Teprotumumab for Thyroid Eye Disease-related Strabismus.

PURPOSE: To assess and quantify teprotumumab's effect on thyroid eye disease-related strabismus by change in measured horizontal and vertical deviations and change in extraocular motility. METHODS: We reviewed a series of patients with thyroid eye disease-related strabismus treated with teprotumumab. Exclusion criteria included age under 18 years, strabismus of alternate etiology, or thyroid eye disease-related reconstructive surgery during the treatment course. Primary outcomes were absolute (prism diopters) and relative (%) differences in horizontal and vertical deviations in primary position at distance, as well as change in ductions of the more affected eye. Secondary outcomes included incidence and timing of strabismus surgery postteprotumumab. RESULTS: Thirty-one patients were included, with mean age 63 years and thyroid eye disease duration 10 months. After teprotumumab, there was 6 prism diopters (39%) mean reduction in vertical deviation ( p < 0.001), without significant change in mean horizontal deviation ( p = 0.75). Supraduction, abduction, adduction, and infraduction significantly improved in the more restricted eye ( p < 0.01, p < 0.01, p = 0.04, and p = 0.01, respectively). Thirty-five percent of patients underwent strabismus surgery posttreatment, at an average 10 months after last infusion. CONCLUSIONS: Teprotumumab produced a statistically significant reduction in vertical but not horizontal strabismus angles in primary position at distance. Extraocular motility in all 4 ductions also improved. A substantial minority of patients still required strabismus surgery following teprotumumab.

MACULAR MICROVASCULAR NETWORKS IN HEALTHY PEDIATRIC SUBJECTS.

PURPOSE: To report optical coherence tomography angiography (OCTA) values in healthy pediatric eyes and to identify factors that may modify these values. METHODS: In this prospective observational cross-sectional study, macular OCTA images were acquired from healthy pediatric patients. Main outcome measures were 1) foveal avascular zone (FAZ) area at the level of the superficial retinal capillary plexus (SCP); 2) SCP and deep retinal capillary plexus (DCP) perfusion density (based on the area of vessels); 3) SCP and DCP vessel density (based on a map with vessels of 1-pixel width); and 4) CC perfusion density. Multiple regression analysis was performed to assess the effect of age, sex, ethnicity, refraction, and foveal macular thickness (FMT) on OCTA parameters. RESULTS: Seventy-seven eyes from 52 subjects (23 male and 29 female) were included in analysis. Mean age was 11.1 ± 3.3 years (range = 5.0-17.0 years). Twenty-nine (55.8%) subjects were white, 14 (27.0%) Hispanic, 8 (15.4%) Asian, and 1 (1.8%) African-American. Mean refraction was -0.1 ± 2.4 diopters (D) (range = -5.75 to +9.0 D). Mean FMT was 248.6 ± 18.6 µm. Larger FAZ area was significantly associated with older age (P = 0.014). Furthermore, larger FAZ area was associated with reduced FMT (P < 0.0001). Male sex was associated only with increased SCP perfusion density (P = 0.042). Increased CC perfusion density was associated with younger age (P = 0.022). CONCLUSION: We report data for pediatric OCTA parameters in healthy subjects. Several variables influence the density of macular microvascular networks, and these factors should be considered in the OCTA study of pediatric eye disorders.

Accuracy of Diagnostic Imaging Modalities for Classifying Pediatric Eyes as Papilledema Versus Pseudopapilledema.

PURPOSE: To identify the most accurate diagnostic imaging modality for classifying pediatric eyes as papilledema (PE) or pseudopapilledema (PPE). DESIGN: Prospective observational study. SUBJECTS: Nineteen children between the ages of 5 and 18 years were recruited. Five children (10 eyes) with PE, 11 children (19 eyes) with PPE owing to suspected buried optic disc drusen (ODD), and 3 children (6 eyes) with PPE owing to superficial ODD were included. METHODS: All subjects underwent imaging with B-scan ultrasonography, fundus photography, autofluorescence, fluorescein angiography (FA), optical coherence tomography (OCT) of the retinal nerve fiber layer (RNFL), and volumetric OCT scans through the optic nerve head with standard spectral-domain (SD OCT) and enhanced depth imaging (EDI OCT) settings. Images were read by 3 masked neuro-ophthalmologists, and the final image interpretation was based on 2 of 3 reads. Image interpretations were compared with clinical diagnosis to calculate accuracy and misinterpretation rates of each imaging modality. MAIN OUTCOME MEASURES: Accuracy of each imaging technique for classifying eyes as PE or PPE, and misinterpretation rates of each imaging modality for PE and PPE. RESULTS: Fluorescein angiography had the highest accuracy (97%, 34 of 35 eyes, 95% confidence interval 92%-100%) for classifying an eye as PE or PPE. FA of eyes with PE showed leakage of the optic nerve, whereas eyes with suspected buried ODD demonstrated no hyperfluorescence, and eyes with superficial ODD showed nodular staining. Other modalities had substantial likelihood (30%-70%) of misinterpretation of PE as PPE. CONCLUSIONS: The best imaging technique for correctly classifying pediatric eyes as PPE or PE is FA. Other imaging modalities, if used in isolation, are more likely to lead to misinterpretation of PE as PPE, which could potentially result in failure to identify a life-threatening disorder causing elevated intracranial pressure and papilledema.

OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY OF THE FOVEA IN CHILDREN BORN PRETERM.

PURPOSE: To compare the foveal avascular zone (FAZ) area measured by optical coherence tomography angiography in children who had been born preterm with age-matched controls. METHODS: In this cross-sectional observational comparative case series, 43 eyes of 26 children (28 eyes of 15 former preterm infants and 15 eyes of 11 former term infants) between the ages of 4 and 12 years old were included. Optical coherence tomography angiography with a scan size of 3 × 3 was performed for all eyes. Foveal avascular zone area was measured using the Optovue RTVue AVANTI instrument (Optovue Inc, Fremont, CA) software. Inner and outer retinal thicknesses were measured with the instrument caliper. RESULTS: A distinct FAZ was absent in 12 eyes (42.8%) of children with a history of preterm birth, however, it was present in all (100%) control eyes. The FAZ area was significantly correlated with gestational age (r = 0.82, P < 0.001) and birth weight (r = 0.80, P < 0.001). The gestational age was less than 29 weeks and birth weight was less than 1,480 grams in eyes with no distinct FAZ. Mean central foveal vessel density in the superficial capillary plexus was 41.8 ± 4.4% in the preterm group and 32.8 ± 5.8% in the control group (P < 0.001). In all eyes, a significant negative correlation was found between the central foveal vessel density and gestational age (r = -0.63, P = 0.001) and birth weight (r = -0.59, P = 0.002). On spectral domain optical coherence tomography examination, the foveal depression was absent and the inner retinal layers were preserved in all eyes with absent FAZ. In all eyes, a significant negative correlation was found between the inner retinal thickness and gestational age (r = -0.68, P < 0.001) and birth weight (r = -0.61, P = 0.001). Ten eyes of 6 preterm children had a history of laser therapy for retinopathy of prematurity. A distinct FAZ was absent in six eyes (60%) with retinopathy of prematurity with history of laser therapy, and six eyes (33.3%) with preterm birth without laser therapy. Eyes with history of laser therapy had a statistically significantly higher inner retinal thickness and central foveal vessel density and smaller FAZ compared with the eyes with preterm birth without laser therapy (P < 0.001, P = 0.04 and P = 0.03, respectively). CONCLUSION: Optical coherence tomography angiography is a novel modality for noninvasive visualization of the retinal vasculature in pediatric patients and expands our knowledge of foveal abnormalities in retinopathy of prematurity. A small or absent FAZ seems to be a distinct sign of prematurity.

Strabismus Surgery in Patients With Ocular Neuromyotonia: Potential Unmasking of the Condition and Effective Management Tool.

BACKGROUND: Ocular neuromyotonia (ONM) is a rare motility disorder in which paroxysms of tonic extraocular muscle contraction from abnormal ocular motor nerve firing result in episodic diplopia and strabismus. Medical therapy with membrane-stabilizing agents has varied success. A surgical approach to treatment has not yet been described. We report the outcomes of strabismus surgery in patients with ONM. METHODS: We describe 3 patients with sixth nerve paresis and ONM of the affected lateral rectus muscle who underwent strabismus surgery. All patients had a history of radiation therapy for intracranial tumors. Ophthalmologic and orthoptic examinations were performed with appropriate medical and neuroradiologic evaluation. Preoperative and postoperative data are presented and analyzed. RESULTS: Two patients were noted to have ONM after their first strabismus surgery for a sixth nerve palsy. Patients 1 and 2 had 3 surgeries, whereas Patient 3 had 1 operation. Extraocular muscles operated on included the medial rectus and lateral rectus. Preoperative primary gaze baseline esotropia ranged from 35 to 75 prism diopters (Δ). All patients achieved improvement in ocular alignment and motility. Postoperative primary gaze deviations ranged from orthotropia to 20Δ of esotropia. Abduction deficits were unchanged or improved. The follow-up period ranged from 15 months to 2 years. CONCLUSIONS: Patients with ONM of a paretic rectus muscle can achieve binocular fusion with strabismus surgery. ONM may manifest postoperatively in patients with a sixth nerve palsy and a contractured medial rectus who, preoperatively, were not noted to have ONM.

