ABSTRACTPurpose: To report four cases of early onset sixth-nerve palsy all of whom had eccentric fixation.Methods: A retrospective case note review was undertaken of all cases presenting to the senior author's private and NHS practice with early onset sixth palsy between 2006 and 2012. As well as demographic information, details of ophthalmic, orthoptic, electrophysiological examinations, and radiological investigations that were extracted from the records.Results: Four children with unilateral or asymmetric early onset sixth-nerve palsy were identified, of which three were congenital. All four had MRI and only one had a normal MRI. Age at presentation ranged from 14–42 months, but all four had marked esotropia and poor visual acuities in the worst affected eye with eccentric fixation, which became more easily or only noticeable after surgical correction. Three patients with congenital sixth-nerve palsy underwent vertical muscle transposition with Botulinum Toxin A(BTXA) to the ipsilateral medial rectus, and two of these patients also had Foster sutures to the transposed vertical muscles. The fourth patient had unilateral medial rectus recession and lateral rectus resection. The mean preoperative measurement was 55Δ ET (range 50–60Δ), and the mean postoperative measurement was 11Δ ET (range 16XT-25ET) at near, and 2Δ XT (range 15XT-14ET) at distance.Conclusions: We speculate that early onset paralytic strabismus due to congenital sixth-nerve palsy results in an inability to cross fixate which results in the development of eccentric fixation. Attempts to use reverse occlusion to negate the eccentric fixation failed. We therefore recommend early surgery for this condition to avoid this sequelae.

Pediatric nystagmus may be the harbinger of systemic or neurologic disease with significant morbidity and potential mortality. This paper reviews pediatriconset nystagmus, which may be associated w...

Congenital cranial dysinnervation disorders (CCDD) is a new term describing a collection of non-progressive neurogenic syndromes. Initially referred to as congenital fibrosis syndrome, it was thought that the primary problem was extraocular muscular maldevelopment. Recent advancements in genetics and neuro-radiology have now determined the initial observation of fibrotic muscles is secondary to a primary lack of innervation from deficient, absent, or misguided cranial nerves. This presentation provides an overview of the known genes and phenotypes currently recognized within the CCDD domain. It will also highlight areas of current research being done in the area of cranial nerve development. Increased knowledge and awareness of these disorders has resulted in more research being conducted. These studies have provided a more complete understanding of efferent motor system development and are leading to improved treatment strategies for patients.

ABSTRACTBackground and Purpose: Early onset exodeviations in systemically and ocularly healthy young children, diagnosed at less than 1 year of age, may be of the constant, “infantile XT” type, or early X(T) type. The onset of common childhood X(T) is not clearly known. The purpose of this lecture is to discuss theories and characteristics of early onset exodeviations, and report on our observations of infantile XT and early X(T) at Children's Eye Care in Michigan.Patients and Methods: A retrospective review of 470 cases of childhood exodeviations (ages 6 months to 15 years) were reviewed and met inclusion criteria of no prior surgical treatment, no ocular, CNS or craniofacial disease, and no significant prematurity. Thirty-nine cases were diagnosed at less than 1 year of age: thirty-five patients with early X(T) and four patients with infantile XT, based upon a motility evaluation at 6 m and 1/3 m fixation using dissociative methods. The clinical characteristics and outcomes of these two groups were described and compared.Results: Comparing infantile XT and early X(T) groups, reported onset by caregivers was significantly younger in the infantile XT group (3 months vs. 6 months), and size of the deviation at both distance and near fixation ranges was significantly larger in the infantile XT group (XT-43/XT′-48Δ vs. X(T)-25/X(T)′-23Δ). Three of 4 infantile XT patients received surgery, one spontaneously resolved, and all resulted in small, residual XT, and DVD without measurable stereoacuity. Many patients with early X(T) demonstrated good/excellent control at near range and fair/poor control at distance range. Four early X(T) patients who did not receive surgical correction either resolved, remained the same, or decompensated. Surgical correction for X(T) resulted in a 50% success rate for one procedure with a minimum of 2 years postoperative follow-up. Stereoacuity outcomes did not appear to correlate with quality of control. Conclusions: Most healthy children with X(T) are diagnosed by age 5 years, although many have a reported onset by caregivers of less than 1 year of age. Good control of X(T) at near range may preclude early examinations. Motility evaluation by dissociative methods at near and far-range fixation may facilitate early diagnosis. Infantile XT is less common than early X(T), by a ratio of 1:10. Characteristics of infantile XT and early X(T) have significant differences in report onset, deviation size, and outcomes with and without surgical intervention. Patients with either infantile XT or early X(T) may spontaneously resolve over time.

