Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
33 Cards in this Set
- Front
- Back
|
scaphocephaly
|
boat shaped skull
Frontal bossing, prominent occiput, palpable keel-like sagittal ridge close skull in the womb sagittal Craniosynostosis (CSO) 80% male most common CSO |
|
|
Coronal Craniosynostosis (CSO)
|
Accounts for about 20% of craniosynostoses
More common in females Can be unilateral or bilateral |
|
|
plagiocephaly
|
Unilateral Coronal CSO
The forehead on the affected side is flattened or may be concave above the eye |
|
|
brachycephaly
|
Bilateral results in brachycephaly (broad flattened forehead)
Coronal CSO |
|
|
Crouzon’s Disease
|
Coronal CSO with abnormalities of the sphenoid, orbital, and facial bones
|
|
|
Apert’s Syndrome
|
Multiple CSOs with syndactyly
|
|
|
Lissencephaly
|
Disorders of Migration
Maldevelopment of cerebral convolutions Likely due to arrest of cortical development at an early fetal age |
|
|
Agyria
|
Surface of the brain is smooth
Disorders of Migration |
|
|
Pachygyria
|
Thickened gyri
Reduced in number Disorders of Migration |
|
|
Polymicrogyria
|
Small gyri
Increased in number Disorders of Migration |
|
|
Heterotopia
|
Islands (blobs) of neuronal tissue where they don’t belong
Commonly associated with epilepsy Disorders of Migration |
|
|
Anancephlay
|
Neural Tube Defects
Due to failure of fusion of the anterior neuropore Neither the cranial vault nor the scalp covers the malformed brain tissue Universally fatal |
|
|
Encephalocele
|
Neural Tube Defects
Herniation of meningeal and central nervous system tissue through a defect in the skull |
|
|
Spina Bifida
|
Result from failure of the posterior vertebral arch to fuse
Most commonly occur in lumbosacral region If meninges bulge through defect can result in a meningocele or myelomeningocele May be associated with caudal displacement of medulla and cerebellum Can result in hydrocephalus |
|
|
Meningocele
|
does not contain spinal cord elements
|
|
|
Of patients with a meningomyelocele
|
contains spinal cord and nerve roots
Of patients with a meningomyelocele 1/3 have complete paralysis and loss of sensation below the level of the defect 1/3 have preservation of distal segments below the level of the defect 1/3 have an incomplete lesion 90% of children develop urinary problems |
|
|
Clinical features of Spina bifida
|
Occurs in 2-3 per 1000 live births
Can be detected prenatally by increased serum alpha-fetoprotein Spinal defect is clinically obvious Can result in various degrees of: Limb weakness Sensory loss Joint dislocation and contractures Urinary disorders |
|
|
Spina Bifida Occulta
|
Most common form of spina bifida
True prevalence is unclear Isolated laminar defects are seen on about 5% of lumbar spine x-rays The spinal cord is usually normal Only clinical sign is often a tuft of hair or dimple at the site of the defect Neurological deficit is rare May present with subtle neurological abnormalities such as enuresis or incontinence |
|
|
Arachnoid Cyst
|
Often referred to as leptomeningeal cyst
Arise during development from splitting of arachnoid membrane Two histological types “simple”: lined with cells that are capable of secreting cerebral spinal fluid Cysts with more complex lining (neuroglia, ependyma, etc…) Commonly found incidentally on CT or MRI If symptomatic Seizures Increased intracranial pressure Focal signs of space occupying lesion Hydrocephalus Sudden deterioration due hemorrhage into cyst or cystic rupture |
|
|
Porencephalic Cyst
|
Cystic lesion lined with glial or connective tissue that communicates with the ventricular system
Usually due to trauma or vascular event |
|
|
Hydranencephaly
|
Large portions of the cerebrum replaced by cerebrospinal fluid
Due to destructive process such as ischemic infarcts or infection |
|
|
Hydocephalus
|
Due to accumulation of cerebrospinal fluid (CSF)
Obstruction of CSF flow Decreased absorption Rapid deteroriation, in hours. Pluming procedure. Ventricular peritoneum shunt. |
|
|
Hydrocephalus
|
In infants and children, monitor head circumference
If due to obstruction, rapid deterioration may occur Treatment is shunt placement |
|
|
Type I
Chiari Malformation |
AKA primary cerebellar ectopia
Caudal displacement of the cerebellum with herniation of the tonsils below the foramen magnum Can be associated with syringomyelia |
|
|
Clinical presentation (Type I)
Chiari Malformation |
Average age at presentation about 40
Most common symptom is pain (70%) Usually in the suboccipital region Other signs/symptoms Weakness Lhermitte’s sign Upper motor neuron signs Spasticity |
|
|
Type 2
Chiari Malformation |
Caudally dislocated cervicomedullary junction, pons, fourth ventricle, and medulla
Brainstem and lower cranial nerve dysfunction Possible hydrocephalus Rarely presents in adulthood In neonates, there can be rapid neurological deterioration Apnea, swallowing difficulties, stridor, aspiration, pooling of secretions, limb weakness |
|
|
Chiari Malformation treatment
|
Suboccipital decompression
Shunt for hydrocephalus |
|
|
Dandy-Walker Malformation
|
Due to atresia of foramina of Magendie and Luschka
Results in agenesis (no development) of the cerebellar vermis with a large posterior fossa cyst that communicates with an enlarged fourth ventricle Hydrocephalus occurs in 90% of cases Usually associated with some degree of mental retardation, poor fine motor skills, ataxia, and spasticity |
|
|
Agenesis of the Corpus Callosum
|
Forms from rostrum (genu) to splenium
May be complete or incomplete May be found incidentally and may be of no clinical significance Can be part of complex of malformations (for example, Aicardi Syndrome: agenesis of CC, seizures, retardation, retinal pigment abnormalities) |
|
|
Syringomyelia
|
A cystic cavitation of the spinal cord
If it extends into the brainstem it is called a syringobulbia Communicating: primary dilitation of the central canal of the spinal cord Most common type associated with Chiari malformation Noncommunicating: arises in the cord substance and does not communicate with the central canal of the spinal cord. |
|
|
Tethered Cord
|
Abnormally low conus medullaris associated with a thickened filum terminale
May also be associated with an intradural lipoma Commonly seen with myelomeningocele Most cases present in childhood Progressive scloiosis Foot deformities (club foot) Gait problems (regression) Urinary problems (dribbling, incontinence) Cutaneous stigmata of dysraphism (tuft of hair,dimple) Back and lower extremity pain Adulthood presentation is very rare Significant perianal and perineal shock-like pain Urinary problems |
|
|
Split Cord-Type 1(diastematomyelia)
|
Two hemicords in their own dural tube separated by a bony medium septum
Associated with abnormalities at the level of the split Absent disc, tuft of hair |
|
|
Split Cord-Type 2 (diplomyelia)
|
Two hemicords within a single dural tube separated by a fibrous median septum
Usually no abnormalities at the level of the split, but most affected individuals have spina bifida occulta in the lumbosacral region |
