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CVJ ANATOMY-

CLASSIFICATION&
CLINICAL SIGNS

DR NILESH JAIN
23.06.2007
THE TERM ‘CV JUNCTION’ REFERS
TO THE OCCIPITAL BONE
THAT SURROUNDS THE FORAMEN MAGNUM
AND THE ATLAS AND
THE AXIS VERTEBRAE
HISTORICAL ASPECT

CHAMBERLAIN , 1939 : BASILAR INVAGINATION

GREENBERG , 1968 : CLASSIFIED ATLANTOAXIAL


ANOMALIES

BELL , 1830 : FIRST DESCRIBED SPONTANEOUS


ATLANTOAXIAL DISLOCATION

MECKEL , 1815 : MANIFESTATION OF OCCIPITAL


VERTEBRAE
History

 1886 – Giacomini described the first


case of congenital AAD
 1960 – Wadia-congenital AADs
 1968 – Greenberg – classification of
AADs
CV jn -Embryology

 Develops from the 4 occipital and upper 2 cervical somites.

The mesoderm caudal to neural plate condense into four occipital

somites, these are the precursors of ocipital sclerotomes.

First Two - Basiocciput

Third - Jugular tubercles

* Fourth occipital sclerotome



Proatlas
Proatlas

Hypocentrum Centrum Neural arch

Ant tubercle of the Apex of the dens Ventral Dorsal


clivus & Apical ligament Rostral Caudal
First spinal sclerotome
Atlas vertebra primarily formed from this sclerotome.
Sclerotome division

Hypocentrum Centrum Neural Arch

Anterior arch C1 Dens Inferior portion of


(mid portion the posterior arch
Of the odontoid
process and
fused with axis
Second spinal sclerotome

Develops into axis vertebra

Sclerotome division

Hypocentrum Centrum Neural Arch

Disappears Body of axis Facets &


Posterior arch of axis
Surgical anatomy
 Constituents of CV jn
 Osseous components and their articulations
 Ligamento-muscular elements
 Neuro-vascular structures
 Characteristics of CV jn
 Mobility at the cost of stability
 Constantly changing structure and kinematics –
even in the post natal period
 Vital neuro-vascular relations
Atlas
* Named after the mythical giant who carried the earth on his shoulder.
* Thin Anterior and posterior arches
•Sturdy Lateral masses – made up of a column of superior and inferior
articular facets placed in a vertical line
•No body
Axis:
Forms the axis of rotation
Dens is a divorced body of C1
Bifid spinous process
Inferior facet more posterior than superior facet
External craniovertebral ligaments
Internal Craniovertebral Ligaments
LIGAMENTS OF CVJ
POSTERIOR
- POST. ATLANTOOCCIPITAL MEMBRANE:
EXTENDS FROM OCCIPITAL BONE TO
POST. ARCH OF ATLAS
SHARP & THIN & DIRECT CONTACT
WITH ANT. CORTEX OF POST. ARCH OF
ATLAS
ANTERIOR LIGAMENTS
ANTERIOR LONGITUDINAL
LIGAMENT EXTENDING
FROM LOWER BORDER OF
ANT.ARCH C1 TO BODY
OF AXIS

ANTERIOR ATLANTO
-OCCIPITAL MEMBRANE
EXTENDS FROM
ANT.EDGE OF FM TO
ANT.ARCH C1
ANTERIOR LIGAMENTS

 TECTORIAL MEMBRANE
CEPHALIC EXTENSION
OF PLL
INSERTED INTO
PROCESSUS BASILARIS
1 –2 cm ABOVE BASION
CRUCIATE LIGAMENT
 OCCIPITOTRANSVERSE
LIGAMENT: TO BASION
 INFERIOR LOGITUDINAL
BAND: TO AXIS BODY
 TRUE TRANSVERSE
LIGAMENT
TRUE TRANSVERSE LIGAMENT
 STRONG HORIZONTAL
PORTION
 MAINTAINS THE
POSITION OF DENS IN
SAGITTAL &
CRANIOCAUDAL
DIRECTION
 ARTICULATES WITH
ODONTOID FACET
 INSERTED LATERALLY
IN BONY PROMINENCE
IN INNER
ASPECT OF CONDYLES
 IT IS 8mm IN HEIGHT
AND 2-3 MM THICK IN
MIDLINE
ANTERIOR LIGAMENTS
 BARKOWS LIG- FROM TIP
OF DENS TO ANT.LAT. FM
RIM
 APICAL LIG- TIP OF DENS
TIP OF DENS TO MIDDLE
PART OF FM RIM
 GRUBERS LIG- TRANSVERSE
LIG TO TIP OF DENS
ALAR LIGAMENT-
 VERY STRONG
LIGAMENT
 6 – 8 mm IN

