Cranio-Vertebral Cranio-Vertebral AnomaliesAnomalies

DR. ANKUR NANDAN VARSHNEYDR. ANKUR NANDAN VARSHNEY

IMS, BHUIMS, BHU

VaranasiVaranasi

Dedicated to Prof. Dedicated to Prof. B.C. KatiyarB.C. Katiyar

ContentsContents

Classification of CV AnomaliesClassification of CV Anomalies Anatomical and radiological aspectsAnatomical and radiological aspects Specific anomalies – AA dislocation, Specific anomalies – AA dislocation,

Dens dysplasia, KFSDens dysplasia, KFS ACM, SyrinxACM, Syrinx Clinical featuresClinical features ManagementManagement

ClassificationClassificationI.I. Bony AnomaliesBony Anomalies

A.A. Major AnomaliesMajor Anomalies

1. Platybasia1. Platybasia

2. Occipitalization2. Occipitalization

3. Basilar 3. Basilar InvaginationInvagination

4. Dens Dysplasia4. Dens Dysplasia

5. Atlanto- axial 5. Atlanto- axial dis.dis.

B. Minor Anomalies1.Dysplasia of Atlas2.Dysplasia of occipital condyles, clivus, etc.

II. Soft Tissue anomalies1. Arnold-Chiari Malformation2. Syringomyelia/ Syringobulbia

Anatomical and Anatomical and Radiological AspectsRadiological Aspects

• Anatomical LandmarksAnatomical Landmarks• Nasion, Tuberculum Nasion, Tuberculum

Sellae, Clivus, Basion, Sellae, Clivus, Basion, Opisthion, IndionOpisthion, Indion

• Lines , angles and Lines , angles and indexesindexes

• Chamberlain’s LineChamberlain’s Line• McGregor’s LineMcGregor’s Line• McRae’s LineMcRae’s Line• Klaus Height IndexKlaus Height Index• AT IndexAT Index

•Basal Angle•Boogard’s angle•Bull’s angle•A-O interval•EDFM

Table : Lines and angles used in radiologic diagnosis of C.V anomalies.

Parameter Normal range limits

A. PLATYBASIA

B. BASILAR INVAGINATION

C. ATLANTO-AXIAL DISLOCATION *

• Basal angle < 150 degree

• Boogard’s angle < 136 degree

• Bull’s angle < 13 degree

• Chamberlain’s line < one third of odontoid above this line• Mcgregor’s line < 5 mm• Mcrae line odontoid lies below this• Klaus height index > 35 mm• Atlanto-temporo > 22mm. mandibular index

• Atlanto-odontoid space upto 3 mm in adults upto 5 mm in children• EDFM > 19mm

* May be reducible, partially reducible or irreducible

Atlanto-Axial DislocationAtlanto-Axial Dislocation– TraumaticTraumatic– Spontaneous (Hyperemic)Spontaneous (Hyperemic)– CongenitalCongenital

Wadia Classification (1973)Wadia Classification (1973)• Group 1- associated with occipitalization Group 1- associated with occipitalization

& frequent fusion of C2,C3& frequent fusion of C2,C3• Group 2- Associated with Dens Dysplasia- Group 2- Associated with Dens Dysplasia-

frequently totally reduciblefrequently totally reducible• Group 3- No Congenital Bony Group 3- No Congenital Bony

abnormalityabnormality

Type 1Type 1 : Syringomyelia with obstruction of Foramen Magnum : Syringomyelia with obstruction of Foramen Magnum and dilatation of central canal ( and dilatation of central canal ( Developmental TypeDevelopmental Type))

1.1. With type 1 Chiari malformationsWith type 1 Chiari malformations

2.2. With other obstructive lesion of foramen magnumWith other obstructive lesion of foramen magnum

Type 2Type 2 : Syringomyelia without obstruction of Foramen : Syringomyelia without obstruction of Foramen MagnumMagnum ( ( Idiopathic Developmental TypeIdiopathic Developmental Type))

Type 3Type 3: : Syringomyelia with other diseases of spinal cord Syringomyelia with other diseases of spinal cord ( ( Acquired Types Acquired Types ))

1.1. Spinal cord tumors ( usually intramedullary Spinal cord tumors ( usually intramedullary hemangioblastoma)hemangioblastoma)

2.2. Traumatic myelopathyTraumatic myelopathy

3.3. Spinal Arachdnoitis and pachymeningitis.Spinal Arachdnoitis and pachymeningitis.

4.4. Secondary myelomalacia from cord compression ( tumour , Secondary myelomalacia from cord compression ( tumour , spondylosis ) , infarction , hematomyeliaspondylosis ) , infarction , hematomyelia

Type 4Type 4 : Pure hydromyelia ( developmental dilatation of the : Pure hydromyelia ( developmental dilatation of the cental canal ) with or without hydocephalus.cental canal ) with or without hydocephalus.

Types of dens dysplasiaTypes of dens dysplasia

Type 1 (Os odontoideum) separate odontoid Type 1 (Os odontoideum) separate odontoid

processprocess

Type 2 (Ossiculum terminale) failure of fusion Type 2 (Ossiculum terminale) failure of fusion

of of . . apical segment with its baseapical segment with its base

Type 3 – Agenesis of odontoid base & apical Type 3 – Agenesis of odontoid base & apical

segment segment . . . . lies separately.lies separately.

Type 4 – Agenesis of odontoid apical segmentType 4 – Agenesis of odontoid apical segment

Type 5 –Total agenesis of odontoid process. Type 5 –Total agenesis of odontoid process.

