cv junction anomaly

AN INTERESTING AN INTERESTING CASE OF CASE OF

QUADRIPARESISQUADRIPARESIS

PROF. RAMASAMY’S UNITPROF. RAMASAMY’S UNIT

Dr.BetsyDr.Betsy

M 3M 3

• VELAN , 18 yr male VELAN , 18 yr male • C/O- difficulty in using limbs – 2 yrsC/O- difficulty in using limbs – 2 yrs• Difficulty in using lower limbs initially (2 Difficulty in using lower limbs initially (2

yrs) followed by difficulty in using upper yrs) followed by difficulty in using upper limbs (1 yr) : gradual in onset ; slowly limbs (1 yr) : gradual in onset ; slowly progressing , proximal & distal ; spastic progressing , proximal & distal ; spastic

• H/o restriction of neck movements H/o restriction of neck movements • No h/o s/o HMF / cranial nerve involvementNo h/o s/o HMF / cranial nerve involvement• No h/o s/o sensory or cerebellar No h/o s/o sensory or cerebellar

involvementinvolvement• No h/o bladder/ bowel involvementNo h/o bladder/ bowel involvement• No h/o LOC, seizures, feverNo h/o LOC, seizures, fever• No h/o traumaNo h/o trauma• No significant past h/o / family h/oNo significant past h/o / family h/o

• O/EO/EConscious and cooperativeConscious and cooperativeVitals stable , no pallorVitals stable , no pallorShort stature – 152 cm , moderately nourishedShort stature – 152 cm , moderately nourishedLow hairline , Height neck ratio- 15Low hairline , Height neck ratio- 15No webbing of neck / torticollis No webbing of neck / torticollis No neurocutaneous markersNo neurocutaneous markersHigher mental functions- NlHigher mental functions- NlCranial nerve examinaton – NlCranial nerve examinaton – NlMotor system- Motor system- No evident wastingNo evident wasting Hypertonia all 4 limbsHypertonia all 4 limbs No clonusNo clonus Muscle power –Muscle power – Upper limbs – 4 +/ 5 B/LUpper limbs – 4 +/ 5 B/L Lower limbs – 4 +/ 5 B/LLower limbs – 4 +/ 5 B/L

Reflexes – B/L DTR briskReflexes – B/L DTR brisk B/L plantar extensorB/L plantar extensorSensory – decreased below C 2Sensory – decreased below C 2Romberg’s sign –positiveRomberg’s sign –positiveCerebellum – normalCerebellum – normalNo involuntary movementsNo involuntary movementsSpastic gaitSpastic gaitNo signs of meningeal irritationNo signs of meningeal irritationFundus- normalFundus- normalSpine and cranium- movements of cervical Spine and cranium- movements of cervical

spine restrictedspine restrictedNo kyphosis/ scoliosisNo kyphosis/ scoliosis

• CARDIOVASCULAR SYSTEMCARDIOVASCULAR SYSTEM

Apical impulse in 5Apical impulse in 5thth LICS in MCL LICS in MCL

No thrill / parasternal heaveNo thrill / parasternal heave

S1 S2 +S1 S2 +

Pan systolic murmur grade 3/6 best Pan systolic murmur grade 3/6 best heard in the left lower sternal borderheard in the left lower sternal border

• RESPIRATORY SYSTEMRESPIRATORY SYSTEM

Within normal limitsWithin normal limits• GASTROINTESTINAL SYSTEMGASTROINTESTINAL SYSTEM

Within normal limitsWithin normal limits

PROVISIONAL PROVISIONAL DIAGNOSISDIAGNOSIS

Spastic quadriparesisSpastic quadriparesisLow hair lineLow hair lineShort neck Short neck

Restriction of neck movementsRestriction of neck movementsVSDVSD

CRANIOVERTEBRAL JUNCTION ANOMALY

INVESTIGATIONSINVESTIGATIONS

• Complete blood count – normalComplete blood count – normal• Renal Function test- normalRenal Function test- normal• Cardiology opinion:Cardiology opinion: Chest X- ray / ECG / Echo – Chest X- ray / ECG / Echo –

