- •Foreword
- •Preface
- •Contributors
- •Contents
- •1. Epidemiology of Pediatric Strabismus
- •1.1 Introduction
- •1.2 Forms of Pediatric Strabismus
- •1.2.1 Esodeviations
- •1.2.1.1 Congenital Esotropia
- •1.2.1.2 Accommodative Esotropia
- •1.2.1.3 Acquired Nonaccommodative Esotropia
- •1.2.1.4 Abnormal Central Nervous System Esotropia
- •1.2.1.5 Sensory Esotropia
- •1.2.2 Exodeviations
- •1.2.2.1 Intermittent Exotropia
- •1.2.2.2 Congenital Exotropia
- •1.2.2.4 Abnormal Central Nervous System Exotropia
- •1.2.2.5 Sensory Exotropia
- •1.2.3 Hyperdeviations
- •1.3 Strabismus and Associated Conditions
- •1.4.1 Changes in Strabismus Prevalence
- •1.4.2 Changes in Strabismus Surgery Rates
- •1.5 Worldwide Incidence and Prevalence of Childhood Strabismus
- •1.6 Incidence of Adult Strabismus
- •References
- •2.1 Binocular Alignment System
- •2.1.2 Vergence Adaptation
- •2.1.3 Muscle Length Adaptation
- •2.2 Modeling the Binocular Alignment Control System
- •2.2.1 Breakdown of the Binocular Alignment Control System
- •2.2.4 Changes in Basic Muscle Length
- •2.2.6 Evidence Against the “Final Common Pathway”
- •2.3 Changes in Strabismus
- •2.3.1 Diagnostic Occlusion: And the Hazard of Prolonged Occlusion
- •2.3.2.1 Supporting Evidence for Bilateral Feedback Control of Muscle Lengths
- •2.4 Applications of Bilateral Feedback Control to Clinical Practice and to Future Research
- •References
- •3.1 Dissociated Eye Movements
- •3.2 Tonus and its relationship to infantile esotropia
- •3.5 Pathogenetic Role of Dissociated Eye Movements in Infantile Esotropia
- •References
- •4.1 Introduction
- •4.2.1 Binocular Correspondence: Anomalous, Normal, or Both?
- •4.3 MFS with Manifest Strabismus
- •4.3.1 Esotropia is the Most Common Form of MFS
- •4.3.2 Esotropia Allows for Better Binocular Vision
- •4.3.3 Esotropia is the Most Stable Form
- •4.4 Repairing and Producing MFS
- •4.4.1 Animal Models for the Study of MFS
- •References
- •5.1 Esotropia as the Major Type of Developmental Strabismus
- •5.1.2 Early Cerebral Damage as the Major Risk Factor
- •5.1.3 Cytotoxic Insults to Cerebral Fibers
- •5.1.5 Development of Binocular Visuomotor Behavior in Normal Infants
- •5.1.6 Development of Sensorial Fusion and Stereopsis
- •5.1.7 Development of Fusional Vergence and an Innate Convergence Bias
- •5.1.8 Development of Motion Sensitivity and Conjugate Eye Tracking (Pursuit/OKN)
- •5.1.9 Development and Maldevelopment of Cortical Binocular Connections
- •5.1.10 Binocular Connections Join Monocular Compartments Within Area V1 (Striate Cortex)
- •5.1.11 Too Few Cortical Binocular Connections in Strabismic Primate
- •5.1.12 Projections from Striate Cortex (Area V1) to Extrastriate Cortex (Areas MT/MST)
- •5.1.15 Persistent Nasalward Visuomotor Biases in Strabismic Primate
- •5.1.16 Repair of Strabismic Human Infants: The Historical Controversy
- •5.1.18 Timely Restoraion of Correlated Binocular Input: The Key to Repair
