Epidemiology of Pediatric Ocular Surface Inflammatory Diseases in the United States Using the Optum Labs Data Warehouse.

PURPOSE: To benchmark the epidemiologic features of pediatric ocular surface inflammatory diseases (POSID). DESIGN: Retrospective cohort study. PARTICIPANTS: Patients 18 years of age or younger with a medical claim for a diagnosis of POSID in the Optum Labs Data Warehouse between 2007 and 2020. METHODS: Patients with claims of blepharokeratoconjunctivitis (BKC), herpes simplex keratoconjunctivitis (HSK), or vernal keratoconjunctivitis (VKC) were included. Those with less than 6 months of follow-up before the initial diagnosis of POSID were excluded. Odds ratios (ORs) were derived from multivariable logistic regression analyses evaluating the associations between epidemiologic variables and POSID development. MAIN OUTCOME MEASURES: The primary outcome was the estimated prevalence of POSID. Prevalence of POSID subtypes and changes in prevalence over time were also evaluated. RESULTS: Two thousand one hundred sixty-eight patients with POSID were identified from 2018 through 2019, yielding an estimated prevalence of 3.32 per 10 000. The prevalence of POSID was higher among children between 5 and 10 years of age, male children, those of Asian descent, and those living in the Northeast and the West census regions of the United States. The prevalence (per 10 000) of BKC, HSK, and VKC in the same period were 0.59 (95% confidence interval [CI], 0.53-0.65), 0.74 (95% CI, 0.68-0.81), and 1.99 (95% CI, 1.88-2.10), respectively, and significant differences were found in terms of age, sex, racial, ethnic, and regional distributions among the diagnoses. Between 2008 through 2009 and 2018 through 2019, a significant increase in POSID was noted among Asians (from 6.26 [95% CI, 5.28-7.36] to 11.80 [95% CI, 10.40-13.34]) driven by changes in VKC. Multivariable analysis demonstrated that age older than 5 years (OR, 2.57-3.75; 95% CI, 2.17-4.34), male sex (OR, 1.38; 95% CI, 1.26-1.50), Asian descent (OR, 3.12; 95% CI, 2.70-3.60), and Black or African American descent (OR, 1.26; 95% CI, 1.02-1.55) were associated with POSID development. CONCLUSIONS: This study provides an estimated prevalence of POSID and its 3 common subtypes in the United States, with important epidemiologic differences among them. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Recombinant Human Nerve Growth Factor for Pediatric Neurotrophic Keratopathy.

ABSTRACT: A 4-year-old boy presented with right neurotrophic corneal ulcer, lagophthalmos, and facial palsy 8 months after neurosurgery for synchronous brain tumors. Initial treatment with topical antibiotics, topical corticosteroids, lubrication, and lateral tarsorrhaphy successfully treated the corneal epithelial defect; however, the cornea continued to demonstrate diffuse epitheliopathy and a dense stromal opacity and remained insensate on Cochet-Bonnet esthesiometry. After a course of topical cenegermin, central corneal sensation normalized, and the corneal epitheliopathy was markedly improved. Two years after the completion of cenegermin, corneal sensation was maintained; there were no recurrences of epithelial defects, and the stromal opacity had markedly improved. In vivo confocal microscopy (IVCM) demonstrated the presence of subbasal corneal innervation. This report highlights the safety and prolonged effects of cenegermin for the treatment of pediatric iatrogenic neurotrophic keratopathy, as evidenced by the clinical course and IVCM.

Dominant variants in PRR12 result in unilateral or bilateral complex microphthalmia.

Complex microphthalmia is characterized by small eyes with additional abnormalities that may include anterior segment dysgenesis. While many genes are known, a genetic cause is identified in only 4-30% of microphthalmia, with the lowest rate in unilateral cases. We identified four novel pathogenic loss-of-function alleles in PRR12 in families affected by complex microphthalmia and/or Peters anomaly, including two de novo, the first dominantly transmitted allele, as well as the first splicing variant. The ocular phenotypes were isolated with no additional systemic features observed in two unrelated families. Remarkably, ocular phenotypes were asymmetric in all individuals and unilateral (with structurally normal contralateral eye) in three. There are only three previously reported PRR12 variants identified in probands with intellectual disability, neuropsychiatric disorders, and iris anomalies. While some overlap with previously reported cases is seen, nonsyndromic developmental ocular anomalies are a novel phenotype for this gene. Additional phenotypic expansions included short stature and normal development/cognition, each noted in two individuals in this cohort, as well as absence of neuropsychiatric disorders in all. This study identifies new associations for PRR12 disruption in humans and presents a genetic diagnosis resulting in unilateral ocular phenotypes in a significant proportion of cases.

