Anisometropia and Amblyopia Outcomes in Early Versus Late Resolution of Congenital Nasolacrimal Duct Obstruction in Older Infants.

PURPOSE: Congenital nasolacrimal duct obstruction is a known risk factor for amblyopia and anisometropia. The purpose of this study was to investigate whether the rate of anisometropia and amblyopia development differed based on the age at CNLDO resolution in older infants. METHODS: This retrospective chart review at a single tertiary children's hospital from 2007 to 2017 compared early versus late spontaneous resolution (cutoff 12 months) and intervention (cutoff 15 months) groups presenting at ≥9 months of age, comparing visual outcomes, including anisometropia (≥1 D of sphere or cylinder) and amblyopia (≥2 levels difference in Teller acuity or optotype testing). Parents/guardians were contacted by phone for missing data on spontaneous resolution or intervention status. RESULTS: A total of 462 patients were included (152 early; 310 late group). The early group presented at a median age of 12.0 (interquartile range: 10.0, 13.0) months, while the late group presented at 21.0 (interquartile range: 15.0, 32.0) months. Unilateral disease occurred in 62% and 59%, respectively. Anisometropia was seen in (12/102) 12% of early versus (25/243) 10% of late patients (p = 0.686, 95% CI: -0.059, 0.088), and amblyopia in (4/131) 3% of early versus (14/286) 5% of late patients (p = 0.322, 95% CI: -0.061, 0.018). In patients presenting <24 months without undergoing surgery, spontaneous resolution occurred in 76% between 12 and 24 months (n = 41). CONCLUSIONS: Anisometropia and amblyopia rates did not significantly differ between early and delayed intervention for congenital nasolacrimal duct obstruction in this retrospective cohort presenting beyond 9 months of age to a children's hospital. This study found frequent late spontaneous resolution.

Clinical Outcomes in Children With Orbital Cellulitis and Radiographic Globe Tenting.

PURPOSE: Axial displacement of the globe with tenting centered on the optic nerve-globe junction is a predictor of visual loss in adults. The purpose of this study was to determine the visual outcomes of children with orbital cellulitis and globe tenting. METHODS: The records of 46 consecutive children with orbital cellulitis at a single tertiary children's hospital were reviewed retrospectively. Initial and final visual acuities were available for 34 of 46 patients (74%). Globe tenting was defined by an angle of 130° or less at the optic nerve-globe junction as derived from sagittal CT or MRI. Visual acuities of 4 children with globe tenting (mean age, 10.3 ± 3.3 years) were compared with those of 30 children without globe tenting (mean age, 10.8 ± 3.5 years). Final logarithm of the minimum angle of resolution visual acuities were analyzed. RESULTS: The mean posterior globe angle was 124.5° ± 8.0° in patients with globe tenting, compared with 145.6° ± 7.4° in the affected eye of the patients without globe tenting (p = 0.002). Final visual acuity was logarithm of the minimum angle of resolution = 0 following treatment in patients with globe tenting and logarithm of the minimum angle of resolution = 0.02 in patients without tenting (p = 0.70). DISCUSSION: We propose that the increased elastic compliance of the optic nerve sheath and sclera in children may contribute to better visual outcomes. CONCLUSIONS: Pediatric orbital cellulitis with globe tenting may not lead to devastating vision loss as previously seen in adults.

Diagnostic Role of Oral Fluorescein Angiography in Pediatric Ambulatory Clinics.

Oral ingestion of fluorescein can be done in ambulatory pediatric clinics. We show that oral ultra-widefield fluorescein angiography is a non-invasive approach to rapidly diagnose and manage a diverse set of pediatric retinal vascular diseases.

Vitreoretinal biomarkers of retinopathy of prematurity using handheld optical coherence tomography: a review.

Retinopathy of prematurity (ROP) is caused by abnormal retinal vascularization in premature infants that has the potential for severe long-term vision impairment. Recent advancements in handheld optical coherence tomography (OCT) have enabled noninvasive, high-resolution, cross-sectional imaging of the infant eye at the bedside. The use of handheld OCT devices in the diagnosis of ROP in premature infants has furthered our understanding of disease state and progression. This review discusses the known and novel biomarkers of ROP severity in premature infants identified through handheld OCT and potential for future directions.