- •References
- •6. Neuroanatomical Strabismus
- •6.1 General Etiologies of Strabismus
- •6.2 Extraocular Myopathy
- •6.2.1 Primary EOM Myopathy
- •6.2.2 Immune Myopathy
- •6.2.4 Neoplastic Myositis
- •6.2.5 Traumatic Myopathy
- •6.3 Congenital Pulley Heterotopy
- •6.4 Acquired Pulley Heterotopy
- •6.5 “Divergence Paralysis” Esotropia
- •6.5.1 Vertical Strabismus Due to Sagging Eye Syndrome
- •6.5.2 Postsurgical and Traumatic Pulley Heterotopy
- •6.5.3 Axial High Myopia
- •6.6 Congenital Peripheral Neuropathy: The Congenital Cranial Dysinnervation Disorders (CCDDs)
- •6.6.1 Congenital Oculomotor (CN3) Palsy
- •6.6.3 Congenital Trochlear (CN4) Palsy
- •6.6.4 Duane’s Retraction Syndrome (DRS)
- •6.6.5 Moebius Syndrome
- •6.7 Acquired Motor Neuropathy
- •6.7.1 Oculomotor Palsy
- •6.7.2 Trochlear Palsy
- •6.7.3 Abducens Palsy
- •6.7.4 Inferior Oblique (IO) Palsy
- •6.8 Central Abnormalities of Vergence and Gaze
- •6.8.1 Developmental Esotropia and Exotropia
- •6.8.2 Cerebellar Disease
- •6.8.3 Horizontal Gaze Palsy and Progressive Scoliosis
- •References
- •7.1 Congenital Cranial Dysinnervation Disorders: Facts About Ocular Motility Disorders
- •7.1.1 The Concept of CCDDs: Ocular Motility Disorders as Neurodevelopmental Defects
- •7.1.1.1 Brainstem and Cranial Nerve Development
- •7.1.1.2 Single Disorders Representing CCDDs
- •7.1.1.3 Disorders Understood as CCDDs
- •7.2 Congenital Cranial Dysinnervation Disorders: Perspectives to Understand Ocular Motility Disorders
- •7.2.1.1 Brown Syndrome
- •Motility Findings
- •Saccadic Eye Movements
- •Comorbidity
- •Epidemiologic Features
- •Laterality
- •Sex Distribution
- •Incidence
- •Heredity
- •Potential Induction of the Syndrome
- •Radiologic Findings
- •Natural Course in Brown Syndrome
- •Intra-and Postoperative Findings
- •References
- •8.1 Amblyopia
- •8.2 What Is Screening?
- •8.2.1 Screening for Amblyopia, Strabismus, and/or Refractive Errors
- •8.2.1.1 Screening for Amblyopia
- •8.2.1.2 Screening for Strabismus
- •8.2.1.3 Screening for Refractive Error
- •8.2.1.4 Screening for Other Ocular Conditions
- •8.3 Screening Tests for Amblyopia, Strabismus, and/or Refractive Error
- •8.3.1 Vision Tests
- •8.3.3 Stereoacuity
- •8.3.4 Photoscreening and/or Autorefraction
- •8.3.6 Who Should Administer the Screening Program?
- •8.4 Treatment of Amblyopia
- •8.4.1 Type of Treatment
- •8.4.2 Refractive Adaptation
- •8.4.3 Conventional Occlusion
- •8.4.4 Pharmacological Occlusion
- •8.4.5 Optical Penalization
- •8.4.7 Treatment Compliance
- •8.4.8 Other Treatment Options for Amblyopia
- •8.4.9 Recurrence of Amblyopia Following Therapy
- •8.5 Quality of Life
- •8.5.1 The Impact of Amblyopia Upon HRQoL
- •8.5.3 Reading Speed and Reading Ability in Children with Amblyopia
- •8.5.4 Impact of Amblyopia Upon Education
- •8.5.6 The Impact of Strabismus Upon HRQoL
- •8.5.7 Critique of HRQoL Issues in Amblyopia
- •8.5.8 The Impact of the Condition or the Impact of Treatment?
