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A rare but an important side effect of recombinant growth hormone therapy is pseudotumor cerebri syndrome and related vision loss. For the first time, we report the follow-up of recombinant growth hormone therapy with optical coherence tomography and its dose correlation with retinal nerve fiber layer thickness., Purpose: To report the correlation between recombinant growth hormone (rhGH) dosage and retinal nerve fiber layer (RNFL) thickness values measured by optical coherence tomography in a case of pseudotumor cerebri syndrome (PTCS) after rhGH. Methods: An 11-year-old girl was receiving rhGH for panhypopituitarism. The patient developed PTCS, and her rhGH dose was adjusted using optical coherence tomography RNFL thickness measurements. The linear correlation coefficient (r) and coefficient of determination (r2) were calculated to assess the relationship between RNFL thickness and rhGH dose. Results: As the rhGH dosage was increased, the RNFL thickness values also increased, especially when acetazolamide was excluded because of its confounding effect. (r = 0.64) In separate subgroup analysis, a higher acetazolamide dosage strongly correlated with reduced RNFL thickness (r = 0.77). Conclusion: Although PTCS is a rare complication after rhGH therapy, its detrimental effects cannot be ignored. In our case report, we used optical coherence tomography RNFL values in addition to clinical findings to carefully titrate the rhGH dosage to prevent a flare-up of PTCS. Despite the obvious need for larger studies, our case report shows the value of RNFL thickness measured by optical coherence tomography and the valuable additional data it provides to refine rhGH therapy as an adjunct noninvasive method in PTCS.

This study aimed to develop a fully automated deep learning ciliary body segmentation and assessment approach in three-dimensional ultrasound biomicroscopy (3D-UBM) images.Each 3D-UBM eye volume was aligned to the optic axis via multiplanar reformatting. Ciliary muscle and processes were manually annotated, and Deeplab-v3+ models with different loss functions were trained to segment the ciliary body (ciliary muscle and processes) in both en face and radial images.We trained and tested the models on 4320 radial and 3864 en face images from 12 cadaver eye volumes. Deep learning models trained on radial images with Dice loss achieved the highest mean F1-score (0.89) for ciliary body segmentation. For three-class segmentation (ciliary muscle, processes, and background), radial images with Dice loss achieved the highest mean F1-score (0.75 for the ciliary process and 0.82 for the ciliary muscle). Part of the ciliary muscle (10.9%) was misclassified as the ciliary process and vice versa, which occurred owing to the difficulty in differentiating the ciliary muscle-processes border, even by experts. Deep learning segmentation made further editing by experts at least seven times faster than a fully manual approach. In eight cadaver eyes, the average ciliary muscle, process, and body volumes were 56 ± 9, 43 ± 13, and 99 ± 18 mm3, respectively. The average surface area of the ciliary muscle, process, and body were 346 ± 45, 363 ± 83, and 709 ± 80 mm2, respectively. We performed transscleral cyclophotocoagulation in cadaver eyes to shrink the ciliary processes. Both manual and automated measurements from deep learning segmentation show a decrease in volume, surface area, and 360° cross-sectional area measurements.The proposed deep learning segmentation of the ciliary body and 3D measurements showed transscleral cyclophotocoagulation-related changes in the ciliary body.Automated ciliary body assessment using 3D-UBM has the translational potential for ophthalmic treatment planning and monitoring.

The prevalence of myopia keeps increasing during the COVID-19 pandemic. We aimed to map the worldwide treatment preferences of ophthalmologists managing myopia control during the first wave of the pandemic.An online questionnaire inquiring about pharmacological and optical treatment patterns during the first half of 2020 was sent to pediatric ophthalmology as well as general ophthalmology memberships worldwide. The results among pediatric ophthalmologists were compared to a previous study we performed before the pandemic.A total of 2269 respondents from 94 countries were included. Most respondents were pediatric ophthalmologists (64.6%), followed by ophthalmologists from other subspecialties (32.3%). The preferred modality for all geographical regions was a combination therapy of pharmacological and optical treatments. When evaluated independently, the pharmacological treatment was more popular than the optical treatment in most regions other than East Asia (P 0.001). Compared to a pre-pandemic questionnaire, the participation of pediatric ophthalmologists affiliated with non-university hospitals increased. Additionally, the prevalence of respondents utilizing either any type of pharmacological treatment and those that using only evidence-based treatments increased globally. Although a decline in the use of optical treatment was evident worldwide, the use of evidence-based optical treatments increased.Ophthalmologists around the world preferred a combination therapy of pharmacological and optical treatments. More pediatric ophthalmologists treated myopia progression and preferred a better evidence-based approach to control myopia. These trends reflect a positive response and more awareness of the rising prevalence of myopia due to the increased burden of myopia imposed by the COVID-19 pandemic.