Dry eye disease in the young: A narrative review.

Dry eye disease (DED), a multifactorial ocular disease that significantly impacts quality of life, is most commonly reported in adults. This review describes the prevalence, risk factors, diagnosis and management of DED in children. A literature search, conducted from January 2000-December 2022, identified 54 relevant publications. Using similar diagnostic criteria to those reported in adults, namely standardized questionnaires and evaluation of tear film homeostatic signs, the prevalence of DED in children ranged from 5.5% to 23.1 %. There was limited evidence for the influence of ethnicity in children, however some studies reported an effect of sex in older children. Factors independently associated with DED included digital device use, duration of digital device use, outdoor time and urban living, Rates of DED were higher in children with ocular allergy and underlying systemic diseases. Compared with similar studies in adults, the prevalence of a prior DED diagnosis or a diagnosis based on signs and symptoms was lower in children, but symptoms were commonly reported. Treatment options were similar to those in adults, including lifestyle modifications, blinking, management of lid disease and unpreserved lubricants in mild disease with escalating treatment with severity. Management requires careful exploration of symptoms, medical history and the diagnosis and management of ocular comorbidities such as allergy and anterior blepharitis. Appropriately powered population-based studies are required to understand the prevalence of and risk factors for DED in children. Development of age-appropriate thresholds for signs and symptoms of DED would support better diagnosis of disease and understanding of natural history.

Inferior Rectus Muscle Y-Split for Thyroid-Related Vertical Strabismus.

Incomitant hypotropia in thyroid eye disease can be difficult to manage, especially in the presence of orthotropia with fusion in down gaze and reading position. Recessing the affected ipsilateral inferior rectus muscle may result in an undesirable downgaze diplopia secondary to a hypertropia in downgaze. Various surgical techniques have been described to manage this potential complication including asymmetric recession of both inferior rectus muscles, posterior myoscleropexy operation, and the Scott recess/resect procedure of the contralateral inferior rectus. In 2004, Hoerantner et al. introduced the y-split recession of the medial rectus muscle for near esotropic deviations. The anterior portion of muscle is split and secured in a y-shaped configuration, which reduces the muscle lever arm and helps minimize incomitance and muscle slippage. Unlike the traditional Cüppers Faden, a y-split recession results in torque reduction in all gaze positions. In addition, a y-split recession does not involve scleral passes posteriorly reducing the risk of globe perforation. We report a patient with incomitant strabismus secondary to thyroid eye disease who underwent a combination of traditional recession and y-splitting recession of the contralateral inferior rectus muscle, resulting in good functional alignment in primary gaze and in the reading position.

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.

Outcomes of Strabismus Surgery Following Teprotumumab Therapy.

PURPOSE: To investigate the results of patients undergoing surgical treatment for strabismic diplopia in thyroid eye disease (TED) following teprotumumab. DESIGN: Multicenter, retrospective, case series. METHODS: We report 28 patients who underwent extraocular muscle surgery for strabismic diplopia after treatment with teprotumumab at 7 different academic centers. Elapsed time from last teprotumumab dose to the date of surgery, previous orbital decompression, primary preoperative horizontal and vertical deviation, surgical procedure, and 2-month postoperative results were collected from the patient records. RESULTS: Sixteen (57%) patients were diplopia-free after 1 surgery. Three (11%) chose prism spectacles to correct residual diplopia, 2 (7%) used compensatory head posture to resolve diplopia, and 1 (4%) had intermittent diplopia and was functionally improved (choosing no prisms or further surgery). These were considered treatment successes. Three (11%) patients required reoperation, and all were diplopia-free after their second procedure. CONCLUSIONS: Most patients requiring surgery for strabismic diplopia following teprotumumab achieve good outcomes with success rates comparable to series published before the availability of teprotumumab.

Asymmetric inferior oblique anterior transposition for incomitant asymmetric dissociated vertical deviation.