DIAMETER
 DENS TIP TO

LATERAL PART OF RIM

OF FM
BLOOD SUPPLY
VERTIBRAL ARTERIES - ANT.
& POST. ASCENDING a.
CAROTID ARTERY : ANT.
ASCENDING a.
FORMS AN APICAL ARTERIAL
ARCADE IN THE REGION OF
ALAR LIGAMENT & SEND
PERFORATORS

VENOUS DRAINAGE :
PERIODONTAL VENOUS
PLEXUS &
SUBOCCIPITAL
EPIDURAL SINUS DRAIN TO
PHARYNGOVERTIBRAL
VEINS
LYMPHATIC DRAINAGE :

CV JUNCTION DRAINS TO RETROPHARYNGEAL


LYMPH NODES & THENCE TO
DEEP JUGULAR CERVICAL CHAIN

RETROGRADE INFECTION OF CV JUNCTION FROM


PHARYNX , SINUSES & RETROPHARYNGEAL AREAS :
GRISEL’S SYNDROME
CLASSIFICATION OF
CRANIOVERTEBRAL
JUNCTION ANOMALIES
Classification of AAD
 Menezes classified CV jn anomalies
into two broad categories
 Developmental(Primary)
 Congenital and Acquired(Secondary)
 CV jn anomalies and AAD classifications
overlap
 I Congenital anomalies & malformations
of the cranio vertebral junction
 1.Manifestation of Occipital bone
 a.Clivus segmentations
 b.Remnants around FM.
 c.Atlas variants.
 d.Dens segmentation anomalies.
 2.Basilar invagination
 3.Condylar hypoplasia
 4.Assimilation of atlas
 B Malformation of atlas
 1.Assimilation of atlas.
 2.Atlantoaxial fusion
 3.Aplasia of atlas arches
 C.Malformation of axis
 1.Irregular atlantoaxial segmentation.
 2.Dens dysplasias
 a.Ossiculum terminale persistens.
 b.Os odontoideum
 c.Hypoplasia-aplasia
 3.Segmentation failure of C2-C3
 II Developmental & acquired
abnormalities of the CV Junction
 A.Abnormalities of Foramen magnum
 1.Sec. Basilar invagination (e.g.
Pagets disease, osteomalacia,renal
resistance rickets)
 2.Foraminal stenosis
(e.g.achondroplasia
 B.Atlantoaxial instability
 1.errors of metabolism(e.g Morquio s
disease)
 2.Downs syndrome.
 3.Infections(e.g Grisels syndrome)
 4.Inflammatory (e.g Rheumatoid
arthritis)
 5.Traumatic occipitoatlantal &
atlantoaxial dislocation;Os
odontoideum)

6.Tumors(neurofibromatosis,syringomy
elia)
 7.Miscellaneous(e.g fetal warfarin
syndrome,Conradis syndrome)
Greenberg’s Classification of AAD
I Incompetence of odontoid process
A. Congenital
1. Type I Separate odontoid: OS odontoideum
2. Type II Free apical segment: Ossiculum Terminale
3. Type III Agenesis of odontoid base
4. Type IV Agenesis of Apical segment
5. Type V Agenesis of odontoid process totally
B. Traumatic
1. Acute
2. Chronic
C. Infectious
e.g Tuberculosis
D. Tumors
1. Primary
2. Metastatic
II Incompetence of Transverse Atlantal ligament

A. Congenital:
1. Idiopathic
2. Mongolism

B. Traumatic
1. Acute - Rupture of TAL
2. Chronic - Assimilation of atlas
- Block vertebrae C2 & C3

C. Hyperaemic
1.Infection –Bacterial/viral(Grisel’s syndrome)/granulomatous
2.Rheumatoid arthritis
Wadia proposed the following classification