Ventricular septal defectVentricular septal defect• USG Abdomen – normalUSG Abdomen – normal• Audiogram – normalAudiogram – normal• Radiologic investigations –Radiologic investigations – Complex craniovertebral Complex craniovertebral

junction anomalyjunction anomaly

CRANIOVERTEBRAL CRANIOVERTEBRAL JUNCTION ANOMALIESJUNCTION ANOMALIES

CRANIOVERTEBRAL JUNCTIONCRANIOVERTEBRAL JUNCTION1.1. Foramen magnum Foramen magnum 2.2. Adjacent surrounding occipital bone Adjacent surrounding occipital bone

(basiocciput)(basiocciput)3.3. First cervical vertebrae (atlas)First cervical vertebrae (atlas)4.4. Second cervical vertebrae (axis)Second cervical vertebrae (axis)• Transition zone between a mobile cranium Transition zone between a mobile cranium

and relatively rigid spinal columnand relatively rigid spinal column• Site of craniospinal junctionSite of craniospinal junction Anomalies may be defects of development Anomalies may be defects of development

and not necessarily congenital and so may and not necessarily congenital and so may not manifest at birthnot manifest at birth

CLASSIFICATION OF CVJ CLASSIFICATION OF CVJ ANOMALIES ANOMALIES

1.1. CONGENITAL MALFORMATIONSCONGENITAL MALFORMATIONS Malformations of occipital bone Malformations of occipital bone

Malformations of atlasMalformations of atlas

•Basilar Basilar invaginationinvagination•Remnants around Remnants around foramen magnumforamen magnum

•Condylar Condylar hypoplasiahypoplasia•Clivus Clivus segmentationssegmentations

•Failure of atlas segmentation from Failure of atlas segmentation from occiput(assimilation)occiput(assimilation)

•Atlantoaxial fusionAtlantoaxial fusion•Aplasia or hypoplasia of atlas archesAplasia or hypoplasia of atlas arches

CVJ ANOMALIES CVJ ANOMALIES CLASSIFICN (CONTD)CLASSIFICN (CONTD)

Malformations of axisMalformations of axis• Atlantoaxial segmentation failureAtlantoaxial segmentation failure• Segmentation failure of C2-C3Segmentation failure of C2-C3• Dens dysplasias – os odontoideumDens dysplasias – os odontoideum

odontoid odontoid hypoplasia/aplasiahypoplasia/aplasia

ossiculum ossiculum terminale persistensterminale persistens


2.2. DEVELOPMENTAL AND DEVELOPMENTAL AND ACQUIRED ABNORMALITIESACQUIRED ABNORMALITIES

Foramen magnum abnormalitiesForamen magnum abnormalities• Foraminal stenosis (eg. Foraminal stenosis (eg.

achondroplasia ,MPS )achondroplasia ,MPS )• Secondary basilar invaginationSecondary basilar invagination

eg. Paget’s disease, osteomalacia, eg. Paget’s disease, osteomalacia, hyperparathyroidismhyperparathyroidism


ATLANTOAXIAL INSTABILITYATLANTOAXIAL INSTABILITY

•Errors of Errors of metabolism metabolism (Morquio’s (Morquio’s syndrome)syndrome)•Infections(Grisel’s Infections(Grisel’s synd)synd)•Traumatic Traumatic atlantoaxial / atlantoaxial / occipitoatlantal occipitoatlantal dislocationdislocation•Degenerative(ligaDegenerative(ligamentous disruption mentous disruption at CV junction)at CV junction)

•Inflammatory (RA, Inflammatory (RA, ankylosing ankylosing spondylitis)spondylitis)•Down’s syndromeDown’s syndrome•Tumours Tumours ( chordoma, ( chordoma, syringomyelia, NF )syringomyelia, NF )•Misc (fetal Misc (fetal warfarin syndrome, warfarin syndrome, Conradi’s synd)Conradi’s synd)

