Cv Junction Anomaly
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Cv Junction Anomaly Cv Junction Anomaly Presentation Transcript

  • AN INTERESTING CASE OF QUADRIPARESIS PROF. RAMASAMY’S UNIT Dr.Betsy M 3
    • VELAN , 18 yr male
    • C/O- difficulty in using limbs – 2 yrs
    • Difficulty in using lower limbs initially (2 yrs) followed by difficulty in using upper limbs (1 yr) : gradual in onset ; slowly progressing , proximal & distal ; spastic
    • H/o restriction of neck movements
    • No h/o s/o HMF / cranial nerve involvement
    • No h/o s/o sensory or cerebellar involvement
    • No h/o bladder/ bowel involvement
    • No h/o LOC, seizures, fever
    • No h/o trauma
    • No significant past h/o / family h/o
    • O/E
    • Conscious and cooperative
    • Vitals stable , no pallor
    • Short stature – 152 cm , moderately nourished
    • Low hairline , Height neck ratio- 15
    • No webbing of neck / torticollis
    • No neurocutaneous markers
    • Higher mental functions- Nl
    • Cranial nerve examinaton – Nl
    • Motor system-
    • No evident wasting
    • Hypertonia all 4 limbs
    • No clonus
    • Muscle power –
    • Upper limbs – 4 +/ 5 B/L
    • Lower limbs – 4 +/ 5 B/L
    • Reflexes – B/L DTR brisk
    • B/L plantar extensor
    • Sensory – decreased below C 2
    • Romberg’s sign –positive
    • Cerebellum – normal
    • No involuntary movements
    • Spastic gait
    • No signs of meningeal irritation
    • Fundus- normal
    • Spine and cranium- movements of cervical spine restricted
    • No kyphosis/ scoliosis
    • CARDIOVASCULAR SYSTEM
    • Apical impulse in 5 th LICS in MCL
    • No thrill / parasternal heave
    • S1 S2 +
    • Pan systolic murmur grade 3/6 best heard in the left lower sternal border
    • RESPIRATORY SYSTEM
    • Within normal limits
    • GASTROINTESTINAL SYSTEM
    • Within normal limits
  •  
  •  
  • PROVISIONAL DIAGNOSIS Spastic quadriparesis Low hair line Short neck Restriction of neck movements VSD CRANIOVERTEBRAL JUNCTION ANOMALY
  • INVESTIGATIONS
    • Complete blood count – normal
    • Renal Function test- normal
    • Cardiology opinion:
    • Chest X- ray / ECG / Echo – Ventricular septal defect
    • USG Abdomen – normal
    • Audiogram – normal
    • Radiologic investigations –
    • Complex craniovertebral junction anomaly
  • CRANIOVERTEBRAL JUNCTION ANOMALIES
    • CRANIOVERTEBRAL JUNCTION
    • Foramen magnum
    • Adjacent surrounding occipital bone (basiocciput)
    • First cervical vertebrae (atlas)
    • Second cervical vertebrae (axis)
    • Transition zone between a mobile cranium and relatively rigid spinal column
    • Site of craniospinal junction
    • Anomalies may be defects of development and not necessarily congenital and so may not manifest at birth
  • CLASSIFICATION OF CVJ ANOMALIES
    • CONGENITAL MALFORMATIONS
    • Malformations of occipital bone
    • Malformations of atlas
    • Basilar invagination
    • Remnants around foramen magnum
    • Condylar hypoplasia
    • Clivus segmentations
    • Failure of atlas segmentation from occiput(assimilation)
    • Atlantoaxial fusion
    • Aplasia or hypoplasia of atlas arches
  • CVJ ANOMALIES CLASSIFICN (CONTD)
    • Malformations of axis
    • Atlantoaxial segmentation failure
    • Segmentation failure of C2-C3
    • Dens dysplasias – os odontoideum
    • odontoid hypoplasia/aplasia
    • ossiculum terminale persistens
  • CVJ ANOMALIES CLASSIFICN (CONTD)
