DOCSLIB.ORG

The Management of Squint

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
The Management of Squint Arch Dis Child: first published as 10.1136/adc.64.3.413 on 1 March 1989. Downloaded from Archives of Disease in Childhood, 1989, 64, 413-418 Personal practice The management of squint A R FIELDER Department of Ophthalmology, University of Leicester Medical School, Leicester Squint may be the first sign of a serious ocular or early neonatal life about 32% of infants are diver- systemic disorder, and yet it is also one of the most gent and only 3% convergent.4 As the incidence of common ocular conditions of childhood. It follows divergence is higher in preterm neonates it is likely that while most ocular deviations have no such that during development the eyes are initially sinister connotations, an understanding of the range divergent but that this resolves over the first few of associations is essential to be prepared for this weeks of life. comparatively infrequent but important possibility. Apart from those seen in the neonatal period, Screening for squint has been covered recently' squints are comparatively uncommon in the first few and in this article its presence will be assumed. The months of life, and the term 'infantile' is preferred clinical evaluation of the child with a squint will be to 'congenital' because this type of esotropia is considered first, followed by a discussion of the aims rarely if ever congenital but develops between 3 and and methods of treatment. The main thrust will be 6 months of age. Characteristically the deviation is directed towards the squint itself, but as amblyopia large, constant, and there is no appreciable refrac- is such a frequent and important association this tive error.5 topic will also be covered briefly. In order to avoid Apart from the infantile type, the onset of extensive and tedious repetition the associations of esotropia in childhood is most common between 11/2 squint will be listed using a predominantly system and 3 years of age. The deviation may range from based approach. Subdivision into paralytic and non- the hardly detectable (microtropia) to the cosmeti- paralytic types is only done at a later stage as both cally obvious manifest squint, and may be intermit- http://adc.bmj.com/ may occur in certain categories. Inevitably this tent or constant. In some the deviation increases- classification has limitations and inaccuracies, but or is only present-on accommodation (partially hopefully it will help the paediatrician to consider and fully accommodative squints respectively). the possible implications of a squint. For further Many, but by no means all, are associated with reference the reader may wish to consult two refractive errors. excellent standard texts.2 3 Exotropia is less common than esotropia at all ages and has less propensity to cause amblyopia. In Squint and its associations infancy ocular divergence should alert the clinician on October 1, 2021 by guest. Protected copyright. to the possibility of a severe visual impairment. SQUINT AS AN ISOLATED ANOMALY Paralytic squints do occur as an isolated anomaly Recently emphasis has been placed on early referral in an otherwise healthy child, but a careful search of infants and children with squint, but this has not for an underlying cause must always be undertaken taken into account that during the neonatal period (see acute central nervous system disease). Most, such a deviation may be a normal event. During but not all, paralytic squints in children are con- genital rather than acquired, although the role of birth trauma as a causal factor has probably been greatly overemphasised. A sixth cranial nerve palsy In terms will be used this article the following may present at this interchangeably: squint and strabismus, esotropia birth but characteristically and convergent squint, exotropia and divergent resolves over the ensuing few weeks. Apart from squint, comitant and non-paralytic squint, and this clinically obvious and rare exception, the most incominant and paralytic squint. Comitance will be common palsy, of the fourth nerve, is usually not assumed unless the terms incominant or paralytic diagnosed for a year or so as the ocular deviation is are used. difficult to elicit in infancy and the presenting sign is usually a compensatory head posture. Third nerve 413 Arch Dis Child: first published as 10.1136/adc.64.3.413 on 1 March 1989. Downloaded from 414 Fielder palsy is less common but more obvious, and in may be unilateral, bilateral, and concomitant or children is more likely to be congenital than acquired. paralytic, or both. Causes include raised intracranial A form of the latter that usually commences in pressure, intracranial tumours, trauma, degenera- childhood is ophthalmoplegic migraine. Benign tive and inflammatory diseases.6 12 recurrent sixth nerve palsy, thought to be a post- infective mononeuropathy as it usually follows a Neurodevelopmental disorders-In general, the inci- febrile illness, is included here because at presenta- dence of ocular abnormalities in children with tion there are no associated systemic or neurological neurodevelopmental disorders including