Introduction and PurposeAnisometropia, a relative difference in the refractive state of the two eyes, is common in hyperopic patients. We investigated the association between ocular dominance (sighting dominance) and refractive asymmetry in patients with hyperopia.MethodsThis retrospective study included 223 hyperopic patients with a mean age of 10.1 ± 3.6 years (range 3 to 21 years). Refractive error was measured with cycloplegic refraction, and axial length was measured with IOLMaster® (Carl Zeiss Meditec, Dublin, CA). Ocular dominance was assessed with the hole-in-the-card test. The amount of hyperopic anisometropia was subdivided into four groups: less than 0.50 D, 0.50–0.99 D, 1.00–1.99 D, and 2.00 D or greater.ResultsOcular dominance of the right and left eye was seen in 66% and 34% of the patients, respectively. The nondominant eye had higher hyperopia, astigmatism, and shorter axial length than the dominant eye (P < 0.001). In the group with spherical equivalent anisometropia of ≥0.50 D in particular, the nondominant eye was significantly more hyperopic and had shorter axial length than the dominant eye (both P < 0.001).ConclusionsThe current study revealed that the nondominant eye had a greater hyperopic refractive error and shorter axial length than the dominant eye, in patients who had a high degree of anisometropia in particular.

Background and PurposeAlthough adverse psychosocial effects are well documented among patients afflicted with strabismus, recent studies have shown that children with intermittent exotropia, unlike the more prevalent forms of esotropia, are at an increased risk for developing frank mental illness by early adulthood. The purpose of the study was to provide a brief overview of current findings regarding the nonophthalmic aspects of this common condition.Patients and MethodRecent reports will be reviewed regarding psychosocial effects and mental illness among children with intermittent exotropia.ResultsChildren with intermittent exotropia have been shown to have a nearly three-fold increased incidence of developing mental illness, compared to controls, by the third decade of life. Moreover, males had significantly more mental health morbidity than females. Although quality of life measures have demonstrated improved psychosocial outcomes among children who underwent successful strabismus surgery, frank mental illness appears to be less amenable to such intervention.ConclusionIn addition to the adverse psychosocial affects observed in children with strabismus, intermittent exotropia, especially among boys, appears to confer a specific risk for developing frank mental illness. Clinicians managing such patients should consider the entire physical and psychological aspects of this condition and develop strategies for minimizing adverse psychosocial outcomes.

Introduction and PurposeTo compare binocular vision measurements between Chronic Fatigue Syndrome (CFS) patients and healthy controls.MethodsForty-one CFS patients referred by the Reference Centre for Chronic Fatigue Syndrome of the Antwerp University Hospital and forty-one healthy volunteers, matched for age and gender, underwent a complete orthoptic examination. Data of visual acuity, eye position, fusion amplitude, stereopsis, ocular motility, convergence, and accommodation were compared between both groups.ResultsPatients with CFS showed highly significant smaller fusion amplitudes (P < 0.001), reduced convergence capacity (P < 0.001), and a smaller accommodation range (P < 0.001) compared to the control group.ConclusionIn patients with CFS binocular vision, convergence and accommodation should be routinely examined. CFS patients will benefit from reading glasses either with or without prism correction in an earlier stage compared to their healthy peers. Convergence exercises may be beneficial for CFS patients, despite the fact that they might be very tiring. Further research will be necessary to draw conclusions about the efficacy of treatment, especially regarding convergence exercises. To our knowledge, this is the first prospective study evaluating binocular vision in CFS patients.