s of the Ophthalmic Literature Compiled by: Katherine Fray, C.O. Alexandra Huebner, C.O. Cindy Cline, c.o. Jacqueline Shimko, C.O. Ingrid Rocha-Gamba, C.O. Cindy Pritchard, C.O. Colleen Gallagher, C.O. Kyle Arnoldi, C.O. ALIO JL, ORTIZ D, ABDELRAHMAN A, DELUCA A: Optical analysis of visual improvement after correction of anisometropic amblyopia with aphakic intraocular lens in adult patients. Ophthalmology 2007; 114:643-647. . This was a retrospective study of 59 eyes of 48 patients with anisometropic amblyopia implanted with an angle-supported phakic intraocular lens. Inclusion criteria consisted of anisometropia of at least 3 diopters and a best-corrected visual acuity of 0.7 or less in the best eye. Mean gain in visual acuity was three lines with a range of 0 to 7 lines. Ninety-one percent of eyes gained at least one line of visual acuity, and no eyes lost lines of visual acuity. The change in best-corrected visual acuity did not correlate with preoperative best-corrected visual acuity or with the degree of anisometropia. Calculations utilizing a Kooijman eye model suggested that the increase in visual acuity was due to magnification caused by the intraocular lens. All subjects were myopic preoperatively. The authors conclude that the visual acuity of myopic patients with anisometropic amblyopia increases after implantation of an intraocular lens and that this improvement in vision is explained in terms of magnification and changes in aberrations. Jorge L. Alio, M.D., Ph.D., Research and Investigation Dept., Instituto Oftalmologico Alicante, Vissum Corporation, AVDA. DENIA son, Edificio Vissum, 03016 Alicante, Spain. ALWAN A, MEJICO LJ: Ophthalmoplegia, proptosis, and lid retraction caused by cranial nerve hypertrophy in chronic inflammatory demyelinating polyradicuJoneuropathy. .J Neuroophthalmol 2007; 27:99-103. The authors discuss the course of a 53-year-old woman whose past medical history was significant for episodes of proximal and distal limb weakness, ptosis of the left upper lid, and numbness in her extremities. She ultimately received the diagnosis of CIDP (chronic inflammatory demyelinating polyradiculoneuropathyJ. Her systemic symptoms improved with intravenous immunoglobulin treatment; however, the ocular signs of diplopia, proptosis, eyelid retraction, and ophthalmoplegia were the first symptoms to worsen between treatments. She was evaluated by ophthalmology for the possibility of Graves' ophthalmopathy. An MRI was performed approximately 25 years after the initial onset ofneurologic symptoms that showed "enhancement and ~) 2008 Board f)ff«~~l'ntsor thcUruversitv of Wisconsin System. American Orthoptic .lournul, Volume fiB, 200H, ISSN 006!l·9fitlX. E·)SSN 1!i!la-4448
There are a number of ways orthoptists in pediatric ophthalmology practices can help patients with nystagmus. Treatment of amblyopia by conventional patching or with atropine or Bangerter filters can be monitored by the orthoptist. Optical treatment including manipulation of refractive error or use of contact lenses has been proven to improve visual function. Also, prisms can help determine a surgical plan for an abnormal head posture. If surgery is not indicated, then prisms can improve vision or eliminate the need for a compensatory head posture. This paper will describe in detail the use of prisms in patients with nystagmus.
PURPOSE: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.
Background and Purpose: Nystagmus is a commonly encountered clinical finding both in children and adults. Despite its complexities, a fundamental understanding of the basic etiology and underlying pathophysiology of nystagmus is critical to manage these patients appropriately.Patients and Method: The subset of ocular motility disorders that constitutes true nystagmus is identified and delineated from saccadic intrusions. Once defined, we explore the causes of commonly encountered forms of nystagmus and their clinical features.Results: Three separate mechanisms work to integrate sensory input and calibrate ocular movements: the afferent visual pathway, the neural integrator, and the vestibular system. Specific neuroanatomical pathways and corresponding pathologic changes are reviewed.Conclusion: A basic understanding of the underlying pathophysiology of nystagmus is essential to clinical practice. Three main mechanisms utilize multifactorial sensory input to produce a normally functioning ocular motor system. Both congenital and acquired forms of nystagmus result from pathology within these systems causing insufficient or asymmetric input to the central nervous system.
