- •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
treatment because they tend to resolve spontaneously; however, resolution can take months or years. Lesions causing conjunctivitis can be treated by incising each lesion and debriding the central core. For young children, such treatment usually requires general anesthesia.
Figure 21-4 Molluscum contagiosum. A, Eyelid lesions. B, Secondary follicular conjunctivitis. (Part A courtesy of Edward L. Raab,
MD; part B courtesy of Gregg T. Lueder, MD.)
Inflammatory Disease
Blepharitis
Though less common in children than in adults, blepharitis is a common cause of chronic conjunctivitis in children. The signs and symptoms in children are similar to those in adults and include ocular irritation, morning crusting, eyelid margin erythema, and meibomian gland obstruction. Intermittent blurred vision may be present because of tear-film instability. Inferior keratitis may develop in more severe cases. Recurrent chalazia in children may indicate underlying blepharitis. Acne rosacea in children may present with chronic blepharitis and facial telangiectasias, papules, and pustules. Demodex (human mites that inhabit the hair follicles) may play a role in the pathogenesis of blepharitis.
Initial treatment of blepharitis includes warm compresses, eyelid scrubs with baby shampoo, and topical antibiotic ointment. Severe cases may benefit from either oral erythromycin or tetracycline. Erythromycin is most commonly used in young children to avoid the potential dental staining associated with use of tetracycline. Judicious use of topical steroids may be indicated in patients with corneal disease. Dietary supplementation with flaxseed oil (omega-3 fatty acid) may benefit some patients.
Jones SM, Weinstein JM, Cumberland P, et al. Visual outcome and corneal changes in children with chronic blepharokeratoconjunctivitis. Ophthalmology. 2007;114(12):2271–2280.
Ocular Allergy
Allergies are thought to affect approximately 20% of the US population, and more than 50% of
patients who seek treatment present with ocular symptoms. Allergic ocular disease is a common problem in children and is often associated with asthma, allergic rhinitis, and atopic dermatitis. Marked itching and bilateral conjunctival inflammation of a chronic, recurrent, and possibly seasonal nature are hallmarks of external ocular disease of allergic origin. Other signs and symptoms may be nonspecific and include tearing, stinging, burning, and photophobia.
Three specific types of ocular allergy are discussed in this section: seasonal allergic conjunctivitis, vernal keratoconjunctivitis (VKC), and atopic keratoconjunctivitis (AKC). All have some element of a type I hypersensitivity reaction caused by the interaction between an allergen and specific immunoglobulin E (IgE) antibodies on the surface of mast cells in the conjunctiva. This interaction initiates a cascade of biochemical events involved in mediation of the allergic response. Among the mediators released is histamine, which causes much of the itching, vasodilation, and edema that are characteristic of the ocular allergic response.
Seasonal allergic conjunctivitis
Seasonal allergic conjunctivitis is a common clinical entity that affects approximately 40 million people in the United States, including many children. It occurs in the spring and fall and is triggered by environmental contact with specific airborne allergens such as pollens from grasses, flowers, weeds, and trees. Patients typically present with red, watery eyes; boggy-appearing conjunctiva; and ocular itching. Blue-gray to purple discoloration of the lower eyelids, termed allergic shiners, can occur secondary to venous stasis from nasal congestion. Perennial allergic conjunctivitis is similar to seasonal allergic conjunctivitis and is a type I hypersensitivity reaction that occurs after contact with ubiquitous household allergens, such as dust mites and dander from domestic pets. This condition is diagnosed based on clinical presentation. Conjunctival scrapings reveal eosinophils, a finding that is almost diagnostic of an allergic response.
Treatment of all ocular allergy disorders is fundamentally similar to that of other allergy-related disorders. The most effective treatment is to remove the offending allergens from the patient’s environment. Unfortunately, attempts at removal may not be adequate to alleviate the patient’s symptoms. Medical treatment can be systemic or topical. Although oral antihistamines may be less effective at relieving specific ocular symptoms, they are often better tolerated in children, because many have an aversion to eyedrops.
Topical medications include mast-cell stabilizers, H1-receptor blockers, vasoconstrictors, corticosteroids, nonsteroidal anti-inflammatory agents, or combinations of these drugs (Table 21-2). Mast-cell stabilizers are often effective, but they must be used for a few days before an effect is seen. Nonsteroidal anti-inflammatory drops should be used with caution because associated corneal perforation has been reported. Topical steroid drops used in pulsed doses can effectively reduce severe allergic ocular symptoms, but patients must be closely monitored for adverse effects, including glaucoma and cataracts.
Table 21-2
Vernal keratoconjunctivitis
VKC is caused by type I and type IV hypersensitivity reactions. This condition most commonly affects males in the first 2 decades of life and, like seasonal allergic conjunctivitis, usually occurs in the spring and fall. It occurs in 2 forms: palpebral and limbal (or bulbar). Both types present with severe itching. The limbal form is more common in patients of African or Asian decent and is more prevalent in hotter climates.
Clinically, the palpebral form of VKC preferentially affects the tarsal conjunctiva of the upper eyelid (Fig 21-5). In the early stages, the eye may be diffusely injected, with little discharge. There may be no progression beyond this stage. However, papillae may multiply, covering the tarsal area with a mosaic of flat papules. A thick, ropy, whitish discharge may develop.
