- •Foreword
- •Preface
- •Contents
- •Chapter 1
- •The Apparently Blind Infant
- •Introduction
- •Hereditary Retinal Disorders
- •Leber Congenital Amaurosis
- •Joubert Syndrome
- •Congenital Stationary Night Blindness
- •Achromatopsia
- •Congenital Optic Nerve Disorders
- •Cortical Visual Insufficiency
- •Causes of Cortical Visual Loss
- •Perinatal Hypoxia-Ischemia
- •Postnatal Hypoxia-Ischemia
- •Cerebral Malformations
- •Head Trauma
- •Twin Pregnancy
- •Metabolic and Neurodegenerative Conditions
- •Meningitis, Encephalitis, and Sepsis
- •Hydrocephalus, Ventricular Shunt Failure
- •Preictal, Ictal, or Postictal Phenomena
- •Associated Neurologic and Systemic Disorders
- •Characteristics of Visual Function
- •Neuro-Ophthalmologic Findings
- •Diagnostic and Prognostic Considerations
- •Role of Visual Attention
- •Neuroimaging Abnormalities and their Implications
- •Subcortical Visual Loss (Periventricular Leukomalacia)
- •Perceptual Difficulties
- •Dorsal and Ventral Stream Dysfunction
- •Pathophysiology
- •Intraventricular Hemorrhage
- •Hemianopic Visual Field Defects in Children
- •Delayed Visual Maturation
- •Blindsight
- •The Effect of Total Blindness on Circadian Regulation
- •Horizons
- •References
- •Chapter 2
- •Congenital Optic Disc Anomalies
- •Introduction
- •Optic Nerve Hypoplasia
- •Segmental Optic Nerve Hypoplasia
- •Excavated Optic Disc Anomalies
- •Morning Glory Disc Anomaly
- •Optic Disc Coloboma
- •Peripapillary Staphyloma
- •Megalopapilla
- •Optic Pit
- •Congenital Tilted Disc Syndrome
- •Optic Disc Dysplasia
- •Congenital Optic Disc Pigmentation
- •Aicardi Syndrome
- •Doubling of the Optic Disc
- •Optic Nerve Aplasia
- •Myelinated (Medullated) Nerve Fibers
- •The Albinotic Optic Disc
- •References
- •Chapter 3
- •The Swollen Optic Disc in Childhood
- •Introduction
- •Papilledema
- •Pathophysiology
- •Neuroimaging
- •Primary IIH in Children
- •Secondary IIH
- •IIH Secondary to Neurological Disease
- •IIH Secondary to Systemic Disease
- •Malnutrition
- •Severe Anemia
- •Addison Disease
- •Bone Marrow Transplantation
- •Renal Transplantation
- •Down Syndrome
- •Gliomatosis Cerebri
- •Systemic Lupus Erythematosis
- •Sleep Apnea
- •Postinfectious
- •Childhood IIH Associated with Exogenous Agents
- •Atypical IIH
- •Treatment of IIH in Children
- •Prognosis of IIH in Children
- •Optic Disc Swelling Secondary to Neurological Disease
- •Hydrocephalus
- •Neurofibromatosis
- •Spinal Cord Tumors
- •Subacute Sclerosing Panencephalitis
- •Optic Disc Swelling Secondary to Systemic Disease
- •Diabetic Papillopathy
- •Malignant Hypertension
- •Sarcoidosis
- •Leukemia
- •Cyanotic Congenital Heart Disease
- •Craniosynostosis Syndromes
- •Nonaccidental Trauma (Shaken Baby Syndrome)
- •Cysticercosis
- •Mucopolysaccharidosis
- •Infantile Malignant Osteopetrosis
- •Malaria
- •Paraneoplastic
- •Uveitis
- •Blau Syndrome
- •CINCA
- •Kawasaki Disease
- •Poststreptococal Uveitis
- •Intrinsic Optic Disc Tumors
- •Optic Disc Hemangioma
- •Tuberous Sclerosis
- •Optic Disc Glioma
- •Combined Hamartoma of the Retina and RPE
- •Retrobulbar Tumors
- •Optic Neuritis in Children
- •History and Physical Examination
- •Postinfectious Optic Neuritis
- •Acute Disseminated Encephalomyelitis
- •MS and Pediatric Optic Neuritis
- •Devic Disease (Neuromyelitis Optica)
- •Prognosis and Treatment
- •Course of Visual Loss and Visual Recovery
- •Systemic Prognosis
- •Systemic Evaluation of Pediatric Optic Neuritis
- •Treatment
- •Leber Idiopathic Stellate Neuroretinitis
- •Ischemic Optic Neuropathy
- •Autoimmune Optic Neuropathy
- •Pseudopapilledema
- •Optic Disc Drusen
- •Epidemiology
- •Ophthalmoscopic Appearance in Children
- •Distinguishing Buried Disc Drusen from Papilledema
- •Fluorescein Angiographic Appearance
- •Neuroimaging
- •Histopathology
- •Pathogenesis
- •Ocular Complications
- •Systemic Associations
- •Natural History and Prognosis
- •Systemic Disorders Associated with Pseudopapilledema
- •Down Syndrome
- •Alagille Syndrome
- •Kenny Syndrome
- •Leber Hereditary Neuroretinopathy
- •Mucopolysaccharidosis
- •Linear Sebaceous Nevus Syndrome
- •Orbital Hypotelorism