    • DEVELOPMENTAL AND ACQUIRED ABNORMALITIES
    • Foramen magnum abnormalities
    • Foraminal stenosis (eg. achondroplasia ,MPS )
    • Secondary basilar invagination
    • eg. Paget’s disease, osteomalacia, hyperparathyroidism
  • CVJ ANOMALIES CLASSIFICN (CONTD)
    • ATLANTOAXIAL INSTABILITY
    • Errors of metabolism (Morquio’s syndrome)
    • Infections(Grisel’s synd)
    • Traumatic atlantoaxial / occipitoatlantal dislocation
    • Degenerative(ligamentous disruption at CV junction)
    • Inflammatory (RA, ankylosing spondylitis)
    • Down’s syndrome
    • Tumours ( chordoma, syringomyelia, NF )
    • Misc (fetal warfarin syndrome, Conradi’s synd)
  • CLASSIFICATION
    • SKELETAL ANOMALIES
    • Platybasia
    • Basilar invagination ( 1 0 / 2 0)
    • Klippel-feil anomaly
    • Occipitalisation of atlas
    • Atlanto-axial dislocation
    • NEURAXIAL ANOMALIES
    • Arnold Chiari malformation
    • Dandy Walker syndrome
    • Occipito cervical myelo meningiocoele
    • Posterior fossa cysts
  • SIGNS AND SYMPTOMS
    • Abnormal general physical appearance:
    • Head may be cocked to one side
    • Short neck
    • Scoliosis
    • Neurological :
    • M.C- Posterior occipital headache that worsens with neck flexion or extension
    • Myelopathy
    • Brainstem and lower cranial nerve deficits
    • Vascular symptoms
    • Intermittent attacks of altered consciousness
    • Confusion and transient loss of visual fields
    • Vertigo
  • KLIPPEL FEIL SYNDROME
    • Congenital fusion of cervical vertebrae
    • Failure of normal segmentation of the cervical vertebrae/somite between 3 rd and 8 th weeks of fetal development (rather than a secondary fusion)
    • Maurice Klippel and Andre Feil – 1912
    • Incidence – 1 in 42,000 births ; more in females
    • Autosomal dominant inheritance – C2-C3 fusion. Autosomal recessive – C5- C6 fusion
    • FEIL’S TRIAD :
    • Low posterior hair line
    • Short neck
    • Limitation of head and neck movements / decreased range of motion in cervical spine
  • CLASSIFICATION
    • Feil’s classification
    • Type I – massive fusion of many cervical and upper thoracic vertebrae with synostosis
    • Type II – fusion of only 1 or 2 vertebrae (with hemivertebrae , scoliosis, occipito atlantoid fusion)
    • Type III – presence of lower thoracic and upper lumbar spine anomalies with I/II
    • Type IV – sacral agenesis
    • Samartzis’s classification (2006)
    • To clarify prognosis
    • Type I – single congenitally fused cervical segment
    • Type II – multiple non-contiguous fused segments
    • Type III – multiple contiguous fused segments
  • CLINICAL FEATURES
    • Patients with upper cervical spine involvement tend to present at an earlier age than those whose with lower cervical spine involvement
    • Rotational loss and lateral bending is usually more pronounced than loss of flexion and extension because latter movements take place mostly between occiput and atlas
    • Scoliosis – some patients congenital due to involvement of thoracic spine , others scoliosis compensatory to cervical scoliosis
  • CLINICAL FEATURES
    • Webbing of soft tissues on each side of the neck (extending from mastoid process to acromion of shoulders)- ‘pterygium colli’
    • Assocd torticollis due to contracture of sternocleidomastoid muscle or bony abnormalities
    • Facial asymmetry
    • Sprengel deformity/ high scapula
    • Scoliosis and/or kyphosis
  • CLINICAL FEATURES CONTD..