What's trending: a review of social media in ophthalmology.

PURPOSE OF REVIEW: With social media use continuing to increase in popularity, ophthalmologists use social media daily for interactions with patients, colleagues, and the academic community. RECENT FINDINGS: The potential reach of social media is overwhelmingly encouraging, but academic organizations have much work to do in order to compete for viewership on social media platforms, and users need to remain vigilant of easily spread misinformation. Individual ophthalmology practices can tailor their social media presence to attract and educate patients. Using hashtags to supplement the experience of academic conferences has boosted engagement both of attendees and other interested parties. As an effective indicator of the popularity of different subjects in medicine, new studies are leveraging social media for epidemiological models. Finally, social media is emerging as a powerful tool for patient advocacy in ophthalmology. SUMMARY: The accessibility of social media uniquely positions it to educate patients, disseminate public eye health initiatives, and increase the reach of individual physicians. It is also able to enhance the academic experience of conferences, connecting new research colleagues, and is becoming the subject of epidemiologic studies itself. Whether using social media for patient education, research, clinical practice, or patient advocacy, ophthalmologists will find social media an increasingly important workplace contributor.

Visual Outcomes and Complications of Type I Boston Keratoprosthesis in Children: A Retrospective Multicenter Study and Literature Review.

PURPOSE: To report outcomes and complications of Boston type 1 keratoprosthesis (KPro) implantation in children. DESIGN: Retrospective, multicenter case series. PARTICIPANTS: All children 16 years of age or younger who underwent KPro surgery at 3 ophthalmology centers in Canada between January 2010 and November 2014. METHODS: Records of patients having undergone KPro implantation were reviewed. Data on preoperative characteristics, surgical procedure(s) performed, and postoperative outcomes were collected and analyzed. MAIN OUTCOME MEASURES: Intraoperative and postoperative complications, device retention, and best-corrected visual acuity (BCVA). RESULTS: The KPro was implanted in 11 eyes of 11 patients 0.9 to 15.5 years of age, with 6 being primary corneal procedures. Best-corrected visual acuity recorded before surgery ranged from 20/600 to light perception (LP), and vision in 2 eyes was fix and follow. All patients had been diagnosed with glaucoma and 6 eyes had glaucoma drainage devices (GDDs) inserted before KPro implantation. At last follow-up (mean, 41.8 months; range, 6.5-85.0 months), 2 eyes retained BCVA of 20/400 or better, whereas 5 eyes lost LP. Postoperative complications included retroprosthetic membrane (9 eyes), corneal melt (5 eyes), infectious keratitis (3 eyes), endophthalmitis (3 eyes), GDD erosion (2 eyes), and retinal detachment (5 eyes). The initial KPro was retained in 4 eyes (36.4%). CONCLUSIONS: Boston type 1 keratoprosthesis implantation in children is associated with a substantially higher rate of complications, higher chance of device failure, and worse visual outcomes than observed in adults. In view of these results, the authors do not recommend the use of the KPro in the pediatric population.

Evaluation of 3D tablet-based stereoacuity test ASTEROID in children with normal and abnormal visual acuity.

