Ch. 11 - Developmental Abnormalities Flashcards

1
Q

Arachnonid Cyst Definition

A

benign developmental cysts along craniospinal axis.

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2
Q

most common location of arachnoid cyst.

A

Sylvian fissure (50%) > cerebellopontine angle = quadrigeminal= supra sellar (10%) > other

location determines sx

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3
Q

sylvian fissure arachnoid cyst presentation

A

male > female. can be asymptomatic

classic: raised ICP and seizures
rare: rupture = focal neuro deficits

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4
Q

cerebellopontine angle arachnoid cyst presentation

A

sensorineural hearing loss, impairment of CNV, ataxia

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5
Q

suprasellar arachnoid cyst presentation

A

hydrocephalus, visual impairment, endocrine dysfxn

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6
Q

cerebral convexity arachnoid cyst presentation

A

seizures, HA, progressive hemiparesis

kids: asymmetrical enlargement of head

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7
Q

quadreminal arachnoid cyst presentation

A

mimic pineal masses. obstructive hydrocephalus and increased ICP

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8
Q

dx arachnoid cysts

A

CT/MRI, usually incidentalomas

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9
Q

tx arachnoid cyst

A
  1. nothing if asymptomatic/no obstruction, regular follow up

2. craniotomy and drainage vs shunting

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10
Q

chiari malformation types

A

type 1: caudal displacement of cerebellar tonsils below foramen magnum
type 2: caudal displacement of cerebellar vermis, 4th vent, and medulla
type 3: caudal displacement of cerebellum and brainstem

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11
Q

syringomyelia

A

cavitation w/i spinal cord but outside central canal

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12
Q

hydromyelia

A

dilation of central canal 2/2 CSF cannot exit @ foramen of lushka/magendie and are transmitted down central canal

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13
Q

chiari malformation common association

A

syringomyelia, hydrocephalus

uncommon: CV anomolies, imperforate anus

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14
Q

causes of hydrocephalus in chiari

A

aqueduct stenosis/atresia/forking, fusion of superior/inferior colliculi, compression in posterior fossa

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15
Q

theories behind chiari malformation

A
  1. tethered cord

2. differential pressure in intracranial/intraspinal fluid

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16
Q

Chiari I presentation

A

adolescent/adult-onset HA cape-like loss of pain/temp, long track signs (LE spasticity, UE paralysis), bulbar features 2/2 syrinx formation

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17
Q

Chiari II presentation

A

infant-onset. assoc. w/ myelomeningocele, progressive hydrocephalus, brainstem dysfxn (apnea, decreased gag, nystagmus, spastic paresis), adolescent/adult onset similar to chiari 1

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18
Q

How to dx chiari malformation

A

MRI - displacement of cerebellum into upper cervical canal

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19
Q

cause of hydromyelia

A

chiari malformations

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20
Q

tx chiari malformation

A
  1. posterior fossa and upper cervical decompression

2. shunt syrinx

21
Q

craniovertebral jxn abnormalities

A

jxn of foramen magnum, occipital bone and atlas (C1)/axis (C2) resulting in underlying neurologic compression

22
Q

basilar invagination

A

upward invagination of base of skull near foramen magnum into posterior fossa, shortening of clivus, odontoid protrusion

23
Q

causes of basilar invagination

A

Paget’s disease, osteomalacia, hyperPTH, Osteogenesis imperfecta

24
Q

presentation basilar invagination

A

quadriparesis, dysphagia, respiratory difficulty, nystagmus, occipital HA

25
Q

platybasia

A

obtuse basal angle joining the plain of the clivus with the plane of the anterior fossa

26
Q

atlantoaxial dislocation associated with…

A

fusion of occiput to atlas and fusion of C2-C3 causes adjacent joint dz and instability, rheumatoid arthritis, trauma

27
Q

Dandy Walker cyst

A

cystic enlargement of 4th vent, hypoplasia of cerebellum, hydrocephalus of 3rd and lateral ventricles

28
Q

dandy walker cyst presentation

A

infantile: hydrocephalus
childhood: ataxia, delayed motor development

29
Q

dandy walker cyst diagnosed via

A

CT/MRI

30
Q

Dandy walker cyst tx

A

shunting of cyst

31
Q

spinal dysraphism

A

incomplete or faulty closure of dorsal midline ambryologic structures ie (myelomeningocele, meningocele, lipomyelomeningocele, occult spinal dysaphism)

32
Q

spina bifida occulta

A

bony defect of lamina in lumbosacral spine, asx, 20% of adults

33
Q

most common spinal dysraphism

A

myelomeningocele: protrusion of neural elements through vertebral defect into meningeal lined sac

34
Q

myelomeningocele presentation

A

irreversible neuro deficit 2/2 spinal cord injury

35
Q

tx myelomeningocele

A

untether cord, reduce neural tissue into intervertebral canal

36
Q

lipomyelomeningocele

A

lipomatous tissue extends intradurally and interwoven with rootlets of cauda equina

37
Q

lipomyelomeningocele presentation

A

progressive neuro deficits (bowel/bladder dysfxn, back pain, progressive paralysis 2/2 tethered cord

38
Q

lipomyelomeningocele tx

A

surgical resection of tumor, untether spinal cord

39
Q

meningocele

A

cystic lesion lined by meninges and contains CSF, no neural tissue

40
Q

occult spinal dysraphism

A

lumbar spinal disorders that produce progressive neurologic dysfunction 2/2 tethered cord (intraspinal lipoma, dermoid tumors, diastematomyelia)

41
Q

most common cutaneous finding of occult spinal dysraphism

A

overlying skin lesions: dimple, sinus tract, fatty mass tuft of hair

42
Q

sx. occult spinal dysraphism

A

bowel and bladder disturbance, progressive weakness of legs/feet, back pain, sensory disability, progressive scoliosis

43
Q

diastematomyelia

A

spinal cord bifid –> progressive neurologic dysfunction

44
Q

craniosynostosis

A

premature closure of cranial sutures

45
Q

normal closure time for antereior and posterior fontanelles

A

anterior: 3-6 months, posterior 16-18 mo

46
Q

most common primary craniosynostosis

A

sagittal synostosis = head growth in occipitofrontal diameter = long narrow head
common in males

47
Q

cranial synostosis

A

head expands superiorly and laterally

48
Q

cranial synostosis SE

A

increased ICP

49
Q

cranial synostosis tx

A

correct cranial deformity, relieve effects of raised ICP