OBJECTIVE: This study evaluates the 24 month follow up for the NICHD Neonatal Research Network (NRN) Inositol for Retinopathy Trial. STUDY DESIGN: Bayley Scales of Infants Development-III and a standardized neurosensory examination were performed in infants enrolled in the main trial. Moderate/severe NDI was defined as BSID-III Cognitive or Motor composite score

To report a case of recurrent chalazia and Herpes Simplex conjunctivitis in a patient with DOCK8 deficiency.We report the case of a 15-year-old male patient with genetically diagnosed DOCK8 deficiency who presented with a left lower eyelid mass and left eye conjunctivitis. The lesion did not resolve despite aggressive measures, including topical steroids, topical antibiotics, and multiple debulking procedures but eventually resolved after the initiation of intravenous acyclovir. The diagnosis of HSV infection was supported by pathology findings from the eyelid mass.Patients with DOCK8 deficiency can present with a variety of cutaneous viral infections. In our case, a DOCK8-deficient patient with chalazia and conjunctivitis resistant to antibiotics, steroids and debulking responded to intravenous acyclovir.

Ophthalmic manifestations of disseminated intravascular coagulation (DIC) in a newborn are rare. Ocular involvement typically involves the posterior segment bilaterally and manifests as choroidal and/or retinal hemorrhages associated with fibrin thrombi in the choriocapillaris. We present the case of a newborn girl with DIC secondary to sepsis who subsequently developed nonclearing hyphema, related secondary cataract, 360° posterior synechiae, and nonclearing vitreous hemorrhage in the right eye and diffuse retinal hemorrhage in the left eye. The right fundus was not visible because of intraocular bleeding. The patient underwent cataract surgery at 39 days of life and was left aphakic. Two weeks later, she required subsequent vitrectomy because of a nonclearing vitreous hemorrhage. She developed amblyopia of the right eye and a right esotropia that required strabismus surgery. At 7 years of age, the patient's visual acuity was 20/50 in the right eye and 20/20 in the left eye.

OBJECTIVEApproximately 10% of patients with subarachnoid hemorrhage (SAH) become permanently, legally blind. The average cost of lifetime support and unpaid taxes for each blind person amounts to approximately $900,000. This study evaluates the feasibility and potential role of bedside optical coherence tomography (OCT) in Terson’s syndrome (TS) in patients with acute SAH (aSAH) and its potential role in blindness prevention.METHODSThe authors conducted an open-label pilot study, in which 31 patients with an angiographic diagnosis of aSAH were first screened for TS with dilated funduscopy and then with OCT in the acute phase and at 6-week follow-up visits. Outpatient mood assessments (Patient Health Questionnaire–depression module, Hamilton Depression Scale), and quality of life general (NIH Patient-Reported Outcomes Measurement Information System) and visual scales (25-item National Eye Institute Visual Functioning Questionnaire) were measured at 1 and 6 weeks after discharge. Exclusion criteria included current or previous history of severe cataracts, severe diabetic retinopathy, severe macular degeneration, or glaucoma.RESULTSOCT identified 7 patients with TS, i.e., a 22.6% incidence in our aSAH sample: 7 in the acute phase, including a large retinal detachment that was initially missed by funduscopy and diagnosed by OCT in follow-up clinic. Dilated retinal funduscopy significantly failed to detect TS in 4 (57.1%) of these 7 cases. Intraventricular hemorrhage was significantly more common in TS cases (85.7% vs 25%). None of the participants experienced any complications from OCT examinations. Neither decreased quality of life visual scale scores nor a depressed mood correlated with objective OCT pathological findings at the 6-week follow-up after discharge. There were no significant mood differences between TS cases and controls.CONCLUSIONSOCT is the gold standard in retinal disease diagnosis. This pilot study shows that bedside OCT examination is feasible in aSAH. In this series, OCT was a safe procedure that enhanced TS detection by decreasing false-negative/inconclusive funduscopic examinations. It allows early diagnosis of macular holes and severe retinal detachments, which require acute surgical therapy to prevent legal blindness. In addition, OCT aids in ruling out potential false-positive visual deficits in individuals with a depressed mood at follow-up.

