Update on thyroid eye disease and management

Thyroid eye disease is a heterogeneous autoimmune orbital reaction typically manifesting in middle age. The inflammation may parallel or remain isolated from a related inflammatory cascade in the thyroid called Graves’ disease. The orbital manifestations can lead to severe proptosis, dry eyes, strabismus, and optic neuropathy. In this article, we will discuss this unique condition including the ophthalmic findings and management

Outcomes of Bilateral Cataract Surgery in Infants 7 to 24 Months of Age Using the Toddler Aphakia and Pseudophakia Treatment Study Registry

Purpose To evaluate outcomes of bilateral cataract surgery in children aged 7 to 24 months and compare rates of adverse events (AEs) with other Toddler Aphakia and Pseudophakia Study (TAPS) registry outcomes. Design Retrospective clinical study at 10 Infant Aphakia Treatment Study (IATS) sites. Statistical analyses comparing this cohort with previously reported TAPS registry cohorts. Participants Children enrolled in the TAPS registry between 2004 and 2010. Methods Children underwent bilateral cataract surgery with or without intraocular lens (IOL) placement at age 7 to 24 months with 5 years of postsurgical follow-up. Main Outcome Measures Visual acuity (VA), occurrence of strabismus, AEs, and reoperations. Results A total of 40 children (76 eyes) who underwent bilateral cataract surgery with primary posterior capsulectomy were identified with a median age at cataract surgery of 11 months (7–23); 68% received a primary IOL. Recurrent visual axis opacification (VAO) occurred in 7.5% and was associated only with the use of an IOL (odds ratio, 6.10; P = 0.005). Glaucoma suspect (GS) was diagnosed in 2.5%, but no child developed glaucoma. In this bilateral cohort, AEs (8/40, 20%), including glaucoma or GS and VAO, and reoperations occurred in a similar proportion to that of the published unilateral TAPS cohort. When analyzed with children aged 1 to 7 months at bilateral surgery, the incidence of AEs and glaucoma or GS correlated strongly with age at surgery (P = 0.011/0.004) and glaucoma correlated with microcornea (P = 0.040) but not with IOL insertion (P = 0.15). Conclusions Follow-up to age 5 years after bilateral cataract surgery in children aged 7 to 24 months reveals a low rate of VAO and very rare glaucoma or GS diagnosis compared with infants with cataracts operated at < 7 months of age despite primary IOL implantation in most children in the group aged 7 to 24 months. The use of an IOL increases the risk of VAO irrespective of age at surgery

Outcomes of Bilateral Cataracts Removed in Infants 1 to 7 Months of Age Using the Toddler Aphakia and Pseudophakia Treatment Study Registry

Purpose To evaluate outcomes of bilateral cataract surgery in infants 1 to 7 months of age performed by Infant Aphakia Treatment Study (IATS) investigators during IATS recruitment and to compare them with IATS unilateral outcomes. Design Retrospective case series review at 10 IATS sites. Participants The Toddler Aphakia and Pseudophakia Study (TAPS) is a registry of children treated by surgeons who participated in the IATS. Methods Children underwent bilateral cataract surgery with or without intraocular lens (IOL) placement during IATS enrollment years 2004 through 2010. Main Outcome Measures Visual acuity (VA), strabismus, adverse events (AEs), and reoperations. Results One hundred seventy-eight eyes (96 children) were identified with a median age of 2.5 months (range, 1–7 months) at the time of cataract surgery. Forty-two eyes (24%) received primary IOL implantation. Median VA of the better-seeing eye at final study visit closest to 5 years of age with optotype VA testing was 0.35 logarithm of the minimum angle of resolution (logMAR; optotype equivalent, 20/45; range, 0.00–1.18 logMAR) in both aphakic and pseudophakic children. Corrected VA was excellent (<20/40) in 29% of better-seeing eyes, 15% of worse-seeing eyes. One percent showed poor acuity (≥20/200) in the better-seeing eye, 12% in the worse-seeing eye. Younger age at surgery and smaller (<9.5 mm) corneal diameter at surgery conferred an increased risk for glaucoma or glaucoma suspect designation (younger age: odds ratio [OR], 1.44; P = 0.037; and smaller cornea: OR, 3.95; P = 0.045). Adverse events also were associated with these 2 variables on multivariate analysis (younger age: OR, 1.36; P = 0.023; and smaller cornea: OR, 4.78; P = 0.057). Visual axis opacification was more common in pseudophakic (32%) than aphakic (8%) eyes (P = 0.009). Unplanned intraocular reoperation occurred in 28% of first enrolled eyes (including glaucoma surgery in 10%). Conclusions Visual acuity after bilateral cataract surgery in infants younger than 7 months is good, despite frequent systemic and ocular comorbidities. Although aphakia management did not affect VA outcome or AE incidence, IOL placement increased the risk of visual axis opacification. Adverse events and glaucoma correlated with a younger age at surgery and glaucoma correlated with the presence of microcornea

Extraocular Muscles in Patients With Infantile Nystagmus Adaptations at the Effector Level