- •References
- •9. The Brückner Test Revisited
- •9.1 Amblyopia and Amblyogenic Disorders
- •9.1.1 Early Detection of Amblyopia
- •9.1.2 Brückner’s Original Description
- •9.2.1 Physiology
- •9.2.2 Performance
- •9.2.3 Shortcomings and Pitfalls
- •9.3.1 Physiology
- •9.3.2 Performance
- •9.3.3 Possibilities and Limitations
- •9.4.1 Physiology
- •9.4.2 Performance
- •9.4.3 Possibilities and Limitations
- •9.5 Eye Movements with Alternating Illumination of the Pupils
- •References
- •10. Amblyopia Treatment 2009
- •10.1 Amblyopia Treatment 2009
- •10.1.1 Introduction
- •10.1.2 Epidemiology
- •10.1.3 Clinical Features of Amblyopia
- •10.1.4 Diagnosis of Amblyopia
- •10.1.5 Natural History
- •10.2 Amblyopia Management
- •10.2.1 Refractive Correction
- •10.2.2 Occlusion by Patching
- •10.2.3 Pharmacological Treatment with Atropine
- •10.2.4 Pharmacological Therapy Combined with a Plano Lens
- •10.3 Other Treatment Issues
- •10.3.1 Bilateral Refractive Amblyopia
- •10.3.3 Maintenance Therapy
- •10.4 Other Treatments
- •10.4.1 Filters
- •10.4.2 Levodopa/Carbidopa Adjunctive Therapy
- •10.5 Controversy
- •10.5.1 Optic Neuropathy Rather than Amblyopia
- •References
- •11.1 Introduction
- •11.1.2 Sensory or Motor Etiology
- •11.1.4 History
- •11.1.5 Outcome Parameters
- •11.2 Outcome of Surgery in the ELISSS
- •11.2.1 Reasons for the ELISSS
- •11.2.2 Summarized Methods of the ELISSS
- •11.2.3 Summarized Results of the ELISSS
- •11.2.4 Binocular Vision at Age Six
- •11.2.5 Horizontal Angle of Strabismus at Age Six
- •11.2.6 Alignment is Associated with Binocular Vision
- •11.3 Number of Operations and Spontaneous Reduction into Microstrabismus Without Surgery
- •11.3.1 The Number of Operations Per Child and the Reoperation Rate in the ELISSS
- •11.3.2 Reported Reoperation Rates
- •11.3.3 Test-Retest Reliability Studies
- •11.3.6 Spontaneous Reduction of the Angle
- •11.3.7 Predictors of Spontaneous Reduction into Microstrabismus
- •Appendix
- •References
- •12.1 Overview
- •12.1.2 Manifest Latent Nystagmus (MLN)
- •12.1.2.1 Clinical Characteristics of Manifest Latent Nystagmus (MLN)
- •12.1.3 Congenital Periodic Alternating Nystagmus (PAN)
- •12.1.3.1 Clinical characteristics of congenital periodic alternating nystagmus
- •12.2 Compensatory Mechanisms
- •12.2.1 Dampening by Versions
- •12.2.2 Dampening by Vergence
- •12.2.3 Anomalous Head Posture (AHP)
- •12.2.3.4 Measurement of AHP
- •12.2.3.6 Testing AHP at Near
- •12.3 Treatment
- •12.3.1 Optical Treatment
- •12.3.1.1 Refractive Correction
- •12.3.1.2 Spectacles and Contact Lenses (CL)
- •12.3.1.3 Prisms
- •12.3.1.4 Low Visual Aids
- •12.3.2 Medication
- •12.3.3 Acupuncture
- •12.3.4 Biofeedback
- •12.3.6 Surgical Treatment of Congenital Nystagmus
- •12.3.6.1 Management of Horizontal AHP
- •12.3.6.2 Management of Vertical AHP
- •12.3.6.3 Management of Head Tilt
- •Retro-Equatorial Recession of Horizontal Rectus Muscles
- •The Tenotomy Procedure
- •References
- •13.1 Dissociated Deviations