Down's signs.6 In the superior oblique tendon sheath syn- syndrome and cerebral palsy ranges from about 40% drome (Brown's syndrome) elevation of the eye in to 90%.12 Of these squint is the most common with adduction is limited. The aetiology of this condition an average incidence of about 40%,12 15 depending may differ between cases from a congenital anomaly both on patient selection and the nature of the of the tendon sheath to an acquired swelling of the neurodevelopmental disorder. Although both comi- tendon. tant and incomitant deviations occur the former are far more common. SQUINT AND OCULAR DISEASE Squint may be the presenting sign of serious ocular Brain stem dysfunction-Two conditions need to be disease. The eyes of a blind infant are commonly, considered in this category: Duane's and Mobius' but not invariably, divergent. Most important, syndromes. Duane's syndrome is relatively common squint either convergent or divergent may be the and superficially resembles a sixth nerve palsy and first sign of a treatable and potentially life threaten- its identification can obviate the need for extensive ing condition such as retinoblastoma. Thus squint investigation Often an isolated anomaly, it can be can be associated with unilateral or bilateral ocular associated with other congenital abnormalities such disease, including cataract and retinal disorders such as Goldenhar's syndrome, spina bifida, and deafness. as regressed retinopathy of prematurity (see below). Mobius' syndrome is either a developmental defect Esotropia also develops in some children with or an acquired hypoxic insult to the cranial nerve congenital nystagmus. nuclei and is characterised by bilateral facial weak- ness, horizontal gaze palsies, and esotropia in some SQUINT AND SYSTEMIC CONDITIONS cases. Affected children are usually mentally re- Many systemic conditions can be associated with tarded, deaf, and exhibit limb malformations. strabismus and it would not be sensible or indeed possible to list them all here. Instead only a few will Muscle disease http://adc.bmj.com/ be considered and these will be grouped into broad Uncommon in routine clinical practice and can categories. easily therefore be misdiagnosed. Extraocular muscle involvement in myasthenia usually involves Prematurity the levator muscle of the eyelid resulting in ptosis The incidence of comitant squint is increased in and may also affect any other extraocular muscle infants born prematurely with figures quoted from leading to diplopia. Characteristically myasthenic -9 with and the can be 11% to 19%. The pathogenesis of this association symptoms increase fatigue signs on October 1, 2021 by guest. Protected copyright. is not well understood but is almost certainly due in unilateral or bilateral. Ocular motility hIay be part to both the perinatal neurological insults and affected in other muscle disorders and esotropia retinopathy of prematurity to which these infants may occur in myotonia congenita. Ophthalmoplegia are susceptible. A high incidence of squint has also is seen in a number of muscle disorders including been reported in term and preterm infants who congenital and other myopathies, dystrophia myo- have had phototherapy for the treatment of tonia and mitochondrial cytopathy. As in these last hyperbilirubinaemia,l° but a causal relationship mentioned conditions the limitation of ocular move- between these two and squint has not been defined ments is usually symmetrical, diplopia is not a particularly as many of these babies will also have common symptom. Rarely only the extraocular suffered the aforementioned complications. muscles are involved as in the congenital fibrosis syndrome. Disorders of the central nervous system Acute diseases of the central nervous system-The ORBITAL CONDITIONS association between squint and acute serious disease In this group of conditions certain ocular move- of the central nervous system is well known, and it ments are limited, due not to a neurogenic cause, must always be remembered that it may be the first but to mechanical restriction of the muscle move- sign of serious neurological disease. The deviation ment. Examples include injury such as orbital blow Arch Dis Child: first published as 10.1136/adc.64.3.413 on 1 March 1989. Downloaded from The management of squint 415 out fractures and infections. Squint may be the first caution as children can confuse physiological and sign of an orbital tumour such as rhabdomyosarcoma. pathological diplopia. The term diplopia is also sometimes used to describe blurred vision, as in the Investigation uncorrected myope. As children relatively rarely complain of diplopia, indirect evidence such as Even now the evaluation of a squint, and its. effect closing one eye when looking in a particular on the visual system, remains essentially clinical. direction can be helpful. Often there is a disparity The obvious priority is to identify first those con- between history and findings concerning the follow- ditions that require urgent treatment, and inves- ing signs: squint, ptosis, compensatory head pos- tigation should consequently be directed initially ture, proptosis, and pupil inequality.