CLASSIFICATIONCLASSIFICATION

SKELETAL SKELETAL ANOMALIESANOMALIES

• PlatybasiaPlatybasia• Basilar invagination ( Basilar invagination (

110 0 / 2/ 20)0)

• Klippel-feil anomalyKlippel-feil anomaly• Occipitalisation of Occipitalisation of

atlasatlas• Atlanto-axial Atlanto-axial

dislocationdislocation

NEURAXIAL NEURAXIAL ANOMALIESANOMALIES

• Arnold Chiari Arnold Chiari malformationmalformation

• Dandy Walker Dandy Walker syndromesyndrome

• Occipito cervical Occipito cervical myelo myelo meningiocoelemeningiocoele

• Posterior fossa cystsPosterior fossa cysts

SIGNS AND SYMPTOMSSIGNS AND SYMPTOMS• Abnormal general physical appearance:Abnormal general physical appearance: Head may be cocked to one sideHead may be cocked to one side Short neckShort neck ScoliosisScoliosis• Neurological :Neurological : M.C- Posterior occipital headache that M.C- Posterior occipital headache that

worsens with neck flexion or extensionworsens with neck flexion or extension MyelopathyMyelopathy Brainstem and lower cranial nerve deficitsBrainstem and lower cranial nerve deficits• Vascular symptomsVascular symptoms Intermittent attacks of altered consciousnessIntermittent attacks of altered consciousness Confusion and transient loss of visual fieldsConfusion and transient loss of visual fields VertigoVertigo

KLIPPEL FEIL KLIPPEL FEIL SYNDROMESYNDROME

• Congenital fusion of cervical vertebraeCongenital fusion of cervical vertebrae• Failure of normal segmentation of the cervical Failure of normal segmentation of the cervical

vertebrae/somite between 3vertebrae/somite between 3rdrd and 8 and 8thth weeks of weeks of fetal development (rather than a secondary fetal development (rather than a secondary fusion) fusion)

• Maurice Klippel and Andre Feil – 1912Maurice Klippel and Andre Feil – 1912• Incidence – 1 in 42,000 births ; more in Incidence – 1 in 42,000 births ; more in

femalesfemales• Autosomal dominant inheritance – C2-C3 Autosomal dominant inheritance – C2-C3

fusion. Autosomal recessive – C5- C6 fusionfusion. Autosomal recessive – C5- C6 fusion• FEIL’S TRIAD :FEIL’S TRIAD :1.1. Low posterior hair lineLow posterior hair line2.2. Short neckShort neck3.3. Limitation of head and neck movements / Limitation of head and neck movements /

decreased range of motion in cervical spinedecreased range of motion in cervical spine

CLASSIFICATIONCLASSIFICATION

Feil’s classificationFeil’s classification• Type I – massive fusion of many cervical and Type I – massive fusion of many cervical and

upper thoracic vertebrae with synostosisupper thoracic vertebrae with synostosis• Type II – fusion of only 1 or 2 vertebrae (with Type II – fusion of only 1 or 2 vertebrae (with

hemivertebrae , scoliosis, occipito atlantoid hemivertebrae , scoliosis, occipito atlantoid fusion)fusion)

• Type III – presence of lower thoracic and upper Type III – presence of lower thoracic and upper lumbar spine anomalies with I/IIlumbar spine anomalies with I/II

• Type IV – sacral agenesisType IV – sacral agenesisSamartzis’s classification (2006)Samartzis’s classification (2006)To clarify prognosisTo clarify prognosis• Type I – single congenitally fused cervical Type I – single congenitally fused cervical

segmentsegment• Type II – multiple non-contiguous fused segmentsType II – multiple non-contiguous fused segments• Type III – multiple contiguous fused segmentsType III – multiple contiguous fused segments