- •References
- •Chapter 4
- •Optic Atrophy in Children
- •Introduction
- •Epidemiology
- •Optic Atrophy Associated with Retinal Disease
- •Congenital Optic Atrophy Vs. Hypoplasia
- •Causes of Optic Atrophy in Children
- •Compressive/Infiltrative Intracranial Lesions
- •Optic Glioma
- •Craniopharyngioma
- •Noncompressive Causes of Optic Atrophy in Children with Brain Tumors
- •Postpapilledema Optic Atrophy
- •Paraneoplastic Syndromes
- •Radiation Optic Neuropathy
- •Hydrocephalus
- •Hereditary Optic Atrophy
- •Dominant Optic Atrophy (Kjer Type)
- •Leber Hereditary Optic Neuropathy
- •Recessive Optic Atrophy
- •X-Linked Optic Atrophy
- •Behr Syndrome
- •Wolfram Syndrome (DIDMOAD)
- •Toxic/Nutritional Optic Neuropathy
- •Neurodegenerative Disorders with Optic Atrophy
- •Krabbe’s Infantile Leukodystrophy
- •Canavan Disease (Spongiform Leukodystrophy)
- •PEHO Syndrome
- •Neonatal Leukodystrophy
- •Metachromatic Leukodystrophy
- •Pantothenate Kinase-Associated Neurodegeneration
- •Neuronal Ceroid Lipofuscinoses (Batten Disease)
- •Familial Dysautonomia (Riley–Day Syndrome)
- •Infantile Neuroaxonal Dystrophy
- •Organic Acidurias
- •Propionic Acidemia
- •Cobalamin C Deficiency with Methylmalonic Acidemia
- •Spinocerebellar Degenerations
- •Hereditary Polyneuropathies
- •Mucopolysaccharidoses
- •Optic Atrophy due to Hypoxia-Ischemia
- •Traumatic Optic Atrophy
- •Vigabatrin
- •Carboplatin
- •Summary of the General Approach to the Child with Optic Atrophy
- •References
- •Chapter 5
- •Transient, Unexplained, and Psychogenic Visual Loss in Children
- •Introduction
- •Transient Visual Loss
- •Migraine
- •Migraine Aura
- •Amaurosis Fugax as a Migraine Equivalent
- •Migraine Versus Retinal Vasospasm
- •Migraine Headache
- •Complicated Migraine
- •Pathophysiology
- •Genetics
- •Sequelae
- •Treatment
- •Epilepsy
- •Epileptiform Visual Symptoms with Seizure Aura
- •Ictal Cortical Blindness
- •Postictal Blindness
- •Distinguishing Epilepsy from Migraine
- •Vigabitrin-Associated Visual Field Loss
- •Posttraumatic Transient Cerebral Blindness
- •Cardiogenic Embolism
- •Nonmigrainous Cerebrovascular Disease
- •Transient Visual Obscurations Associated with Papilledema
- •Anomalous Optic Discs
- •Entoptic Images
- •Media Opacities
- •Retinal Circulation
- •Phosphenes
- •Uhthoff Symptom
- •Alice in Wonderland Syndrome
- •Charles Bonnet Syndrome
- •Lilliputian Hallucinations
- •Palinopsia
- •Peduncular Hallucinosis
- •Hypnagogic Hallucinations
- •Posterior Reversible Encephalopathy Syndrome
- •Neurodegenerative Disease
- •Multiple Sclerosis
- •Schizophrenia
- •Hallucinogenic Drug Use
- •Cannabinoid Use
- •Toxic and Nontoxic Drug Effects
- •Antimetabolites and Cancer Therapy
- •Digitalis
- •Erythropoietin
- •Atropine (Anticholinergic Drugs)
- •Carbon Monoxide
- •Summary of Clinical Approach to the Child with Transient Visual Disturbances
- •Unexplained Visual Loss in Children
- •Transient Amblyogenic Factors
- •Refractive Abnormalities
- •Cornea
- •Retina
- •Optic Nerve
- •Central Nervous System
- •Psychogenic Visual Loss in Children
- •Clinical Profile
- •Neuro-Ophthalmologic Findings
- •Group 1: The Visually Preoccupied Child
- •Group 2: Conversion Disorder
- •Group 3: Possible Factitious Disorder
- •Group 4: Psychogenic Visual Loss Superimposed on True Organic Disease
- •Interview with the Parents
- •Interview with the Child
- •When to Refer Children with Psychogenic Visual Loss for Psychiatric Treatment
- •Horizons
- •References
- •Chapter 6
- •Ocular Motor Nerve Palsies in Children
- •Introduction
- •Oculomotor Nerve Palsy
- •Clinical Anatomy
- •Nucleus
- •Fascicle
- •Clinical Features
- •Isolated Inferior Rectus Muscle Palsy
- •Isolated Inferior Oblique Muscle Palsy
- •Isolated Internal Ophthalmoplegia
- •Isolated Divisional Oculomotor Palsy
- •Oculomotor Synkinesis
- •Etiology
- •Congenital Third Nerve Palsy
- •Congenital Third Nerve Palsy with Cyclic Spasm
- •Traumatic Third Nerve Palsy
- •Meningitis
- •Ophthalmoplegic Migraine
- •Recurrent Isolated Third Nerve Palsy
- •Cryptogenic Third Nerve Palsy in Children
- •Vascular Third Nerve Palsy in Children