Klippel- Feil SyndromeKlippel- Feil Syndrome

• Type 1Type 1- Massive fusion of cervical and - Massive fusion of cervical and often upper thoracic vertebra. often upper thoracic vertebra. Associated with short neck, low hairline Associated with short neck, low hairline and restricted neck movementsand restricted neck movements

• Type 2Type 2- associated with fusion of several - associated with fusion of several cervical vertebracervical vertebra

• Type 3Type 3 – associated with fusion of 2 – associated with fusion of 2 cervical vertebra.cervical vertebra.

Other associated anomalies:- Sprengel Other associated anomalies:- Sprengel shouldershoulder

Cervical canal stenosis in Cervical canal stenosis in CV anomaliesCV anomalies

Foramen Magnum: Foramen Magnum: 25-30mm25-30mm C1C1 25-30mm25-30mm C2C2 20-25mm20-25mm C3-C7C3-C7 ≥ 14mm≥ 14mm EDFM EDFM >19mm>19mm

Clinical manifestations of Clinical manifestations of CV anomaliesCV anomalies

Age Age SexSex Familial occurrenceFamilial occurrence Precipitating factorsPrecipitating factors Onset Onset

Presenting FeaturesPresenting Features

A.A. Cervical symptoms and signsCervical symptoms and signsB.B. Myelopathic Features- long tract Myelopathic Features- long tract

involvement and wastinginvolvement and wastingC.C. CN involvement- IX, X,XI,XII,V, AND CN involvement- IX, X,XI,XII,V, AND

rarely VIII,VIIrarely VIII,VIID.D. Cerebellar symptoms/signs- Nystagmus, Cerebellar symptoms/signs- Nystagmus,

Ataxia, intention tremor, dysarthriaAtaxia, intention tremor, dysarthriaE.E. Transient Attack of V-B insufficiencyTransient Attack of V-B insufficiencyF.F. Features of Raised ICT- usually seen in Features of Raised ICT- usually seen in

Pts. Having basilar impresssion and/or Pts. Having basilar impresssion and/or ACMACM

Importants Points to rememberImportants Points to remember

1.1. Short neck, low hairline, restricted neck Short neck, low hairline, restricted neck movements are frequently seen in KF movements are frequently seen in KF anomaly, Occipitalization, and basilar anomaly, Occipitalization, and basilar invaginationinvagination

2.2. Transient Attacks of VB insufficiency are Transient Attacks of VB insufficiency are usually encountered in Pts. With A-A usually encountered in Pts. With A-A dislocation.dislocation.

3.3. Several bony and soft tissue anomalies often Several bony and soft tissue anomalies often co-existco-exist

4. 4. Neurological deficit is usually produced by Neurological deficit is usually produced by A-A dislocation, Basilar Invagination, ACM A-A dislocation, Basilar Invagination, ACM and Syringomyelia / Syringobulbiaand Syringomyelia / Syringobulbia

Arnold-Chiari Arnold-Chiari MalformationMalformation

• Type 1Type 1- Cerebellomedullary malformation - Cerebellomedullary malformation without meningocoele with variable without meningocoele with variable downward displacement of cerebellar tonsils downward displacement of cerebellar tonsils into spinal canalinto spinal canal

• Type 2Type 2. Cerebello-medullary malformation . Cerebello-medullary malformation with meningocoele or meningomyelocoele with meningocoele or meningomyelocoele along with variable downward displacement along with variable downward displacement of inferior vermis of cerebellum, brainstem of inferior vermis of cerebellum, brainstem and IVth ventricle into cervical canaland IVth ventricle into cervical canal

ACM - Diagnosis

ACM DiagnosisACM Diagnosis

Syringomyelia/ Syringomyelia/ SyringobulbiaSyringobulbia

Progressive degenerative/ developmental disorder of Progressive degenerative/ developmental disorder of the spinal cord, characterized clinically by brachial the spinal cord, characterized clinically by brachial amyotrophy, suspended dissociative sensory loss, and amyotrophy, suspended dissociative sensory loss, and pathologically by cavitation in the central region of the pathologically by cavitation in the central region of the spinal cord. 90% cases associated with ACM type I.spinal cord. 90% cases associated with ACM type I.

Types I: Syrinx with obstn of the foramen magnumTypes I: Syrinx with obstn of the foramen magnum

Type II: Syrinx without obstn of the foramen magnumType II: Syrinx without obstn of the foramen magnum

Type III: Syrinx with other diseases of the spinal cord Type III: Syrinx with other diseases of the spinal cord

Type IV: Pure hydromyeliaType IV: Pure hydromyelia

Syringobulbia: the lower brainstem equivalent of Syringobulbia: the lower brainstem equivalent of syringomyelia, usually co-existssyringomyelia, usually co-exists

Syringomyelia - Syringomyelia - pathologicalpathological

Syringomyelia -clinicalSyringomyelia -clinical

Syringomyelia -Myelogram

Syringomyelia - MRISyringomyelia - MRI

Treatment of CV Treatment of CV anomaliesanomalies

1.1. Treatment of A-A dislocationTreatment of A-A dislocationa) Conservative treatment- For patients a) Conservative treatment- For patients

having only cervical symptoms or having only cervical symptoms or transient VB insufficiency with or without transient VB insufficiency with or without mild neurological deficit maybe initially mild neurological deficit maybe initially managed conservatively using –managed conservatively using –

1.1. Cervical CollarCervical Collar

2.2. Head- Halter Traction- if there is Head- Halter Traction- if there is associated myelopathic featuresassociated myelopathic features

b) Surgical Managementb) Surgical Management

Treatment of Basilar Treatment of Basilar Invagination and ACMInvagination and ACM

A.A. Conservative managementConservative management

B.B. Surgical treatmentSurgical treatmentA.A. Upper cervical laminectomy and Upper cervical laminectomy and

enlargement of Foramen Magnumenlargement of Foramen Magnum

Treatment of CV Treatment of CV anomaliesanomalies

THANK YOUTHANK YOU