PURPOSE: To assess the utility of 3D, tablet-based, glasses-free Accurate STEReotest (ASTEROID) in children compared with the Titmus test. METHODS: Children aged 5-13 years were enrolled in a single-center, nonrandomized, observational comparison study and analyzed by age (5-7 vs 8-13 years) and visual acuity (20/25 or better in both eyes vs abnormal). Each participant underwent both the ASTEROID and Titmus stereoacuity tests. Stereoacuity was defined as fine (≤60 arcsec), moderate (61-200 arcsec), coarse (201-1199 arcsec), or very coarse to nil (≥1200 arcsec). Agreement between the tests was assessed using a weighted kappa (κ) statistic based on all four categories. RESULTS: A total of 112 children were included: 28 aged 5-7 with normal visual acuity, 30 aged 5-7 with abnormal visual acuity, 34 aged 8-13 with normal visual acuity, and 20 aged 8-13 with abnormal visual acuity. Mean ASTEROID score was 688 ± 533 arcsec (range, 13-1200 arcsec). Agreement between ASTEROID and Titmus test scores for participants overall was moderate (κ = 0.52). By subgroup, agreement was fair for children 5-7 with abnormal visual acuity (κ = 0.31), moderate for children 5-7 with normal visual acuity (κ = 0.47) and children 8-13 with normal visual acuity (κ = 0.42), and substantial for children 8-13 with abnormal visual acuity (κ = 0.76). Where ASTEROID and Titmus score group varied, ASTEROID score was poorer in 94% (47/50) of cases. CONCLUSIONS: ASTEROID is a digital, tablet-based test that evaluates global stereopsis, does not require glasses, and provides a continuum of scores. Among children, ASTEROID has good agreement with the Titmus test; however, it may be more sensitive at detecting stereovision deficits. Further study is necessary to determine which test is more accurate.

Co-existence of Neurotrophic Keratopathy in Eyes with Limbal Stem Cell Deficiency.

PURPOSE: To investigate whether neurotrophic keratopathy is present in limbal stem cell deficiency (LSCD) by measuring corneal sensation and characterizing corneal subbasal nerve plexus. DESIGN: Prospective, cross-sectional "case-control" comparative study. METHODS: Forty-six eyes with LSCD and 14 normal eyes were recruited from 2019 to 2022. Corneal sensation was measured using a Cochet-Bonnet esthesiometer and subbasal nerve plexus was imaged using in vivo confocal microscopy (IVCM) at the central cornea and 4 limbal regions. Subbasal nerve density (SND, number of nerve/mm2), subbasal nerve length (SNL, total length of nerve/mm2) and subbasal nerve branch density (SNBD, number of branch/mm2) were quantified. LSCD was graded to stage 1, 2 and 3 using a previously established staging method` consisting of clinical scores, basal cell density, central corneal epithelial thickness and SNL. RESULTS: The mean (±SD) cornea sensation in the central cornea and limbus were 29.2 ± 21.5 and 33.6 ± 15.1 mm in the LSCD group and 57.6 ± 5.8 and 54.3 ± 4.7 mm in the control group, respectively (all P < 0.001). In sectoral LSCD, the sensation in the affected regions (29.1 ± 17.6 mm) decreased significantly compared to the unaffected regions (41.4 ± 18.2 mm, P < 0.001). Central corneal SND, SNL and SNBD were reduced by 84.6%, 82.6%, and 89.2%, respectively in LSCD compared to the control (all P < 0.05). The central corneal sensation negatively correlated with the severity of LSCD (rho = -0.64, P < 0.0001) and positively correlated with SND, SNL, and SNBD (rho=0.63, 0.66, and 0.56, respectively; all P < 0.001). CONCLUSIONS: Corneal sensation was reduced in eyes with LSCD. The degree of corneal sensation reduction positively correlated with the severity of LSCD. This finding demonstrated the co-existence of neurotropic keratopathy in LSCD.

Clinical management, monitoring, and prophylaxis of adverse events of special interest associated with datopotamab deruxtecan.

Antibody drug conjugates (ADCs) are an emerging class of treatments designed to improve efficacy and decrease toxicity compared with other systemic therapies through the selective delivery of cytotoxic agents to tumor cells. Datopotamab deruxtecan (Dato-DXd) is a novel ADC comprising a topoisomerase I inhibitor payload and a monoclonal antibody directed to trophoblast cell-surface antigen 2 (TROP2), a protein that is broadly expressed in several types of solid tumors. Dato-DXd is being investigated across multiple solid tumor indications. In the ongoing, first-in-human TROPION-PanTumor01 phase I study (ClinicalTrials.gov: NCT03401385), encouraging and durable antitumor activity and a manageable safety profile was demonstrated in patients with advanced/metastatic hormone receptor-positive/human epidermal growth factor receptor2-negative breast cancer (HR+/HER2- BC), triple-negative breast cancer (TNBC), and non-small cell lung cancer (NSCLC). Improved understanding of the adverse events (AEs) that are associated with Dato-DXd and their optimal management is essential to ensure safe and successful administration. Interstitial lung disease/pneumonitis, infusion-related reactions, oral mucositis/stomatitis, and ocular surface events have been identified as AEs of special interest (AESIs) for which appropriate prevention, monitoring, and management is essential. This article summarizes the incidence of AESIs among patients with HR+/HER2- BC, TNBC, and NSCLC reported in TROPION-PanTumor01. We report our recommendations for AESI prophylaxis, early detection, and management, using experience gained from treating AESIs that occur with Dato-DXd in clinical trials.

