Cvj Anomalies Flashcards
(27 cards)
Cvj components
Occipital bone with opening - foramen Magnum
Axis
Atlas
Cvj anomaly definition
Anomaly results due to malrelation of base of occiput with atlas c1, axis c2, odontoid process of axis (dens), their articulation facets and ligaments.
Itself doesn’t cause any neurological lesion but increases the chance of basilar invagination
Classification of cvj anomalies
Skeletal
Neuraxial
Combined
Skeletal anomalies
Platybasia
Klippel feil anomaly
Occipitalisation of atlas
AAD
Neuraxial
Arnold chiari
Dandy walker
Posterior fossa cysts
Occipitocervical meningomyelocoele
Platybasia
Flattening of base of skull
Angle at base of skull >135°
Angle between lines:
1. Line joining nasion to centre of Pit Fossa (copf)
2. Line from copf to basion/Ant lip of foramen magnum
Normal 115-135
Causes of cvj anomaly
Congenital
Craniofacial anomalies
OI
Arnold chiari
Acquired
Pagets
Rickets
Osteomalacia
Basilar invagination/impression
Foramen magnum and adjacent skull base pushed cephalad such that they invaginate into post cranial fossa
Causes of BI
Mostly congenital
Acquired
Rickets
OM
Pagets
Achondroplasia
MPS
Neoplastic
Hypothyroid
Hyperparathyroid
Clinical features of BI
Any age
Symptomatic/asymptomatic
Occipital headache
Hyperalgesia along C2 distribution
Cerebellar # ataxia, tremors, incordination, nystagmus, scanning speech
Corticospinal tract # Quadriparesis, spasticity
Lower CN #
Bulbar # diplopia, dysphagia, vertigo, drop attacks
Klippel feil anomaly
Congenital fusion of cervical vertebra
Can involve 2 Segments or entire cervical spine
Clinical features of KF
Feils triad
Low posterior hairline
Short neck
Limited neck movements
Associated disorders of KF
Scoliosis/kyphosis 60% mc
Urinary tract (absent kidney, hun)
Hearing loss (microtia, absent eac)
CHD VSD
Synkinesia
Springel deformity - high scapula
Facial asymmetry
Torticollis
Webbed neck
Short neck
Birds index = height/distance from EOP to C7 spine
Normal < 12.8
Short neck if > 13.6
Low posterior hairline
If hairline below C5
Or
Ratio of distance of EOP to post hairline/post hairline to C7 is
Men > 1/6
Female > 1/4
C2-c3
C5-c6
Inheritance
C2-c3 AD
C5-c6 AR
Feils classification
1 fusion of many cervical and upper thoracic vertebrae
2 fusion of only 1-2 vertebrae
3 a/w anomalies in lower thoracic and upper lumbar spine
4 sacral agenesis
Diagnosis
Cervical xray f/b flexion/extension bilateral x rays
MRI - head flexed/extended - more sensitive
Occipitalisation of atlas/atlantooccipital synostosis
Partial/complete fusion of ring of atlas to base of occiput
Anterior arch to clivus
Post arch to occiput
Clinical features of OOA/AOS
Torticollis
Short neck
Low hairline
ROM in neck
Appear in 2nd decade due to increase laxity of ligaments and instability with aging
Appear suddenly, ppt by minor trauma
AAD classification
IA normal odontoid with occipitalisation of atlas
IB normal odontoid without occipitalisation
II detachment of odontoid from body of axis, agenesis of part or whole of odontoid
Dx - atlas-dens interval > 5mm
Causes of AAD
Acquired
Trauma
TB
RA
AS
Downs
Morqiuo
Clinical features of AAD
Following minor trauma
Pain or tenderness in AAX
Reduced rotation of head
Spastic quadriparesis
Low post column sensation
No cranial nerve involvement
Mechanism of neurological symptoms by cvj anomalies
1 abnormalities of bone, soft tissue cause direct pressure on medulla or cervical cord
2. Associated developmental anomalies if CNS independent of bone anomalies
3. Increased ICT secondary to impaired flow of CSF