Load more

Recommended publications
  • The Official Scientific Journal of the Delhi Ophthalmologycal Society DJO Vol
    DJO Vol.20, No. 2, October-December 09 DJO Vol.20, No. 2, October-December 09 October-December 2, No. Vol.20, DJO The Official Scientific Journal of the Delhi Ophthalmologycal Society DJO Vol. 20, No. 2, October-December, 2009 Delhi Journal of Ophthalmology Editor Editorial Board Rohit Saxena Rajvardhan Azad Ramanjit Sihota Managing Editor Vimla Menon Divender Sood Rajesh Sinha Atul Kumar Rishi Mohan Ashok K Grover Namrata Sharma Editorial Committee Mahipal S Sachdev Tanuj Dada Parijat Chandra M.Vanathi Lalit Verma Rajinder K hanna Tushar Agarwal Prakash Chand Agarwal Sharad Lakhotia Harbans Lal Chandrashekhar Kumar Swati Phuljhele P V Chaddha Amit Khosla Shibal Bhartiya Reena Sharma Dinesh Talwar B Ghosh Munish Dhawan Twinkle Parmar K.P.S Malik Kirti Singh Harinder Sethi Varun Gogia Pradeep Sharma B P Guliani Raghav Gupta Sashwat Ray V P Gupta S P Garg Ashish Kakkar Saptrishi Majumdar S. Bharti Arun Baweja General Information Delhi Journal of Ophthalmology (DJO), once called Visiscan, is a quarterly journal brought out by the Delhi Opthalmological Society. The journal aims at providing a platform to its readers for free exchange of ideas and information in accordance with the rules laid out for such publication. The DJO aims to become an easily readable referenced journal which will provide the specialists with up to date data and the residents with articles providing expert opinions supported with references. Contribution Methodology Delhi Journal of Opthalmology (DJO) is a quarterly journal. Author/Authors must have made significant contribution in carrying out the work and it should be original. It should be accompanied by a letter of transmittal.
    [Show full text]
  • Duane Retraction Syndrome
    Med. J. Cairo Univ., Vol. 78, No. 1, June: 331-336, 2010 www.medicaljournalofcairouniversity.com Duane Retraction Syndrome KARIMA L. SHALABY, M.D.* and MOSTAFA BAHGAT, M.D.** The Pediatric Ophthalmology Section*, Tripoli Eye Hospital and the Department of Ophthalmology**, Faculty of Medicine, Cairo University. Abstract Electromyography studies have shown paradox- ical innervations of Lateral rectus muscle and Purpose of Study: To evaluate and to manage if manage- anomalous synergistic innervations of medial rec- ment indicated for cases of Duane retraction syndrome. tus, inferior rectus, superior rectus and oblique Patients and Methods: 15 Duane retraction syndrome muscles [7,8] . (DRS) patients seen in Pediatric clinic in Tripoli Eye Hospital in period from January 2006-December 2006. Complete In most cases of DRS the entire 6 th nerve atro- ophthalmic examination including ortho-optic assessment for phy instead of post half of 6 th nerve (without all cases. specific teratogenic stimulus) 95% of DRS cases Results: Patients age ranged 1 year to 20 years in this this is the only initial abnormality. In about 5% of group of study, females were affected more than males with cases other abnormalities seen (e.g. nerve deafness). 2 to 1 ratio. Type 1 (esotropic) is the most common 80% of cases. Left eye was affected more than right eye. Bilateral in Most cases of DRS are sporadic [5,9] . 13.3% of cases. DRS clinical picture varies widely, surgical intervention will not eliminate the abnormality but will lessen Etiology: it. Two cases were operated upon to improve alignment in Etiology of DRS has been proposed by several primary position.
