- •Contents
- •General Introduction
- •Objectives
- •1 The Pediatric Eye Examination
- •Preparation
- •Examination: General Considerations and Strategies
- •Examination: Specific Elements
- •Visual Acuity Assessment
- •Alternative Methods of Visual Acuity Assessment in Preverbal Children
- •Red Reflex Examination (Brückner Test)
- •Dynamic Retinoscopy
- •Visual Field Testing
- •Pupil Testing
- •Anterior Segment Examination
- •Intraocular Pressure Measurement
- •Cycloplegic Refraction
- •Fundus Examination
- •Examination of the Uncooperative Child
- •2 Strabismus Terminology
- •Prefixes and Suffixes
- •Prefixes
- •Suffixes
- •Strabismus Classification Terms
- •Age of Onset
- •Fixation
- •Variation of the Deviation Size With Gaze Position or Fixating Eye
- •Miscellaneous Terms
- •Abbreviations for Types of Strabismus
- •3 Anatomy of the Extraocular Muscles
- •Horizontal Rectus Muscles
- •Vertical Rectus Muscles
- •Oblique Muscles
- •Levator Palpebrae Superioris Muscle
- •Relationship of the Rectus Muscle Insertions
- •Blood Supply of the Extraocular Muscles
- •Arterial System
- •Venous System
- •Structure of the Extraocular Muscles
- •Orbital and Fascial Relationships
- •Adipose Tissue
- •Muscle Cone
- •Muscle Capsule
- •The Tenon Capsule
- •Pulley System
- •Anatomical Considerations During Surgery
- •4 Amblyopia
- •Epidemiology
- •Detection and Screening
- •Pathophysiology
- •Classification
- •Strabismic Amblyopia
- •Refractive Amblyopia
- •Visual Deprivation Amblyopia
- •Evaluation
- •Treatment
- •Cataract Removal
- •Refractive Correction
- •Occlusion and Penalization
- •Complications of Therapy
- •5 Motor Physiology
- •Basic Principles and Terms
- •Axes of Fick and Ocular Rotations
- •Positions of Gaze
- •Extraocular Muscle Action
- •Eye Movements
- •Motor Units
- •Monocular Eye Movements
- •Binocular Eye Movements
- •Supranuclear Control Systems for Eye Movement
- •6 Sensory Physiology and Pathology
- •Physiology of Normal Binocular Vision
- •Retinal Correspondence
- •Fusion
- •Selected Aspects of the Neurophysiology of Vision
- •Visual Development
- •Effects of Abnormal Visual Experience on the Retinogeniculocortical Pathway
- •Abnormalities of Binocular Vision
- •Visual Confusion
- •Diplopia
- •Sensory Adaptations in Strabismus
- •Suppression
- •Anomalous Retinal Correspondence
- •Monofixation Syndrome
- •History and Presenting Features of Strabismus
- •Assessment of Ocular Alignment
- •Positions of Gaze
- •Cover Tests
- •Corneal Light Reflex Tests
- •Subjective Tests
- •Assessment of Eye Movements
- •Ocular Rotations
- •Convergence
- •Fusional Vergence
- •Special Tests
- •Motor Tests
- •Assessment of the Field of Single Binocular Vision
- •3-Step Test
- •Prism Adaptation Test
- •Torticollis: Differential Diagnosis and Evaluation
- •Ocular Torticollis
- •Tests of Sensory Adaptation and Binocular Cooperation
- •Red-Glass Test
- •Bagolini Lenses
- •4Δ Base-Out Prism Test
- •Afterimage Test
- •Amblyoscope Testing
- •Worth 4-Dot Test
- •Stereoacuity Testing
- •Related Videos
- •8 Esodeviations
- •Epidemiology
- •Pseudoesotropia
- •Infantile (Congenital) Esotropia
- •Pathogenesis
- •Evaluation
- •Management
- •Accommodative Esotropia
- •Pathogenesis and Types of Accommodative Esotropia
- •Evaluation
- •Management
- •Acquired Nonaccommodative Esotropias
- •Basic Acquired Nonaccommodative Esotropia
- •Cyclic Esotropia
- •Sensory Esotropia
- •Divergence Insufficiency