Group I: AAD with


* Occipitalization of atlas
* Fusion of C2, C3 vertebrae
* Odontoid process dislocated posteriorly
Group II: AAD with
* No occipitalization of atlas
* No Fusion of vertebrae
*Odontoid process dislocated because of its mal
development
Group III: AAD with
* No occipitalization
* No fusion of vertebrae
* Odontoid is normal in shape and size to body of the axis.
I & II are usually developmental and III is acquired
Other classifications
 Biomechanical
 Translatory
 Rotary
 Radiological
 Mobile
 Fixed
 Clinical
 Reducible
 Irreducible
Common Bony CV Junction
Anomalies
Basilar Invagination:
 The term Basilar Invagination was used
by Chamberlain in 1939 .
 This is a primary defect implying
prolapse of the vertebral column into
the skull at the base.
 Two types of Basilar invagination
 A Ventral:In this there is shortening of the
basiocciput so that clivus is short &
horizontally oriented thus displacing the FM
in an upward direction.
 B.Paramesial :The condylar hypoplasia may
be present so that clivus become dorsally
displaced into posterior fossa but may be of
normal length.
 BI is commonly associated with an
abnormal odontoid process invaginating
into posterior fossa which leads to
indentation on the pons,medulla or
cervicomedullary junctionin a ventral
manner.
 Chiari Malformation is associated with
BI in 25 – 30%cases.
OS Odontoideum

Definition - Dens has developed necessarily but has failed to fuse with body
of the axis.

Two types:
A. Orthotopic variety: OS lies in place of dens and moves with atlas and axis.
B. Dystopic variety: OS lies near the skull-base and moves with clivus with
which it may be fused.

Congenital Os odontoideum Traumatic Os Odontoidem


1. H/o Trauma - often present Always present
2. Site of specification - Usually between base of dens and
usually between the base body of the axis (below SAF)
with apical segment of the dens
(above supra articular facet)
3. Line of separation - Always smooth Acutely irregular and not corticated
and corticated
4. Associated cong. Anomaly - Absent
often present
AAD -Definition
 AAD is not a disease per se , rather it’s a
manifestation of a spectrum of pathological
states.
This is a condition in which the atlas(C1) slips over the axis(C2) in

the antero-posterior direction resulting in neural structure

compression between the two vertebrae.


 Bone & ligamentous structure help
stabilize the atlanto axial region.
 The transverse ligament is the most
important structure.
 Other structure involved are alar
ligament ,accessory atlanoaxial
ligaments & tectorial membrane.
 A distance of >3mm in an adult &
>4.5mm in a child between posterior
surface of ant. Arch of C1 & ant.
surface of dens is thought to be
incompetence of TL with associated
instability.
Clinical Presentation of CV
junction anomalies
 The most interesting feature of the clinical
presentation is the diversity.This is due to
compression of the lower brainstem,cervical
spinal cord,cranial nerves,cervical nerve
roots,& vascular supply.
 Presentation may be insidious,or as false
localizing sign, infrequently a rapid
neurological progression followed by death.
 The most common symptom is neck
pain originating in sub occipital region
with radiation to cranial vertex region
-85%.
 False localising signs:Usually motor
monoparesis,paraparesis,&
quadripresis.
Clinical features
GENERAL EXAM : Abnormal general apperance.

KLIPPEL- FEIL SYNDROME :Traid of Low posterior hair line, short

neck and limitation of neck movements

OTHER DYSPLASTIC FEATURES:

high arch palate

poly/syndactyly

pes cavus

scoliosis

sprengel shoulder,
Myelopathic features
 Motor deficits- legs more involved
 Cruciate paralysis
 Posterior tract symptoms- Lhermitte
sign
 Central cord syndrome
 Neck pain/ cough headcahe
Cranial nerve symptoms
 Lower cranial nerve paresis
 Hearing loss(most common)-25%
 Hypoglossal paralysis (Klaus 1969)
Brain stem/cerebellar signs
 Sleep apnea and dysphagia
 Nystagmus
 Gait ataxia
Vascular symptoms
 Syncope
 Vertigo
 Episodic paresis
 Transient visual loss.
 Due to vertebro basilar insufficiency
 Present in 15 – 25% of cases.
 The phenomenon of basilar migraine
affects about 25% of children with BI &
compression of the medulla.
 This is usually involves compression of
vertebro basilar arterial system.
THANK YOU

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