- •Postviral Third Nerve Palsy
- •Differential Diagnosis
- •Management
- •Amblyopia
- •Ocular Alignment
- •Ptosis
- •Trochlear Nerve Palsy
- •Clinical Anatomy
- •Clinical Features
- •Head Posture
- •Three-Step Test
- •Bilateral Trochlear Nerve Palsy
- •Etiology
- •Traumatic Trochlear Nerve Palsy
- •Congenital Trochlear Nerve Palsy
- •Large Vertical Fusional Vergence Amplitudes
- •Facial Asymmetry
- •Synostotic Plagiocephaly
- •Hydrocephalus
- •Idiopathic
- •Compressive Lesions
- •Rare Causes of Trochlear Nerve Palsy
- •Differential Diagnosis
- •Treatment
- •Abducens Nerve Palsy
- •Clinical Anatomy
- •Clinical Features
- •Causes of Sixth Nerve Palsy
- •Congenital Sixth Nerve Palsy
- •Traumatic Sixth Nerve Palsy
- •Benign Recurrent Sixth Nerve Palsy
- •Pontine Glioma
- •Elevated Intracranial Pressure
- •Infectious Sixth Nerve Palsy
- •Inflammatory Sixth Nerve Palsy
- •Rare Causes of Sixth Nerve Palsy
- •Differential Diagnosis
- •Duane Retraction Syndrome
- •Genetics
- •Other Clinical Features of Duane Syndrome
- •Upshoots and Downshoots
- •Y or l Pattern
- •Synergistic Divergence
- •Rare Variants
- •Systemic Associations
- •Etiology of Duane Syndrome
- •Classification of Duane Syndrome on the Basis of Range of Movement
- •Embryogenesis
- •Surgical Treatment of Duane Syndrome
- •Esotropia in Duane Syndrome
- •Duane Syndrome with Exotropia
- •Bilateral Duane Syndrome
- •Management of Sixth Nerve Palsy
- •Multiple Cranial Nerve Palsies in Children
- •Horizons
- •References
- •Chapter 7
- •Complex Ocular Motor Disorders in Children
- •Introduction
- •Strabismus in Children with Neurological Dysfunction
- •Visuovestibular Disorders
- •Neurologic Esotropia
- •Spasm of the Near Reflex
- •Exercise-Induced Diplopia
- •Neurologic Exotropia
- •Convergence Insufficiency
- •Skew Deviation
- •Horizontal Gaze Palsy in Children
- •Congenital Ocular Motor Apraxia
- •Vertical Gaze Palsies in Children
- •Downgaze Palsy in Children
- •Upgaze Palsy in Children
- •Diffuse Ophthalmoplegia in Children
- •Myasthenia Gravis
- •Transient Neonatal Myasthenia
- •Congenital Myasthenic Syndromes
- •Juvenile Myasthenia
- •Olivopontocerebellar Atrophy
- •Botulism
- •Bickerstaff Brainstem Encephalitis
- •Tick Paralysis
- •Wernicke Encephalopathy
- •Miscellaneous Causes of Ophthalmoplegia
- •Transient Ocular Motor Disturbances of Infancy
- •Transient Neonatal Strabismus
- •Transient Idiopathic Nystagmus
- •Tonic Downgaze
- •Tonic Upgaze
- •Neonatal Opsoclonus
- •Transient Vertical Strabismus in Infancy
- •Congenital Ptosis
- •Congenital Fibrosis Syndrome
- •Möbius Sequence
- •Monocular Elevation Deficiency, or “Double Elevator Palsy”
- •Brown Syndrome
- •Other Pathologic Synkineses
- •Internuclear Ophthalmoplegia
- •Cyclic, Periodic, or Aperiodic Disorders Affecting Ocular Structures
- •Ocular Neuromyotonia
- •Eye Movement Tics
- •Eyelid Abnormalities in Children
- •Congenital Ptosis
- •Excessive Blinking in Children
- •Hemifacial Spasm
- •Eyelid Retraction
- •Apraxia of Eyelid Opening
- •Pupillary Abnormalities
- •Congenital Bilateral Mydriasis
- •Accommodative Paresis
- •Adie Syndrome
- •Horner Syndrome
- •References
- •Chapter 8
- •Nystagmus in Children
- •Introduction
- •Infantile Nystagmus
- •Clinical Features
- •Onset of Infantile Nystagmus
- •Terminology
- •History and Physical Examination
- •Relevant History
- •Physical Examination
- •Hemispheric Visual Evoked Potentials
- •Immature Infantile Nystagmus Waveforms
- •Mature Infantile Nystagmus Waveforms
- •Fixation in Infantile Nystagmus
- •Smooth Pursuit System in Infantile Nystagmus
- •Vestibulo-ocular Reflex in Infantile Nystagmus
- •Saccadic System in Infantile Nystagmus
- •Suppression of Oscillopsia in Infantile Nystagmus
- •Albinism
- •Achiasmia
- •Isolated Foveal Hypoplasia
- •Congenital Retinal Dystrophies
- •Cone and Cone-Rod Dystrophies
- •Achromatopsia
- •Blue Cone Monochromatism
- •Leber Congenital Amaurosis
- •Alström Syndrome
- •Rod-Cone Dystrophies
- •Congenital Stationary Night Blindness
- •Medical Treatment
- •Optical Treatment
- •Surgical Treatment
- •Surgery to Improve Torticollis
- •Surgery to Improve Vision