- •Spasm of the Near Reflex
- •Consecutive Esotropia
- •Nystagmus and Esotropia
- •Incomitant Esotropia
- •Sixth Nerve Palsy
- •Other Forms of Incomitant Esotropia
- •9 Exodeviations
- •Pseudoexotropia
- •Exophoria
- •Intermittent Exotropia
- •Clinical Characteristics
- •Evaluation
- •Classification
- •Treatment
- •Convergence Weakness Exotropia
- •Constant Exotropia
- •Infantile Exotropia
- •Sensory Exotropia
- •Consecutive Exotropia
- •Other Forms of Exotropia
- •Exotropic Duane Retraction Syndrome
- •Neuromuscular Abnormalities
- •Dissociated Horizontal Deviation
- •Convergence Paralysis
- •10 Pattern Strabismus
- •Etiology
- •Clinical Features and Identification
- •V Pattern
- •A Pattern
- •Y Pattern
- •X Pattern
- •λ Pattern
- •Management
- •General Principles
- •Treatment of Specific Patterns
- •11 Vertical Deviations
- •A Clinical Approach to Vertical Deviations
- •Incomitant Vertical Tropias
- •Overelevation and Overdepression in Adduction
- •Superior Oblique Muscle Palsy
- •Inferior Oblique Muscle Palsy
- •Other Incomitant Vertical Tropias
- •Comitant Vertical Tropias
- •Monocular Elevation Deficiency
- •Orbital Floor Fractures
- •Other Comitant Vertical Tropias
- •Dissociated Vertical Deviation
- •Clinical Features
- •Management
- •Related Videos
- •12 Special Forms of Strabismus
- •Congenital Cranial Dysinnervation Disorders
- •Duane Retraction Syndrome
- •Congenital Fibrosis of the Extraocular Muscles
- •Möbius Syndrome
- •Miscellaneous Special Forms of Strabismus
- •Brown Syndrome
- •Third Nerve Palsy
- •Sixth Nerve Palsy
- •Thyroid Eye Disease
- •Chronic Progressive External Ophthalmoplegia
- •Myasthenia Gravis
- •Esotropia and Hypotropia Associated With High Myopia
- •Internuclear Ophthalmoplegia
- •Ocular Motor Apraxia
- •Superior Oblique Myokymia
- •Strabismus Associated With Other Ocular Surgery
- •13 Childhood Nystagmus
- •General Features
- •Nomenclature
- •Evaluation
- •History
- •Ocular Examination
- •Types of Childhood Nystagmus
- •Congenital Nystagmus
- •Acquired Nystagmus
- •Nystagmus-Like Disorders
- •Convergence-Retraction Nystagmus
- •Opsoclonus
- •Treatment
- •Prisms
- •Surgery for Nystagmus
- •14 Surgery of the Extraocular Muscles
- •Evaluation
- •Indications for Surgery
- •Planning Considerations
- •Visual Acuity
- •General Considerations
- •Incomitance
- •Cyclovertical Strabismus
- •Prior Surgery
- •Surgical Techniques for the Extraocular Muscles and Tendons
- •Approaches to the Extraocular Muscles
- •Rectus Muscle Weakening Procedures
- •Rectus Muscle Strengthening Procedures
- •Rectus Muscle Surgery for Hypotropia and Hypertropia
- •Adjustable Sutures
- •Oblique Muscle Weakening Procedures
- •Oblique Muscle Tightening (Strengthening) Procedures
- •Stay Sutures
- •Transposition Procedures
- •Posterior Fixation
- •Complications of Strabismus Surgery
- •Diplopia
- •Unsatisfactory Alignment
- •Iatrogenic Brown Syndrome
- •Anti-Elevation Syndrome
- •Lost and Slipped Muscles
- •Pulled-in-Two Syndrome
- •Perforation of the Sclera
- •Postoperative Infections
- •Foreign-Body Granuloma and Allergic Reaction
- •Epithelial Cyst
- •Conjunctival Scarring
- •Adherence Syndrome
- •Dellen
- •Anterior Segment Ischemia
- •Change in Eyelid Position
- •Refractive Changes
- •Anesthesia for Extraocular Muscle Surgery
- •Methods
- •Postoperative Nausea and Vomiting
- •Oculocardiac Reflex
- •Malignant Hyperthermia
- •Chemodenervation Using Botulinum Toxin
- •Pharmacology and Mechanism of Action
