- •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
cysts account for approximately 16% of childhood iris cysts. The visual prognosis is guarded.
Secondary iris cysts Secondary iris cysts have been reported in childhood after trauma; they are also associated with tumors and iris nevi.
Ciliary Body
Medulloepithelioma
A medulloepithelioma (diktyoma) originates from the nonpigmented epithelium of the ciliary body and most often presents as an iris mass during the first decade of life. Secondary glaucoma, hyphema, and ectopia lentis et pupillae or sectoral cataract (Fig 20-17) are less frequent initial manifestations. This rare lesion shows a spectrum of clinical and pathologic characteristics, ranging from benign to malignant. Although distant metastasis is rare, local invasion can lead to death. Teratoid elements are often present. Enucleation is usually required and is curative in most cases.
Figure 20-17 Sectoral cataract adjacent to medulloepithelioma. (Courtesy of Ken K. Nischal, MD.)
Miscellaneous Clinical Signs
Pediatric Iris Heterochromia
The differential diagnosis of pediatric iris heterochromia is extensive. Causes can be classified based on whether the condition is congenital or acquired and whether the affected eye is hypopigmented or hyperpigmented (Fig 20-18; Table 20-3). Trauma, chronic iridocyclitis, intraocular surgery, and use of topical prostaglandin analogues are important causes of acquired hyperpigmented heterochromia. Whether congenital or acquired, hypopigmented heterochromia that is associated with a more miotic pupil and ptosis on the ipsilateral side should prompt a workup for Horner syndrome (see the discussion later in this chapter).
Figure 20-18 Iris heterochromia. The left iris has become darker since development of a traumatic cataract. (Courtesy of John
W. Simon, MD.)
Table 20-3
Anisocoria
Inequality in the diameters of the 2 pupils is called anisocoria. For a detailed discussion of anisocoria and the following conditions, see BCSC Section 5, Neuro-Ophthalmology.
Physiologic anisocoria
Physiologic anisocoria is a common cause of a difference in size between the 2 pupils. This difference is usually less than 1 mm and can vary from day to day in an individual. The inequality does not change significantly when the patient is in dim light or bright light.
Tonic pupil
Features of a tonic pupil include anisocoria that is greater in bright light and a pupil that is sluggishly and segmentally responsive to light and more responsive to near effort. Greater than normal
constriction in response to dilute pilocarpine is diagnostic. Possible etiologic causes in children include varicella-zoster virus and Adie syndrome with absence of deep tendon reflexes.
Horner syndrome
A lesion at any location along the oculosympathetic pathway may lead to Horner syndrome. Affected patients have anisocoria that is greater in dim light and ptosis secondary to paralysis of the Müller muscle. Congenital cases may be associated with iris heterochromia in which the affected iris is lighter in color. However, the heterochromia may not be present in infants because the normal pupil needs time to acquire pigment.
The diagnosis of Horner syndrome can be confirmed with the use of topical cocaine or apraclonidine drops. Apraclonidine reverses the anisocoria, causing dilation of the affected (smaller) pupil and having no effect on the normal pupil. This agent should be used with caution in young children, as it may cause excessive sedation due to its central nervous system effects. Additional pharmacologic testing may not be necessary in the presence of typical clinical findings.
Horner syndrome in children may be idiopathic or due to trauma, surgery, or the presence of neuroblastoma affecting the sympathetic chain in the chest. For children with acquired Horner syndrome but no history of trauma or surgery that could explain the anisocoria, evaluation should include imaging studies of the brain, neck, and chest. The value of measuring catecholamine excretion has been questioned because some patients with catecholamine measurements that are not abnormal have been found to have neuroblastomas.