Hallermann-Streiff Syndrome in Concordant Monozygotic Twins With Congenital Cataracts, Exudative Retinal Detachments, and One Case of Corneal Perforation Requiring Keratoplasty.

PURPOSE: We describe the management of Hallermann-Streiff syndrome in monozygotic female twins with congenital cataracts, exudative retinal detachments, and 1 case of corneal descemetocele with associated dellen and subsequent perforation. METHODS: This study was a case report and review of the literature. RESULTS: Twins 1 and 2 exhibited all 7 cardinal characteristics of Hallermann-Streiff syndrome, presenting with spontaneous lenticular resorption, anterior uveitis, and glaucoma. They underwent bilateral cataract extraction with near total capsulectomy. Both twins experienced recurrent glaucoma, for which twin 1 underwent successful endocyclophotocoagulation in both eyes and twin 2 in the left eye alone. The fellow eye developed 2 sites of perilimbal corneal descemetoceles with associated dellen at the inferotemporal limbal corneal junction leading to spontaneous perforation of 1 site, requiring a full-thickness corneal graft. Both twins developed recurrent bilateral exudative retinal detachments unresponsive to oral prednisolone. Twin 1's last best-corrected visual acuity with aphakic spectacles was 20/260 in the right eye and 20/130 in the left eye at age 4 years and 8 months. Twin 2's last best-corrected visual acuity was 20/130 in each eye at age 4 years and 11 months, over a year after right eye penetrating keratoplasty. CONCLUSIONS: We describe 2 rare cases of Hallermann-Streiff syndrome in monozygotic twins complicated by corneal perforation requiring penetrating keratoplasty in 1 eye of 1 twin. Although corneal opacities have been described in this condition, this is the first case of corneal descemetocele in Hallermann-Streiff syndrome. The cornea was stabilized with a relatively favorable visual outcome over 1 year later.

Flipped classroom approach to global outreach: cross-cultural teaching of horizontal strabismus to Chinese ophthalmology residents.

AIM: To evaluate the flipped classroom model for teaching horizontal strabismus didactics in an ophthalmology residency program in China as part of a visiting professorship from the United States. METHODS: Residents from an ophthalmology residency program in China were invited to participate in flipped classroom sessions taught by an experienced American ophthalmology faculty in 2018. Residents were instructed to watch a pre-class video lecture prior to the in-class-case-based activity. Content tests (5 Ophthalmic Knowledge Assessment Program style questions) and surveys were administered before and after the classroom sessions (100% response rate). These results were compared to that of an American cohort who were taught the same content. RESULTS: The Chinese cohort of 12 residents preferred the flipped classroom to the traditional classroom at higher rates than the American cohort of 40 residents (92% vs 55%, P=0.04) and felt that all ophthalmology topics would be appropriate for the flipped classroom teaching style (P-values between 0.008 and <0.001). In both Chinese and American cohorts, we found that the exotropia curriculum saw a small but significant improvement in performance following the flipped classroom session (P=0.025 for Chinese residents; P=0.001 for US residents), whereas scores in both groups for the esotropia course did not significantly improve. CONCLUSION: This is the first study to evaluate the flipped classroom model implemented by a visiting ophthalmology professor in a global outreach setting. The flipped classroom sessions are viewed favorably by the Chinese residents relative to the US cohort with a modest impact on knowledge. Decreased in-person interpreter requirement and increased student engagement make this model valuable in cross-cultural visiting professorship settings. Finally, the flipped classroom may lend itself well to a virtual format to prevent the transmission of COVID-19, although such a format requires further study.

Semi-Automated Analysis of Foveal Maturity in Premature and Full-Term Infants Using Handheld Swept-Source Optical Coherence Tomography.