- •Indications, Techniques, and Results
- •Complications
- •Related Videos
- •15 Growth and Development of the Eye
- •Normal Growth and Development
- •Dimensions of the Eye
- •Refractive State
- •Orbit and Ocular Adnexa
- •Cornea, Iris, Pupil, and Anterior Chamber
- •Intraocular Pressure
- •Extraocular Muscles
- •Retina
- •Visual Acuity and Stereoacuity
- •Abnormal Growth and Development
- •16 Decreased Vision in Infants and Children
- •Normal Visual Development
- •Evaluation of the Infant With Decreased Vision
- •Classification of Visual Impairment in Infants and Children
- •Delayed Visual Maturation
- •Pregeniculate Visual Impairment
- •Retrogeniculate Visual Impairment, or Cerebral Visual Impairment
- •Pediatric Low Vision Rehabilitation
- •17 Eyelid Disorders
- •Congenital Eyelid Disorders
- •Telecanthus
- •Dystopia Canthorum
- •Cryptophthalmos
- •Ablepharon
- •Congenital Coloboma of the Eyelid
- •Ankyloblepharon
- •Congenital Ectropion
- •Congenital Entropion
- •Epiblepharon
- •Congenital Tarsal Kink
- •Distichiasis
- •Euryblepharon
- •Epicanthus
- •Palpebral Fissure Slants
- •Blepharophimosis–Ptosis–Epicanthus Inversus Syndrome
- •Congenital Ptosis
- •Marcus Gunn Jaw-Winking Syndrome
- •Infectious and Inflammatory Eyelid Disorders
- •Neoplasms and Other Noninfectious Eyelid Lesions
- •Capillary Malformations
- •Congenital Nevocellular Nevi of the Skin
- •Other Acquired Eyelid Conditions
- •Trichotillomania
- •Excessive Blinking
- •18 Orbital Disorders
- •Craniosynostosis
- •Nonsynostotic Craniofacial Conditions
- •Infectious and Inflammatory Conditions
- •Preseptal Cellulitis
- •Orbital Cellulitis
- •Childhood Orbital Inflammation
- •Neoplasms
- •Differential Diagnosis
- •Primary Malignant Neoplasms
- •Metastatic Tumors
- •Hematopoietic, Lymphoproliferative, and Histiocytic Neoplasms
- •Benign Tumors
- •Ectopic Tissue Masses
- •Cystic Lesions
- •Teratoma
- •Ectopic Lacrimal Gland
- •19 Lacrimal Drainage System Abnormalities
- •Congenital and Developmental Anomalies
- •Atresia of the Lacrimal Puncta or Canaliculi
- •Congenital Lacrimal Fistula
- •Dacryocystocele
- •Nasolacrimal Duct Obstruction
- •Clinical Features
- •Nonsurgical Management
- •Surgical Management
- •20 Diseases of the Cornea, Anterior Segment, and Iris
- •Congenital and Developmental Anomalies of the Cornea
- •Abnormalities of Corneal Size and Shape
- •Abnormalities of Peripheral Corneal Transparency
- •Abnormalities of Central and Diffuse Corneal Transparency
- •Treatment of Corneal Opacities
- •Congenital and Developmental Anomalies of the Globe
- •Microphthalmos
- •Anophthalmos
- •Nanophthalmos
- •Abnormalities of the Iris
- •Abnormalities in the Size, Shape, or Location of the Pupil
- •Acquired Corneal Conditions
- •Keratitis
- •Systemic Diseases Affecting the Cornea or Iris
- •Metabolic Disorders Affecting the Cornea or Iris
- •Other Systemic Diseases Affecting the Cornea or Iris
- •Tumors of the Cornea, Iris, and Anterior Segment
- •Cornea
- •Iris
- •Ciliary Body
- •Miscellaneous Clinical Signs
- •Pediatric Iris Heterochromia
- •Anisocoria
- •21 External Diseases of the Eye
- •Infectious Conjunctivitis
- •Ophthalmia Neonatorum
- •Bacterial Conjunctivitis
- •Viral Conjunctivitis
- •Inflammatory Disease
- •Blepharitis
- •Ocular Allergy
- •Ligneous Conjunctivitis
- •Miscellaneous Conjunctival Disorders
- •Papillomas
- •Conjunctival Epithelial Inclusion Cysts
- •Conjunctival Nevi