    [Show full text]
  • G:\All Users\Sally\COVD Journal\COVD 37 #3\Maples
    Essay Treating the Trinity of Infantile Vision Development: Infantile Esotropia, Amblyopia, Anisometropia W.C. Maples,OD, FCOVD 1 Michele Bither, OD, FCOVD2 Southern College of Optometry,1 Northeastern State University College of Optometry2 ABSTRACT INTRODUCTION The optometric literature has begun to emphasize One of the most troublesome and long recognized pediatric vision and vision development with the advent groups of conditions facing the ophthalmic practitioner and prominence of the InfantSEE™ program and recently is that of esotropia, amblyopia, and high refractive published research articles on amblyopia, strabismus, error/anisometropia.1-7 The recent institution of the emmetropization and the development of refractive errors. InfantSEE™ program is highlighting the need for early There are three conditions with which clinicians should be vision examinations in order to diagnose and treat familiar. These three conditions include: esotropia, high amblyopia. Conditions that make up this trinity of refractive error/anisometropia and amblyopia. They are infantile vision development anomalies include: serious health and vision threats for the infant. It is fitting amblyopia, anisometropia (predominantly high that this trinity of early visual developmental conditions hyperopia in the amblyopic eye), and early onset, be addressed by optometric physicians specializing in constant strabismus, especially esotropia. The vision development. The treatment of these conditions is techniques we are proposing to treat infantile esotropia improving, but still leaves many children handicapped are also clinically linked to amblyopia and throughout life. The healing arts should always consider anisometropia. alternatives and improvements to what is presently The majority of this paper is devoted to the treatment considered the customary treatment for these conditions.
    [Show full text]
  • Sixth Nerve Palsy
    COMPREHENSIVE OPHTHALMOLOGY UPDATE VOLUME 7, NUMBER 5 SEPTEMBER-OCTOBER 2006 CLINICAL PRACTICE Sixth Nerve Palsy THOMAS J. O’DONNELL, MD, AND EDWARD G. BUCKLEY, MD Abstract. The diagnosis and etiologies of sixth cranial nerve palsies are reviewed along with non- surgical and surgical treatment approaches. Surgical options depend on the function of the paretic muscle, the field of greatest symptoms, and the likelihood of inducing diplopia in additional fields by a given procedure. (Comp Ophthalmol Update 7: xx-xx, 2006) Key words. botulinum toxin (Botox®) • etiology • sixth nerve palsy (paresis) Introduction of the cases, the patients had hypertension and/or, less frequently, Sixth cranial nerve (abducens) palsy diabetes; 26% were undetermined, is a common cause of acquired 5% had a neoplasm, and 2% had an horizontal diplopia. Signs pointing aneurysm. It was noted that patients toward the diagnosis are an who had an aneurysm or neoplasm abduction deficit and an esotropia had additional neurologic signs or increasing with gaze toward the side symptoms or were known to have a of the deficit (Figure 1). The diplopia cancer.2 is typically worse at distance. Measurements are made with the Anatomical Considerations uninvolved eye fixing (primary deviation), and will be larger with the The sixth cranial nerve nuclei are involved eye fixing (secondary located in the lower pons beneath the deviation). A small vertical deficit may fourth ventricle. The nerve on each accompany a sixth nerve palsy, but a side exits from the ventral surface of deviation over 4 prism diopters the pons. It passes from the posterior Dr. O’Donnell is affiliated with the should raise the question of cranial fossa to the middle cranial University of Tennessee Health Sci- additional pathology, such as a fourth fossa, ascends the clivus, and passes ence Center, Memphis, TN.