Comparative analysis of European residency programs: benchmarking and harmonizing ophthalmology training in Europe.

BACKGROUND/OBJECTIVES: This study aims to benchmark the training programs of European ophthalmology residents. SUBJECTS/METHODS: An online survey, aimed at European ophthalmology residents in training and those within two years of completion, was sent through the national representatives of the European Society of Ophthalmology, Young Ophthalmologists section (SOE-YO). The study involved 214 subjects representing 36 of the 44 European countries offering ophthalmology training programs. RESULTS: Among the surveyed, 74.8% of participants had an official national curriculum; 55.8% had a national specialty examination to accredit their training as ophthalmologists. 45.8% were satisfied or very satisfied with the clinical skills acquired, while 42.1% were completely dissatisfied with the surgical skills achieved. Considering the 4th year residents (mean duration of the residency), many of them did not perform phacoemulsification surgery (34%), pterygium excision (46.9%), or repair of eyelid laceration (31.3%). CONCLUSIONS: There is great heterogeneity in the competencies achieved by residents in training according to their country of origin, especially in terms of surgical competences.

Harmonizing ophthalmic residency surgical training across Europe: A proposed surgical curriculum.

BACKGROUND: One of the core aims of the European Union of Medical Specialists is to harmonize training across Europe by creating European Training Requirements for all medical specialties including Ophthalmology. The theoretical part is already defined by the EBO, however as ophthalmology also includes surgical skills, we herein propose a surgical minimum curriculum for ophthalmology residents in Europe. METHODS: National and international ophthalmic training curricula which are publicly available in English were reviewed and compared. The final proposal was created from 5 criteria: 1. Disease prevalence; 2. Patient safety; 3. Case-trainee ratio; 4. Skill transfer; and 5. Technical difficulty. RESULTS: In total 7 different training curricula from across the world were compared. Among the surgical procedures, cataract surgery has the highest median number of procedures required to be completed during residency: 86 procedures (50-350). Followed by oculoplastics: 28 procedures (10-40) and panretinal photocoagulation: 27.5 procedures (10-49) Full procedural competence is proposed in 9 surgical skills, including YAG laser posterior capsulotomy, retinal argon laser, intravitreal injection, corneal foreign body removal, removal of corneal sutures, facial and periocular laceration repair, eyelid laceration repair, minor eyelid procedures, and punctal occlusion. These procedures are deemed essential and feasible for all ophthalmology residents in Europe to perform independently upon completion of their training. CONCLUSION: This proposal should be regarded as a recommendation based on comparable surgical curricula in use worldwide to establish standards across European countries and may serve as valuable insight to those responsible for compiling ETRs for ophthalmology, or their national curriculums.

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.

Evaluation of dry eye disease in children with blepharokeratoconjunctivitis.

OBJECTIVE: To evaluate the symptoms and signs of dry eye disease (DED) in children diagnosed with blepharokeratoconjunctivitis (BKC). DESIGN: Prospective case-controlled study PARTICIPANTS: Consecutive patients with BKC and normal controls. METHODS: All participants underwent a comprehensive dry eye assessment including the Canadian Dry Eye Assessment (CDEA) questionnaire, tear film osmolarity test, Schirmer's test without anesthesia, slit lamp examination, tear film break-up time, corneal fluorescein staining (CFS), and lissamine green conjunctival staining (LGCS), according to the Sjögren's International Collaborative Clinical Alliance ocular staining score. For each test the result of the more severe eye was included in the statistical analysis. RESULTS: Twenty-five patients were recruited-11 with BKC and 14 healthy controls. No difference in symptoms was found between children with BKC (CDEA score 6.1 ± 5.5) and normal controls (CDEA score 3.6 ± 3.2; p = 0.16). Children with BKC had significantly higher mean CFS (1.1 ± 1.6 vs 0.1 ± 0.4; p = 0.04) but similar mean LGCS (1.4 ± 1.8 vs 1.5 ± 2.1; p = 0.81) than normal controls. No statistically significant differences were observed in other tests between the 2 groups. CDEA scores were significantly correlated to CFS in normal controls (r = 0.59, p = 0.03), and approached significance in children with BKC (r = 0.56, p = 0.07). CONCLUSIONS: The only test that can distinguish DED in patients with BKC from children without BKC is the CFS score. This should guide management and monitoring of this unique patient population with DED symptoms and signs.