Purpose: To develop a semi-automated method of measuring foveal maturity using investigational handheld swept source-optical coherence tomography (SS-OCT). Methods: In this prospective, observational study, full-term newborns and preterm infants undergoing routine retinopathy of prematurity screening were imaged. Semi-automated analysis measured foveal angle and chorioretinal thicknesses at the central fovea and average two-sided parafovea by three-grader consensus, correlating with OCT features and demographics. Results: One hundred ninety-four imaging sessions from 70 infants were included (47.8% girls, 37.6 ± 3.4 weeks postmenstrual age, 26 preterm infants with birth weight 1057 ± 325.0, gestational age 29.0 ± 3.0 weeks). Foveal angle (96.1 ± 22.0 degrees) steepened with increasing birth weight (P = 0.003), decreasing inner retinal layer thickness, and increasing gestational age, postmenstrual age, and foveal and parafoveal choroidal thickness (all P < 0.001). Inner retinal fovea/parafovea ratio (0.4 ± 0.2) correlated with increasing inner foveal layers, decreasing postmenstrual age, gestational age, and birth weight (all P < 0.001). Outer retinal F/P ratio (0.7 ± 0.2) correlated with ellipsoid zone presence (P < 0.001), increased gestational age (P = 0.002), and birth weight (P = 0.003). Foveal (447.8 ± 120.6 microns) and parafoveal (420.9 ± 109.2) choroidal thicknesses correlated with foveal ellipsoid zone presence (P = 0.007 and P = 0.01, respectively), postmenstrual age, birth weight, gestational age, and decreasing inner retinal layers (all P < 0.001). Conclusions: Foveal development is dynamic and partially observed through semi-automated analysis of handheld SS-OCT imaging. Translational Relevance: Semi-automated analysis of SS-OCT images can identify measures of foveal maturity.

Multivariate Models to Diagnose Early Referral-Warranted Retinopathy of Prematurity With Handheld Optical Coherence Tomography.

Purpose: The purpose of this study was to create multivariate models predicting early referral-warranted retinopathy of prematurity (ROP) using non-contact handheld spectral-domain optical coherence tomography (OCT) and demographic data. Methods: Between July 2015 and February 2018, infants ≤1500 grams birth weight or ≤30 weeks gestational age from 2 academic neonatal intensive care units were eligible for this study. Infants were excluded if they were too unstable to participate in ophthalmologic examination (2), had inadequate image quality (20), or received prior ROP treatment (2). Multivariate models were created using demographic variables and imaging findings to identify early referral-warranted ROP (referral-warranted ROP and/or pre-plus disease) by routine indirect ophthalmoscopy. Results: A total of 167 imaging sessions of 71 infants (45% male infants, gestational age 28.2+/-2.8 weeks, and birth weight 995.6+/-292.0 grams) were included. Twelve of 71 infants (17%) developed early referral-warranted ROP. The area under the receiver operating characteristic curve (AUC) was 0.94 for the generalized linear mixed model (sensitivity = 95.5% and specificity = 80.7%) and 0.83 for the machine learning model (sensitivity = 91.7% and specificity = 77.8%). The strongest variables in both models were birth weight, image-based Vitreous Opacity Ratio (an estimate of opacity density), vessel elevation, and hyporeflective vessels. A model using only birth weight and gestational age yielded an AUC of 0.68 (sensitivity = 77.3% and specificity = 63.4%), and a model using only imaging biomarkers yielded 0.88 (sensitivity = 81.8% and specificity = 84.8%). Conclusions: A generalized linear mixed model containing handheld OCT biomarkers can identify early referral-warranted ROP. Machine learning produced a less optimal model. Translational Relevance: With further validation, this work may lead to a better-tolerated ROP screening tool.

Topical adapalene for the treatment of follicular conjunctivitis due to periocular molluscum contagiosum in children.

