A Pilot Randomized Clinical Trial of Base-in Relieving Prism Spectacle Treatment of Intermittent Exotropia.

SIGNIFICANCE: This pilot randomized trial, the first to evaluate a specific base-in relieving prism treatment strategy for childhood intermittent exotropia, did not support proceeding to a full-scale clinical trial. Defining and measuring prism adaptation in children with intermittent exotropia are challenging and need further study. PURPOSE: This study aimed to determine whether to proceed to a full-scale trial of relieving base-in prism spectacles versus refractive correction alone for children with intermittent exotropia. METHODS: Children 3 years old to those younger than 13 years with distance intermittent exotropia control score of ≥2 points on the Intermittent Exotropia Office Control Scale (Strabismus 2006;14:147-150; 0 [phoria] to 5 [constant]), ≥1 episode of spontaneous exotropia, and 16 to 35∆ by prism-and-alternate-cover test, who did not fully prism adapt on a 30-minute in-office prism-adaptation test were randomized to base-in relieving prism (40% of the larger of distance and near exodeviations) or nonprism spectacles for 8 weeks. A priori criteria to conduct a full-scale trial were defined for the adjusted treatment group difference in mean distance control: "proceed" (≥0.75 points favoring prism), "uncertain" (>0 to <0.75 points favoring prism), or "do not proceed" (≥0 points favoring nonprism). RESULTS: Fifty-seven children (mean age, 6.6 ± 2.2 years; mean baseline distance control, 3.5 points) received prism (n = 28) or nonprism (n = 29) spectacles. At 8 weeks, mean control values were 3.6 and 3.3 points in prism (n = 25) and nonprism (n = 25) groups, respectively, with an adjusted difference of 0.3 points (95% confidence interval, -0.5 to 1.1 points) favoring nonprism (meeting our a priori "do not proceed" criterion). CONCLUSIONS: Base-in prism spectacles, equal to 40% of the larger of the exodeviations at distance or near, worn for 8 weeks by 3- to 12-year-old children with intermittent exotropia did not yield better distance control than refractive correction alone, with the confidence interval indicating that a favorable effect of 0.75 points or larger is unlikely. There was insufficient evidence to warrant a full-scale randomized trial.

Ipsilateral gaze deviation as a risk factor for surgical overcorrection in two-muscle surgery for unilateral superior oblique palsy.

PURPOSE: To investigate the angle of deviation in various gaze positions as a risk factor for overcorrection of moderate-angle unilateral trochlear nerve palsies treated with two-muscle surgery. METHODS: The medical records of consecutive patients with presumed unilateral moderate-angle trochlear nerve palsy who underwent two-muscle surgery were retrospectively reviewed. Patients with overcorrection, defined as reversal of hyperdeviation by prism alternate cover testing at distance (straight ahead) or near measured at 6 weeks, were compared to non-overcorrected patients for their preoperative torsion and ocular alignment at near and distance. RESULTS: A total of 45 patients (age range, 12-77 years; 24 [53%] males) with deviation ranging from 14Δ to 25Δ in primary position underwent two-muscle surgery, of whom 8 (18%) experienced surgical overcorrection by 6 weeks' follow-up. The preoperative angle of deviation was similar between overcorrected and non-overcorrected patients for eight of nine cardinal distance positions and near gaze; however, patients with smaller deviations in ipsilateral gaze were more likely to be overcorrected with two-muscle surgery (8.5 vs 16.0 [P = 0.029]). Cut point analysis determined that an ipsilateral gaze of ≤9Δ was significantly associated with overcorrection. Greater lateral incomitance also trended toward overcorrection (15.0 vs 9.0 [P = 0.059]). Torsion was not a clinically significant indicator of overcorrection (3.5 vs 6 [P = 0.083]). CONCLUSIONS: A preoperative ipsilateral angle of ≤9Δ was associated with overcorrection in patients undergoing two-muscle surgery for moderate angle unilateral trochlear nerve palsies.

Intermittent Esotropia in 4 Patients With Allan-Herndon-Dudley Syndrome.

Allan-Herndon-Dudley syndrome is a rare X-linked neurologic condition caused by mutations in monocarboxylate transporter 8 ( MCT8), which leads to deficient thyroid hormone transport. Typical features include severe cognitive impairment, truncal hypotonia, spastic paraplegia, weakness, and speech difficulties. Minimal literature exists describing the ocular findings in patients with Allan-Herndon-Dudley syndrome. We describe 4 male siblings affected with Allan-Herndon-Dudley syndrome with a novel nonsense mutation (Q90X) in the MCT8 protein. All affected siblings presented with classic findings of Allan-Herndon-Dudley syndrome, and each of the siblings also developed intermittent esotropia. This group of affected siblings represents the first consistent documentation of strabismus in Allan-Herndon-Dudley syndrome, suggesting a possible association between this clinical finding and the neurologic syndrome.

