Unilateral Horizontal Rectus Muscle Recessions for Pediatric Comitant Strabismus.

INTRODUCTION: Compared to bilateral horizontal rectus muscle recessions (BHR), a unilateral horizontal rectus muscle recession (UHR) confers shorter operating time and anesthesia exposure, limits complication risks to one eye, and requires less recovery from the patient. METHODS: Retrospective chart review of pediatric patients who underwent unilateral medial rectus recession (UMR) for esotropia (ET), or unilateral lateral rectus recession (ULR) for exotropia (XT). Primary successes were defined as maximum distance deviation at post-operative month 3: -12 to +5 for ET, -5 to +12 for XT. Rates of postoperative horizontal incomitance (HI) >5 PD and success for small medium, and large (in mm, respectively, ET: <5, 5-6 mm, >6; XT: <8, 8-10, >10) recessions were analyzed. RESULTS: Seventeen ETs and 40 XTs were analyzed. Overall primary success was 71.9% (p = .02). Significantly, 80% (95% CI: 67.60,92.40) XTs succeeded. ETs were equally likely to succeed (53.9%) or fail (47.1%) (p = .22). For patients without significant preoperative HI, average postoperative HI was 3.90 PD (95% CI: 0.20, 7.60) for ETs; 5.48 PD (95% CI: 3.65, 7.32) for XTs. CONCLUSIONS: UHR was 71.9% successful in treating pediatric comitant strabismus. ULR for XT, particularly small to medium deviations, was most likely to succeed. In contrast to prior reports, large UMR and ULR were less likely to succeed and post-operative incomitance was frequent but rarely clinically significant.

Does Early Glasses Wear Improve Visual Outcome in OCA1A?

Purpose: Oculocutaneous albinism type 1A (OCA1A), with lifelong absent melanin in skin, hair, and eyes, is the most severe type of albinism with greatest ametropia and poorest vision. We evaluated the relationship between age when spectacles were begun and visual outcome, in addition to status of refraction, in OCA1A. Methods: After IRB approval, a retrospective review of 70 consecutive charts of patients with OCA1A identified 24 fitting inclusion criterion of BCVA recorded at age 10-12 years. Exclusion criteria were those with other vision-threatening diagnoses and patients seen for a single visit. We recorded sex, age at beginning glasses, and refraction and BCVA at age 10-12 and most recent visit. Data were arbitrarily grouped by those initiating glasses at ≤ age 12 months and > age 12 months. Results: Regression analysis showed a larger degree of astigmatism was weakly associated with worse vision at age 10-12 years. A weakly positive relationship was found between poorer BCVA at last visit and older age at which glasses were initiated. All receiving glasses by age 1 and only half receiving glasses when older had improved visual acuity from age 10-12 years to last follow up. Conclusion: Additional study of a larger sample of this rare disorder is needed to determine if early glasses wear improves later BCVA.

Strabismus Patterns in Patients with Intrauterine Drug Exposure.

Purpose: This retrospective study evaluates the patterns of strabismus, the incidence of amblyopia, and prevalence of anatomical ocular pathology in patients with intrauterine drug exposure (IDE) presenting to a tertiary referral center for an ophthalmic evaluation.Methods: We reviewed charts of patients who presented to our pediatric ophthalmology clinic from 8/2017-10/2018 with reported IDE. The mean age at presentation was 29 months. We documented the type of exposure, birth history, social history, and past medical and surgical history. Visual acuity, motility, fundus exam, and cycloplegic refraction were reviewed.Results: Thirty-eight patients met the criteria. Motility exam showed that 23 patients were exotropic, 10 were esotropic, and 5 were orthophoric. Average angle of the exotropic deviation was 27ΔXT (range X 10 to XT 50). Amblyopia, nystagmus, and anatomical ocular anomalies also occurred frequently.Conclusion: Strabismus and other eye diseases are common in patients with IDE. Exodeviations were the most common finding and occurred in 60.5% of our cohort. Patients with IDE are at a higher risk for treatable conditions such as strabismus and amblyopia. Possible history of IDE should be considered in infants who present with congenital strabismus, particularly exotropia.

Don't Miss This! Red Flags in the Pediatric Eye Examination: Subnormal Acuity.

Subnormal vision can be a harbinger of threating disease. Recognizing certain "red flags" on history or examination can facilitate early diagnosis in the pediatric patient. These objective tests or subjective complaints may help differentiate true organic causes of vision loss from possible functional overlay, amblyopia or poor results due to an uncooperative child.

A Cluster of Cyclic Esotropia: White Matter Changes on MRI and Surgical Outcomes.