Purpose Ultrasound biomicroscopy (UBM) is an important ophthalmic imaging modality due to its ability to see behind pigmented iris and to visualize anterior chamber when the eye's transparency is compromised. We created a three-dimensional UBM (3D-UBM) system and acquired example images to illustrate its potential. Methods A commercial 50-MHz two-dimensional UBM (2D-UBM) system was attached to a precision translation stage and translated across the eye to acquire an image volume. The stage was mounted on a surgical microscope, which enabled safe, stable positioning. Image processing steps included image alignment, noise reduction, and calibration. 3D visualization included alignment of the optic axis, multiplanar reformatting at arbitrary orientations, and volume rendering with optimized transfer functions. Scans were performed on cadaver and rabbit eyes. Results 3D-UBM allowed visualization of the anterior segment tissues within a 3D anatomical context, unlike 2D-UBM. En face views and interactive slicer operations suggested an ability to plan and assess treatments, including lens placement and microcatheter cannulation of Schlemm's canal. Interactive software allowed us to make accurate measurements of tissue structures (e.g., iridocorneal angles, cyst volumes). In addition, unique measurements of ciliary tissues included single ciliary process volumes of 0.234 ± 0.093 mm3 with surface areas of 3.02 ± 1.07 mm2 and ciliary muscle volume of 67.87 mm3. Conclusions 3D-UBM imaging of the anterior segment can be used to enable unique visualization and quantification of anterior segment structures. Translational relevance 3D-UBM provides informative 3D imaging of tissues in the eye that are invisible to light to potentially provide physicians with improved diagnosis, treatment planning, and treatment assessment as compared to conventional 2D-UBM.

High frequency ultrasound biomicroscopy (UBM) images are used in clinical ophthalmology due to its ability to penetrate opaque tissues and create high resolution images of deeper intraocular structures. Because these inexpensive, high frequency (50 MHz) systems use single ultrasound elements, there is a limitation in visualizing small structures and anatomical landmarks, especially outside focal area, due to the lack of dynamic focusing. The wide and axially variant point spread function degrade image quality and obscure smaller structures. We created a fast, generative adversarial network (GAN) method to apply axially varying deconvolution for our 3D ultrasound biomicroscopy (3D-UBM) imaging system. Original images are enhanced using a computationally expensive axially varying deconvolution, giving paired original and enhanced images for GAN training. Supervised generative adversarial networks (pix2pix) were trained to generate enhanced images from originals. We obtained good performance metrics (SSIM = 0.85 and PSNR = 31.32 dB) in test images without any noticeable artifacts. GAN deconvolution runs at about 31 msec per frame on a standard graphics card, indicating that near real time enhancement is possible. With GAN enhancement, important ocular structures are made more visible.

Ultrasound biomicroscopy is a unique imaging modality used for high-resolution visualization of opaque tissues in the anterior segment of the eye. Due to the lack of dynamic focusing and wide spatially varying point spread function in conventional single element probe, small ocular structures are blurred. Computationally expensive deconvolution approaches can enhance the images but are not suitable for real-time use. We created a supervised generative adversarial network-based deconvolution approach that maps an input image to an enhanced image. We achieved a structural similarity index of 0.85 in our test images without significant degradation. The proposed GAN based deconvolution approach can enhance a single image frame at 31 msec making deconvolution almost real-time.

We developed a methodology for 3D assessment of ciliary body of the eye, an important, but understudied tissue, using our new 3D ultrasound biomicroscopy (3D-UBM) imaging system. The ciliary body produces aqueous humor, which if not drained properly, can lead to increased intraocular pressure and glaucoma, a leading cause of blindness. Most medications and some surgical procedures for glaucoma target the ciliary body. Ciliary body is also responsible for focusing-accommodation by muscle contraction and relaxation. UBM is the only imaging modality which can be used to visualize structures behind the opaque iris, such as ciliary body. Our 3D-UBM acquires several hundred high resolutions (50 MHz) 2D-UBM images and creates a 3D volume, enabling heretofore unavailable en face visualizations and quantifications. In this study, we calculated unique 3D biometrics from automated segmentation using deep learning (UNet). Our results show accuracy of 0.93 ± 0.01, sensitivity of 0.79 ± 0.07 and dice score of 0.72 ± 0.07 on deep learning segmentation of ciliary muscle. For an eye, volume of ciliary body was 67.87 mm3, single ciliary process volumes were 0.234 ± 0.093 mm3 with surface areas adjacent to aqueous humor of 3.02 ± 1.07 mm2. Automated and manual measurements of ciliary muscle volume and cross-sectional area are compared which show overestimation in volume measurement but higher agreeability in cross-sectional area measurements.

