- •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
Cerebral Dysgenesis and Intracranial Malformations |
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Fig. 11.27 Basic patterns of brain destruction. (a) porencephaly; (b) hydranencephaly; (c) encephalomalacia; (d) colpocephaly
often, septations in the area of damaged brain (Fig. 11.27).47 MR imaging shows reactive gliomas and tissue injury that are dark on T1-weighted images and bright on T2-weighted images. These findings signify injury to the mature brain, which has acquired the capacity to react to injury with significant astroglial proliferation. Neuro-ophthalmologically, encephalomalacia most often correlates with cortical visual insufficiency associated with hypoxic–ischemic injury to the visual system at term.
Colpocephaly
Colpocephaly was once considered to be a distinct anatomic finding in the brain manifested by occipital horns that are disproportionately enlarged when compared with other parts of the lateral ventricles (Fig. 11.27).389 However, it is really a descriptive term with no inherent diagnostic significance. Colpocephaly is most often seen in association with agenesis of the corpus callosum, and can accompany a variety of degen-