- •Tenotomy with Reattachment
- •Four Muscle Recession
- •Artificial Divergence Surgery
- •When to Obtain Neuroimaging Studies in Children with Nystagmus
- •Treatment
- •Spasmus Nutans
- •Russell Diencephalic Syndrome of Infancy
- •Monocular Nystagmus
- •Nystagmus Associated with Infantile Esotropia
- •Torsional Nystagmus
- •Horizontal Nystagmus
- •Latent Nystagmus
- •Treatment of Manifest Latent Nystagmus
- •Nystagmus Blockage Syndrome
- •Treatment of Nystagmus Blockage Syndrome
- •Vertical Nystagmus
- •Upbeating Nystagmus in Infancy
- •Congenital Downbeat Nystagmus
- •Hereditary Vertical Nystagmus
- •Periodic Alternating Nystagmus
- •Seesaw Nystagmus
- •Congenital versus Acquired Seesaw Nystagmus
- •Saccadic Oscillations that Simulate Nystagmus
- •Convergence-Retraction Nystagmus
- •Opsoclonus and Ocular Flutter
- •Causes of Opsoclonus
- •Kinsbourne Encephalitis
- •Miscellaneous Causes
- •Pathophysiology
- •Voluntary Nystagmus
- •Ocular Bobbing
- •Neurological Nystagmus
- •Pelizaeus-Merzbacher Disease
- •Joubert Syndrome
- •Santavuori-Haltia Disease
- •Infantile Neuroaxonal Dystrophy
- •Down Syndrome
- •Hypothyroidism
- •Maple Syrup Urine Disease
- •Nutritional Nystagmus
- •Epileptic Nystagmus
- •Summary
- •References
- •Chapter 9
- •Torticollis and Head Oscillations
- •Introduction
- •Torticollis
- •Ocular Torticollis
- •Head Tilts
- •Incomitant Strabismus
- •Synostotic Plagiocephaly
- •Spasmus Nutans
- •Infantile Nystagmus
- •Benign Paroxysmal Torticollis of Infancy
- •Dissociated Vertical Divergence
- •Ocular Tilt Reaction
- •Photophobia, Epiphora, and Torticollis
- •Down Syndrome
- •Spasmodic Torticollis
- •Head Turns
- •Seizures
- •Cortical Visual Insufficiency
- •Congenital Ocular Motor Apraxia
- •Vertical Head Positions
- •Refractive Causes of Torticollis
- •Neuromuscular Causes of Torticollis
- •Congenital Muscular Torticollis
- •Systemic Causes of Torticollis
- •Head Oscillations
- •Head Nodding with Nystagmus
- •Spasmus Nutans
- •Infantile Nystagmus
- •Head Nodding without Nystagmus
- •Bobble-Headed Doll Syndrome
- •Cerebellar Disease
- •Benign Essential Tremor
- •Paroxysmal Dystonic Head Tremor
- •Autism
- •Infantile Spasms
- •Congenital Ocular Motor Apraxia
- •Opsoclonus/Myoclonus
- •Visual Disorders
- •Blindness
- •Intermittent Esotropia
- •Otological Abnormalities
- •Labyrinthine Fistula
- •Systemic Disorders
- •Aortic Regurgitation
- •Endocrine and Metabolic Disturbances
- •Nasopharyngeal Disorders
- •Organic Acidurias
- •References
- •Chapter 10
- •Introduction
- •Neuronal Disease
- •Neuronal Ceroid Lipofuscinosis
- •Infantile NCL (Santavuori-Haltia Disease)
- •Late Infantile (Jansky–Bielschowsky Disease)
- •Juvenile NCL (Batten Disease)
- •Lysosomal Diseases
- •Gangliosidoses
- •GM2 Type I (Tay–Sachs Disease)
- •GM2 Type II (Sandhoff Disease)
- •GM2 Type III
- •Niemann–Pick Disease
- •Gaucher Disease
- •Mucopolysaccharidoses
- •MPS1H (Hurler Syndrome)
- •MPS1S (Scheie Syndrome)
- •MPS2 (Hunter Syndrome)
- •MPS3 (Sanfilippo Syndrome)
- •MPS4 (Morquio Syndrome)
- •MPS6 (Maroteaux–Lamy Syndrome)
- •MPS7 (Sls Syndrome)
- •Sialidosis
- •Subacute Sclerosing Panencephalitis
- •White Matter Disorders
- •Metachromatic Leukodystrophy
- •Krabbe Disease
- •Pelizaeus–Merzbacher Disease
- •Cockayne Syndrome
- •Alexander Disease
- •Sjögren–Larsson Syndrome
- •Cerebrotendinous Xanthomatosis
- •Peroxisomal Disorders
- •Zellweger Syndrome
- •Adrenoleukodystrophy
- •Basal Ganglia Disease
- •Wilson Disease
- •Maple Syrup Urine Disease
- •Homocystinuria
- •Abetalipoproteinemia
- •Mitochondrial Encephalomyelopathies
- •Myoclonic Epilepsy and Ragged Red Fibers (MERRF)
- •Mitochondrial Depletion Syndrome
- •Congenital Disorders of Glycosylation
- •Horizons
- •References
- •Chapter 11
- •Introduction
- •The Phakomatoses
- •Neurofibromatosis (NF1)
- •Neurofibromatosis 2 (NF2)
- •Tuberous Sclerosis
- •Sturge–Weber Syndrome
- •von Hippel–Lindau Disease
- •Ataxia Telangiectasia
- •Linear Nevus Sebaceous Syndrome
- •Klippel–Trenauney–Weber Syndrome
- •Brain Tumors
- •Suprasellar Tumors
- •Pituitary Adenomas
- •Rathke Cleft Cysts
- •Arachnoid Cysts