PURPOSE: Periocular molluscum contagiosum can cause a chronic secondary follicular conjunctivitis or keratoconjunctivitis that rarely leads to corneal scarring and visual impairment. We describe two cases of follicular conjunctivitis due to periocular molluscum contagiosum that were successfully treated with topical adapalene 0.1%. OBSERVATIONS: Case 1 is a 9-year old female with a history of leg molluscum contagiosum who presented with three 1mm flesh-colored umbilicated papules on the periocular skin of the right eye with associated follicular conjunctivitis and diffuse corneal punctate epithelial erosions. Ocular symptoms were persistent for 6 months. Case 2 is a 4-year old female with a 3-month history of right periocular bumps and one month of conjunctival redness with eyelid edema. Examination revealed umbilicated flesh colored nodules on the right upper and lower eyelids with associated trace conjunctival injection. Both patients experienced rapid resolution of both eyelid involvement and conjunctivitis following the use of twice daily topical adapalene 0.1% to the eyelid lesions, with no reported side effects. CONCLUSIONS AND IMPORTANCE: Topical adapalene 0.1% is a cost-effective, convenient, and non-toxic over-the-counter retinoid cream that should be considered for first-line therapy in the treatment of periocular molluscum contagiosum and any associated conjunctivitis.

Vitreous opacities in infants born full-term and preterm by handheld swept-source optical coherence tomography.

PURPOSE: To compare vitreous opacity density in infants born at term and in infants born prematurely using an investigational handheld swept-source optical coherence tomography (SS-OCT). METHODS: Infants born at term underwent imaging once between 12 and 48 hours after birth; infants born prematurely were imaged at each routine retinopathy of prematurity (ROP) examination. Three masked, trained graders analyzed images. Semiautomated methods were used to quantify vitreous opacity density, which was correlated with ROP severity based on indirect ophthalmoscopy, other SS-OCT findings, and medical comorbidities. RESULTS: Between April 2018 and June 2019, 251 SS-OCT imaging sessions were performed on 78 infants (49% female; 36% preterm, with mean birth weight of 1018 ± 338 g and gestational age of 28.6 ± 3.2 weeks). All SS-OCT sessions produced images of adequate quality. Punctate vitreous opacities were present in 25 of 28 term infants (89%) and 41 of 50 premature infants (82%). Dice coefficient and F1 scores for intergrader agreement were 0.99 ± 0.03 and 0.77 ± 0.31, respectively. Vitreous opacity density was 0.118 ± 0.187 in prematurely born infants and 0.031 ± 0.118 in infants born at term (P = 0.009). In the former, vitreous opacity density was associated with ROP zone (P = 0.044) and stage (P = 0.031), intraventricular hemorrhage (P = 0.028), subchorionic hemorrhage (P = 0.026), and African American race (P = 0.023). In the latter, vitreous opacity density was associated with maternal diabetes (P = 0.049). CONCLUSIONS: Our investigational handheld SS-OCT achieved high-quality vitreoretinal images. In our study cohort, punctate vitreous opacities were a frequent finding in infants born at term and those born prematurely, with increased density in those born prematurely, particularly those with severe ROP.

EXUDATIVE RETINAL DETACHMENT DOCUMENTED BY HANDHELD SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY AFTER RETINAL LASER PHOTOCOAGULATION FOR RETINOPATHY OF PREMATURITY.

BACKGROUND: Few cases of exudative retinal detachments have been reported in the literature after laser treatment for retinopathy of prematurity, and none of the cases include optical coherence tomography images. METHODS: Case report. RESULTS: A 469-g birth weight, 25-week gestational age baby girl at 35-week postmenstrual age received a retinal laser (1,724 spots in the right eye and 1,287 spots in the left eye) for bilateral Stage 3 Zone II plus disease retinopathy of prematurity. The patient developed a large macular exudative retinal detachment seen in the right eye at the first follow-up, 5 days after the laser. Serial handheld optical coherence tomography demonstrated resolution of retinal detachment and intraretinal fluid over the course of 4 weeks with only topical tobramycin/dexamethasone ophthalmic ointment 3 to 4 times daily for 2 weeks. The patient was left with a residual subretinal scar and outer retinal loss on optical coherence tomography, with a later appearance of a dragged macula on funduscopic examination noted at 52-week postmenstrual age. It is unclear whether systemic steroid therapy, intravitreal bevacizumab, or surgery would hasten resolution of exudative retinal detachment after the retinopathy of prematurity laser and avoid photoreceptor damage seen on optical coherence tomography in this case. CONCLUSION: This case suggests that post-retinopathy of prematurity laser exudative retinal detachments can resolve without aggressive intervention, but may cause permanent retinal sequelae.

