Development, Clinical features & Controversies in the Management of Atlanto-axial dislocations

Definition
AAD is not a disease per se , rather its 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.

History
1830 Bell described the first case of spontaneous rotary AAD 1857 Malgaigne attempted first classification( traumatic vs pathological) 1868 Blasius suggested all were traumatic 1886 Giacomini described the first case of congenital AAD 1890s 3 types of AAD(traumatic/congenital and spontaneous) 1964 Dastur&Bharucha-congenital AADs 1968 Greenberg classification of AADs

Embryology
Development starts following gastrulation(3rd week) Para-axial mesoderm undergoes segmentation into somites. 42 somite stage reached by 4th week. Subsequently a host of segmentation and fusion processes occur in a sequential and coordinated manner influenced by various signaling molecules. Overall genetic control

Embryology- CV jn development
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 clivus

Apex of the dens & Apical ligament

Ventral Rostral

Dorsal Caudal

First spinal sclerotome

Atlas vertebra primarily formed from this sclerotome.

Sclerotome division

Hypocentrum Anterior arch C1

Centrum

Neural Arch

Dens Inferior portion of (mid portion the posterior arch Of the odontoid process and fused with axis

Ossification of atlas * From three centres * Appear between 7th-9th IU Life * Two halves unite in the 3rd & 4th year * The anterior arch is cartilageous at birth * Lateral mass unites between 6th - 8th year

Second spinal sclerotome Develops into axis vertebra Sclerotome division

Hypocentrum Disappears

Centrum Body of axis

Neural Arch Facets & Posterior arch of axis

ODONTOID PROCESS

At birth - Odontoid process is separated from the body of the axis by a cartilagenous band

Vestigial disc
Neurocentral synchondrosis present at birth but disappear after 8 years of age. Tip of odontoid process is not ossified at birth Represented by a small ossification centre seen at 3 years of age called - Ossiculum terminale If fuses with remainder dens by the age of 12 years If fails to fuse with odontoid process Called Ossiculum terminale

Genetic control
Role of Hox and Pax genes However these genes are not specific and mutations in them can produce a host of developmental anomalies in addition to those of the CV junction.

Clinical Implications
Developmental defects can be
Segmentation defects Fusion anomalies Hypoplasia/aplasia Ankylosis

Co-existent neural defects

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

 Occiput

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 inferieor 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

Vascular and lymph drainage

Biomechanics
Most mobile portion of the axial skeleton * Motion is controlled by

- The geometry of the surfaces

- Ligaments and their elastic properties The range of motion of the occipito-atlanto-axial complex Complex Occipito-Atlantal Joint Type of Motion * Flexio-Extension * Lateral Bending * Axial Rotation * Flexio-Extension * Lateral Bending * Axial Rotation Degree of Motion 130-150 80 00 (Negligible) 100 00 370 - 420(Negligible)

Atlanto-axial Joint

Coupling
* Axial Rotation + Translation (Motion along an axis) * Occur at Atlanta axial joint because of facet articulation * Axial Rotation of the atlas around the axis upward and transverse motion of the dens in relation to the atlas with rotatary displacement of the atlas beyond 34 to 400 Produce excessive translation of the axis-dens process relative descent of the cervicomedullary junction (mis diagnosis of the chiari-I mulformation) * Lateral rotation * Upto 900 possible * First 20-30 - upper cervical spine * rest is achieve by lower cervical spine

 Role of ligaments
Transverse ligament
Strongest. Tensile strength double that of alar lig Failure leads to AAD

Alar ligament
Second line of defense. Role in Rotary AAD

Apical ligament Capsular ligaments

Role of muscles
Clamp like action of the dorsal cervical spinal musculature

In 1968 - Steel In a patient with incompetence TAL Alar ligaments - second line of defence

Rule of third

1/3 - For cord 1/3 - For odontoid process 1/3 - Space - represent a safe zone

Safe zone - After disruption of TAL

Odontoid process displacement occur in this safe zone without neurological deficit In Chronic cases - of AAD It exceeds the safe zone and enters the area of impending spinal cord compression At this point - The second line of defense has failed, No margin of safety present

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 Greenbergs classification Wadia Radiological Jains

Greenbergs Classification of AAD I Incompetence of odontoid process A. Congenital 1. Type I Separate odontoid: OS odontoiderm 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 Bacteria/viral(Grisels 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

Magnitude of the problem

In India wadia, Gaj singh etc In West

Clinical presentation
Variable. No characteristic syndrome. Anatomical basis
Neural compression Vascular occlusion Obstruction of CSF flow

Acute deterioration and vital center compromise Important to realize the dynamic and intermittent nature of instability CV jn markers

Radiology

Radiology of AAD

Note: F-E X-rays to be conducted by voluntary by the patient particularly those with neurological deficit. It should be careful supervised motion studied

Red AAD

General outline- AAD Management

Needs to be individualized Depends on
Etiology peculiarities and specific treatment Instability Reducibility (radiological and clinical) Associated soft tissue compression

Various procedures available

Aims of treatment
Relieve the symptoms Reverse and prevent neurological deficits Provide stability Prevent recurrence Maintain the normal anatomy and kinematics as far as possible.