- •13.2 Surgical Alternatives to Treat Patients with DVD
- •13.2.1 Symmetric DVD with Good Bilateral Visual Acuity, with No Oblique Muscles Dysfunction
- •13.2.2 Bilateral DVD with Deep Unilateral Amblyopia
- •13.2.3 DVD with Inferior Oblique Overaction (IOOA) and V Pattern
- •13.2.4 DVD with Superior Oblique Overaction (SOOA) and A Pattern
- •13.2.5 Symmetric vs. Asymmetric Surgeries for DVD
- •13.3 Dissociated Horizontal Deviation
- •13.4 Dissociated Torsional Deviation. Head tilts in patients with Dissociated Strabismus
- •13.5 Conclusions
- •References
- •14.1 Introduction
- •14.2 Clinical and Theoretical Investigations
- •References
- •15.1 General Principles of Surgical Treatment in Paralytic Strabismus
- •15.1.1 Aims of Treatment
- •15.1.2 Timing of Surgery
- •15.1.3 Preoperative Assessment
- •15.1.4 Methods of Surgical Treatment
- •15.2 Third Nerve Palsy
- •15.2.1 Complete Third Nerve Palsy
- •15.2.2 Incomplete Third Nerve Palsy
- •15.3 Fourth Nerve Palsy
- •15.4 Sixth Nerve Palsy
- •References
- •16.1 Graves Orbitopathy (GO): Pathogenesis and Clinical Signs
- •16.1.1 Graves Orbitopathy is Part of a Systemic Disease: Graves Disease (GD)
- •16.1.2 Graves Orbitopathy−Clinical Signs
- •16.1.2.1 Clinical Changes Result in Typical Symptoms
- •16.1.3 Clinical Examination of GO
- •16.1.3.1 Signs of Activity
- •16.1.3.2 Assessing Severity of GO
- •16.1.3.3 Imaging
- •16.2 Natural History
- •16.3 Treatment of GO
- •16.3.1.1 Glucocorticoid Treatment
- •16.3.1.2 Orbital Radiotherapy
- •16.3.1.3 Combined Therapy: Glucocorticoids and Orbital Radiotherapy
- •16.3.1.4 Other Immunosuppressive Treatments and New Developments
- •16.3.2 Inactive Disease Stages
- •16.3.2.1 Orbital Decompression
- •16.3.2.2 Extraocular Muscle Surgery
- •16.3.2.3 Lid Surgery
- •16.4 Thyroid Dysfunction and GO
- •16.5.1 Relationship Between Cigarette Smoking and Graves Orbitopathy
- •16.5.2 Genetic Susceptibility
- •16.6 Special Situations
- •16.6.1 Euthyroid GO
- •16.6.2 Childhood GO
- •16.6.3 GO and Diabetes
- •References
9.3 Fundus Red Reflex (Brückner Reflex) |
119 |
Table 9.2. Sensitivity (50 trials for each condition) and false-positive findings (in 225 trials) of the Brückner reflex to detect unilateral spherical ametropia [34]
Simulated unilateral |
Experts 1 m (%) |
Experts 4 m (%) |
Students 1 m (%) |
Students 4 m (%) |
ametropia |
|
|
|
|
Hypermetropia 1 diopters |
34 |
52 |
8 |
60 |
Hypermetropia 2 diopters |
58 |
94 |
40 |
100 |
Hypermetropia 3 diopters |
76 |
96 |
56 |
100 |
Hypermetropia 4 diopters |
80 |
98 |
64 |
100 |
Myopia 1 diopters |
60 |
100 |
32 |
68 |
Myopia 2 diopters |
80 |
100 |
28 |
100 |
Myopia 3 diopters |
74 |
98 |
40 |
100 |
Myopia 4 diopters |
82 |
100 |
36 |
100 |
False-positive tests |
3.1 |
4.0 |
1.5 |
3.0 |
Results for unilateral astigmatism showed also the higher detection rates at 4 m distance (Table 9.3). On the basis of these results, it is recommendable to perform the test also at a distance of 4 m to detect refractive error more sensitively [34].