CLINICAL FEATURESCLINICAL FEATURES

• Patients with upper cervical spine Patients with upper cervical spine involvement tend to present at an earlier involvement tend to present at an earlier age than those whose with lower cervical age than those whose with lower cervical spine involvementspine involvement

• Rotational loss and lateral bending is usually Rotational loss and lateral bending is usually more pronounced than loss of flexion and more pronounced than loss of flexion and extension because latter movements take extension because latter movements take place mostly between occiput and atlasplace mostly between occiput and atlas

• Scoliosis – some patients congenital due to Scoliosis – some patients congenital due to involvement of thoracic spine , others involvement of thoracic spine , others scoliosis compensatory to cervical scoliosisscoliosis compensatory to cervical scoliosis

CLINICAL FEATURESCLINICAL FEATURES

• Webbing of soft tissues on each side of Webbing of soft tissues on each side of the neck (extending from mastoid the neck (extending from mastoid process to acromion of shoulders)- process to acromion of shoulders)- ‘pterygium colli’‘pterygium colli’

• Assocd torticollis due to contracture of Assocd torticollis due to contracture of sternocleidomastoid muscle or bony sternocleidomastoid muscle or bony abnormalitiesabnormalities

• Facial asymmetryFacial asymmetry• Sprengel deformity/ high scapulaSprengel deformity/ high scapula• Scoliosis and/or kyphosis Scoliosis and/or kyphosis

CLINICAL FEATURES CLINICAL FEATURES CONTD..CONTD..

• Musculoskeletal sys- cervical rib, congenital Musculoskeletal sys- cervical rib, congenital fusion of ribs, abnormal costovertebral joints, fusion of ribs, abnormal costovertebral joints, syndactyly, hypoplastic thumb, supernumerary syndactyly, hypoplastic thumb, supernumerary digits, hypoplasia of pectoralis major, digits, hypoplasia of pectoralis major, hemiatrophy of upper or lower limbs, CTEV, hemiatrophy of upper or lower limbs, CTEV, sacral agenesissacral agenesis

• Urinary tract abnormalities – agenesis of Urinary tract abnormalities – agenesis of kidney, horseshoe kidney, hydronephrosis, kidney, horseshoe kidney, hydronephrosis, tubular ectasia, renal ectopia, double tubular ectasia, renal ectopia, double collecting systemcollecting system

• Cardiovascular- VSD, PDA, coarctation of Cardiovascular- VSD, PDA, coarctation of aorta, patent foramen ovaleaorta, patent foramen ovale

CLINICAL FEATURES CLINICAL FEATURES CONTD..CONTD..

• Deafness (absence of auditory canal Deafness (absence of auditory canal and microtia)and microtia)

• Synkinesia- involuntary paired Synkinesia- involuntary paired movements of the hand ( mirror movements of the hand ( mirror movements)movements)

• Neurologic deficit- facial nerve Neurologic deficit- facial nerve Palsy, rectus muscle palsy, ptosis of Palsy, rectus muscle palsy, ptosis of eye, cleft palate, etceye, cleft palate, etc

RADIOLOGICAL RADIOLOGICAL FINDINGSFINDINGS

• Cervical spine routine x-ray followed by Cervical spine routine x-ray followed by flexion/extension lateral X-rays. These may show flexion/extension lateral X-rays. These may show flattening and widening of vertebrae, hemivertebrae flattening and widening of vertebrae, hemivertebrae or block vertebrae, instability. or block vertebrae, instability.

• MRI with head flexed and extended will most MRI with head flexed and extended will most accurately access subluxation and cord compression accurately access subluxation and cord compression along with cord anomalies. along with cord anomalies.

• Wasp-waist sign- anterior concave indentation at the Wasp-waist sign- anterior concave indentation at the site of the absent or fused interspace between the site of the absent or fused interspace between the fused vertebrae. fused vertebrae.