- •Cavernous Sinus Lesions
- •Hemispheric Tumors
- •Hemispheric Astrocytomas
- •Gangliogliomas and Ganglioneuromas
- •Supratentorial Ependymomas
- •Primitive Neuroectodermal Tumors
- •Posterior Fossa Tumors
- •Medulloblastoma
- •Cerebellar Astrocytoma
- •Ependymoma
- •Brainstem Tumors
- •Tumors of the Pineal Region
- •Meningiomas
- •Epidermoids and Dermoids
- •Gliomatosis Cerebri
- •Metastasis
- •Hydrocephalus
- •Hydrocephalus due to CSF Overproduction
- •Noncommunicating Hydrocephalus
- •Communicating Hydrocephalus
- •Aqueductal Stenosis
- •Tumors
- •Intracranial Hemorrhage
- •Intracranial Infections
- •Chiari Malformations
- •Chiari I
- •Chiari II
- •Chiari III
- •The Dandy–Walker Malformation
- •Congenital, Genetic, and Sporadic Disorders
- •Clinical Features of Hydrocephalus
- •Ocular Motility Disorders in Hydrocephalus
- •Dorsal Midbrain Syndrome
- •Visual Loss in Hydrocephalus
- •Effects and Complications of Treatment
- •Vascular Lesions
- •AVMs
- •Clinical Features of AVMs in Children
- •Natural History
- •Treatment
- •Cavernous Angiomas
- •Intracranial Aneurysms
- •Isolated Venous Ectasia
- •Craniocervical Arterial Dissection
- •Strokes in Children
- •Cerebral Venous Thrombosis
- •Cerebral Dysgenesis and Intracranial Malformations
- •Destructive Brain Lesions
- •Porencephaly
- •Hydranencephaly
- •Encephalomalacia
- •Colpocephaly
- •Malformations Due to Abnormal Stem Cell Proliferation or Apoptosis
- •Schizencephaly
- •Hemimegalencephaly
- •Lissencephaly
- •Gray Matter Heterotopia
- •Malformations Secondary to Abnormal Cortical Organization and Late Migration
- •Polymicrogyria
- •Holoprosencephaly
- •Absence of the Septum Pellucidum
- •Hypoplasia, Agenesis, or Partial Agenesis of the Corpus Callosum
- •Focal Cortical Dysplasia
- •Anomalies of the Hypothalamic–Pituitary Axis
- •Posterior Pituitary Ectopia
- •Empty Sella Syndrome
- •Encephaloceles
- •Transsphenoidal Encephalocele
- •Orbital Encephalocele
- •Occipital Encephalocele
- •Cerebellar Malformations
- •Molar Tooth Malformation
- •Rhombencephalosynapsis
- •Lhermitte–Duclos Disease
- •Miscellaneous
- •Congenital Corneal Anesthesia
- •Reversible Posterior Leukoencephalopathy
- •Cerebroretinal Vasculopathies
- •Syndromes with Neuro-Ophthalmologic Overlap
- •Proteus Syndrome
- •PHACE Syndrome
- •Encephalocraniocutaneous Lipomatosis
- •References
- •Index
240 |
5 Transient, Unexplained, and Psychogenic Visual Loss in Children |
|
|
involved, and to report adjustment difficulties and obsessive personality traits in adolescence. Bullying and loss of an important figure seemed to be important concomitants.436
Many afflicted children report that school is the source of their stress. However, these school anxieties sometimes represent a displacement from the real source of the problem, which is often the home,323,325 as evidenced by the fact that the temporal distribution of psychogenic visual loss in children is virtually even throughout the school year rather than skewed toward the beginning.255 Decreased acuity is often noted initially when teachers report that the child complains of difficulty seeing the blackboard and doing schoolwork, while parents note that there seems to be no difficulty watching television or playing games.325 The child is moved closer to the blackboard with little symptomatic improvement. Vision testing at school reveals bilaterally decreased acuity, and the child is referred for ophthalmologic examination.
The child’s affliction may result in considerable secondary gain. According to Rabinowicz,323 “the deterioration of grades when an ‘A’ student begins to perform at a ‘B’ or ‘C’ level is certainly something that will immediately focus parental and school attention on the child, and the deteriorated vision brings forth sympathy, of which many of these children feel deprived. The child’s visual symptoms may bring about a temporary cease-fire in an ongoing interparental war.” While such explanations sound plausible and may indeed be applicable in some cases, it is reasonable to assume that the intricate psychological details of each case are difficult to uncover.