- •Ocular Melanocytosis
- •Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
- •22 Pediatric Glaucomas
- •Genetics
- •Classification
- •Primary Childhood Glaucoma
- •Primary Congenital Glaucoma
- •Juvenile Open-Angle Glaucoma
- •Secondary Childhood Glaucoma
- •Glaucoma Associated With Nonacquired Ocular Anomalies
- •Glaucoma Associated With Nonacquired Systemic Disease or Syndrome
- •Secondary Glaucoma Associated With an Acquired Condition
- •Glaucoma Following Cataract Surgery
- •Treatment
- •Surgical Therapy
- •Medical Therapy
- •Prognosis and Follow-Up
- •Pediatric Cataracts
- •General Features
- •Morphology
- •Evaluation
- •Examination
- •Cataract Surgery in Pediatric Patients
- •Timing of the Procedure
- •Intraocular Lens Use in Children
- •Management of the Anterior Capsule
- •Lensectomy Without Intraocular Lens Implantation
- •Lensectomy With Intraocular Lens Implantation
- •Postoperative Care
- •Complications
- •Visual Outcome After Cataract Extraction
- •Structural or Positional Lens Abnormalities
- •Congenital Aphakia
- •Spherophakia
- •Coloboma
- •Dislocated Lenses in Children
- •Isolated Ectopia Lentis
- •Ectopia Lentis et Pupillae
- •Marfan Syndrome
- •Homocystinuria
- •Weill-Marchesani Syndrome
- •Sulfite Oxidase Deficiency
- •Treatment
- •24 Uveitis in the Pediatric Age Group
- •Epidemiology and Genetics
- •Classification
- •Anterior Uveitis
- •Juvenile Idiopathic Arthritis
- •Tubulointerstitial Nephritis and Uveitis Syndrome
- •Kawasaki Disease
- •Other Causes of Anterior Uveitis
- •Intermediate Uveitis
- •Posterior Uveitis
- •Toxoplasmosis
- •Toxocariasis
- •Panuveitis
- •Sarcoidosis
- •Familial Juvenile Systemic Granulomatosis
- •Vogt-Koyanagi-Harada Syndrome
- •Other Causes of Posterior Uveitis and Panuveitis
- •Masquerade Syndromes
- •Evaluation of Pediatric Uveitis
- •Treatment of Pediatric Uveitis
- •Management of Inflammation
- •Surgical Treatment of Uveitis Complications
- •25 Disorders of the Retina and Vitreous
- •Congenital and Developmental Abnormalities
- •Persistent Fetal Vasculature
- •Retinopathy of Prematurity
- •Hereditary Retinal Disease
- •Hereditary Macular Dystrophies
- •Hereditary Vitreoretinopathies
- •Infections
- •Herpes Simplex Virus and Cytomegalovirus
- •Human Immunodeficiency Virus
- •Tumors
- •Choroidal and Retinal Pigment Epithelial Lesions
- •Retinoblastoma
- •Acquired Disorders
- •Coats Disease
- •Diabetes Mellitus
- •Albinism
- •26 Optic Disc Abnormalities
- •Developmental Anomalies
- •Optic Nerve Hypoplasia
- •Morning Glory Disc Anomaly
- •Coloboma of the Optic Nerve
- •Myelinated Retinal Nerve Fibers
- •Tilted Disc Syndrome
- •Bergmeister Papilla
- •Megalopapilla
- •Peripapillary Staphyloma
- •Optic Nerve Aplasia
- •Melanocytoma
- •Optic Atrophy
- •Dominant Optic Atrophy, Kjer Type
- •Recessive Optic Atrophy
- •Behr Optic Atrophy
- •Leber Hereditary Optic Neuropathy
- •Optic Neuritis
- •Papilledema
- •Idiopathic Intracranial Hypertension
- •Pseudopapilledema
- •Drusen
- •27 Ocular Trauma in Childhood
- •Accidental Trauma
- •Superficial Injury
- •Penetrating Injury
- •Blunt Injury
- •Orbital Fractures
- •Traumatic Optic Neuropathy
- •Nonaccidental Trauma
- •Abusive Head Trauma
- •Ocular Injury Secondary to Nonaccidental Trauma
- •28 Ocular Manifestations of Systemic Disease
- •Diseases due to Chromosomal Abnormalities
- •Inborn Errors of Metabolism
- •Familial Oculorenal Syndromes
- •Phakomatoses
- •Neurofibromatosis