• In the young child (<5y) the fusion is more apparent In the young child (<5y) the fusion is more apparent in the posterior elements. in the posterior elements.

• X-rays of the T-spine because of extension of X-rays of the T-spine because of extension of synostoses below the neck. synostoses below the neck.

ASSIMILATION OF ATLASASSIMILATION OF ATLAS OCCIPITALIZATION OF ATLAS/ ATLANTO-OCCIPITALIZATION OF ATLAS/ ATLANTO-

OCCIPITAL SYNOSTOSISOCCIPITAL SYNOSTOSIS• Failure of segmentation between the last occipital Failure of segmentation between the last occipital

sclerotome and the first spinal sclerotome – sclerotome and the first spinal sclerotome – partial/completepartial/complete

• Anterior arch to clivus : posterior arch to occiputAnterior arch to clivus : posterior arch to occiput• Signs and symptoms only in second decade of life due to Signs and symptoms only in second decade of life due to

gradual increasing ligament laxity and instability with gradual increasing ligament laxity and instability with agingaging

• Onset of clinical symptoms can be sudden and precipitated Onset of clinical symptoms can be sudden and precipitated by relatively minor trauma – loss of movt b/w atlas and by relatively minor trauma – loss of movt b/w atlas and occiput increases stress at atlanto axial joint => instabilityocciput increases stress at atlanto axial joint => instability

• Neurological symptoms due to Odontoid process projecting Neurological symptoms due to Odontoid process projecting into foramen magnuminto foramen magnum

• Assocd – basilar invagination, occipital vertebra, Klippel Assocd – basilar invagination, occipital vertebra, Klippel FeilFeil

• Radiology: cervical spine x-ray (flexion – extension ), CT, Radiology: cervical spine x-ray (flexion – extension ), CT, MRIMRI

• Treatment – non-operative / surgeryTreatment – non-operative / surgery

DENS DYSPLASIASDENS DYSPLASIAS

• OSSICULUM TERMINALE OSSICULUM TERMINALE PERSISTANSPERSISTANS

• OS ODONTOIDEUMOS ODONTOIDEUM

:Dystopic – fuses with the occipital :Dystopic – fuses with the occipital bone and moves with clivusbone and moves with clivus

:Orthotopic – lies in the position of :Orthotopic – lies in the position of normal dens and moves with atlas and normal dens and moves with atlas and axis vertebraeaxis vertebrae

• HYPOPLASIA/ AGENESIS OF DENSHYPOPLASIA/ AGENESIS OF DENS

ATLANTO AXIAL ATLANTO AXIAL DISLOCATIONDISLOCATION

• CLASSIFICATION:CLASSIFICATION:

Type I A- normal odontoid with Type I A- normal odontoid with occipitalisation of atlasoccipitalisation of atlas

Type I B- normal odontoid without Type I B- normal odontoid without occipitalisationoccipitalisation

Type II- detachment of odontoid from body Type II- detachment of odontoid from body of axis or agenesis of part or whole of densof axis or agenesis of part or whole of dens

• Diagnosis is radiological. Atlas-Dens interval Diagnosis is radiological. Atlas-Dens interval of more than 5 mm in children and more of more than 5 mm in children and more than 3 mm in adults is diagnosticthan 3 mm in adults is diagnostic


• Errors of metabolism (Morquio’s syndrome)Errors of metabolism (Morquio’s syndrome)• Infections(Grisel’s synd):Infections(Grisel’s synd):associated with

inflammation of adjacent soft tissues of the neck • Traumatic atlantoaxial / occipitoatlantal Traumatic atlantoaxial / occipitoatlantal

dislocationdislocation• Degenerative(ligamentous disruption at CV Degenerative(ligamentous disruption at CV

junction)junction)• Inflammatory (RA, ankylosing spondylitis): Inflammatory (RA, ankylosing spondylitis): From

laxity of the ligaments and destruction of the articular cartilage

• Down’s syndrome :dDown’s syndrome :due to laxity of transverse ligament

• Tumours ( chordoma, syringomyelia, NF )Tumours ( chordoma, syringomyelia, NF )• Misc (fetal warfarin syndrome, Conradi’s synd)Misc (fetal warfarin syndrome, Conradi’s synd)


The gap between the occipital condyles and the condylar surface of the atlas is widened to more than 5 mm.