Bizzare forms of psychogenic visual loss referable to specific behaviors are occasionally described.252 (The complaint of eyestrain in children may have a strong psychogenic component, as evidenced by a recent study that found that most children with eyestrain have normal eye examinations, while most patients with refractive error, amblyopia, or strabismus were free of eyestrain).178
Neuro-Ophthalmologic Findings
The primary goal of the neuro-ophthalmologic examination is to rule out organic causes of unexplained visual loss. This process requires the examiner to be familiar with organic conditions that may masquerade as psychogenic visual loss in children and to have the necessary clinical and ancillary tests to diagnose them. Major inconsistencies between the current test results and those of previous examinations often provide an early clue to the psychogenic nature of the child’s symptoms. The next goal is to determine whether the child’s vision is better than he or she reports.
Unlike adults, children with nonorganic visual loss are rarely malingering (i.e., deliberately feigning a visual prob-
Table 5.3 Different clinical profiles of psychogenic visual loss in children versus adults
Children |
Adults |
Malingering uncommon |
Malingering common |
Strong predilection toward girls |
Affects both men and women |
Clusters around puberty |
Occurs at any age |
Visual loss usually bilateral |
Visual loss unilateral or bilateral |
Normal confrontation visual |
Tubular visual field constriction |
fields, except in older |
|
teenagers |
|
Usually resolves with reassurance |
Variable response to reassurance |
Recurrences rare |
Recurrences common |
|
|
lem in order to obtain some desired goal) (Table 5.3). When they complain of visual difficulties, they generally believe they are afflicted. They often display genuine bafflement regarding the nature of their visual problems, and they try very hard to cooperate and please the examiner.255,323
Ophthalmologic examination usually reveals defective distance acuity in the range of 20/30–20/100.323 The Snellen chart is often read in a hesitant manner, with wrinkling of the forehead and facial grimacing, even when the line falls well within the range of the child’s purported acuity.352 Some letters are read quickly, while similar-sized letters seem to present insurmountable difficulty.323 Attempts to guess the letters are often inappropriate (the child will call an 0 an E rather than a D). Some children complain of headaches prior to or during the examination.323
Both children and adults with nonorganic visual loss display visual field constriction when tested with Goldmann or automated perimetry.30,363 Goldmann or tangent screen testing may show the characteristic spiraling of isopters. However, it has been our experience that, with encouragement, most prepubescent children with psychogenic visual loss demonstrate normal confrontation visual fields. Older teenagers, like adults, may display functional visual field constriction when tested with confrontation techniques.30,298,363 Even when kinetic visual fields are normalized, static visual fields may remain abnormal, showing false-negative errors and short-term fluctuations.298 Bourke and Gole40 have noted that children with psychogenic visual loss are unable to see the Ishihara numbers while maintaining perfect color vision to shapes (which subtend the same visual angle).
Unlike malingering adults, who may have to be tricked into seeing using a variety of tests,56 one can often persuade the suggestible child with psychogenic visual loss to improve his or her performance on a visual test. Titmus stereoacuity is often initially poor, but many children can be persuaded to identify all Titmus circles with encouragement. Normal Titmus stereoacuity is a valuable finding, as it demonstrates that visual acuity is at least 20/30 in each eye, and it demonstrates that the child’s visual loss, at least in part, is psychogenic.
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In attempting to determine the child’s actual visual acuity, it is helpful to place a negligible corrective lens in the phoropter (plano + 0.50 × 90°) and urge the child to read an isolated 20/10 letter on the Snellen line. When the child is unable to read the letter, the examiner can make use of suggestion by offering an isolated 20/15 letter as a major concession, thereby implying that the child’s failure to read the letter represents a major visual loss.383 When an “enormous” letter from the 20/25 line appears on the screen, the child often readily identifies it. In performing this exercise, it is important to use a single letter viewed through a phoropter in a dark room, which removes external cues as to the size of the letter. If the vision fails to improve, it is helpful to repeat the process after dilation, with the suggestion that the pupils are “huge” so that “extra light” can enter the eyes. The ability of the examiner to use negligible refractive lenses to improve acuity is further evidence of psychogenic visual loss.
The visuscope is a valuable and underutilized diagnostic tool in the evaluation of psychogenic visual loss in children. In this test, the child is instructed to follow the star from the visuoscope as the examiner observes the position of the star on the macula. Children with early Stargardt disease display eccentric (nonfoveal) fixation on the star, while those with psychogenic visual loss “lock on” to the star and maintain foveal fixation as the star is moved.
In a child with monocular visual loss that is suspected to be nonorganic, a useful test for nonorganic monocular visual loss is to place red-green glasses on the child, with the green filter over the eye with decreased vision. The red-green colored filter bar in the projector is placed over the Snellen line with the red filter over the first three letters, and the green filter over the last three. The glasses allow the child to see only the red letters through the red filter, while all letters are visible through the green filter. The child with psychogenic monocular visual loss may demonstrate the nonorganic nature of his or her visual loss by reading the entire line. As an optional second test, the examiner can place the green filter in front of the normal eye. Some children read only half the letters, despite the fact that all letters can be seen through the green filter.