SHORT-TERM COMPUTER-ASSISTED QUANTIFICATION OF PLUS DISEASE AFTER TREATMENT OF TYPE 1 RETINOPATHY OF PREMATURITY WITH INTRAVITREAL BEVACIZUMAB OR RETINAL LASER PHOTOCOAGULATION.

PURPOSE: To compare objectively measured changes in plus disease after bevacizumab and laser for Type 1 retinopathy of prematurity. METHODS: ROPtool (a computer program) analyzed fundus images at baseline, 1 week, and subsequent examinations. RESULTS: Six infants (9 eyes) were treated with bevacizumab and 7 (12 eyes) with laser. One week after treatment, bevacizumab compared with laser resulted in a greater median percent change from pretreatment in tortuosity (-53.8 vs. -0.2%, P < 0.001) and overall plus disease (-20.1 vs. -3.1%, P < 0.001). Change in dilation did not differ (-3.5 vs. -5.5%, P = 0.48). After week 1, all median ROPtool parameters continued to decrease for both groups. At last follow-up (median 3, range: 2-10 weeks), both bevacizumab and laser significantly decreased tortuosity (-54.3 and -23.8%), dilation (-8.7 and -7.5%), and overall plus disease (-27.3 and -10.3%). CONCLUSION: Improvement in plus disease, in particular tortuosity, likely occurs more quickly after bevacizumab compared with laser, particularly at 1 week after treatment. These results guide clinical expectations for plus disease resolution after both treatment modalities.

Survey of current retinopathy of prematurity practices in China.

AIM: To understand retinopathy of prematurity (ROP) screening and treatment preferences among Chinese ophthalmologists. METHODS: A Chinese language survey was administered anonymously using WebQ (Catalyst, Seattle, WA, USA) among Chinese ROP screeners from December 2016 to January 2017. RESULTS: Among 70 ophthalmologists contacted, 65 responded (93%; 78% female, mean age 40y, 57% pediatric ophthalmologists and 25% retina specialists). Most used screening criteria of birth weight ≤2 kg (62%) with variation in cut-off gestational age (≤37wk, 34%; ≤34wk, 22%; ≤32wk, 31%). RetCam (Natus Medical Incorporated, Pleasanton, CA, USA) wide-field fundus photography assisted most screeners (72%) and was exclusively used by many (29%). Among 55 ophthalmologists treating ROP, anti-vascular endothelial growth factor (VEGF) was preferred over laser for both zone I (76% vs 24%) and zone II ROP (58% vs 42%). Retina specialists (P=0.004) and ophthalmologists with >3mo of training (P=0.03) were more likely to use anti-VEGF over laser for zone I ROP. Lack of laser training (8/20, 40%), access (6/20, 30%) and anesthesia (4/20, 20%) were common barriers to laser treatment. CONCLUSION: Chinese ROP screeners favor anti-VEGF injection and RetCam imaging for ROP management. A better understanding of ROP screening and treatment informs future research and education efforts in China.

Choroidal Thickness by Handheld Swept-Source Optical Coherence Tomography in Term Newborns.

Purpose: To describe normative values for choroidal thickness in newborns and characterize their relationship to vitreoretinal features. Methods: Term newborns underwent awake, handheld swept-source optical coherence tomography (SS-OCT) in this prospective cohort study. An automated segmentation algorithm followed by manual adjustments measured choroidal thickness at the fovea and five perifoveal locations. Two masked, trained graders, with a third mediating disagreements, analyzed scans for vitreoretinal findings. OCT vitreoretinal findings, including dome-shaped macula, subretinal fluid, punctate hyperreflective vitreous opacities, persistent inner retinal layers, foveal ellipsoid zone, tractional and non-tractional vitreous bands, epiretinal membrane, cystoid macular edema, vessel elevation, scalloped retinal layers, hyporeflective vessels, and retinal spaces, were assessed and correlated with foveal choroidal thickness using a generalized linear mixed model. Results: Fifty-nine eyes of 39 infants (mean gestational age, 39.5 weeks; 18 male, 46%) were included. Mean foveal choroidal thickness was 455.5 ± 93.9 µm. Choroid was thinner inferonasally (343.6 ± 106.2 µm) compared to superonasally (368.4 ± 92.9 µm; P = 0.03) and superotemporally (369.6 ± 100.6 µm; P = 0.02). Thinner foveal choroidal thickness was associated with absence of a foveal ellipsoid zone (437.1 ± 78.5 µm vs. 553.7 ± 93.9 µm; P = 0.02). Choroidal thickness was not significantly associated with other OCT findings. Conclusions: We identified an association between thinner choroid and foveal immaturity. Additional study is needed to determine whether choroidal development impacts visual outcomes. Translational Relevance: Handheld SS-OCT achieved normative measurements for choroidal thickness across the macula in term newborns, providing a foundation for future investigations into the role of choroidal development in infancy.