    [Show full text]
  • Strabismus: a Decision Making Approach
    Strabismus A Decision Making Approach Gunter K. von Noorden, M.D. Eugene M. Helveston, M.D. Strabismus: A Decision Making Approach Gunter K. von Noorden, M.D. Emeritus Professor of Ophthalmology and Pediatrics Baylor College of Medicine Houston, Texas Eugene M. Helveston, M.D. Emeritus Professor of Ophthalmology Indiana University School of Medicine Indianapolis, Indiana Published originally in English under the title: Strabismus: A Decision Making Approach. By Gunter K. von Noorden and Eugene M. Helveston Published in 1994 by Mosby-Year Book, Inc., St. Louis, MO Copyright held by Gunter K. von Noorden and Eugene M. Helveston All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without prior written permission from the authors. Copyright © 2010 Table of Contents Foreword Preface 1.01 Equipment for Examination of the Patient with Strabismus 1.02 History 1.03 Inspection of Patient 1.04 Sequence of Motility Examination 1.05 Does This Baby See? 1.06 Visual Acuity – Methods of Examination 1.07 Visual Acuity Testing in Infants 1.08 Primary versus Secondary Deviation 1.09 Evaluation of Monocular Movements – Ductions 1.10 Evaluation of Binocular Movements – Versions 1.11 Unilaterally Reduced Vision Associated with Orthotropia 1.12 Unilateral Decrease of Visual Acuity Associated with Heterotropia 1.13 Decentered Corneal Light Reflex 1.14 Strabismus – Generic Classification 1.15 Is Latent Strabismus
    [Show full text]
  • Advanced Anatomy & Physiology of The
    By Diane F. Drake, LDO, ABOM, NCLEM, FNAO Introduction Terminology Presbyopia Anatomy Muscle Imbalances Refractive Errors Unequal refractive errors Spherical Correction Visualizing the Rx Cylindrical Correction Visual Angle and Minimum Visual Angle Minimum Visual Angle Visual Angle Subtend To extend under or to be opposite to The angle which is opposite the object being observed Minimum Detail The detail that must be detected on an object to positively identify or distinguish the object Minimum Detail E Minimum Detail Resolution The ability to detect minimum detail The resolving power of the normal eye is a minimum visual angle of 1 minute E The minimum visual angle of the letter is 1 minute The visual angle of the letter is 5 minutes Visual Acuity The measure of the angle subtended by the outer limits of rays of light coming from the minimum detail of an object as they enter the eye 20/20 or 6/6 Snell’s Law of Refraction n1 sin i = n2 sin r The Snellen Fraction The distance at which the test is made divided by the distance at which the smallest letter read subtends an angle of 5 minutes Snellen Letters Snellen Letters Minimum Angle of Resolution Landolt Ring (C) and the Illiterate E Contrast Sensitivity Terminology Emmetropia Ametropia Myopia Hyperopia Terminology Astigmatism Simple myopic Corneal astigmatism astigmatism Lenticular astigmatism Compound myopic Regular astigmatism astigmatism Irregular astigmatism Simple hyperopic astigmatism Compound hyperopic astigmatism Terminology Presbyopia Greek Presby
    [Show full text]
  • Guidelines for Universal Eye Screening in Newborns Including RETINOPATHY of Prematurity
    GUIDELINES FOR UNIVERSAL EYE SCREENING IN NEWBORNS INCLUDING RETINOPATHY OF PREMATURITY RASHTRIYA BAL SWASthYA KARYAKRAM Ministry of Health & Family Welfare Government of India June 2017 MESSAGE The Ministry of Health & Family Welfare, Government of India, under the National Health Mission launched the Rashtriya Bal Swasthya Karyakram (RBSK), an innovative and ambitious initiative, which envisages Child Health Screening and Early Intervention Services. The main focus of the RBSK program is to improve the quality of life of our children from the time of birth till 18 years through timely screening and early management of 4 ‘D’s namely Defects at birth, Development delays including disability, childhood Deficiencies and Diseases. To provide a healthy start to our newborns, RBSK screening begins at birth at delivery points through comprehensive screening of all newborns for various defects including eye and vision related problems. Some of these problems are present at birth like congenital cataract and some may present later like Retinopathy of prematurity which is found especially in preterm children and if missed, can lead to complete blindness. Early Newborn Eye examination is an integral part of RBSK comprehensive screening which would prevent childhood blindness and reduce visual and scholastic disabilities among children. Universal newborn eye screening at delivery points and at SNCUs provides a unique opportunity to identify and manage significant eye diseases in babies who would otherwise appear healthy to their parents. I wish that State and UTs would benefit from the ‘Guidelines for Universal Eye Screening in Newborns including Retinopathy of Prematurity’ and in supporting our future generation by providing them with disease free eyes and good quality vision to help them in their overall growth including scholastic achievement.