Scott and Egan354 found a high frequency of organic visual disorders in children who manifest with psychogenic visual loss. Early-onset macular dystrophies (cone dystrophy, Stargardt’s disease), and Leber hereditary optic neuropathies are easily misdiagnosed as psychogenic visual loss.236 As mentioned above, children with craniopharyngiomas that compress the optic nerve but have not yet produced visible optic atrophy can behave functionally, as can children with cortical visual loss that selectively involves the dorsal streams in the visual association areas.138 In the child whose vision fails to normalize over time, the possibility of organic underlay necessitates periodic reevaluation. Although the psychiat-
ric underpinnings are difficult to elucidate, functional imaging studies have shown a positive pathophysiologic substrate in patients with “hysterical” disorders, suggesting that any true distinction between “organic” and psychogenic remains tenuous. For all these reasons, the clinician should never become cavalier about the diagnosis of psychogenic visual loss.
Categories of Psychogenic Visual Loss
in Children
We have found it useful to conceptualize children with psychogenic visual loss as falling into one of the four groups:
Group 1: The Visually Preoccupied Child
Most children with psychogenic visual loss have, for unknown reasons, become preoccupied with their vision and concerned about their visual health. They start to believe their visual function has changed for the worse. These children can be compared with adults who become concerned about their cardiac function and find that their pulse rate is high whenever they measure it.
Aside from their concern about their vision and the anxiety it engenders, these children seem to have no serious personality disorder that interferes with their day-to-day functioning. Simple reassurance leads to gradual resolution of their symptoms and normalization of their acuity. One might speculate that such cases represent a physiological adjustment period (reminiscent of the general physical awkwardness one sees during puberty) during which hormonal/physiological alterations somehow under lies this phenomenon in predisposed individuals. It is likely that the psychodynamics differ in these children from those whose visual symptomatology lingers for years despite reassurance.
Occasionally, we examine children who are concerned about their ability to function visually and have become convinced that glasses are the solution. These are usually younger children with friends who have recently received glasses. If asked, these children volunteer that they would like to wear glasses. (Although such children may have negligible refractive errors, we sometimes prescribe glasses for them after a frank discussion with the parents and after reaffirming for the child that he or she seems to see normally without glasses). It is difficult to know what symbolic value wearing glasses may have for a given child. If one assumes that this child is expressing some kind of need and that glasses will not harm the child, then one may decide to give glasses and reevaluate the situation after several months.
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Group 2: Conversion Disorder
Conversion disorder is a psychiatric term that indicates an unconscious loss of neurologic function (e.g., visual loss) for secondary gain, which is also unconscious. For example, a child may believe that he or she cannot see. The gain is that the child no longer has to go to school, where he or she may be experiencing intolerable conflict with the teacher or harassment by students. Children with conversion reactions are more likely to be girls.
A conversion symptom manifests as a disturbance of bodily functioning that does not correspond to concepts of the anatomy of the pathways of the central or peripheral nervous system.148 Generally, it occurs in the setting of psychological stress and produces considerable impairment. Although conversion reactions may simulate neurological disease, they are not associated with the usual pathological neurodiagnostic signs, but instead, the signs and symptoms correspond to the child’s concept of the medical condition. A conversion reaction transforms psychic energy from the turmoil of an acute conflict into somatic symptoms and sometimes leaves the child calm (la belle indifference).
Some forms of psychogenic visual loss may represent a conversion reaction to a previous experience of sexual abuse, in which the visual loss may a signal that the child has seen something inadmissible or unacceptable.30 Sexual abuse as a cause of psychogenic visual loss is uncommon but well recognized;30,236 the prevalence of sexual abuse as a precipitant of psychogenic visual loss has not been studied. If a history of sexual abuse is elicited, psychiatric evaluation is warranted.
Group 3: Possible Factitious Disorder
Parents of children with psychogenic visual loss occasionally display behavior that is reminiscent of a factitious disorder by proxy. This disorder (sometimes called Munchausen syndrome by proxy) refers to the intentional production or feigning of physical or psychiatric signs or symptoms in another person who is under the individual’s care for the purpose of indirectly assuming the sick role.13 This possibility should be considered when one or both parents seem overly invested in the child’s disability and appear to be actively driving the symptom. At an unconscious level, the child cooperates with the parents and may come to share the belief.352 The parents may become hostile and sometimes violent when the physician suggests that the visual loss is nonorganic, and “sabotage” the physician’s reassurances by telling the child that the doctor does not believe the symptoms are real. These parents often refuse psychiatric consultation and fail to return for follow-up appointments.
Group 4: Psychogenic Visual Loss Superimposed on True Organic Disease
Several studies have noted that approximately one-fourth of children with conversion symptoms have true organic disease.189,236,324,354 In these children, the psychogenic component can conceal or distract from a true organic visual loss. Such children have organically decreased vision as the cause of their symptoms, and in trying to bring attention to the problem, they exaggerate it to the point at which the symptoms appear nonorganic. Visual symptoms that are long-standing, progressive, and relatively nonfluctuating should arouse suspicion of organicity.324 An organic etiology is also suggested if the child complains of symptoms while engaged in activities he particularly enjoys (e.g., sports). Long-term follow-up of children with psychogenic visual loss is important to detect the subgroup with true organic disorders.189,236,324,354
Management of Psychogenic Visual Loss
in Children
Interview with the Parents
Many children with psychogenic visual loss see several ophthalmologists and/or neurologists before the psychogenic nature of the symptoms becomes evident. The parents have frequently consulted numerous health care professionals and incurred a large medical bill. Parental anxiety induced by the child, whose vision seems to be declining, intensifies with successive consultations and tests.323 In this context, the process of informing the parents that there is no organic basis for the symptoms becomes a delicate matter.