BASILAR BASILAR INVAGINATIONINVAGINATION

• Primary developmental defectPrimary developmental defect• Deformation of the basiocciput – upward Deformation of the basiocciput – upward

indentation or invagination of base of skull indentation or invagination of base of skull into posterior fossa into posterior fossa

• Prolapse of the vertebral column upward into Prolapse of the vertebral column upward into the skull at the basethe skull at the base

BASILAR IMPRESSION – Acquired form of BASILAR IMPRESSION – Acquired form of basilar invagination secondary to softening of basilar invagination secondary to softening of skull Eg. Paget’s disease, OI, skull Eg. Paget’s disease, OI, hyperparathyroidism, osteomalacia, ricketshyperparathyroidism, osteomalacia, rickets

PLATYBASIA – flattening of the skull - PLATYBASIA – flattening of the skull - Abnormal obtuse basal angle formed by clivus Abnormal obtuse basal angle formed by clivus and floor of anterior fossa – if exceeds 145and floor of anterior fossa – if exceeds 14500

BASILAR INVAGINATION BASILAR INVAGINATION CONTD..CONTD..

• Two types- Two types- 1. Anterior / Ventral – shortening of 1. Anterior / Ventral – shortening of

basiocciput such that clivus is short, horizontally basiocciput such that clivus is short, horizontally oriented ; and displaces the plane of foramen oriented ; and displaces the plane of foramen magnum upward in relation to the spinal column. magnum upward in relation to the spinal column. Posterior fossa structures become crowded. Posterior fossa structures become crowded. Often assocd wuth platybasia Often assocd wuth platybasia

2. Paramedian – maldevelopment of occipital 2. Paramedian – maldevelopment of occipital bones and occipital condylar hypoplasia. Clivus bones and occipital condylar hypoplasia. Clivus dorsally displaced into posterior fossa and is of dorsally displaced into posterior fossa and is of normal length. Clivus invagination is normal length. Clivus invagination is compensated by excessive downward curvature compensated by excessive downward curvature of lateral squamous occipital bone.of lateral squamous occipital bone.

• Clinical features due to compression of Clinical features due to compression of neural structures at the neural structures at the cervicomedullary junctioncervicomedullary junction

• Suboccipital headache due to irritation of Suboccipital headache due to irritation of 22ndnd cranial nerve, lower cranial nerve cranial nerve, lower cranial nerve symptoms, cerebellar symptoms, cerebellar symptoms( nystagmus ), progressive symptoms( nystagmus ), progressive quadriparesisquadriparesis

• Often associated with other Often associated with other developmental bony anomalies developmental bony anomalies

CRANIOMETRIC LINESCRANIOMETRIC LINES• CHAMBERLAIN’S CHAMBERLAIN’S

LINELINE (Palato-occipital line)(Palato-occipital line) - joins posterior tip of - joins posterior tip of

hard palate to posterior hard palate to posterior rim of foramen magnumrim of foramen magnum

Normally tip of dens lies Normally tip of dens lies 3.6 mm below this line. 3.6 mm below this line. Upto one third of the Upto one third of the dens may be above this dens may be above this line.line.

In basilar invagination, In basilar invagination, the odontoid process the odontoid process bisects Chamberlain’s bisects Chamberlain’s lineline

Hard palate

ClivusOccipu

t

CRANIOMETRIC LINES CRANIOMETRIC LINES contd..contd..