    [Show full text]
  • Anatomy & Physiology of The
    Anatomy & Physiology of The Eye 2017-2018 Done By: 433 Team Abdullah M. Khattab Important Doctor’s Notes Extra Abdullah AlOmair Resources: Team 433, Doctors Notes, Vaughan & Asbury’s General ophthalmology. Editing File Embryology of The Eye ............................................................................................. 2 ● Defects: ........................................................................................................................... 2 Development of The Eye After Birth .......................................................................... 3 ● Refractive power depends on two factors: ...................................................................... 3 The Orbit ................................................................................................................... 4 ● Seven bones contribute the bony orbit and surrounded by nasal sinuses. .................... 4 ● The orbital wall, pear-like shaped, formed by: ................................................................ 4 ● Structures Passing Through the Optic Openings: ........................................................... 4 Extraocular Muscles .................................................................................................. 1 ● Anatomy .......................................................................................................................... 1 ● Notes: .............................................................................................................................. 1 ● Field of action:
    [Show full text]
  • Disclosure Ocular Anatomy and Motility Ocular Anatomy Overview the Human Vision System the Human Vision System Lids and Lacrimal
    10/19/2012 Disclosure Ocular Anatomy and Motility Jenean Carlton BA, ABOC, NCLC 9President, Carlton & Associates, LLC – Carlton and Associates, 6 Course #214 Monterey Symposium LLC provides communications and educational materials for the optical industry 9Contributing Editor for Refractive Eyecare magazine. 6 Jenean Carlton BA, ABOC, NCLC 9Contributing Author Eyecare Business magazine. 9Communications Committee member OWA. 9Has been in the optical industry for 30 years with more than half of this time working in practices. 9Vision Monday’s Most Influential Women Award in Optical 2005 1 Ocular Anatomy Overview The Human Vision System 9 The eye is an amazing organ that works like a camera. 9 Think of the cornea and crystalline lens as the lenses of a camera, the iris as the shutter, and the retina as the photographic film. The Human Vision System Lids and Lacrimal System Vision takes place in the occipital lobe of The tear film has 3 layers: the brain Outer: the lipid layer (meibomian 9The brain can be glands in lids) is the oil layer. thought of as the development Middle: the aqueous layer center because it (lacrimal glands) is mainly water and provides oxygen converts and nutrients. electrical signals from the retina Inner: the mucin layer (goblet cells in conjunctiva) helps the tears into vision. adhere to the cornea. It is the mucus layer. 1 10/19/2012 Dry Eye Syndrome Lid Disorders What causes dry eyes? Ectropion= Out Treatments Entropion= In Ptosis- a drooping of the lid. Lids can have other problems too like turning out, in. Lids Disorders Lid Disorders Blepharitis is a chronic Hordeolum (stye) - inflammation- not an acute inflammation of a infection.
    [Show full text]
  • Vertical Rectus Transposition in Duane&Rsquo
    Eye (2015) 29, 839–842 & 2015 Macmillan Publishers Limited All rights reserved 0950-222X/15 www.nature.com/eye Sir, 5 Souza-Dias C. Congenital VI nerve palsy is Duane syndrome CORRESPONDENCE Vertical rectus transposition in Duane’s syndrome: does until disproven. Binocul Vis Strabismus Q 1992; 7:70. co-contraction worsen? 6 Sharma P, Tomar R, Menon V, Saxena R, Sharma A. Evaluation of periosteal fixation of lateral rectus and partial 1 We read with interest the article by Akar et al. We would VRT for cases of exotropic Duane’s retraction syndrome. like to make the following observations/queries. Indian J Ophthalmol 2014; 62: 204–208. In patients with Duane’s retraction syndrome there is some degree of subnormal and some degree of V Bhambhwani, PK Pandey, S Sood and K Rana anomalous innervation of the lateral rectus (LR) muscle. The extent and severity of the two may be variable. Guru Nanak Eye Centre and Maulana Azad Medical Presumably, subnormal innervation may lead to deficient College, New Delhi, India abduction and anomalous innervation may lead to E-mail: [email protected] co-contraction with globe retraction, palpebral aperture narrowing, or retraction equivalents like upshoots and Eye (2015) 29, 839; doi:10.1038/eye.2014.309; downshoots. published online 6 February 2015 In their article the authors describe patients of type 1 Duane syndrome to be with esotropia of 20 pd or more, an AHP larger than 201, limited abduction, and no significant upshoots or downshoots in the adducted Sir, position. There is no objective grading used for the Reply: Vertical rectus transposition in Duane’s measurement of shoots or palpebral aperture changes.