To demonstrate the utility of using eye-movement data to reveal the diagnostic characteristics of infantile nystagmus syndrome (INS), determine treatment, and both estimate and document therapeutic improvements in three patients with well-developed foveation periods, fairly broad, lateral gaze "nulls," head turns, strabismus, and complex, multiplanar nystagmus.Infrared reflection, magnetic search coil, and high-speed digital video systems were used to record the eye movements of INS patients, pre- and post-Kestenbaum null-point correction surgery (horizontal or vertical). Data were analyzed and estimations made, using the eXpanded Nystagmus Acuity Function (NAFX) that is part of the OMtools toolbox for MATLAB.In all three subjects (S1-S3), both peak NAFX and longest foveation domain (LFD) improved from their pre-Kestenbaum values. S1: 0.700-0.745 (6.4%) and 25-34° (36%), respectively. S2: 0.445-0.633 (42.4%) and40° to50° (10%), respectively. S3: 0.250-0.300 (20%) and 13° to ≫18° (see text), respectively.S1: Even at the high ends of the pre-therapy NAFX and LFD spectra, INS foveation (and therefore, visual-function) improvements may be adequate to justify nystagmus surgery and provide clinical improvements beneficial to the patient. S2: INS foveation improvements in the vertical plane are equal to those originally estimated using the horizontal data in prior patients. S3: Two apparent NAFX peaks can be converted into a very broad peak by surgery based on the preferred lower peak.

PurposeTo test the hypothesis that augmented tenotomy and reattachment surgery (AT-R), which involves placing an additional suture in each distal tendon during the 4-muscle tenotomy and reattachment (T-R) or other infantile nystagmus syndrome (INS) procedures, could increase the beneficial effects of many types of extraocular muscle (EOM) surgery to treat INS. MethodsBoth infrared reflection and high-speed digital video systems were used to record the eye movements in 4 patients with INS before and after AT-R surgery. Data were analyzed using the eXpanded Nystagmus Acuity Function (NAFX) that is part of the OMtools software. ResultsPlacement of the augmentation suture did not interfere with Kestenbaum, Anderson, bilateral medial rectus muscle recession, or T-R surgeries. The therapeutic effects of AT-R were similar to but not equal to those from the traditional single-suture surgeries (ie, broadening longest foveation domain [LFD] but no improvement of NAFX peak). The average of the NAFX percent improvements after AT-R was within 31% of those estimated from NAFX values before T-R; the average of the percent broadenings of the LFD values after AT-R was within 16%. ConclusionsThe AT-R does not improve the foveation quality in INS above the traditional T-R surgery. It is not improved by an additional suture; indeed, some improvements may be diminished by the added suture. The hypothesized augmented-tendon suture technique (sans tenotomy) has been modified and remains to be tested.

To report the newest ophthalmic manifestations of a mother-daughter pair diagnosed with toe syndactyly, telecanthus, anogenital and renal malformations (STAR) syndrome, a rare X-linked developmental disorder.The medical and ophthalmic records were reviewed for a mother-daughter pair diagnosed with FAM58A confirmed STAR syndrome on chromosome Xq28.The mother at birth had left foot syndactyly, telecanthus, anal stenosis, and clitoromegaly and was told at 19 she had a hypoplastic left kidney. The daughter, born at 38 weeks after a complication of oligohydramnios, had a more severe presentation, demonstrating toe syndactyly, telecanthus, anal stenosis, clitoromegaly, bilateral renal hypoplasia, ureteral reflux, urogenital sinus, and congenital heart disease amongst others. The pair shared similar ophthalmic findings, though those of the daughter were more pronounced. They included bilateral, medial upper eyelid prominences with madarosis, mild peripapillary atrophy, and soft macular drusen with the daughter also displaying optic nerve hypoplasia and peripheral anterior synechia in the iridocorneal angle.These ophthalmic findings are the first reported to our knowledge in association with STAR syndrome. The literature frequently demonstrates that patients with developmental anomalies often have ocular manifestations, warranting a full ophthalmic examination when the diagnosis of STAR syndrome has been made or is being considered.