BACKGROUND: Inferior oblique anterior transposition (IOAT) is indicated in patients with incomitant dissociated vertical deviation (DVD) larger in adduction. In general, bilateral surgery is recommended in patients with DVD unless there is deep monocular amblyopia. The purpose of this study is to evaluate the results of asymmetric IOAT in patients with asymmetric incomitant DVD larger in adduction. METHODS: Retrospective chart review of the records of all patients with incomitant asymmetric DVD associated with inferior oblique (IO) overaction who underwent asymmetric IO weakening procedure. In all patients, the eye with more DVD in adduction underwent IOAT to the temporal corner of the insertion of the inferior rectus (IR) muscle, and the eye with less DVD underwent IOAT to a position 3-4 mm posterior to the insertion of the IR. No other muscles were operated simultaneously. No patient had previous surgery on any cyclovertical extracular muscle. RESULTS: Fourteen patients were included. Mean age at surgery was 10.3 ± 8.8 years (range 4-33). Primary position DVD preoperatively was 18 ± 2 PD in the eye with the larger DVD compared to 1.1 ± 1.0 PD postoperatively (p < 0.0001). DVD asymmetry between the lateral gaze with the largest DVD and the lateral gaze with the smallest DVD was 9.8 ± 3.1 PD (range 5-14 PD) preoperatively vs 1.1 ± 1.0 PD (range 0-2 PD), (p < 0.0001). Ten patients had preoperative V-pattern >10 PD (24.7 ± 8.7 PD, range 12-50 PD) preoperatively vs no patients postoperatively (mean V-pattern 4.4 ± 2.0 PD), (p < 0.0001). Postoperative follow up was 1.6 ± 0.7 years (range 1-3 years). CONCLUSION: In patients with asymmetric incomitant DVD, asymmetric IOAT improves lateral incomitance without increasing the risk of antielevation, limitation in upgaze rotation, or hypertropia, or worsening the DVD in the eye with less deviation preoperatively.

Long-term follow-up of strabismus surgery for patients with ocular myasthenia gravis.

BACKGROUND: Patients with ocular myasthenia gravis (OMG) may develop strabismus and diplopia. We completed a retrospective observational case series to evaluate long-term surgical outcomes in patients with OMG. METHODS: The medical records of all patients with OMG who underwent strabismus surgery with at least 6 months of postoperative follow-up were reviewed. Nine patients met the study inclusion criteria. The main outcomes, including ocular alignment, number of surgeries, and sensory status were evaluated. RESULTS: Of these patients, initially 2 had horizontal strabismus alone, 3 had vertical strabismus alone, 3 had both vertical and horizontal strabismus, and 1 patient had vertical and torsional strabismus. The length of preoperative stability was 2.0 ± 2.5 years (range: 0.1-8.0 years). The mean preoperative horizontal and vertical deviations were 40.5 ± 32.5 prism diopters (PD; range: 0-90 PD) and 25.6 ± 36.7 PD (range: 0-120 PD), respectively. The average length of the follow-up after the first surgery was 5.7 ± 4.2 years (range: 0.7-10.6 years). Four patients (44%) underwent 2 operations. For patients requiring a second operation, the time to second operation was 2.3 years (range: 0.4-5.0 years). Six patients (67%) were within 10 PD of orthotropia at distance in primary position at the final visit. Five patients (55%) had single vision after their surgeries. CONCLUSION: Strabismus surgery can achieve good long-term binocular alignment in patients with OMG.

A Potentially Adjustable Modification of the Nishida Procedure.

For patients with a complete, chronic abducens nerve palsy and resulting abduction deficit, a transposition procedure is often the procedure of choice. One such transposition procedure involves transposing the superior rectus (SR) and inferior rectus (IR) laterally without disinserting or splitting either muscle. While effective, this procedure - like many transposition procedures - carries with it the risk of induced torsional or vertical misalignment. Here, we describe an adjustable variation of the above transposition procedure, one which potentially would allow for post-operative correction of induced vertical or torsional deviations.

Optical Coherence Tomography Findings in Cherry-Red Spot: Implications for Understanding Pathophysiology and Visual Prognosis.