•McRae’s LINEMcRae’s LINE(Foramen Magnum line)-(Foramen Magnum line)- Joins anterior and Joins anterior and posterior edges of posterior edges of foramen magnum: sagittal foramen magnum: sagittal diameter of foramen diameter of foramen magnum. (Avg – 35mm)magnum. (Avg – 35mm)Tip of dens must be below Tip of dens must be below this linethis lineIf effective sagittal space If effective sagittal space for cervicomedullary for cervicomedullary junction is less than 20 junction is less than 20 mm in a child older than 8 mm in a child older than 8 yrs, neurological deficit is yrs, neurological deficit is usually presentusually present


• MCGREGOR’S MCGREGOR’S LINE (Basal line)-LINE (Basal line)-

Joins hard palate to Joins hard palate to lowest point of lowest point of occipital boneoccipital bone

Tip of dens should not Tip of dens should not exceed 5 mm above exceed 5 mm above this linethis line

Basilar invaginationBasilar invaginationLine position may vary Line position may vary

with flexion and with flexion and extensionextension


• WACKENHEIM’S LINEWACKENHEIM’S LINE( Clivus canal line)( Clivus canal line)Line drawn along clivus Line drawn along clivus

and extrapolated into and extrapolated into cervical spinal canal. cervical spinal canal. Odontoid process should Odontoid process should be ventral or tangential be ventral or tangential to it.to it.

Odontoid process Odontoid process transects this line in transects this line in basilar invagination, basilar invagination, atlantoaxial dislocation atlantoaxial dislocation and anterior occipito and anterior occipito atlantal dislocationatlantal dislocation

Clivus

Odontoid process


• FISHGOLD’S DIGASTRIC LINEFISHGOLD’S DIGASTRIC LINE(Biventer line)- measured on frontal (Biventer line)- measured on frontal

projectionprojectionConnects the digastric grooves ( fossae for Connects the digastric grooves ( fossae for

digastric muscles on undersurface of skull digastric muscles on undersurface of skull just medial to mastoid process)just medial to mastoid process)

Line is normally 11mm (+/-4mm) above the Line is normally 11mm (+/-4mm) above the atlanto-occipital junction. atlanto-occipital junction.

Upper limit of position for the odontoid tipUpper limit of position for the odontoid tipOblique in unilateral condylar hypoplasiaOblique in unilateral condylar hypoplasia

• FISHGOLD’S BIMASTOID LINEFISHGOLD’S BIMASTOID LINEOdontoid process should be less than 10 mm Odontoid process should be less than 10 mm

above this lineabove this line


• HEIGHT INDEX OF KLAUSHEIGHT INDEX OF KLAUS

Distance between tip of dens and Distance between tip of dens and tuberculum cruciate linetuberculum cruciate line

Nl- 40-41mmNl- 40-41mm

In basilar invagination- In basilar invagination-

<30 mm<30 mm


• SPINOLAMELLAR LINESPINOLAMELLAR LINE(Spinous interlaminar line)- line drawn from (Spinous interlaminar line)- line drawn from

interoccipital ridge above and down along interoccipital ridge above and down along the fused spinous processes of C2 and C3the fused spinous processes of C2 and C3

Normally should intersect posterior arch of Normally should intersect posterior arch of atlasatlas

If atlas is fused, posterior arch is anterior to If atlas is fused, posterior arch is anterior to the line; posterior compression of spinal cord the line; posterior compression of spinal cord may occurmay occur

• SCHMIDT-FISCHER ANGLESCHMIDT-FISCHER ANGLEAngle of axes of atlanto-occipital jointsAngle of axes of atlanto-occipital jointsNormally 124-127 degreesNormally 124-127 degreesAngle is wider in condylar hypoplasiaAngle is wider in condylar hypoplasia

