- •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
childhood require regular ophthalmic examination.
The mechanism that results in aphakic glaucoma is unclear. The anterior chamber angle usually appears open on gonioscopy; the outflow channels are compromised by some combination of abnormal development of the anterior chamber angle and perhaps susceptibility of the infant eye to surgically induced inflammation, loss of lens support, retained lens epithelial cells, or vitreous factors. The children at highest risk for glaucoma development following cataract surgery are those who have surgery during infancy, and the risk appears to be even higher in patients with microcornea or persistent fetal vasculature. Surgery before 6 weeks of age may be an independent risk factor. Contradicting earlier reports, recent studies have not confirmed a lower incidence of glaucoma in pseudophakic compared with aphakic eyes.
Acute or subacute angle closure with iris bombé is a rare form of aphakic glaucoma. Although it usually occurs soon after surgery, onset can be delayed by a year or more. The diagnosis should be apparent with a slit lamp, but examination at the slit lamp may be difficult in young children. Treatment consists of anterior vitrectomy to relieve the pupillary block, often with surgical iridectomy and goniosynechialysis.
Beck AD, Freedman SF, Lynn MJ, Bothun E, Neely DE, Lambert SR; Infant Aphakia Treatment Study Group. Glaucomarelated adverse events in the Infant Aphakia Treatment Study: 1-year results. Arch Ophthalmol. 2012;130(3):300–305.
Chak M, Rahi JS; British Congenital Cataract Interest Group. Incidence of and factors associated with glaucoma after surgery for congenital cataract: findings from the British Congenital Cataract Study. Ophthalmology. 2008;115(6):1013–1018.
Treatment
The primary treatment for most childhood glaucoma is surgery. PCG is usually effectively treated with angle surgery (goniotomy or trabeculotomy). Although angle surgery may be used in some secondary pediatric glaucomas—most notably Axenfeld-Rieger syndrome, SWS, and aniridia—the outcome is often less successful. The treatment of most secondary childhood glaucomas is similar to that of open-angle or secondary glaucomas in adults. Medical treatment often has value prior to surgery and may have long-term benefit, particularly in JOAG and some secondary childhood glaucomas.
Surgical Therapy
Surgical intervention is the treatment of choice for PCG. Angle surgery is the preferred initial procedure. In a goniotomy, an incision is made, under direct gonioscopic visualization, across the trabecular meshwork (Fig 22-5). In a trabeculotomy, an external approach is used to identify and cannulate the Schlemm canal, then connect it with the anterior chamber through incision of the trabecular meshwork (Fig 22-6). A modification of this technique uses a 6-0 polypropylene monofilament suture or illuminated microcatheter to cannulate and open the Schlemm canal for its entire 360° circumference in one surgery. If the cornea is clear, either a goniotomy or a trabeculotomy can be performed at the surgeon’s discretion. Preoperative glaucoma medications or stripping of edematous epithelium from the cornea can temporarily clear the cornea. If the view through the cornea is compromised, trabeculotomy or combined trabeculotomy-trabeculectomy can be performed.
Figure 22-5 Goniotomy needle with its tip in the trabecular meshwork. The trabecular meshwork to the left of the needle has
been incised. (Courtesy of Ken K. Nischal, MD.)
Figure 22-6 Trabeculotomy. A, The trabeculotome has entered the Schlemm canal. B, The trabeculotome has been rotated
into the anterior chamber. (Courtesy of Steven Archer, MD.)
In approximately 80% of infants with PCG presenting from 3 months to 1 year of age, IOP is controlled with 1 or 2 angle surgeries. If the first procedure is not sufficient, at least 1 additional angle surgery is usually performed before a different procedure is used.
For children in whom angle surgery is not successful or is not indicated (as is the case in many secondary glaucomas) and medical therapy is inadequate, additional surgical options include trabeculectomy with or without antifibrotic therapy (eg, mitomycin C [MMC]), glaucoma implant procedures, and cycloablative procedures.
Trabeculectomy with the use of MMC is successful in approximately 50% of children. Reported success rates vary considerably by surgical technique and type of glaucoma and decrease as the length of follow-up increases. Patients younger than 1 year and those who are aphakic are more prone to treatment failure. Although the success rate of trabeculectomy improves with the use of antifibrotics such as MMC, the long-term risk of bleb leaks and endophthalmitis also increases. Longterm risk is reduced by using a fornix-based rather than a limbus-based trabeculectomy flap. The reported success rate of glaucoma implant surgery with Molteno (Molteno Ophthalmic, Dunedin, New Zealand), Baerveldt (Abbott Medical Optics, Abbott Park, IL), and Ahmed (New World Medical, Rancho Cucamonga, CA) devices varies between 54% and 85%. Although most of these children must remain on adjunct topical medical therapy to control IOP after surgery, their blebs are thicker and are less prone to leaking and infection than those of patients undergoing MMC-augmented trabeculectomy. Potential complications include shunt failure, tube erosion and migration, tube– cornea touch, cataract, restrictive strabismus, and endophthalmitis.
Laser cycloablation and cyclocryotherapy are generally reserved for resistant cases or those not amenable to other surgical procedures. These techniques decrease ciliary body production of aqueous
humor. Cyclocryotherapy (freezing the ciliary processes through the sclera) may be successful, but the complication rate is high. Repeated applications are often necessary, and the risk of phthisis and blindness is significant (approximately 10%). Transscleral laser cycloablation with the Nd:YAG or diode laser has a lower risk of complications. The short-term success rate is about 50%. Patients usually require more than one treatment.
Endoscopic cyclophotocoagulation (ECP) has been used in children with glaucomas that are difficult to treat. In ECP, a microendoscope applies laser energy to the ciliary processes under direct observation (Fig 22-7). Success rates of up to 50% have been reported. Although this is an intraocular procedure, the complication rate may be lower than that of external cyclodestructive procedures. Use of the microendoscope is advantageous in eyes with abnormal anterior segment anatomy. Some studies have shown encouraging results for patients with aphakic glaucoma.
Figure 22-7 Endoscopic view of ciliary processes during endoscopic cyclophotocoagulation. The white structure at the
bottom right of the photo is the lens. (Courtesy of Endo Optiks, Little Silver, NJ.)
O’Malley Schotthoefer E, Yanovitch TL, Freedman SF. Aqueous drainage device surgery in refractory pediatric glaucomas: I.