Glaucoma can occur following cataract removal in children, and determining the risk for and factors associated with glaucoma and glaucoma suspect in a large cohort of children after lensectomy can guide clinical practice.To estimate the incidence of glaucoma and glaucoma suspect and describe its management in the first year following lensectomy in children before 13 years of age.A multicenter clinical research registry containing data for 1361 eyes of 994 children who underwent unilateral or bilateral lensectomy between June 2012 and July 2015 at 1 of 61 sites in the United States (n = 57), Canada (n = 3), and the United Kingdom (n = 1). Patients were eligible for inclusion in the study if they were enrolled in the registry within 45 days after lensectomy and had at least 1 office visit between 6 and 18 months after lensectomy. Patient data were reviewed, and glaucoma and glaucoma suspect were diagnosed by investigators using standardized criteria. Statistical analysis was performed between June 2017 and August 2019.Clinical care 6 to 18 months after lensectomy.Incidence risk using standardized definitions of glaucoma and glaucoma suspect after lensectomy.Among 702 patients included in this cohort study, 353 (50.3%) were male and 427 (60.8%) were white; mean age at lensectomy was 3.4 years (range, 0.04-12.9 years). After lensectomy, glaucoma or glaucoma suspect was diagnosed in 66 of 970 eyes (adjusted overall incidence risk, 6.3%; 95% CI, 4.8%-8.3%). Glaucoma was diagnosed in 52 of the 66 eyes, and glaucoma suspect was diagnosed in the other 14 eyes. Mean age at lensectomy in these 66 eyes was 1.9 years (range, 0.07-11.2 years), and 40 of the 66 (60.6%) were eyes of female patients. Glaucoma surgery was performed in 23 of the 66 eyes (34.8%) at a median of 3.3 months (range, 0.9-14.8 months) after lensectomy. The incidence risk of glaucoma or glaucoma suspect was 15.7% (99% CI, 10.1%-24.5%) for 256 eyes of infants 3 months or younger at lensectomy vs 3.4% (99% CI, 1.9%-6.2%) for 714 eyes of infants older than 3 months (relative risk, 4.57; 99% CI, 2.19-9.57; P .001) and 11.2% (99% CI, 7.6%-16.7%) for 438 aphakic eyes vs 2.6% (99% CI, 1.2%-5.6%) for 532 pseudophakic eyes (relative risk, 4.29; 99% CI, 1.84-10.01; P .001). No association was observed between risk of developing glaucoma or glaucoma suspect and any of the following variables: sex, race/ethnicity, laterality of lensectomy, performance of anterior vitrectomy, prelensectomy presence of anterior segment abnormality, or intraoperative complications.This study found that glaucoma or glaucoma suspect developed in a small number of eyes in the first year after lensectomy and may be associated with aphakia and younger age at lensectomy. Frequent monitoring for signs of glaucoma following lensectomy is warranted, especially in infants 3 months or younger at lensectomy and in children with aphakia after lensectomy.

Three-dimensional (3D) printers are increasingly being used in medicine for surgical planning, medical education, patient education, research, and device development. We explore the educational value of a 3D printer for trainees in an ophthalmology residency program. A 3D printer was made available to medical students, residents, and clinical and research fellows in the Department of Ophthalmology at Case Western Reserve University. One of the medical students was proficient in the technology and was available to help. Multiple projects were initiated including the manufacturing of slit-lamp cellphone adapters and various interface and integral custom-made parts for research applications. The 3D printer was found to be useful by 20 of 21 trainees surveyed with 17 of 20 saying they would use it after graduation if they had access to one. All the respondents felt that the availability of the technology can contribute to increased innovation in the ophthalmology department. We encourage other ophthalmology departments to experiment with 3D printing as a tool to foster innovation and creativity for trainees.

The lacrimal system comprises of a series of anatomical structures with specific physiologic properties. Tearing from a nasolacrimal duct obstruction (NLDO) is the most common lacrimal system abnormality encountered by pediatric ophthalmologists. Most NLDOs spontaneously improve with conservative management by 12 months of age, but persistent or atypical cases may be treated with a procedure consisting of probing and irrigation of the lacrimal system. Other less common but significant lacrimal system abnormalities are also discussed.

Canalicular lacerations are common complications of eyelid trauma in the pediatric population. Irrigating air, water, and colored or viscous agents through the intact canaliculus have been suggested to identify the torn proximal edge. We report our experience in repairing canalicular lacerations using a novel viscoelastic injection technique with a Monoka monocanalicular stent.The medical records of patients18 years of age who underwent repair of a canalicular laceration with a monocanalicular stent using superficial viscoelastic deployment to locate the torn canaliculus were retrospectively reviewed. Demographics, cause of eyelid injury, surgical management using our novel viscoelastic injection technique, and outcome were analyzed.A total of 38 children with lid lacerations were identified, of whom the 17 with canalicular involvement were included (mean age, 6.27 years). Canalicular injury in these 17 was due to dog bite (9 patients) and shearing trauma (8 patients). In 11 patients, the injury was located in the lower lid; in 4, the upper lid; and in 2, combined upper and lower lids. All patients had good anatomic repair and on follow-up had negative dye disappearance tests and were free of tearing.Deploying viscoelastic superficially near, and injecting into the injured canaliculus can improve visualization of the operative field by retracting the surrounding tissue and tamponading any bleeding, which aids in location and dilation of the torn canaliculus initially and in subsequent steps, eases intubation into the lubricated torn canaliculus and nasolacrimal duct, and avoids iatrogenic injury to an uninjured canaliculus.