- •Tuberous Sclerosis
- •Von Hippel–Lindau Disease
- •Sturge-Weber Syndrome
- •Ataxia-Telangiectasia
- •Incontinentia Pigmenti
- •Wyburn-Mason Syndrome
- •Klippel-Trénaunay-Weber Syndrome
- •Intrauterine or Perinatal Infection
- •Toxoplasmosis
- •Rubella
- •Cytomegalovirus
- •Herpes Simplex Virus
- •Syphilis
- •Lymphocytic Choriomeningitis
- •Malignant Disease
- •Leukemia
- •Neuroblastoma
- •Basic Texts
- •Related Academy Materials
- •Requesting Continuing Medical Education Credit
Corneal Light Reflex Tests
Corneal light reflex tests rely on the location of the first Purkinje image of a fixation light to assess the alignment of the eyes. The Hirschberg and Krimsky tests are the main tests of this type. Although they are not as accurate as cover tests, they are a useful alternative for uncooperative patients and those with poor or eccentric fixation, in whom cover testing is not possible.
The Hirschberg test is based on the correlation between the decentration of the corneal light reflection and the ocular deviation. The ratio is about 22Δ/mm but can vary by as much as 20% from one individual to the next. With an uncooperative child, it is not always possible to accurately measure the light reflex displacement, so gross estimates of the deviation are often used: 30Δ if the reflex is at the pupil margin, 60Δ if the reflex is in the middle of the iris, and 90Δ if the reflex is at the limbus (Fig 7-4).
Figure 7-4 Hirschberg test. The extent to which the corneal light reflex is displaced from the center of the pupil provides an approximation of the angular size of the deviation (in this example, a left esotropia). (Modified with permission from Simon JW,
Calhoun JH. A Child’s Eyes: A Guide to Pediatric Primary Care. Gainesville, FL: Triad Publishing Company; 1997:72.)
The Krimsky test uses prisms to quantify the decentration of the corneal reflections and is best suited for near fixation measurement. This is done by holding a prism over either eye. By adjusting
the prism power to center the corneal reflection symmetrically in each eye, it is possible to approximate the near deviation (Fig 7-5).
Figure 7-5 Krimsky test. The right exotropia is measured by the size of the prism required to center the pupillary reflexes, as
shown at bottom. (Reprinted with permission from Simon JW, Calhoun JH. A Child’s Eyes: A Guide to Pediatric Primary Care. Gainesville, FL: Triad Publishing Company; 1997:72.)
The angle kappa (Fig 7-6) can affect corneal light reflex measurements. Angle kappa is the angle between the visual axis and the anatomical pupillary axis of the eye. If the fovea is slightly temporal to the pupillary axis (as is usually the case), the corneal light reflection will be slightly nasal to the center of the cornea. This is termed positive angle kappa. A large positive angle kappa can simulate exotropia. If the position of the fovea is nasal to the pupillary axis, the corneal light reflection will be temporal to the center of the cornea. This is termed negative angle kappa; it simulates esotropia. The angle kappa does not affect any of the cover tests.
Figure 7-6 Angle kappa. A positive angle (in which the corneal light reflex is medial to the vertical line) simulates exotropia, whereas a negative angle (in which the light reflex is lateral to the vertical line) simulates esotropia. (Reprinted with permission from
Parks MM. Ocular Motility and Strabismus. Hagerstown, MD: Harper & Row; 1975.)