    • Musculoskeletal sys- cervical rib, congenital fusion of ribs, abnormal costovertebral joints, syndactyly, hypoplastic thumb, supernumerary digits, hypoplasia of pectoralis major, hemiatrophy of upper or lower limbs, CTEV, sacral agenesis
    • Urinary tract abnormalities – agenesis of kidney, horseshoe kidney, hydronephrosis, tubular ectasia, renal ectopia, double collecting system
    • Cardiovascular- VSD, PDA, coarctation of aorta, patent foramen ovale
  • CLINICAL FEATURES CONTD..
    • Deafness (absence of auditory canal and microtia)
    • Synkinesia- involuntary paired movements of the hand ( mirror movements)
    • Neurologic deficit- facial nerve Palsy, rectus muscle palsy, ptosis of eye, cleft palate, etc
  • RADIOLOGICAL FINDINGS
    • Cervical spine routine x-ray followed by flexion/extension lateral X-rays. These may show flattening and widening of vertebrae, hemivertebrae or block vertebrae, instability.
    • MRI with head flexed and extended will most accurately access subluxation and cord compression along with cord anomalies.
    • Wasp-waist sign- anterior concave indentation at the site of the absent or fused interspace between the fused vertebrae.
    • In the young child (<5y) the fusion is more apparent in the posterior elements.
    • X-rays of the T-spine because of extension of synostoses below the neck.
  • ASSIMILATION OF ATLAS OCCIPITALIZATION OF ATLAS/ ATLANTO-OCCIPITAL SYNOSTOSIS
    • Failure of segmentation between the last occipital sclerotome and the first spinal sclerotome – partial/complete
    • Anterior arch to clivus : posterior arch to occiput
    • Signs and symptoms only in second decade of life due to gradual increasing ligament laxity and instability with aging
    • Onset of clinical symptoms can be sudden and precipitated by relatively minor trauma – loss of movt b/w atlas and occiput increases stress at atlanto axial joint => instability
    • Neurological symptoms due to Odontoid process projecting into foramen magnum
    • Assocd – basilar invagination, occipital vertebra, Klippel Feil
    • Radiology: cervical spine x-ray (flexion – extension ), CT, MRI
    • Treatment – non-operative / surgery
  • DENS DYSPLASIAS
    • OSSICULUM TERMINALE PERSISTANS
    • OS ODONTOIDEUM
    • :Dystopic – fuses with the occipital bone and moves with clivus
    • :Orthotopic – lies in the position of normal dens and moves with atlas and axis vertebrae
    • HYPOPLASIA/ AGENESIS OF DENS
  •  
  • ATLANTO AXIAL DISLOCATION
    • CLASSIFICATION:
    • Type I A- normal odontoid with occipitalisation of atlas
    • Type I B- normal odontoid without occipitalisation
    • Type II- detachment of odontoid from body of axis or agenesis of part or whole of dens
    • Diagnosis is radiological. Atlas-Dens interval of more than 5 mm in children and more than 3 mm in adults is diagnostic
  • ATLANTO AXIAL DISLOCATION
    • Errors of metabolism (Morquio’s syndrome)
    • Infections(Grisel’s synd): associated with inflammation of adjacent soft tissues of the neck
    • Traumatic atlantoaxial / occipitoatlantal dislocation
    • Degenerative(ligamentous disruption at CV junction)
    • Inflammatory (RA, ankylosing spondylitis): From laxity of the ligaments and destruction of the articular cartilage
    • Down’s syndrome :d ue to laxity of transverse ligament
    • Tumours ( chordoma, syringomyelia, NF )
    • Misc (fetal warfarin syndrome, Conradi’s synd)
  • ATLANTO AXIAL DISLOCATION The gap between the occipital condyles and the condylar surface of the atlas is widened to more than 5 mm.