PURPOSE: To report a series of patients with cyclic esotropia, their surgical outcomes, and incidental findings. METHODS: The medical records of five patients with cyclic esotropia presenting over 17 months were reviewed. Age at onset, ocular and motility examinations, brain magnetic resonance imaging (MRI), acetylcholine receptor antibodies, thyroid hormone levels and antibodies, calendars documenting phases, surgical treatments, postoperative alignment, and fusion were documented. RESULTS: Three boys and two girls presented at 3 to 4 years old in 2015-2016. Typical periodicity followed a 48-hour cycle. Duration of cycling varied from 1 to 9 weeks. Mean maximum deviation was 37 prism diopters (PD) of esotropia (range: 35 to 40 PD of esotropia). All patients had normal laboratory studies. MRIs showed an abnormal white matter signal in the frontal lobes in 2 patients and were normal in the others. Bilateral medial rectus recessions for the maximum angle were successful in 4 patients at a minimum follow-up of 13 months; the other patient required reoperation for a residual esotropia. Fusion was present in 4 patients preoperatively and all postoperatively. Stereopsis was stable or improved in all postoperatively. CONCLUSIONS: This is the first report of frontal white matter changes occurring in the setting of cyclic esotropia. MRI with attention to frontal lobe white matter might be considered in the work-up of cyclic esotropia to determine if this is a common finding. Bilateral medial rectus recessions can restore fusion in these patients. [J Pediatr Ophthalmol Strabismus. 2019;56(3):178-182.].

Periocular Asymmetry in Infants with Deformational Posterior Plagiocephaly.

PURPOSE: To analyze the clinical significance of the periorbital features associated with the facial asymmetry that is common in deformational posterior plagiocephaly (DPP). PATIENTS AND METHODS: We identified 32 patients with DPP, photographed their faces and tops of their head, and performed a complete eye examination. Four examiners analyzed the patient's periorbital features on the photographs. RESULTS: Median age was 6.5 months (range 3-12 months). Pseudoptosis was identified in 30 patients and pseudo-brow ptosis in 19. Pseudoptosis was marked in 17 patients. Five patients were misdiagnosed with congenital blepharoptosis and received regular follow-ups for amblyopia checks until the diagnosis of pseudoptosis was established. All patients had normal levator function and symmetric eyelid crease. One patient with pseudoptosis and physiologic anisocoria was diagnosed with pseudo-Horner syndrome after a negative 10% cocaine test. None of the patients developed meridional or occlusion amblyopia. CONCLUSION: DPP is the most frequent form of skull deformation in infants. Its main features are occipital flatness and facial asymmetry. Infants with DPP may present with pseudoptosis and pseudo-brow ptosis on the contralateral side of the occipital flatness. The pseudoptosis in DPP is non-amblyogenic, therefore, ophthalmologic intervention and regular follow-ups are not necessary unless other abnormalities co-exist.

Red Flags in the Assessment of Adult Ophthalmoplegia.

Acquired ophthalmoplegia can represent a harbinger of vision and life-threatening disease. Recognition of select clinical "red flags" on history or examination can facilitate early diagnosis. This article reviews relevant clinical pearls for the detection of dire causes of acquired ophthalmoplegia in adults including myasthenia gravis, carotid cavernous fistula, giant cell arteritis, and thiamine deficiency. Prompt treatment of all of these disorders can either reverse or halt the progression of visual manifestations.

DVD--a conceptual, clinical, and surgical overview.

Dissociated vertical deviation (DVD) is a slow, disconjugate hypertropic deviation of a nonfixating eye. It is usually bilateral, asymmetrical, and often associated with congenital esotropia. The deviating eye elevates, abducts, and excyclotorts. This type of strabismus is often variable, making measurement and clinical quantification difficult. Specific knowledge of the mechanisms and characteristics of the dissociated deviation are required for proper assessment and effective treatment. There is currently no consensus on the mechanisms and pathophysiology of DVD. In this workshop, participants discuss the characteristics and most current methods for assessing and quantifying the deviation and explore the potential etiologies, clinical characteristics, and indications for surgical intervention and nonsurgical management of DVD.

Positive angle kappa: a possible sign of aniridia.