Purpose To compare complication rates of the analgesics fentanyl and morphine in preterm infants undergoing laser therapy for retinopathy of prematurity (ROP). Methods In this observational study, the medical records of consecutive preterm neonates undergoing laser treatment of ROP from June 2007 through September 2010 were retrospectively reviewed. Because a fentanyl-based infusion protocol was initiated in November 2009, there was approximately the same number of treatment sessions with morphine and with fentanyl. In both groups, midazolam was used additionally on a case-by-case basis. Analgesia type, complications, and vital signs were documented at 5-minute intervals for all surgeries. The primary outcome was change in ventilation status. Secondary complications included change in temperature and incidence of apneic, bradycardic, and desaturation events. Results A total of 35 patients were included, with 17 in the morphine group (mean gestational age, 24.8 weeks; mean birth weight, 661 g) and 18 in the fentanyl group (mean gestational age, 24.4 weeks; mean birth weight, 681 g). Overall worsening of ventilation status was noted in 29% of patients in the morphine group and 6% of patients in the fentanyl group ( P = 0.08; 95% confidence interval, −2% to 48%). Temperature instability (outside of 36.5° to 37.4°C range) was noted in 6% of patients in the morphine group and no patients in the fentanyl group. Apneic events were 3.2 times more common and bradycardic events 1.5 times more common in the morphine group. Conclusions We found no difference in safety parameters for fentanyl infusion or morphine for analgesia in preterm infants undergoing ROP laser therapy in the neonatal intensive care unit setting. Although estimates of complication rates suggest that fentanyl may be safer, further study is needed to confirm this premise.

Anterior segment dysgenesis comprises a spectrum of disorders affecting the anterior chamber structures including the cornea, iris, and anterior chamber angle. Importantly, approximately 50 % of patients with anterior segment dysgenesis will develop glaucoma. Conditions with anterior segment dysgenesis include most notably, Axenfeld–Rieger spectrum and Peters anomaly. This chapter will focus on the findings and management of patients with Axenfeld–Rieger spectrum disorders. Classic findings include an anteriorly displaced Schwalbe’s line (posterior embryotoxon), iridocorneal adhesions, and pupillary ectopia and corectopia. However, some patients may present with findings of infantile glaucoma. The condition may or may not be associated with systemic findings. Axenfeld–Rieger spectrum disorders are usually inherited in an autosomal dominant manner with disease-causing mutations in PITX2, FOXC1, and very rarely mutations in PAX6. Significant phenotypic heterogeneity is present, and severity and findings may vary significantly among family members with the same mutation. Ophthalmologic management should include maintaining a clear visual axis, monitoring closely for amblyopia, and glaucoma. Patients with anterior segment dysgenesis should be evaluated by a geneticist for evaluation of possible systemic associated abnormalities and appropriate genetic counseling.

IMPORTANCE: Previous studies of myo-inositol in preterm infants with respiratory distress found reduced severity of retinopathy of prematurity (ROP) and less frequent ROP, death, and intraventricular hemorrhage. However, no large trials have tested its efficacy or safety. OBJECTIVE: To test the adverse events and efficacy of myo-inositol to reduce type 1 ROP among infants younger than 28 weeks’ gestational age. DESIGN, SETTING, AND PARTICIPANTS: Randomized clinical trial included 638 infants younger than 28 weeks’ gestational age enrolled from 18 neonatal intensive care centers throughout the United States from April 17, 2014, to September 4, 2015; final date of follow-up was February 12, 2016. The planned enrollment of 1760 participants would permit detection of an absolute reduction in death or type 1 ROP of 7% with 90% power. The trial was terminated early due to a statistically significantly higher mortality rate in the myo-inositol group. INTERVENTIONS: A 40-mg/kg dose of myo-inositol was given every 12 hours (initially intravenously, then enterally when feeding; n = 317) or placebo (n = 321) for up to 10 weeks. MAIN OUTCOMES AND MEASURES: Type 1 ROP or death before determination of ROP outcome was designated as unfavorable. The designated favorable outcome was survival without type 1 ROP. RESULTS: Among 638 infants (mean, 26 weeks’ gestational age; 50% male), 632 (99%) received the trial drug or placebo and 589 (92%) had a study outcome. Death or type 1 ROP occurred more often in the myo-inositol group vs the placebo group (29% vs 21%, respectively; adjusted risk difference, 7% [95% CI, 0%-13%]; adjusted relative risk, 1.41 [95% CI, 1.08-1.83], P = .01). All-cause death before 55 weeks’ postmenstrual age occurred in 18% of the myo-inositol group and in 11% of the placebo group (adjusted risk difference, 6% [95% CI, 0%-11%]; adjusted relative risk, 1.66 [95% CI, 1.14-2.43], P = .007). The most common serious adverse events up to 7 days of receiving the ending dose were necrotizing enterocolitis (6% for myo-inositol vs 4% for placebo), poor perfusion or hypotension (7% vs 4%, respectively), intraventricular hemorrhage (10% vs 9%), systemic infection (16% vs 11%), and respiratory distress (15% vs 13%). CONCLUSIONS AND RELEVANCE: Among premature infants younger than 28 weeks’ gestational age, treatment with myo-inositol for up to 10 weeks did not reduce the risk of type 1 ROP or death vs placebo. These findings do not support the use of myo-inositol among premature infants; however, the early termination of the trial limits definitive conclusions.

