- •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
found in sexually active teenagers in association with chlamydial urethritis or cervicitis. Patients present with follicular conjunctivitis, scant mucopurulent discharge, and preauricular adenopathy. There is no membrane formation. This condition can be diagnosed by culture of conjunctival scrapings, polymerase chain reaction, direct fluorescent antibody tests, and enzyme immunoassays. If untreated, inclusion conjunctivitis resolves spontaneously in 6–18 months. The recommended treatment is oral tetracycline, doxycycline, or azithromycin. The clinician should consider whether the patient has been sexually abused, especially if adult inclusion conjunctivitis is found in a young child.
Viral Conjunctivitis
Adenovirus
Viral conjunctivitis is most often caused by adenovirus, a DNA virus that can cause a range of human diseases, including upper respiratory tract infection and gastroenteritis. The following adenovirus diseases are listed with their associated serotypes: epidemic keratoconjunctivitis (serotypes 8, 19, and 37), pharyngoconjunctival fever (serotypes 3 and 7), acute hemorrhagic conjunctivitis (serotypes 11 and 21), and acute follicular conjunctivitis (serotypes 1, 2, 3, 4, 7, and 10).
Epidemic keratoconjunctivitis Epidemic keratoconjunctivitis (EKC) is a highly contagious conjunctivitis that tends to occur in epidemic outbreaks. This infection is an acute bilateral follicular conjunctivitis that is usually unilateral at onset and associated with preauricular adenopathy. Initial symptoms are foreign-body sensation and periorbital pain. A diffuse superficial keratitis is followed by focal epithelial lesions that stain. After 11–15 days, subepithelial opacities begin to form under the focal epithelial infiltrates. The epithelial component fades by day 30, but the subepithelial opacities may remain for up to 2 years. In severe infections, particularly in infants, a conjunctival membrane forms and marked swelling of the eyelids occurs; these signs must be differentiated from those of orbital or preseptal cellulitis. In severe cases, complications include persistent subepithelial opacities and conjunctival scar formation.
Because EKC is easily transmitted, medical personnel who become infected should be excluded from patient contact for up to 2 weeks, and isolation areas should be designated for examination of patients known or suspected to have adenoviral infections.
Diagnosis is usually based on clinical presentation but can be confirmed in the office by a rapid immunodetection assay. The organism can be recovered from the eyes and throat for 2 weeks after onset, demonstrating that patients are infectious during this period. Treatment is supportive in most cases. Artificial tears and cold compresses can provide symptomatic relief. Topical steroids may be used judiciously to decrease symptoms in severe cases and in cases of decreased vision secondary to subepithelial opacities; however, such agents may prolong the time to full recovery. Steroid use in adenovirus infection is seldom indicated in children.
Pharyngoconjunctival fever Pharyngoconjunctival fever presents with conjunctival hyperemia, subconjunctival hemorrhage, conjunctival edema, epiphora, and eyelid swelling, accompanied by sore throat and fever. Within a few days, a follicular conjunctival reaction and preauricular adenopathy develop. Symptoms may last for 2 weeks or more. Treatment is supportive because no topical or systemic treatment alters the course of the disease.
Herpes simplex virus
Conjunctivitis caused by HSV type 1 is covered in BCSC Section 8, External Disease and Cornea. Neonatal HSV infection is discussed in Chapter 28.
Varicella-zoster virus
Varicella-zoster virus (VZV) is a herpesvirus that can cause varicella and herpes zoster.
Varicella Varicella (chickenpox) is a contagious viral exanthem of childhood caused by primary infection with VZV. It presents with fever and vesicular eruptions of the skin and mucous membranes. Varicella vaccine is very effective in preventing severe disease, but immunized children exposed to VZV may develop mild symptoms. Clinical manifestations of primary VZV infection include fever and characteristic skin lesions. Except for eyelid vesicles and follicular conjunctivitis, ocular involvement is uncommon. Treatment of conjunctival disease is usually not necessary. Intravenous or oral acyclovir may be considered in the treatment of immunocompromised children with varicella.
Herpes zoster Reactivation of latent VZV from dorsal root and cranial nerve ganglia results in herpes zoster. Vesicular lesions may erupt on the periorbital skin in a dermatome configuration, with subsequent ocular involvement (Fig 21-3). Keratitis and anterior uveitis are most likely if the nasociliary branch of cranial nerve V is affected. Oral acyclovir is indicated in healthy children to shorten the course of the illness and decrease the risk of bacterial superinfection. Intravenous antiviral agents (famciclovir, valacyclovir, acyclovir) are indicated in immunocompromised patients or patients with severe disseminated disease. Antiviral medications should be started within 72 hours of onset of symptoms.
Figure 21-3 Herpes zoster.
Epstein-Barr virus
Epstein-Barr virus is a herpesvirus that can cause infectious mononucleosis. This disease usually occurs between ages 15 and 30 years and is benign and self-limited. Findings include fever, widespread adenopathy, pharyngitis, hepatic involvement, and the presence of atypical lymphocytes in the circulating blood. Conjunctivitis is the most common ocular finding. Nummular keratitis may also be seen. Diagnosis is confirmed with detection of immunoglobulin M (IgM) antibodies to viral capsid antigens or with a positive heterophile antibody test. Ocular treatment is cool compresses to the eyes.
Molluscum contagiosum
Molluscum contagiosum is caused by a DNA poxvirus and usually presents as numerous umbilicated skin lesions (Fig 21-4A). Lesions on or near the eyelid margin can release viral particles onto the conjunctival surface, resulting in a follicular conjunctivitis (Fig 21-4B). Most lesions do not require