    [Show full text]
  • Neurocranial Defects with Neuro-Ophthalmic Significance
    11 Neurocranial Defects with Neuro-Ophthalmic Significance Ronald M. Minzter and Edward G. Buckley atients with cranial/skeletal defects often exhibit neuro- Pophthalmic abnormalities, which may be caused by specific anomalies within the spectrum of a given condition, or by an associated malformation of the nervous system, or be secondary to mechanical forces such as hydrocephalus. This chapter reviews the ophthalmic abnormalities found in progressive hemifacial atrophy, which are primarily due to structural defects, as well as ophthalmic abnormalities in Arnold–Chiari malformations, meningomyelocele, platybasia, and the Klippel–Feil syndrome, which are related to both structural and secondary neurological mechanisms. PROGRESSIVE HEMIFACIAL ATROPHY (PARRY–ROMBERG DISEASE) Progressive hemifacial atrophy (PHA), described by Parry in 1825, and by Romberg in 1846 as “trophoneurosis facialis,” is a progressive variable hemiatrophy of facial fat and subcutaneous tissues.102,111 Eulenburg34 later named this condition “progressive facial hemiatrophy.” The atrophy begins in childhood, pro- gresses intermittently and rapidly over the next 2 to 10 years, and usually decelerates by young adulthood.48,49,99 If onset is early enough, bone and cartilage may be affected because the facial structures have not yet fully matured104 (Fig. 11-1, top). In addition to facial atrophy, there can be dental/oral changes, migraine headaches, and neurological disturbances such as 371 372 handbook of pediatric neuro-ophthalmology A B CD FIGURE 11-1A–D. Progressive nature of progressive hemifacial atrophy (PHA) in a patient at 8 years old (A) and again at 15 years (B), showing left-sided atrophy. Fundus photos of the normal contralateral side (C) and the ipsilateral affected side with hypopigmentary disturbances (D), par- ticularly along the inferior arcade.
    [Show full text]
  • Squints Occur When the Eyes Do Not Work As a Team
    Squints occur when the eyes do not work as a team. One eye may look forward while the other looks in a different direction (that eye may turn inwards, outwards, upwards, or even dow nwards, while the other eye looks forward). Medically the term for this is called “Strabismus” (being cross-eyed) and is actually a fairly common occurrence affecting 2 to 3% of the population. Usually it affects younger children between the ages of 18 months and 4 years of age. Risk factors for developing a Causes squint • The exact cause of most childhood squints has • Positive family history (i.e. if a parent never been discovered – this is termed has had a squint or needed glasses from idiopathic an early age, there may be an increased • This, however, does not mean that it cannot chance that their child may also be be corrected affect ed) • Prematurity and low birth weight Certain conditions can result in a squint: • Cerebral palsy and Down syndrome • Refractive errors – this is due to the eye’s • Tumours (in rare cases) inability to focus correctly on an object. Conditions such as short sightedness, far sightedness, and astigmatism may cause one or both eyes to turn inwards (esotropia) or outwards (exotropia). • Any condition that causes one or both eyes to have poor vision. Conditions such as Congenital cataracts or damage to the cornea, optic nerve or retina can adversely affect vision and therefore, the affected eye can no longer look forward, resulting in a squint • Some squints only occur when the eyes are looking in certain directions.
    [Show full text]