X RAY SKULL X RAY SKULL LATERAL LATERAL

VIEWVIEW

OPEN MOUTH X

RAY

ATLANTO OCCIPITAL FUSION

C5 C6 PARTIAL BLOCK VERTEBRA

OS ODONTOIDEUM

MRI BRAIN MRI BRAIN AND AND

CERVICAL CERVICAL SPINESPINE• Os odontoideum Os odontoideum

is notedis noted• No fluid No fluid

collection is notedcollection is noted• Wackenheim’s Wackenheim’s

clivus canal line clivus canal line is transected by is transected by odontoid process- odontoid process- BASILAR BASILAR INVAGINATIONINVAGINATION

• PLATYBASIAPLATYBASIA

MRI BRAIN MRI BRAIN AND CERVICAL AND CERVICAL

SPINESPINE• Chamberlain’s line Chamberlain’s line

bisected by odontoid bisected by odontoid process – process –

• BASILAR BASILAR INVAGINATIONINVAGINATION

• Gross narrowing of Gross narrowing of spinal canal at C1-C2 spinal canal at C1-C2 level, causing spinal level, causing spinal cord compression. Mid cord compression. Mid sagittal AP diameter of sagittal AP diameter of spinal canal at this spinal canal at this level 6-7 mmlevel 6-7 mm

Anterior subluxation of C 1 over C 2 vertebra. Anterior subluxation of C 1 over C 2 vertebra. ATLANTO- DENTAL interspace is 8-10 mmATLANTO- DENTAL interspace is 8-10 mm

MRI REPORTMRI REPORT

• Gross atlanto-axial subluxation Gross atlanto-axial subluxation causing significant compression of causing significant compression of upper cervical spinal cordupper cervical spinal cord

• Atlanto-occipital assimilationAtlanto-occipital assimilation• Basilar invagination/ platybasiaBasilar invagination/ platybasia• Os odontoideumOs odontoideum

TREATMENTTREATMENT

• Medical therapy depends on the congenital Medical therapy depends on the congenital anomalies present in the syndrome. anomalies present in the syndrome.

• Referrals toReferrals to• NephrologyNephrology• UrologyUrology• CardiologyCardiology• ENT may be needed because of the ENT may be needed because of the

associated anomaliesassociated anomalies• NEUROSURGEONNEUROSURGEON

TREATMENTTREATMENT• Minimally involved patients lead normal lives with Minimally involved patients lead normal lives with

only minor restrictions. only minor restrictions. • Should avoid contact sports that place neck at risk. Should avoid contact sports that place neck at risk. • For mechanical symptoms, cervical collar, For mechanical symptoms, cervical collar,

analgesics, NSAIDS, or careful traction can be used. analgesics, NSAIDS, or careful traction can be used. • For neurologic compromise a thorough work-up to For neurologic compromise a thorough work-up to

find the exact area of irritation, then fusion of the find the exact area of irritation, then fusion of the appropriate segments posteriorly. Decompression appropriate segments posteriorly. Decompression may be employed based on the site of the stenosis. may be employed based on the site of the stenosis.

• Dislocations and basilar invagination are treated by Dislocations and basilar invagination are treated by careful traction followed by posterior fusion. careful traction followed by posterior fusion.

• Neurologic deficits and persistent pain are Neurologic deficits and persistent pain are indications for surgery indications for surgery

TREATMENT OF CV TREATMENT OF CV JUNCTION ANOMALIESJUNCTION ANOMALIES

• MANAGEMENT OF REDUCIBLE MANAGEMENT OF REDUCIBLE DEFORMITIESDEFORMITIES

Primary goal is stabilizationPrimary goal is stabilization reducing the compression on reducing the compression on

brainstem and spinal cordbrainstem and spinal cord• MANAGEMENT OF IRREDUCIBLE MANAGEMENT OF IRREDUCIBLE

DEFORMITIESDEFORMITIES DecompressionDecompression• VENTRAL AND DORSAL VENTRAL AND DORSAL

APPROACHES WITH FIXATION IF APPROACHES WITH FIXATION IF NEEDEDNEEDED

THANK YOUTHANK YOU

cv junction anomaly

Health & Medicine