The Brückner test uses the red reflex to test for bifoveal fixation. The direct ophthalmoscope is used to obtain a red reflex simultaneously in both eyes. Foveation of the ophthalmoscope filament dims the red reflex. If strabismus is present, the deviated eye will have a lighter and brighter reflex than the fixating eye. The Brückner test is used mainly by primary care practitioners to screen for strabismus; observation of the red reflex in this setting can also identify opacities in the visual axis and refractive errors. See also Chapter 1.
Subjective Tests
The Maddox rod test uses a device consisting of a series of parallel cylinders that converts a point source of light into a line image. The optical properties of the cylinders cause the streak of light to be situated 90° to the orientation of the parallel cylinders. Because fusion is precluded by the Maddox rod, heterophorias and heterotropias cannot be differentiated. The Maddox rod can be used to test for horizontal and vertical deviations and, when used in conjunction with another Maddox rod, for cyclodeviations.
To test for horizontal deviations, the clinician places the Maddox rod in front of 1 eye (for this example, the right eye) with the cylinders positioned horizontally. The patient fixates on a point source of light; he or she sees a vertical line with the right eye and the point source of white light with the left eye. If the light superimposes on the line, orthophoria is present. If the light is to the left of the line, an esodeviation is present; if the light is to the right of the line, an exodeviation is present. To measure the amount of deviation, the examiner finds the prism that superimposes the point source on the line. The Maddox rod test cannot be used satisfactorily to quantitate horizontal deviations, however, because accommodative convergence cannot be controlled. A similar procedure, with the cylinders aligned vertically, is used to test for vertical deviations.
The double Maddox rod test (Fig 7-7) is used to measure cyclotropia. Maddox rods are placed in a trial frame or phoropter and positioned in front of each eye, with the rods aligned vertically so that the patient sees 2 horizontal lines. The patient or examiner rotates the axes of the rods until the lines are perceived to be parallel. To facilitate the patient’s recognition of the 2 lines, it is often helpful to dissociate the lines by placing a small prism base-up or base-down in front of 1 eye. The angle of rotation that causes the line images to appear parallel determines the magnitude and direction
(intorsion or extorsion) of cyclotropia. Traditionally, a red Maddox rod is placed before the right eye and a white Maddox rod before the left, but evidence suggests that the different colors can cause fixation artifacts; these artifacts do not occur if the same color is used bilaterally. In congenital conditions such as congenital superior oblique palsy, the patient often does not subjectively appreciate the torsion and will not indicate any torsion with the double Maddox rod test. In these cases, observation of the fundus with an indirect ophthalmoscope for torsion can aid diagnosis.
Figure 7-7 Double Maddox rod test. A, The cylinders are aligned vertically to produce 2 horizontal lines. B, Top: View of a patient with a small left hypertropia and no torsion. Bottom: View of a patient with a small left hypertropia and extorsion. (Part A
courtesy of Scott Olitsky, MD; part B courtesy of Steven Archer, MD.)
The Lancaster red-green test (and variations such as the Hess, Harms, and Lee screening tests) uses red-green goggles that can be reversed, a red-slit projector, a greenslit projector, and a screen ruled into squares. The patient’s head is held steady; by convention, the test is begun with the red filter in front of the right eye. The examiner projects a red slit onto the screen, and the patient is asked to place the green slit so that it appears to coincide with the red slit. The relative positions of the 2 streaks are then recorded. The test is repeated for the diagnostic positions of gaze, and the goggles are then reversed so that the deviation that occurs with the fellow eye fixating can be recorded. The Lancaster red-green test is used primarily for patients with complicated incomitant strabismus and requires that the patient have normal retinal correspondence (see Chapter 6).
The major amblyoscope (Fig 7-8A) is a versatile instrument that can be used to measure ocular alignment subjectively or with cover testing. For subjective measurements, dissimilar targets are presented to each eye, and the patient is asked to superimpose them. If the patient has normal retinal correspondence, the horizontal, vertical, and torsional deviations can be read directly from the calibrated scale of the amblyoscope (Fig 7-8B). Also see the section Amblyoscope Testing, later in this chapter.