INTRODUCTION AND PURPOSE: Aniridia is a panocular disorder with variable expressivity, caused by PAX6 mutations. Foveal hypoplasia and nystagmus occur in both aniridia and albinism. Individuals with albinism have a moderately positive angle kappa. This study evaluates the angle kappa in individuals with aniridia. PATIENTS AND METHOD: We performed a retrospective chart review of recorded angle kappa for individuals with aniridia. In addition, we prospectively examined the monocular corneal light reflex in each eye of patients with congenital aniridia and central fixation. We recorded other ocular characteristics and the results of gene testing. Patients with eccentric fixation or corneal surgery were excluded, as were patients with retinal abnormalities that might have influenced the angle kappa. RESULTS: We included sixteen individuals with congenital aniridia and found that all had a positive angle kappa. Two patients underwent pattern visual evoked potential (VEP) testing with monocular stimulation and were found to have a reversal of polarity, indicating misrouting of the retino-striate fibers. CONCLUSION: The phenotype of aniridia is variable. However, a positive angle kappa may be considered to be a frequent clinical feature of aniridia.

Reading acuity in albinism: evaluation with MNREAD charts.

INTRODUCTION: The MNREAD reading acuity (RA) charts use continuous-text reading to measure (1) RA, ie, the smallest size of print that the patient can resolve; (2) maximum reading speed (MRS); and (3) critical print size (CPS), ie, the smallest print that the patient can read with maximum speed. This project used the MNREAD charts to evaluate reading performance in children and adults with albinism to determine the smallest print size recommended to facilitate effortless reading. METHODS: A total of 63 subjects with albinism were administered the MNREAD test to determine the critical print size for effortless reading. The subjects read continuous, high-contrast text print until they could no longer discern the words. The MRS and CPS were determined with the MNREAD protocol. Presence or absence of nystagmus and strabismus was recorded. Distance visual acuity was measured with ETDRS charts. RESULTS: The sample included 25 male and 38 female subjects with a median age of 16 years. Median MRS was 150 words per minute. The mean CPS was 0.84 logMAR, notably larger than the mean RA of 0.53 logMAR. CONCLUSIONS: The MNREAD acuity charts can be used to determine functional reading ability and critical print size for effortless reading in individuals with albinism. The amount of enlargement in print size can be used in educational and vocational recommendations to facilitate reading.

Evaluation of vision-specific quality-of-life in albinism.

INTRODUCTION: Human albinism is a genetic condition associated with visual impairment that affects many aspects of daily life. Office measurements of visual acuity do not necessarily reflect daily visual function and health status. This study used the National Eye Institute Visual Function Questionnaire (NEI-VFQ) to determine the effect of albinism-associated ophthalmopathy on quality of life (QOL). METHODS: We administered the NEI-VFQ, which consists of 25 questions about QOL (VFQ-25) and 14 questions about well-being (VFQ-39), to 44 consenting participants with albinism older than the age of 18 years. RESULTS: Nineteen male and 25 female subjects completed the study. Median age was 30.5 years (range, 18-79 years). Mean best-corrected visual acuity was 20/83 (range, 20/20 to 20/320). Forty-eight percent reported that they were currently able to drive with their condition. Participants perceived midscale problems with their general vision (median subscale score 60.0). Visual acuity correlated moderately with overall NEI-VFQ composite scores (r(s) = 0.40, p = 0.01 for VFQ-25 and r(s) = 0.36, p = 0.02 for VFQ-39). Most notable impairment was recorded for distance acuity, vision-specific mental health, and vision-specific role difficulties (VFQ-39 median subscale scores 66.7, 70.0, and 75.0, respectively). Differences by sex were insignificant. Greater ceiling effects were noted for the VFQ-25 than for the VFQ-39. CONCLUSIONS: The NEI-VFQ-39 is a method to evaluate self-reported effects of vision-related QOL in albinism and may be used as a baseline for evaluating outcomes in interventional studies in these patients.

Positive angle kappa in albinism.

BACKGROUND: Albinism is an inherited disorder of deficient melanin production. There is a high prevalence of strabismus in patients with albinism. We investigated the prevalence of a positive angle kappa in patients with albinism and report its effect on assessment of binocular alignment. METHODS: We retrospectively reviewed the charts of 207 consecutive patients with the diagnosis of albinism seen at the University of Minnesota between 1984 and 2002. Angle kappa and alignment using prism and alternate-cover test (PACT) and Krimsky measurements were recorded. RESULTS: In 99.6% of our patients with albinism, angle kappa was noted to be positive. The mean difference between PACT and Krimsky measurements was 17.11 prism diopters in the more exotropic (or less esotropic) direction per Krimsky test. Because of the high prevalence of a positive angle kappa, esodeviations often appeared less and exodeviations appeared greater than when measured using PACT. CONCLUSIONS: Positive angle kappa can be considered another clinical feature of albinism. Consideration should be given to the effect of positive angle kappa on alignment as observed by Krimsky measurement when planning extraocular muscle surgery, particularly when preoperative sensory testing suggests binocular potential.