Paysse et al. compared the ability of paediatric residents to di erentiate asymmetric from symmetric red reflex in ten patients and six control subjects. Four patients were anisometropic by 2.25–5.5 dioptres without strabismus. In the entire group, paediatric residents achieved a test sensitivity of 61% and a specificity of 71% [3]. Gole and Douglas reported a test sensitivity of 86% and a specificity of not more than 65%. The Brückner test was performed by a medical student [20]. In these two studies, the test distance was 1 m. In a group of anisometropic patients, we achieved a sensitivity of 32.5% at that distance, and a specificity of 93.3%. At a distance of 4 m, sensitivity increased to 77.5% and specificity was 80%
[34]. These rates that depend on patient selection and observer experience are not representative for a real screening situation in early infancy.
9.3.2Performance
It is commonly recommended to perform the test at a distance of about 1 m or less (‘arm’s length distance’) by simultaneously illuminating both eyes of a patient, and to compare colour and brightness of the pupillary red reflexes for symmetry [3, 18, 19, 35, 37, 38, 40, 41]. The room light should be dimmed but the room should not be completely dark [18].
Using a direct ophthalmoscope is mandatory.Otoscope or flashlight illumination will not yield the same optical phenomena because the characteristic of the emitted light is di erent. The light beam must be directed simultaneous
Table 9.3. Sensitivity (50 trials for each condition) and false-positive findings (in 400 trials) of the Brückner reflex to detect unilateral astigmatismus simplex [30]
Simulated astigmatism |
With the rule |
Against rule |
With the rule |
Against rule |
|
1 m (%) |
1 m (%) |
4 m (%) |
4 m (%) |
Hypermetropic 1 diopters |
44 |
44 |
62 |
46 |
Hypermetropic 2 diopters |
58 |
60 |
88 |
72 |
Hypermetropic 3 diopters |
76 |
66 |
100 |
82 |
Hypermetropic 4 diopters |
88 |
72 |
100 |
100 |
Myopic 1 diopters |
50 |
22 |
44 |
74 |
Myopic 2 diopters |
60 |
48 |
74 |
98 |
Myopic 3 diopters |
60 |
70 |
86 |
100 |
Myopic 4 diopters |
70 |
80 |
92 |
100 |
False-positive tests |
5.5 |
|
5.25 |
|
120 |
9 The Brückner Test Revisited |
into both eyes to enable accurate comparison of the red reflexes. Light intensity can be varied during the examination. It should not be too high to avoid glare. Detection of small media opacity is easier at 0.5–0.1 m, examining each
9eye separately and using a convex lens in the ophthalmoscope, if necessary. To improve detection of refractive error, the examiner should then go 4 m backwards continuously observing the pupils simultaneously for luminance of the red reflexes. By the same way, it is possible to check for correct spectacle correction.
9.3.3Possibilities and Limitations
Severe media opacity is visible by lacking or dark fundus red reflex, regardless of distance. Small media opacity and pathologies of the fundus are best visible at a short distance. Any asymmetry in brightness and colour of the reflexes is predictive of amblyogenic risk factors [18, 19, 37, 38].
While the test is very sensitive to detect media opacity, detection of small-angle strabismus is limited. Detection of ametropia (particularly myopia) and anisometropia can be improved by extending the test distance, but nevertheless, isometropic hypermetropia cannot be detected reliably. Inter-ocular asymmetry in the red reflex can be caused by anisocoria and is also frequent in the age range below 8 months.
a
b
c
d
Summary for the Clinician
■The fundus red reflex test is an excellent complement and a possibility for ophthalmologists, orthoptists, paediatricians, and general practitioners to recognize various eye disorders very early. It is also a valuable tool for use in developmental countries. At a short distance relevant media opacity can be reliably detected by darkening of the red reflex. Testing for refractive error is better performed at an extended distance. Unior bilateral partially or completely weak or lacking red reflex is always pathological.