Comparison of EX-PRESS Shunt and Trabeculectomy With Mitomycin-C in Congenital and Juvenile Glaucoma.

PURPOSE: To compare efficacy and safety of EX-PRESS mini glaucoma shunt (Alcon) and trabeculectomy with mitomycin-C (MMC) in the management of pediatric glaucoma. METHODS: The authors retrospectively reviewed patients from a single pediatric ophthalmology practice with either congenital or juvenile glaucoma who underwent surgical treatment with either trabeculectomy or EX-PRESS shunt insertion with MMC. Postoperative intraocular pressure at 6, 18, and 36 months, medication requirements, visual acuities, postoperative interventions, complications, and noncompliance with medications or follow-up was reviewed. RESULTS: A total of 42 cases of glaucoma with either trabeculectomy or EX-PRESS shunt were identified for review (n trabeculectomy=25, n EX-PRESS=17). At 6 months, 62.5% of trabeculectomies and 94.1% of EX-PRESS shunts met criteria for success (P=0.0281). At 18 months, 38.1% of trabeculectomies and 81.3% of EX-PRESS shunts met criteria for success (P=0.0178). At 36 months, trabeculectomies had a success rate of 33.3% versus 66.7% for EX-PRESS shunts (P=0.321). The average decrease in medications postoperatively was 0.375 and 1.63 for trabeculectomy and EX-PRESS shunt, respectively (P=0.06). Preoperative visual acuities compared with postoperative best-corrected visual acuities before additional surgery demonstrated a slight average worsening of 0.11 logMAR in trabeculectomy patients and an average improvement of 0.56 logMAR in EX-PRESS shunt patients (P=0.0037). CONCLUSIONS: In this review, patients with pediatric glaucoma managed with EX-PRESS shunt with MMC compared with trabeculectomy with MMC appear to have better intraocular pressure control, better visual acuities, and fewer complications and reoperations.

Incidence and Types of Pediatric Nystagmus.

PURPOSE: To report the incidence, prevalent subtypes, and clinical characteristics of pediatric nystagmus diagnosed over a 30-year period. DESIGN: Retrospective, population-based study. METHODS: Using the Rochester Epidemiology Project, we reviewed the medical records of all children (<19 years) diagnosed as residents in Olmsted County, Minnesota, with any form of nystagmus from January 1, 1976, through December 31, 2005. RESULTS: Seventy-one children were diagnosed during the 30-year period, yielding an annual incidence of 6.72 per 100,000 younger than 19 years (95% confidence interval [CI], 5.15-8.28) Infantile nystagmus, onset by 6 months, comprised 62 (87.3%) of the study patients, corresponding to a birth prevalence of 1 in 821. The median age at diagnosis for the cohort was 12.7 months (range, 0 days to 18.6 years) and 42 subjects (59.2%) were male. The main types of nystagmus, in declining order, were nystagmus associated with retinal/optic nerve disease in 23 (32.4%), idiopathic or congenital motor nystagmus in 22 (31.0%), manifest latent nystagmus or latent nystagmus in 17 (24.0%), and 2 (2.8%) each associated with Chiari malformation, medication use, tumor of the central nervous system, and no diagnosis. Developmental delay was diagnosed in 31 (43.6%), strabismus in 25 (35.2%), and amblyopia in 10 (14.1%). Eighty percent had 20/40 (or equivalent) or better vision at presentation in at least 1 eye. CONCLUSIONS: This study provides population-based data on incidence and clinical characteristics of childhood nystagmus in North America. Idiopathic and nystagmus associated with retinal/optic nerve disease were the most common presentations, with most patients having good vision. Developmental delay, strabismus, and amblyopia were common in this cohort.

One- Versus Two-Muscle Surgery for Presumed Unilateral Fourth Nerve Palsy Associated With Moderate Angle Hyperdeviations.