Agreement of iCare IC200 tonometry with Perkins applanation tonometry in healthy children.

PURPOSE: To assess interdevice agreement between the iCare IC200 rebound tonometer and Perkins applanation tonometry (gold standard) in a healthy pediatric population. METHODS: A total of 42 eyes of 42 healthy children were assessed using both tonometers. Data was collected on subject's age, sex, best-corrected visual acuity, and central corneal thickness (CCT). Intraclass correlation coefficient (ICC) and Bland-Altman analyses were used to determine agreement between IC200 and Perkins applanation tonometers. Linear regression analyzed the effects of intraocular pressure (IOP) on device difference. RESULTS: The mean age and standard deviation of healthy pediatric subjects was 10.0 ± 3.3 years. The mean difference between IC200 and Perkins tonometers (IC200-Perkins) was 0.72 mm Hg, with a mean of 17.1 ± 3.0 mm Hg and 16.4 ± 2.5 mm Hg, respectively. The absolute agreement, or ICC, between tonometers was 0.63 (95% CI, 0.56-0.70). Bland-Altman analysis showed 95% limits of agreement ranging from -5.2 to +6.6 mm Hg. CCT was not correlated with IOP for either the IC200 (P = 0.35) or the Perkins tonometer (P = 0.052). CONCLUSIONS: Compared to applanation tonometry, IC200 overestimated IOP in healthy children, with a greater frequency of readings > +2 mm Hg than < -2 mm Hg compared to Perkins. There was moderate agreement between tonometers. CCT was not found to influence IOP measurement for either tonometer.

Quantification of Anterior Chamber Cells in Children With Uveitis Using Anterior Segment Optical Coherence Tomography.

PURPOSE: To evaluate the feasibility of anterior segment optical coherence tomography (AS-OCT) for measuring anterior chamber (AC) cells in children with uveitis and to compare different AS-OCT acquisition modes. DESIGN: Validity and reliability analysis. METHODS: We enrolled children younger than 18 years who had uveitis involving the anterior segment and children without eye disease as controls. All underwent clinical grading of AC cells. AC images of each eye were obtained using the Optovue Avanti RTVue XR AS-OCT. Two acquisition modes were used: a single cross-sectional line scan and an 8-line radial scan in an asterisk pattern. Two independent, masked graders counted cells manually on AS-OCT images. Rater agreement was assessed using intraclass correlation (ICC). RESULTS: Included were 30 children (59 eyes) with uveitis (median age 13.0 years, range 3-17 years) and 20 control children (40 eyes, median age 10.5 years, range 4-17 years). The number of eyes assigned each clinical grade of cells were as follows: none, 32 (54%); 0.5+, 12 (20.3%); 1+, 5 (8.5%); 2+, 8 (13.6%); 3+, 2 (3.4%). ICC of graders for line and radial scan protocols were 0.87 and 0.90. There was no significant difference between acquisition modes for pooled grader results (95% CI for difference: -0.04 to 0.14). ICC of cell counts between line and radial scan protocols was 0.85 (95% CI: 0.69-0.90). No control eyes had cells on AS-OCT images. CONCLUSIONS: Quantification of AC cell in children with uveitis is feasible with AS-OCT and has excellent reliability between different graders and acquisition modes.

Limbal Stem Cell Dysfunction in Ichthyosis Follicularis, Alopecia, and Photophobia Syndrome.