Objective Retinopathy of prematurity (ROP), a vasoproliferative disorder of the retina in preterm infants, is associated with multiple factors, including oxygenation level. We explored whether the common intermittent hypoxemic events in preterm infants are associated with the development of ROP. Study design Oxygen desaturation events were quantified in 79 preterm infants (gestational age, 24 to 27-6/7 weeks) during the first 8 weeks of life. Infants were classified as requiring laser treatment for ROP versus having less severe or no ROP. A linear mixed model was used to study the association between the incidence of intermittent hypoxia and laser treatment of ROP, controlling for gestational age, sex, race, multiple births, and initial severity of illness. Results For all infants, hypoxemic events increased with postnatal age ( P .001). Controlling for all covariates, a higher incidence of oxygen desaturation events was found in the infants undergoing laser therapy for ROP ( P .001), males ( P .02), and infants of younger gestational age ( P .003). Conclusions The incidence of hypoxemic events was higher in infants with ROP requiring laser therapy. Therapeutic strategies to optimize oxygenation in preterm infants should include minimization of desaturation episodes, which may in turn decrease serious morbidity in this high-risk population.

Amblyopia is the leading cause of visual loss in children, affecting 2% to 3% of the population. Occlusion of the dominant eye is the primary and best treatment, although efficacy decreases after 6 years of age. As a result, levodopa/carbidopa has been explored as an adjunct to conventional therapy and has been shown to have an immediate impact on visual acuity. Several studies to date have shown mixed results on the benefit of supplementing occlusion therapy with levodopa/carbidopa, although they have primarily studied children. The authors describe the oldest patient (46 years old) documented in the literature to have shown improvement in visual acuity using levodopa/carbidopa. He was given a 16-week course at a dose in line with previous studies while being effectively occluded full time due to a glaucomatous right eye with no light perception. On 3-month follow-up, his left eye improved two lines and stabilized at 6 months. [ J Pediatr Ophthalmol Strabismus. 2015;52:e45–e47.]

We report endothelial cell (EC) characteristics and central corneal thickness (CCT) from the Infant Aphakia Treatment Study (IATS) patients at the 5-year examination.Randomized, controlled trial of the treatment of unilateral cataract with aphakic contact lens (CL) versus primary intraocular lens (IOL) implant.A total of 114 infants with unilateral cataract.The EC density, coefficient of variation (CV), and percent hexagonal cells were measured by noncontact specular microscopy. The CCT was measured using contact pachymetry. Fellow eyes served as controls.Mean differences between treated and fellow eyes of CL and IOL groups were compared with a paired t test. A 1-way analysis of variance model and the Tukey-Kramer multiple comparison procedure were used to assess the effect of a diagnosis of glaucoma or glaucoma suspect.A total of 105 subjects (52 with CLs, 53 with IOLs) had specular microscopy or corneal thickness data recorded. Mean EC densities were higher in aphakic eyes compared with fellow eyes (3921 vs. 3495 cells/mm2, P0.0001). Mean CV was higher (27 vs. 24, P=0.0002) and mean percent hexagonal cells was lower (72% vs. 76%, P=0.002) in aphakic eyes compared with fellow eyes. Mean CCT of aphakic eyes was higher than in controls (637 vs. 563 μm, P0.0001). There was no difference in EC density in eyes treated with IOLs compared with fellow eyes (3445 and 3487 cells/mm2, P=0.68). Means for CV (25 vs. 24, P=0.07) and percent hexagonal cells (74 vs. 76%, P=0.27) were also not significantly different. Mean CCT was higher in eyes with IOLs (605 vs. 571 μm, P0.0001) compared with fellow eyes. Compared with treated eyes without glaucoma or glaucoma suspect, treated eyes with glaucoma had lower EC density (3289 vs. 3783 cells/mm2, P=0.03) and treated eyes with glaucoma suspect had greater mean corneal thickness (660 vs. 612 μm, P=0.0036).Cataract extraction during infancy with IOL implantation was not associated with a reduced EC count in treated compared with fellow eyes, although CCT was increased. Extended-wear aphakic CLs may cause corneal polymegathism with increased EC density and CCT. Glaucoma diagnosis was associated with reduced EC counts and increased CCT.

