surgical details, final intraocular pressure (IOP), and complications were recorded. Success required IOP ≤22 mm Hg and 20% reduction without additional glaucoma surgery or devastating complication. RESULTS: Thirty-five eyes (35 patients) were included: 25 GFCS and 10 JOAG (mean age at surgery 5.6 vs 16.7 years, respectively, P < .001). Success for GFCS and JOAG was 18 of 25 (72%) vs 6 of 10 (60%) eyes at mean follow-up of 31.9 ± 26.1 vs 24.5 ± 19.7 months, respectively. IOP was significantly reduced from baseline for both GFCS and JOAG (31.5 ± 7.5 mm Hg vs 19.2 ± 7.7 mm Hg, P < .001; and 29.5 ± 10.3 mm Hg vs 15.8 ± 6.6 mm Hg, P < .001, respectively). Fewer glaucoma medications were needed after surgery (P = .01) for GFCS but not JOAG. Complications (all but 2 spontaneously resolving) included choroidal effusion (1), vitreous hemorrhage (3), Descemet detachment (1), and persistent hyphema (2). Three-year Kaplan-Meier success for GFCS vs JOAG was 75.3% vs 53.3%, respectively. CONCLUSIONS: Illuminated microcatheter-assisted 360-degree trabeculotomy is a useful, low-risk, modestly successful initial surgical treatment for both medically refractory GFCS and JOAG.

Update on congenital cataract surgery management.

PURPOSE OF REVIEW: The basic procedure of pediatric cataract surgery has not dramatically changed over the past few years. Recent multicenter study results along with technological innovations, however, have increased our understanding and armamentarium of techniques and devices used to improve outcomes. RECENT FINDINGS: We review surgical techniques that have been recently applied to the management of pediatric cataracts and describe newer intraocular lenses that have become available for use in the pediatric population. The 5-year results of the Infant Aphakia Treatment Study, including visual outcomes and complications, as well as other studies comparing intraocular lens implants with contact lenses for infants have shaped our management of congenital cataract. We also discuss how ocular imaging with optical coherence tomography has enhanced our understanding of the microstructural effects on pediatric eyes after cataract surgery and touch on other future innovations. SUMMARY: We review updates in the management of congenital cataract, which remains a major cause of preventable childhood blindness.