Objective: To test the hypothesis that the extraocular muscles (EOMs) of patients with infantile nystagmus have muscular and innervational adaptations that may have a role in the involuntary oscillations of the eyes. Methods: Specimens of EOMs from 10 patients with infantile nystagmus and postmortem specimens from 10 control subjects were prepared for histologic examination. The following variables were quantified: mean myofiber cross-sectional area, myofiber central nucleation, myelinated nerve density, nerve fiber density, and neuromuscular junction density. Results: In contrast to control EOMs, infantile nystagmus EOMs had significantly more centrally nucleated myofibers, consistent with cycles of degeneration and regeneration. The EOMs of patients with nystagmus also had a greater degree of heterogeneity in myofiber size than did those of controls, with no difference in mean myofiber cross-sectional area. Mean myelinated nerve density, nerve fiber density, and neuromuscular junction density were also significantly decreased in infantile nystagmus EOMs. Conclusions: The EOMs of patients with infantile nystagmus displayed a distinct hypoinnervated phenotype. This represents the first quantification of changes in central nucleation and myofiber size heterogeneity, as well as decreased myelinated nerve, nerve fiber, and neuromuscular junction density. These results suggest that deficits in motor innervation are a potential basis for the primary loss of motor control.National Eye Institute [EY15313, EY11375]National Eye InstituteMinnesota Medical FoundationMinnesota Medical FoundationMinnesota Lions and LionessesMinnesota Lions and LionessesFight for SightFight for SightChildrens Hospital Ophthalmology Foundation, Boston, MassachusettsChildren's Hospital Ophthalmology Foundation, Boston, MassachusettsResearch to Prevent BlindnessResearch to Prevent Blindnes

Ultra-low-dose (boom-boom) radiotherapy for management of recurrent ocular post-transplant lymphoproliferative disorder

Purpose: To report a case of recurrent iris post-transplant lymphoproliferative disorder (PTLD) treated with ultra-low-dose (boom-boom) radiotherapy (RT). Observations: A 12-year-old Caucasian male with a history of bilateral, recurrent iris PTLD of the extranodal marginal zone lymphoma (MALT) type presented with persistent bilateral anterior chamber cellular infiltration, which was incompletely controlled on topical corticosteroids, and with elevated intraocular pressure (IOP) in the right eye secondary to steroid response. The patient was diagnosed with PTLD recurrence and was successfully treated with ultra-low-dose RT to both eyes in 2 fractions of 2 Gy. At 15 month follow-up the patient maintained complete disease control with normal IOP off all topical ophthalmic medications. Conclusions and Importance: Ultra-low-dose RT for ocular PTLD of the MALT subtype represents a novel therapeutic approach that may provide a durable treatment response and could be considered as either primary or adjuvant therapy for this rare condition

Diplopic versus nondiplopic strabismus: effects on functional vision and eye-related quality of life in adolescents

Twenty adolescents (12-17 years old) with diplopic strabismus and 20 with nondiplopic strabismus (matched to diplopic subjects for direction and magnitude of ocular deviation) completed the Pediatric Eye Questionnaire (PedEyeQ). Children completed the Child PedEyeQ, and one parent for each child completed the Proxy PedEyeQ and Parent PedEyeQ. PedEyeQ Rasch domain scores were calculated and converted to a scale of 0 (worst) to 100 (best). Distributions of domain scores were compared between diplopic and nondiplopic cohorts using Wilcoxon tests. Diplopic adolescents had significantly lower Child PedEyeQ scores on Functional Vision (72 vs 90; P = 0.008), Bothered by Eyes/Vision (65 vs 90; P = 0.009), and Frustration/Worry (53 vs 75; P 0.06 for each comparison). These findings highlight the importance of addressing diplopia when managing childhood strabismus.12 month embargo; published: 20 August 2021This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Applying normal PedEyeQ thresholds to define reduced quality of life

Patient-reported outcome measures such as the Pediatric Eye Questionnaire (PedEyeQ) are increasingly recognized as important in healthcare assessment. Defining normal PedEyeQ thresholds would allow classification of individual children as having reduced versus normal domain scores. We prospectively enrolled visually normal children (aged 0-17 years; n = 310) to calculate normal PedEyeQ domain thresholds. In addition, 48 children with bilateral visual impairment (VI; best-eye acuity worse than 20/70 or 20/70 or better with limited visual fields) were enrolled for validation. The Child PedEyeQ (four domains) was completed by 5- to 17-year-olds. Parents completed Proxy (five domains) and Parent PedEyeQ (four domains). Each domain was Rasch scored (converted to 0-100); normal thresholds were defined as the 5th percentile of scores in visually normal controls. For Child 5-11 PedEyeQ, 39%-78% of VI children had reduced domain scores, and 88%-100% for 12- to 17-year-olds. For Proxy PedEyeQ, proportions ranged from 55% to 100% and for Parent PedEyeQ ≥83% had reduced scores. High prevalence of reduced PedEyeQ domain scores in the VI cohort, validates the use of normal thresholds. Nevertheless, variability in child self-reporting creates challenges for identifying individual 5- to 11-year-olds with reduced scores.12 month embargo; available online 26 June 2021This item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]