The flipped-classroom approach to teaching horizontal strabismus in ophthalmology residency: a multicentered randomized controlled study.

BACKGROUND: The flipped-classroom involves watching prerecorded lectures at home followed by group learning exercises within the classroom. This study compares the flipped classroom approach with the traditional classroom for teaching horizontal strabismus didactics in ophthalmology residency. METHODS: In this multicenter, randomized controlled survey study from October 2017 to July 2018, 110 ophthalmology residents were taught esotropia and exotropia sequentially, randomized by order and classroom style. Flipped classroom participants were assigned a preclass video lecture prior to the in-class case-based activity. The traditional classroom included a preparatory reading assignment and an in-person lecture. Residents completed three identical 5-question assessments (pretest, post-test, and 3-month retention) and surveys for each classroom. The primary outcome measured residents' preferences for classroom styles; the secondary outcome compared knowledge acquisition. RESULTS: In our study cohort, the flipped classroom resulted in greater at-home preparation than the traditional classroom (P = 0.001) and was preferred by 33 of 53 residents (62%); 45 of 53 (85%) wished to see the flipped classroom used at least 25% of the time. The exotropia flipped classroom scored higher than traditional classroom on the pretest (3.71/5 [74%] vs 2.87/5 [57%]; P < 0.001) and post-test (4.53/5 [91%] vs 4.13/5 [83%]; P = 0.01) but not the 3-month retention test (3.53/5 [71%] vs 3.37/5 [67%]; P = 0.48). The esotropia classroom styles did not differ on pre- or post-test but demonstrated higher scores for the traditional classroom at 3-month retention (3.43/5 [69%] vs 2.92/5 [58%]; P = 0.03). Advantages cited for flipped classroom include being interactive and engaging while incentivizing better classroom preparation. CONCLUSIONS: The flipped classroom method was received favorably by trainees and may complement traditional methods of teaching.

Utilizing a Professionalism Mentor to Address Sexual Harassment in Academic Ophthalmology.

Objective This study assesses a new departmental role-a professionalism mentor-who receives sexual harassment reporting, liaisons with campus resources, and organizes educational sessions. Study Design Multicenter randomized controlled survey study. Methods Academic ophthalmology departments in the United States were randomized to a professionalism mentor group ( n = 9) and a control group ( n = 7). Among both pre- and postsurveys, 605 faculty and trainee responses were received and 546 were complete. The intervention group was assigned a professionalism mentor with educational session for a 6- to 10-month period. Sexual harassment and reporting rate change over time were compared between the two groups. Results Among 546 anonymous responses, 16% experienced workplace sexual harassment during the prior 10 months. Location in the South or Midwest was a risk factor ( p < 0.001). Victims were mostly women (76%), including residents/fellows (46%) and academic attendings (49%); perpetrators included patients (35%) and academic attendings (35%). Departments with and without a professionalism mentor had stable harassment from pre- to postsurvey ( p = 0.95 comparing change). The professionalism mentor group had an increase in reporting to an authority from pre- to postsurvey (7-23%), whereas the control group had a decrease (27-12%; p = 0.07 comparing change). Most faculty and trainees in the interventional arm of this study recommended instituting a professionalism mentor with educational session (66% presurvey and 68% postsurvey), compared with educational session alone (25% presurvey and 23% postsurvey), or neither (9% presurvey and 9% postsurvey). Residency program directors in the professionalism mentor group even more strongly supported instituting both a professionalism mentor and educational program (100% presurvey and 100% postsurvey) as opposed to educational program alone (0% presurvey and 0% postsurvey) or neither (0% presurvey and 0% postsurvey). Conclusion This study identified a high sexual harassment rate in academic ophthalmology departments over a brief period. The presence of a professionalism mentor was viewed favorably and may lead to increased reporting.