PURPOSE: To report optical coherence tomography (OCT) findings of cherry-red spots from Tay-Sachs and Niemann-Pick disease. METHODS: Consecutive patients with Tay-Sachs and Niemann-Pick disease evaluated by the pediatric transplant and cellular therapy team, for whom a handheld OCT scan was obtained, were included. Demographic information, clinical history, fundus photography, and OCT scans were reviewed. Two masked graders evaluated each of the scans. RESULTS: The study included 3 patients with Tay-Sachs disease (5, 8, and 14 months old) and 1 patient with Niemann-Pick disease (12 months old). All patients had bilateral cherry-red spots on fundus examination. In all patients with Tay-Sachs disease, handheld OCT revealed parafoveal ganglion cell layer (GCL) thickening, increased nerve fiber layer, and GCL reflectivity, and different levels of residual normal signal GCL. The patient with Niemann-Pick disease had similar parafoveal findings, but there was a thicker residual GCL. Sedated visual evoked potentials were unrecordable in all 4 patients despite 3 of them demonstrating normal visual behavior for age. Patients with good vision had relative sparing of the GCL on OCT. CONCLUSIONS: The cherry-red spots in lysosomal storage diseases appear as perifoveal thickening and hyperreflectivity of the GCL on OCT. In this case series, residual GCL with normal signal proved to be a better biomarker for visual function than visual evoked potentials and could be considered for future therapeutic trials. [J Pediatr Ophthalmol Strabismus. 2023;60(6):435-440.].

AAPOS Adult Strabismus Committee's Report on the Status of Adult Strabismus Telemedicine.

PURPOSE: To evaluate the utility of telemedicine in the treatment of adult patients with strabismus. METHODS: A 27-question online survey was sent to ophthalmologists of the American Association for Pediatric Ophthalmology and Strabismus (AAPOS) Adult Strabismus Committee. The questionnaire focused on the frequency of telemedicine utilization, the benefits in the diagnosis, follow-up, and treatment of adult strabismus, and barriers of current forms of remote patient visits. RESULTS: The survey was completed by 16 of 19 members of the committee. Most respondents (93.8%) reported 0 to 2 years of experience with telemedicine. Telemedicine was found to be useful for initial screening and follow-up of established patients with adult strabismus, mainly to reduce wait time for a subspecialist visit (46.7%). A successful telemedicine visit could be completed with a basic laptop (73.3%) or a camera (26.7%) or could be assisted by an orthoptist. Most participants agreed that common forms of adult strabismus (cranial nerve palsies, sagging eye syndrome, myogenic strabismus, and thyroid ophthalmopathy) could be examined via webcam. It was easier to analyze horizontal than vertical strabismus. Among the paralytic forms, sixth nerve palsy was the easiest one to assess. Latent forms of strabismus can be partially diagnosed and evaluated using telemedicine, but half of the respondents underlined the importance of in-person examinations in these cases. Sixty-nine percent believed that telemedicine could be a low-cost and time-efficient health service solution. CONCLUSIONS: Most members of the AAPOS Adult Strabismus Committee consider telemedicine to be a useful supplement to the current adult strabismus practice. [J Pediatr Ophthalmol Strabismus. 2023;60(6):386-389.].

Anterior segment ischemia following strabismus surgery in a young adult using gender-affirming estrogen hormone therapy.

Well-known risk factors for anterior segment ischemia (ASI) following strabismus surgery include ipsilateral surgery on three or more rectus muscles, older age, and vasculopathy. ASI is rarely reported in young patients following uneventful strabismus surgery on two ipsilateral rectus muscles. We report a 30-year-old transgender female on long-term estrogen therapy who underwent strabismus surgery involving recessions of both lateral rectus muscles, the right inferior rectus muscle, and the left superior rectus muscle. The left eye developed severe ASI with hypotony maculopathy that was resistant to topical medications, oral steroids, anterior chamber reformation, and intravitreal steroid injection. Following phacoemulsification with intraocular lens and capsular tension ring insertion 1 year later, intraocular pressure and hypotony maculopathy improved.

OPTICAL COHERENCE TOMOGRAPHY AND OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FINDINGS AND VISUAL PROGNOSIS IN TWO PATIENTS WITH POSTERIOR MICROPHTHALMOS.

BACKGROUND/PURPOSE: Posterior microphthalmos is a rare ocular condition characterized by a disproportionately small posterior segment and multiple retinal abnormalities. The diagnosis can be challenging, and novel findings on optical coherence tomography (OCT) angiography may help to identify this unusual disorder. The purpose of this study is to report OCT and OCT angiography findings in two siblings with posterior microphthalmos. METHODS: Case series at an academic eye institute including two affected siblings with posterior microphthalmos. RESULTS: The results of OCT testing in two siblings with posterior microphthalmos illustrated bilateral papillomacular retinal fold and loss of the foveal depression. Optical coherence tomography angiography findings included loss of the foveal avascular zone and complete vascularization across the fovea. CONCLUSION: Unique OCT angiography findings in posterior microphthalmos are described herein. These findings may support the diagnosis of posterior microphthalmos and aid in the understanding of the pathogenesis of this disease.