Strabismus is often found in patients with nystagmus. It is valuable to understand this association to counsel patients. Strabismus can be difficult to quantify with alternate cover testing when there is a large amplitude nystagmus. Hirschberg or Krimsky measurements can be misleading.
Click to increase image sizeClick to decrease image sizeKey Words: Alfred Bielschowskyhistorical vignetteBielschowsky head tilt testBielschowsky phenomenonBielschowsky LectureBielschowsky Society
Nystagmoid eye movements are involuntary eye movements that are not pure forms of nystagmus. Nystagmus, by definition, is involuntary eye movements off of fixation that are initiated by a slow phas...
The most common forms of nystagmus in infancy and childhood are Infantile Nystagmus Syndrome (INS), Fusion Maldevelopment Nystagmus Syndrome (FMNS), and Spasmus Nutans Syndrome (SNS). This review will sumamrize the clinical and eye movement recording characteristics of these disorders, thus reinforcing the importance of eye movement technology in both the discoveries underlying pathophysiology and the guidance of clinical interventions.
Examining a child with nystagmus can be intimidating even for an experienced clinician. The purpose of this paper is to provide examination pearls for the clinical assessment of the pediatric patient with nystagmus. By employing these techniques, the clinician should be able to document the necessary information for determining the underlying cause of the nystagmus and to direct the patient's plan of care.
Infantile nystagmus presents many challenges for clinicians when defining the characteristics, etiology, visual capabilities, and optimal visual aids for the affected individual. However, life beyond the office may present the greatest challenges from the point of view of the child and their family. Research related to the psychosocial implications and quality of life for these individuals, as well as information related to systems that support their educational and social development, are infrequent in the ophthalmic literature. The purpose of this report is to briefly outline our local pediatric ophthalmic approach for these patients, to present a limited review of applicable literature, and to introduce our regional support system that addresses educational and psychosocial challenges for children and families affected by infantile nystagmus syndrome.
BACKGROUND 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.
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.
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 with systemic diseases such as spasmus nutans, opsoclonus/ocular flutter, downbeat nystagmus, convergence retraction nystagmus, and see-saw nystagmus. These unique forms of nystagmus may be indicative of life- or vision-threatening diseases; thus, their recognition will facilitate earlier diagnosis and treatment, resulting in better outcomes.
BACKGROUND:"Dragged-fovea diplopia syndrome" is a type of central binocular diplopia that is secondary to a foveal displacement, caused by epiretinal membranes (ERMs) or other macular diseases. Its management is difficult, because prisms are not effective.CASE REPORTS:Two cases of dragged-fovea diplopia syndrome were presented. Both patients were affected with a unilateral epiretinal membrane. Therefore, the pathophysiology underlying their diplopia was the conflict between central and peripheral fusion mechanisms.CONCLUSIONS:Diplopia caused by ERM "shift" deserves a complex management. We suggest to be careful about subjective symptoms and to optimize the residual visual function to customize the orthoptic management. A strict cooperation between ophthalmologists and orthoptists could lead to a successful outcome.