Dome-shaped macula in premature infants visualized by handheld spectral-domain optical coherence tomography.

PURPOSE: To describe dome-shaped macula and associated clinical findings in premature infants. METHODS: This prospective, observational cohort study included a consecutive sample of premature infants screened for retinopathy of prematurity (ROP) with 9-month follow-up. Handheld spectral domain optical coherence tomography (SD-OCT) was performed at the time of ROP screening. Images were assessed for dome-shaped macula, cystoid macular edema, epiretinal membrane, vitreous bands, and punctate hyperreflective vitreous opacities. Dome height measurements were performed in a subset of images. Teller visual acuity and cycloplegic refraction were performed at an adjusted age of 8-10 months. RESULTS: Of 37 infants (74 eyes; 49% male; mean gestational age 27.8 ± 3.2 weeks; mean birth weight 949 ± 284 g), 24/37 (65%) demonstrated dome-shaped macula in at least one eye (13 both eyes, 5 right eye only, and 6 left eye only). Of the 74 eyes, 26 (35%) could be reliably measured, with a mean dome height of 139.0 ± 72.3 µm (range, 54-369 µm). Presence of dome-shaped macula was associated with a diagnosis of ROP (P = 0.02; OR, 3.03; 95% CI, 1.18-7.82) and pre-plus or plus disease (P = 0.02; OR, 4.20; 95% CI, 1.05-16.78). Infants with dome-shaped macula had lower birth weight compared with those without (877 vs 1081 g; P = 0.04). No associations with other demographics, OCT findings, and 9-month refractive outcomes were found. CONCLUSIONS: Dome-shaped macula was frequently identified by handheld SD-OCT in premature infants, especially those with lower birth weight and severe ROP. The long-term clinical significance of this finding is unknown.

Characterization of the Spectrum of Ophthalmic Changes in Patients With Alagille Syndrome.

Purpose: The purpose of this study was to characterize the phenotypic spectrum of ophthalmic findings in patients with Alagille syndrome. Methods: We conducted a retrospective, observational, multicenter, study on 46 eyes of 23 subjects with Alagille syndrome. We reviewed systemic and ophthalmologic data extracted from medical records, color fundus photography, fundus autofluorescence, optical coherence tomography, visual fields, electrophysiological assessments, and molecular genetic findings. Results: Cardiovascular abnormalities were found in 83% of all cases (of those, 74% had cardiac murmur), whereas 61% had a positive history of hepatobiliary issues, and musculoskeletal anomalies were present in 61% of all patients. Dysmorphic facies were present in 16 patients, with a broad forehead being the most frequent feature. Ocular symptoms were found in 91%, with peripheral vision loss being the most frequent complaint. Median (range) Snellen visual acuity of all eyes was 20/25 (20/20 to hand motion [HM]). Anterior segment abnormalities were present in 74% of the patients; of those, posterior embryotoxon was the most frequent finding. Abnormalities of the optic disc were found in 52%, and peripheral retinal abnormalities were the most frequent ocular finding in this series, found in 96% of all patients. Fifteen JAG1 mutations were identified in 16 individuals; of those, 6 were novel. Conclusions: This study reports a cohort of patients with Alagille syndrome in which peripheral chorioretinal changes were more frequent than posterior embryotoxon, the most frequent ocular finding according to a number of previous studies. We propose that these peripheral chorioretinal changes are a new hallmark to help diagnose this syndrome.

Botulinum toxin for the treatment of cyclic esotropia in a child with Chiari type I malformation.

We report the case of a 4-year-old boy who presented with cyclic esotropia in the setting of a Chiari type I malformation. He was treated with a single administration of botulinum toxin and remained orthotropic at 7-months. To our knowledge, this is the first reported case of cyclic esotropia with Chiari I malformation successfully treated using botulinum toxin.