Prior to discussing the psychogenic nature of the visual symptoms with the parents, we read the hospital chart for social work notes pertaining to previous psychologically traumatic events, such as sexual abuse. The child is then asked to sit outside, and the parents are invited into the examining room. The parents are informed that the eyes are physically normal and that we believe the child’s vision is decreased on a psychological rather than a physical basis. The parents are reassured that psychogenic visual loss is common in children who have high expectations of themselves. We emphasize that the child is concerned about his or her vision, and that this concern is interfering with the child’s ability to see normally.
We explain that the child’s vision is truly impaired on a psychological basis. In explaining this, it is helpful to draw an analogy to the adult who develops real headaches or muscle tension from stress. One should inquire about the child’s previous school performance and whether it has deteriorated since the symptoms began. One should also inquire about
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possible stressors, such as family discord, divorce, or a death in the family, and attempt to determine whether other psychologically traumatic events have taken place. Parents can be told that their child’s visual impairment can be expected to resolve with time. We generally advise parents to de-emphasize the symptomatology by not discussing the child’s visual difficulties and by urging the child’s relatives and teachers to do the same, although some have questioned the efficacy of this approach.55
Interview with the Child
It is counterproductive to tell a child with psychogenic visual loss that his or her vision is normal. The child has teachers, relatives, and friends, who are concerned about his or her visual difficulties. If one “confronts” the child about the absence of evidence of a visual disorder, he or she has little choice but to claim that the symptoms are real.
Notwithstanding whatever secondary gains are present, the child may be searching unconsciously for a path to recovery. According to Rabinowicz,323 “the purpose of the apparent visual loss may have already been served, and the child is often more than ready for recovery. However, a rapid and ‘miraculous’ cure in the physician’s office is likely to provoke rage from the parents, dismay from the school authorities, and sadness, disappointment, and resentment, together with a feeling of having been deceived from the child’s own teacher and friends,” thus stigmatizing the child.
Because children are suggestible, psychogenic visual loss in children is usually a “curable” condition.262 Because these children rarely have serious psychopathology, some authorities feel justified in using placebo therapy to take advantage of the child’s suggestibility.323 This approach may be efficacious, but we believe it is possible to achieve equally good results with patience and reassurance.
In most cases, the child is well-oriented, has normal thought processes (i.e., no hallucinations or delusions), and has a normal affective state. One can then reassure the child that he or she is having a minor visual disturbance but that the eyes are healthy.23 One can state that visual disturbances are common in children but that the vision will recover over several weeks. This reassurance permits the child to gradually experience improved vision while maintaining esteem with parents, teachers, and friends. A return appointment is scheduled for 2 months (which underscores the notion that there is no urgent physical disorder). On follow-up examination, the child usually claims to be relieved of symptoms and cheerfully demonstrates normal acuity.236 The issue of pediatric placebo treatment is highly controversial, with placebos recently marketed specifically for children.19 We dislike the use of placebo therapy for the treatment of psychogenic visual loss in children because it reinforces the notion that
a physical illness is the cause, and most mental health professionals oppose reinforcing the patient’s misperceptions.44
When to Refer Children with Psychogenic Visual Loss for Psychiatric Treatment
The issue of when to obtain psychiatric consultation for the child with psychogenic visual loss is controversial. Because psychiatric disturbances (anxiety, depression, attention-defi- cit hyperactivity disorder (ADHD)) and home and school stress occur commonly in children with psychogenic visual loss; an underlying psychiatric or psychosocial disturbance should be ruled out in children who present with psychogenic visual loss.376 Advocates for early psychiatric intervention believe that psychogenic visual loss should be viewed as a cry for help or a signal that indicates the child has seen or has experienced something disturbing or unacceptable,437 and that there is a possibility of sexual abuse.30 Other stressful events (e.g., marital discord, divorce, illness, death in the family, a poorly kept parental secret that allows the child to sense that something is terribly wrong) may also produce this reaction and be detrimental to the general well-being of the child. They stress that the child may be coping with a deep-rooted emotional conflict and may benefit from professional assistance. Given lack of formal psychiatric training and the time constraints of most ophthalmologists and neurologists, it may be difficult for such physicians to accurately determine which children need psychiatric counseling.
Proponents of limiting initial intervention to reassurance23,55,189,255,325 argue that it is counterproductive to react to psychogenic visual loss in children and point to the consistent efficacy of reassurance, the natural history of resolution, and the infrequent recurrence of such symptoms in children. Others stress that psychiatric intervention could stigmatize the child at school, and make it difficult for the child to face friends and teachers.323 Kathol et al189 point out that there is no hard evidence that a psychiatric referral would substantially hasten the child’s visual (or psychological) recovery.
In our experience, most children with psychogenic visual loss do not require psychiatric consultation because most fall into the benign group of visually preoccupied children that respond well to reassurance. Those who desire but do not need glasses and those who are found to have organically decreased vision with a psychogenic overlay do not generally require additional psychiatric intervention. We reserve psychiatric consultation for children who have (1) a history of previous psychogenic disturbances, (2) signs of a frank mental disorder, (3) significant impairment in daily functioning at school or at home, (4) a history of psychic trauma (e.g., sexual abuse or otherwise), (5) a grossly dysfunctional family, (6) signs of factitious disorder by proxy (Munchausen syndrome by proxy), or (7) a history of