BACKGROUND AND PURPOSE:To assess the possibility of determining the insertion distance from the limbus of previously operated extraocular rectus muscles (EOM) with the Heidelberg Spectralis anterior segment optical coherence tomography (AS-OCT).PATIENT AND METHOD:Subjects with a history of previous strabismus surgery underwent AS-OCT of the EOM before planned additional strabismus surgery. The EOM insertion distances from the limbus were measured pre-operatively on the AS-OCT and compared to the caliper distance measured during the strabismus surgery.RESULTS:Ten previously operated muscles on nine subjects underwent AS-OCT before subsequent additional strabismus surgery. Four additional un-operated muscles subsequently operated on, were also imaged with the AS-OCT pre-operatively. Subject ages ranged from 13-52 years old (mean ± SD; 27.9 ± 13.2). The muscle insertion could be definitely identified in 6/10 muscles previously operated and 4/4 un-operated muscles. The difference between the two measurements of limbus to insertion in previously operated muscles was ≤1mm in 3/6, and ≤1.5mm in 6/6; <1mm in 4/4 un-operated muscles. Of the four insertions not readily identifiable, two revealed the presence of the muscle with scar tissue; the other two, the muscle insertions, were not visible, which showed that the muscle was at least a minimal amount from the limbus.CONCLUSION:Our study showed that the Heidelberg AS-OCT is capable of imaging previously operated EOM, which can give valuable information to the strabismus surgeon. The information from the AS-OCT was useful in all cases. The insertion to limbus measurements between pre-operative and intra-operative were within 1.5mm in all of the cases that the muscle insertion was able to be identified. The ability to accurately image EOM insertions has significant implications for the pre-operative procedure planning in previously operated and complicated strabismus patients.
Myron Yanoff, MD and Jay S. Duker, MD. Mosby Elsevier, Philadelphia, PA, 2009. ISBN 978-0-323-04332-8, $415.00, Scope: This multi-authored text provides a comprehensive review of ophthalmology, both in breadth and depth of coverage, and is sufficient to answer all but the most detailed subspecialty question. The authors follow the directive of the first two editions, aiming this text at trainees, non-ophthalmologists, and comprehensive ophthalmologists. The entries are not as detailed as in subspecialty publications, but the text is more manageable than multivolume series and successful in providing most of the information needed for all but the most detailed reviews. The book is organized into 12 parts by a blend of anatomy and subspecialty, with each part color-coded for ease of navigation. Each part is divided into chapters that use a consistent template to review a disease, procedure, or diagnostic test. Each chapter begins with a definition of the chapter title, key features, and associated features and is full of large, easy-to-read tables, color illustrations, and figures. The chapters are extensively referenced. Strengths: The color-coding and chapter template format make the text easy to follow. The text is well illustrated with a multitude of large and visually appealing color figures that encourage the reader to peruse multiple chapter sections. The size and color of the photography move the reader along. There is excellent coverage of relevant basic science without being excessive and a consistent focus on advances in clinical conditions, diagnostics, and treatments. The sections on genetics, ophthalmic technology, immunology, and tuberculosis have been expanded. The book retains the size of previous editions due to rigorous purging of outdated material. Weaknesses: This text is not able to attain an encyclopedic depth of each area and still remain as a single portable volume. That being said, the practitioner looking to review in such depth would be unlikely to choose a single textbook. Extensive references direct the reader elsewhere. Recommended Audience: The authors have succeeded in reaching their target audience of trainees, non-ophthalmic physicians, and comprehensive ophthalmologists. Ophthalmic subspecialists would, however, also do well to obtain this text to review areas outside their area of expertise and will doubtless pick up pointers in their own field. Critical Appraisal: This book deserves a place as the first source to review any area of interest or any point of uncertainty in clinical ophthalmology. The format allows quick access. It is well written and easily read, interesting, and full of excellent references. Hilary A. Beaver, MD Methodist Eye Associates Houston, Texas
Intermittent exotropia, an intermittent divergent misalignment, is a common form of childhood strabismus in many populations of the world. Assessing the severity of this condition is challenging due to the variable nature of this disease. Parameters used to measure severity of intermittent exotropia include control, angle, and stereoacuity. The assessment of symptoms and quality of life can also be helpful.
Introduction and Purpose Anisometropia, 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. Methods This 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. Results Ocular 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). Conclusions The 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.