e
f
Fig. 9.5 Brückner reflex at 4 m distance in case of emmetropia OU (a) and simulated hypermetropia OS (anisometropia) of 1 diopter (b), 2 diopters (c), 3 diopters (d), and 4 diopters (e). For comparison, simulated myopia OS of 1 diopter (f) [34]
9.4Pupillary Light Reflexes
Pupillary light reflexes are being tested to detect pathologies in the iris, in the e erent branch of the pupillary light reflex loop, in the midbrain, or in the a erent branch of the reflex loop.Regarding strabismus diagnostic,Brückner described two criteria:
1.Dimming of the red reflex when the child is centrally fixating the ophthalmoscope light.
2.Reduced direct pupillary light reflex in the amblyopic eye compared with the direct light reflex in the nonamblyopic eye.
9.4 Pupillary Light Reflexes |
121 |
This step requires monocular illumination of the pupils. Brückner reported pupillary constriction in the deviated eye when the light beam was changed from the fixating eye onto the strabismic eye, as soon as this eye took up fixation. Permanent fixation with the previously illuminated dominant eye yields an eccentric retinal image of the ophthalmoscope light in the deviated eye. Despite dark adaptation of the deviated eye, the pupillomotor e ect of the eccentric illumination can be weaker than that of the central illumination in the fellow eye. So, the response to alternating illumination may either look like relative a erent pupillomotor deficit or dimming of the red reflex in the amblyopic eye occurs after some latency when the amblyopic eye takes up fixation.
9.4.1Physiology
Illumination of one eye causes symmetric constriction of both pupils [42–46]. In unilateral amaurosis, pupillary constriction is lacking in both eyes when only the blind eye is being illuminated. Illumination of the other eye causes normal constriction of the pupils in both eyes. Less severe a erent disorders show a similar pattern except residual reaction to illumination of the (more severely) concerned eye. Discreet a erent disorders can be found by the swinging flash light test [42–44]. Aiming at strabismus diagnostic, the observer has to watch any eye movement occurring after the change of the illumination to the other eye. If the previously deviated eye which is now being illuminated takes up fixation, the movement of this eye may be visible, and the pupils will constrict because foveal illumination
has a stronger pupillomotor e ect compared with paracentral illumination. Pupillary constriction is also induced by the increased light sensitivity of the ‘dark-adapted’ eye. In the clinical situation, it is hardly possible to discriminate between these two mechanisms. If the strabismic eye fails to take up central fixation, an a erent pupillomotor defect component may be simulated when this eye is being illuminated or there is in fact a relative a erent pupillary defect (RAPD) due to amblyopia [47–50]. Figure 9.6 shows that already minimal eccentricity of illumination reduces pupillary constriction compared with a central illumination.
9.4.2Performance
The examiner directs the light cone on the patient’s right eye and observes constriction of each pupil. The procedure is repeated illuminating the patient’s left eye. If both pupils are normally reactive, which is mostly the case, comparison of the direct light reflexes of both eyes will be su cient [51–52]. If only one pupil is reactive, this pupil can be used to compare the constriction with subsequent illumination of the right and the left eye. The pupillary constriction has to be equal in latency, speed and amplitude, regardless of the eye illuminated.
9.4.3Possibilities and Limitations
RAPD is typical of severe asymmetric retinal lesion or asymmetric lesion of the optic nerve including the optic chiasm. Amblyogenic disorders, such as refractive error,
Fig. 9.6 Video-oculographic registration of the change in pupil diameter with alternating fixation of the ophthalmoscope light and low illuminated visual targets 2.5, 5, 7.5, and 10° right (positive values) and left (negative values) of the ophthalmoscope light. Fixation of the ophthalmoscope light induced more pupillary constriction than fixation of a target as few as 2.5° beside
gaze direction |
10º |
5º |
0º |
–5º |
–10º |
pupil diameter
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