PURPOSE: To compare 1-muscle vs 2-muscle surgery for moderate-angle hyperdeviations owing to presumed unilateral fourth nerve palsy. DESIGN: Retrospective chart review. METHODS: Seventy-three patients (aged 5-86 years) underwent either 1- or 2-muscle surgery at our institution for moderate hyperdeviation owing to presumed unilateral fourth nerve palsy, measuring 14-25 prism diopters (PD) in straight-ahead gaze at distance fixation. Six-week and 1-year motor success was defined as zero vertical deviation or 1-4 PD undercorrection at distance, overcorrection as any reversal of hypertropia, and undercorrection as >4 PD. Diplopia success was defined as no diplopia, or only rarely for distance straight ahead and reading. RESULTS: Twenty-eight patients underwent 1-muscle surgery, and 45 patients underwent 2-muscle surgery. Motor success was similar (64% vs 67%, P > .99 at 6 weeks; 47% vs 55%, P = .8 at 1 year, n = 46), but there were more undercorrections at 6 weeks with 1-muscle surgery (36% vs 16%, P = .09) and more overcorrections at 6 weeks with 2-muscle surgery (0% vs 18%, P = .02). Diplopia success was also somewhat similar between 1- and 2-muscle surgery at 6 weeks (73% vs 60%, P = .5) and 1 year (45% vs 59%, P = .5). CONCLUSION: For moderate-angle hyperdeviations owing to presumed unilateral fourth nerve palsy, there appears no clear advantage of 2-muscle surgery for motor outcomes. Diplopia success was similar between 1- and 2-muscle surgery, owing to a greater number of less symptomatic undercorrections with 1-muscle surgery and a smaller number of more symptomatic overcorrections with 2-muscle surgery.

Trauma and substance use disorders in rural and urban veterans.

CONTEXT: Disparities in the prevalence, morbidity, and mortality of multiple mental health conditions have been described between rural and urban populations. However, there is limited information regarding differences in exposure to trauma and trauma-related mental health conditions in these populations. Given the number of veterans who are returning to rural communities after serving in Operation Enduring Freedom and Operation Iraqi Freedom, differences in trauma exposure are of particular relevance. Trauma exposure is related to a variety of mental health disorders including substance use disorders (SUD). PURPOSE: The objectives of this preliminary study were to describe lifetime military and nonmilitary trauma and to compare trauma history between rural and urban veterans in SUD treatment. METHODS: Sixty adults in SUD treatment were enrolled at 3 Veterans Health Administration sites in Nebraska over a 3-month period in 2008. Subjects completed an interview with study staff, which assessed SUD diagnoses and childhood, lifetime, and military trauma. Rural or urban status was determined by self-report of childhood residence. Childhood trauma, lifetime trauma, and response to military trauma were compared between rural and urban veterans. FINDINGS: Although there were no significant differences in trauma exposure between rural and urban groups, there was an association between specific types of trauma and measures typically associated with increased substance abuse severity and poorer SUD treatment outcome. CONCLUSION: This is the first study, to our knowledge, which compared trauma exposure between rural and urban veterans and identified an association between childhood trauma exposure and multiple SUD treatment attempts.

Validation of a new coma scale, the FOUR score, in the emergency department.

OBJECTIVE: Full Outline of Unresponsiveness (FOUR) score has previously been validated scale in the Neurosciences Intensive Care Unit. In this study, we sought to validate the use of FOUR score in the emergency department (ED) using non-neurology staff. We also compared its performance to the Glasgow Coma Scale (GCS) and correlated it to functional outcome at hospital discharge and overall survival. METHODS: We prospectively rated 69 patients with initial neurologic symptoms presenting to the ED. Three types of examiners performed the FOUR score: ED physician, ED resident, and ED nurse. Patients were followed through hospital discharge; functional outcome was measured using modified Rankin Score (mRS). RESULTS: Interrater reliability for FOUR score and GCS was excellent (respectively, kappa(w) = 0.88 and 0.86). Both FOUR score and GCS predicted functional outcome, and overall survival with and without adjustment for age, sex, and alertness group. CONCLUSION: The FOUR score can be reliably used in the ED by non-neurology staff. Both FOUR score and GCS performed equally well, but the neurologic detail incorporated in the FOUR score makes it more useful in management and triage of patients.

S100 as a marker of acute brain ischemia: a systematic review.

BACKGROUND: Studies show S100 as a possible acute ischemic stroke (AIS) marker. OBJECTIVES: Determine (1) whether S100 serum concentrations correlate with stroke symptom onset, infarction volume, stroke severity, functional outcome, or length of hospital stay; (2) whether S100 serial measurements are useful markers for ongoing brain ischemia, and (3) whether S100 levels at various time intervals are higher in AIS patients than controls. METHODS: Literature was searched using OVID and MEDLINE from January 1950 to February 2007, and all relevant reports were included. RESULTS: Eighteen studies (1,643 patients) satisfied entry criteria. S100 peaks from symptom onset between 24 and 120 h with significantly raised values measured from 0 to 120 h. Higher S100 values indicated significantly larger infarction volumes, more severe strokes, and worse functional outcome. There was a significant difference in S100 levels between AIS patients and controls. CONCLUSION: Peak values after stroke onset varied. S100 was significantly increased after stroke onset, and correlates with infarct volume, stroke severity, and functional outcome, and was a possible marker for ongoing ischemia. Its serum concentration during acute stroke is a useful marker of infarct size and long-term clinical outcome.