BACKGROUND Retinopathy of prematurity [ROP] continues to be a significant clinical problem in preterm infants. There is a need for animal models to better understand the roles of hypoxia/hyperoxia in the pathogenesis and management of ROP. OBJECTIVES To test the hypothesis that multiple daily cycles of intermittent hypoxia, followed by brief hyperoxia, would provide a clinically relevant protocol for generation of ROP in a rat pup. METHODS Rat pups were exposed for the first 14 days to one of three protocols: room air [RA], sustained cycles of hyperoxia/hypoxia [SHH] as previously employed to produce ROP in rat pups, and intermittent hypoxia/hyperoxia [IHH] in order to more closely simulate clinical conditions in preterm infants. Retinae were obtained at 18 days and imaged for both avascularization and neovascularization. RESULTS As expected, the SHH group demonstrated significantly increased avascularity [40.9 ± 7.9% of retina] which was minimal in both RA and IHH groups. All SHH exposed pups exhibited neovascularization which occurred in 5/7 IHH exposed retinae versus 0 in the RA group [p = 0.02]. However, mean number of clock hours of neovascularization after IHH was 1.9 ± 2.1 which did not differ from the RA group, and was less than in the SHH group [8.3 ± 1.9, p < 0.001]. CONCLUSION A more clinically relevant intermittent hypoxia/hyperoxia [IHH] protocol does not produce the same degree of ROP as the traditional sustained hypoxia/hyperoxia [SHH] paradigm. Nonetheless, further refinement of our model may provide a suitable model for understanding the lesser degrees of ROP which predominate in preterm infants.

Background: Volunteer ophthalmologists can achieve success with teaching and service programs working with high intensity over a short term. Continuation of initially successful programs may be limited by lack of timely, effective communication and follow-up. In an attempt to overcome these limitations, a total of 6 telemedicine programs were established after a successful trial program at the Ramon Pando Ferrer Hospital in Havana, Cuba. Methods: Two pediatric ophthalmology-strabismus clinics, one in Cuba and one in Romania, were provided a digital camera and a computer in order to obtain and then transmit by e-mail patient images obtained according to a prescribed format. Ophthalmologists in both of these programs were instructed personally during an orientation period in their clinics. Training included use of a digital camera and computer, patient examination, and surgical technique in the operating room. Four additional programs referred patients via digital images after receiving only written and oral instruction. Results: The diagnosis and treatment plan determined by one of us (E.M.H.) for each of the first 15 Cuban patients after study of digital images sent by e-mail was the same as the diagnosis and treatment plan determined by the same observer after in-person examination of the patients. On the basis of the level of confidence attained in these patients, 35 additional patients from a total of 6 clinics were seen by digital consultation only. Conclusion: A store-and-forward telemedicine consultation technique that uses digital images and e-mail holds promise to be an effective means for carrying out consultation for patients with strabismus. (J AAPOS 2001;5:291-6)

Purpose: To examine the waveform and clinical effects of the four-muscle tenotomy and reattachment procedure in fusion maldevelopment nystagmus syndrome (FMNS) and to compare them to those documented in infantile nystagmus syndrome (INS) and acquired nystagmus. Methods: Both infrared reflection and high-speed digital video systems were used to record the eye movements in a patient with FMNS (before and after tenotomy and reattachment). Data were analyzed using the eXpanded Nystagmus Acuity Function (NAFX) that is part of the OMtools software. Model simulations and predictions were performed using the authors’ behavioral ocular motor system model in MATLAB Simulink (The MathWorks, Inc., Natick, MA). Results: The model predicted, and the patient’s data confirmed, that the tenotomy and reattachment procedure produces improvements in FMN waveforms across a broader field of gaze and decreases the Alexander’s law variation. The patient’s tenotomy and reattachment plots of NAFX after surgery versus gaze angle were higher and had lower slope than before surgery. Clinically, despite moderate improvements in both peak measured acuity and stereoacuity, dramatic improvements in the patient’s abilities and lifestyle resulted. Conclusions: The four-muscle tenotomy and reattachment nystagmus surgery produced beneficial therapeutic effects on FMN waveforms that are similar to those demonstrated in INS and acquired nystagmus. These results support the authors’ prior recommendation that tenotomy and reattachment nystagmus should be added to required strabismus procedures in patients who also have FMNS (ie, perform tenotomy and reattachment on all unoperated muscles in the plane of the nystagmus). Furthermore, when strabismus surgery is not required, four-muscle tenotomy and reattachment may be used to improve FMN waveforms and visual function. [J Pediatr Ophthalmol Strabismus 2014;51(3):180–188.]