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.

Visual acuity outcomes in pediatric glaucomas.

PURPOSE: To evaluate and compare the visual acuity prognosis in the various pediatric glaucoma subtypes and to determine risk factors for vision loss. METHODS: The medical records of pediatric glaucoma patients from 2000 to 2010 at Children's Hospital of Philadelphia were retrospectively reviewed. Visual acuities, surgeries, glaucoma subtype, and etiology of vision impairment were recorded. Univariate and multivariate analyses were performed to determine the risk factors for visual impairment. RESULTS: A total of 133 eyes (36.8% primary congenital glaucoma, 28.6% aphakic glaucoma, 12.0% glaucoma associated with anterior segment dysgenesis, 12.0% Sturge-Weber glaucoma) of 88 patients were included. At last follow-up (median length, 5 years), 46.6% eyes achieved excellent (≥20/70) visual acuity. Of the glaucoma subtypes, primary congenital glaucoma conferred the best visual prognosis, with 69.4% eyes with excellent (≥20/70) visual acuity at final follow-up. Factors most associated with visual impairment (<20/200) were unilateral disease, multiple surgeries, poor vision at diagnosis, and other ocular comorbidities. The most common primary etiology for vision impairment was amblyopia (54.9%). CONCLUSIONS: Patients with glaucoma early in life appear to have a better visual acuity prognosis than previously reported, with those with primary congenital glaucoma faring better than other glaucoma subtypes. Recognition of risk factors for visual impairment can better guide clinical management and counseling of patients.

Magnetic resonance imaging of tissues compatible with supernumerary extraocular muscles.

PURPOSE: To determine by magnetic resonance imaging (MRI) the prevalence and anatomy of anomalous extraocular muscle (EOM) bands. DESIGN: Prospective, observational case series. METHODS: High-resolution, multipositional, surface coil orbital MRI was performed using T1 or T2 fast spin echo weighting with target fixation control under a prospective protocol in normal adult subjects and a diverse group of strabismic patients between 1996 and 2009. Images demonstrating anomalous EOM bands were analyzed digitally to evaluate their sizes and paths, correlating findings with complete ophthalmic and motility examinations. RESULTS: Among 118 orthotropic and 453 strabismic subjects, 1 (0.8%) orthotropic and 11 (2.4%) strabismic subjects exhibited unilateral or bilateral orbital bands having MRI signal characteristics identical to EOM. Most bands occurred without other EOM dysplasia and coursed in the retrobulbar space between rectus EOMs such as the medial rectus to lateral rectus, from superior to inferior rectus, or from 1 EOM to the globe. In 2 cases, horizontal bands from the medial rectus to lateral rectus muscles immediately posterior to the globe apparently limited supraduction by collision with the optic nerve. All bands were too deep to be approached via conventional strabismus surgical approaches. CONCLUSIONS: Approximately 2% of humans exhibit on MRI deep orbital bands consistent with supernumerary EOMs. Although band anatomy is nonoculorotary, some bands may cause restrictive strabismus.