PURPOSE: To describe the presentation and management of limbal stem cell dysfunction in ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome. METHODS: A retrospective case report. RESULTS: A 6-day-old male infant was diagnosed with IFAP syndrome based on family history and a mutation detected in the MBTPS2 gene. Initial examination showed hyperkeratotic eyelids, madarosis, and lagophthalmos, but otherwise clear corneas. He developed bilateral central corneal epithelial defects spontaneously 6 months later, which were managed with aggressive lubrication, prophylactic antibiotics, and bilateral permanent lateral tarsorrhaphies at 7 months of age. During the procedure, the patient was noted to have bilateral limbal thickening, peripheral corneal pannus with underlying stromal scarring, and late fluorescein staining of the corneal surface. Anterior segment optical coherence tomography demonstrated a significantly abnormal and hyperreflective epithelial surface overlying a thinned corneal stroma, suggestive of limbal stem cell dysfunction. The corneal surface was maintained with lubrication and tarsorrhaphy and has remained stable since. CONCLUSIONS: The progressive conjunctivalization, spontaneous epithelial defects, and anterior segment optical coherence tomography features are highly suggestive of limbal stem cell dysfunction in IFAP syndrome. Optimizing the ocular surface is of importance in the management of children with this rare disease.

Technique for pediatric corneal crosslinking under general anesthesia.

Corneal cross-linking (CXL) is typically performed under topical anesthesia, which may be unsuitable in children and individuals with severe anxiety, poor cooperation, or neurodevelopmental issues. We describe a technique of CXL under general anesthesia that uses sevoflurane and propofol with laryngeal airway or nasal oxygen cannulas, and forceps or microsponges to stabilize the eye position intraoperatively. We also developed a simple and time-saving technique of bilateral simultaneous CXL, whereby the second eye can undergo CXL concurrently with the first in a staggered fashion. Using this technique, we have been able to eliminate up to 25% of surgical time required in standard bilateral procedures. Postoperatively, multimodal analgesia was administered to ensure patient comfort and prevent excessive eye rubbing. In total, 21 eyes of 13 subjects ≤18 years of age were treated. During a median follow-up of 14.5 months (range, 4-43), the only complication observed was sterile corneal infiltrate in 1 eye that resolved with a short course of corticosteroids.

Myopia and anterior segment optical coherence tomography findings in laser-treated retinopathy of prematurity eyes.

PURPOSE: To evaluate structural features and visual outcomes in eyes with a prior history of laser treatment for retinopathy of prematurity (ROP). METHODS: Laser-treated eyes for type 1 ROP, preterm monitored eyes, and full-term control eyes were included. LogMAR conversion of Snellen best-corrected visual acuity and spherical equivalent based on cycloplegic refraction were measured in children 5-15 years of age. Anterior segment optical coherence tomography (OCT) was used to study structural features, including anterior chamber angle (ACA) in a subset of eyes. RESULTS: A total of 50 eyes of 50 patients were included (19 full-term eyes, 19 laser-treated type 1 ROP eyes, 12 preterm monitored eyes). Of these, 44 eyes had visual outcomes data, and 15 eyes had anterior segment data. There was no significant difference in sex or age at final examination between the three groups. There was no significant difference in gestational age between the laser-treated and preterm monitored groups. Compared with the full-term control group and the preterm monitored group, the laser-treated ROP group had narrower ACA and more myopic refractive error. There was a significant correlation between ACA and spherical equivalent. CONCLUSIONS: Laser treatment may affect angle configuration in ROP eyes. Anterior segment OCT is an easy and useful modality that could aid in screening for visually impairing conditions such as myopia and glaucoma in children with ROP.

Mulitmodal Corneal Imaging of Genetically Confirmed Keratitis-Ichthyosis-Deafness Syndrome.

BACKGROUND: Keratitis-ichthyosis-deafness (KID) syndrome is characterized by a congenital triad of keratitis, ichthyosis, and deafness, and is most commonly associated with mutations in the gap junction protein beta 2 gene (GJB2) on chromosome 13q11-q12. METHODS: Multimodal anterior segment imaging and genetic testing were used to supplement clinical examination findings in the diagnosis and management of a 12-year-old boy with suspected KID syndrome. RESULTS: The patient presented with hearing loss, ichthyosis of the face and extremities, and corneal scarring and keratinization. The corneal limbal stem cell population was found to be normal on in vivo confocal microscopy, whereas the basal epithelium of the cornea demonstrated scarring and areas of cellular loss. Screening of GJB2 revealed a presumed pathogenic heterozygous missense mutation, c.148G>A, confirming the diagnosis of KID syndrome. CONCLUSIONS: Multimodal imaging including in vivo confocal microscopy suggests that dysfunctional corneal basal epithelium maturation might contribute to the pathophysiology of keratopathy in KID syndrome.