General outline- AAD Management

Role of Orthoses Role of traction Ventral decompressive procedures Posterior decompression & stabilization

Cervical orthoses
Soft collar Philadelphia collar Minerva jacket Halo vest best immobilization

Traction

Anterior approaches

Posterior stabilization

Treatment AAD Reducible (Needs - stabilization) Fusion (Posterior) (A) Irreducible (Needs decompression)

Ventral compression Transoral decompression Stable (B) Unstable (C) (D) Posterior fusion

Dorsal compression Posterior decompression Stable (E)

Group A: Reducible anterior compression requires only posterior stabilization

Group B: Non Reducible anterior compression requires anterior decompression but not require stabilization

Group C: Non Reducible anterior compression requires anterior decompression with posterior stabilization

Group D: Non Reducible posterior compression requires posterior decompression with posterior stabilization

Some controversies
Fixed AAD with no neurological deficits
To operate or no

Fixed AAD with neurological deficits which improve on traction though radiologically still not reduced
To decompress anteriorly or no

No two pathological conditions are the same

Neither are two patients with the same disease

Kochs
Common in tropics.Resurgence with HIV. 1% of Spinal Kochs( itself ~ 1-5% of all Kochs) 3 stages (Lifeso) Instability may persist even in healed cases. Caution!!! Radiological indices may be difficult--obscuration of landmarks Need for tissue diagnosis???

Kochs-management
Aims Tissue diagnosis Decompression Correct instability Promote early and correct healing and fusion

ATT is mainstay Flowchart-algorithm(Behari et al)

 Behari et al(2003)- 25 pts CVJ Kochs 15 minor deficits and 10 severe. 14 of 15 with minor def treated conservatively( 1 PF done for red AAD). All 10 severe def operated after traction( 4 one stage ant+post surgery and 6 only PF). 24 improved with one death.1 redo reqd Sinha et al(2003)- 18 pts with Irred TB AAD. All transcervical decompression with PF(1stage).fusion at 3-6mths. All improved. 2 redo reqd. Both studies gave 4-drug ATT Rajshekar et al(2002)- favoured peroral decompression in all cases. NIMHANS exp(Shukla et al..accepted for publication)differs. Individualize the treatment. Per-oral procedure can be

Rheumatoid Arthritis
Common in West Cervical spine inv in 59-80% (Menezes) Erosive synovitis with pannus formation. AAD very common due to ligament incompetence. Natural historystarts early, though may manifest later. Poor outlook with advanced myelopathy Risk of sudden acute medullary compression(Mickulowski et al, 1975)

RA
MRI essential soft tissue contribution to compression even though AAD is reducible Controversy regarding asymptomatic pts with AAD. Should all have anterior decompressive procedures.

RA with AAD

RA

RA-pre and post PF only

RA-complic after only PF

Developmental AAD
Diff b/w Congenital and Developmental Common in India Multiple associated anomalies
C1 assimilation Klippel Feil

Usually not reducible Either due to Odontoid failure or ligamentous incompetence (Greenberg)

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 1. H/o Trauma - often present 2. Site of specification usually between the base with apical segment of the dens (above supra articular facet) 3. Line of separation - Always smooth and corticated 4. Associated cong. Annomaly often present Traumatic Os Odontoidem Always present Usually between base of dens and body of the axis (below SAF)

Acutely irregular and not corticated Absent

Congenital
Refers to non-developmental genetic and metabolic diseases which may predispose to AAD Downs syndrome Morquios disease (Type of MPS) Achondroplasia Spondyloepiphyseal dysplasias

Traumatic

Neoplastic

Rare case- pseudogout with retrodental calcified mass

NIMHANS EXPERIENCE Per oral decompression only Per oral decompression with posterior fusion with contoured rod Posterior decompression with fusion with contoured rod Fusion of C1 and C2 with sublaminar wiring and graft 30

47

44

30

Total

151

NEUROLOGICAL OUTCOME
Post op status Per oral (30) Per oral + fusion (47) Only Fusion (44) C1-C2 fusion (30) Improvement 20 40 Status Quo 5 5 Deteriorated 5 2

35 20

7 5

2 5

COMPLICATIONS
Complications Wound infection Dural tear 3 2 Meningitis Miscellaneous

Per oral Per oral + fusion Occipito cervical Fusion only C1- C2 fusion

2 2

2 1

1 1

5 3

2 6

4 -

1 6

MORTALITY

Mortality Per Oral Per oral + fusion Fusion only C1-C2 fusion

Post Op 3 2 3 -

During F/U 2 3 1 3