  • BASILAR INVAGINATION
    • Primary developmental defect
    • Deformation of the basiocciput – upward indentation or invagination of base of skull into posterior fossa
    • Prolapse of the vertebral column upward into the skull at the base
    • BASILAR IMPRESSION – Acquired form of basilar invagination secondary to softening of skull Eg. Paget’s disease, OI, hyperparathyroidism, osteomalacia, rickets
    • PLATYBASIA – flattening of the skull - Abnormal obtuse basal angle formed by clivus and floor of anterior fossa – if exceeds 145 0
  • BASILAR INVAGINATION CONTD..
    • Two types-
    • 1. Anterior / Ventral – shortening of basiocciput such that clivus is short, horizontally oriented ; and displaces the plane of foramen magnum upward in relation to the spinal column. Posterior fossa structures become crowded. Often assocd wuth platybasia
    • 2. Paramedian – maldevelopment of occipital bones and occipital condylar hypoplasia. Clivus dorsally displaced into posterior fossa and is of normal length. Clivus invagination is compensated by excessive downward curvature of lateral squamous occipital bone.
    • Clinical features due to compression of neural structures at the cervicomedullary junction
    • Suboccipital headache due to irritation of 2 nd cranial nerve, lower cranial nerve symptoms, cerebellar symptoms( nystagmus ), progressive quadriparesis
    • Often associated with other developmental bony anomalies
  • CRANIOMETRIC LINES
    • CHAMBERLAIN’S LINE
    • (Palato-occipital line)
    • - joins posterior tip of hard palate to posterior rim of foramen magnum
    • Normally tip of dens lies 3.6 mm below this line. Upto one third of the dens may be above this line.
    • In basilar invagination, the odontoid process bisects Chamberlain’s line
    Hard palate Clivus Occiput
  • CRANIOMETRIC LINES contd..
    • McRae’s LINE
    • (Foramen Magnum line)-
    • Joins anterior and posterior edges of foramen magnum: sagittal diameter of foramen magnum. (Avg – 35mm)
    • Tip of dens must be below this line
    • If effective sagittal space for cervicomedullary junction is less than 20 mm in a child older than 8 yrs, neurological deficit is usually present
  • CRANIOMETRIC LINES contd..
    • MCGREGOR’S LINE (Basal line)-
    • Joins hard palate to lowest point of occipital bone
    • Tip of dens should not exceed 5 mm above this line
    • Basilar invagination
    • Line position may vary with flexion and extension
  • CRANIOMETRIC LINES contd..
    • WACKENHEIM’S LINE
    • ( Clivus canal line)
    • Line drawn along clivus and extrapolated into cervical spinal canal. Odontoid process should be ventral or tangential to it.
    • Odontoid process transects this line in basilar invagination, atlantoaxial dislocation and anterior occipito atlantal dislocation
    Clivus Odontoid process
  • CRANIOMETRIC LINES contd..
    • FISHGOLD’S DIGASTRIC LINE
    • (Biventer line)- measured on frontal projection
    • Connects the digastric grooves ( fossae for digastric muscles on undersurface of skull just medial to mastoid process)
    • Line is normally 11mm (+/-4mm) above the atlanto-occipital junction.
    • Upper limit of position for the odontoid tip
    • Oblique in unilateral condylar hypoplasia
    • FISHGOLD’S BIMASTOID LINE
    • Odontoid process should be less than 10 mm above this line
  • CRANIOMETRIC LINES contd..
    • HEIGHT INDEX OF KLAUS
    • Distance between tip of dens and tuberculum cruciate line
    • Nl- 40-41mm
    • In basilar invagination-
    • <30 mm
  • CRANIOMETRIC LINES contd..
    • SPINOLAMELLAR LINE
    • (Spinous interlaminar line)- line drawn from interoccipital ridge above and down along the fused spinous processes of C2 and C3
    • Normally should intersect posterior arch of atlas
    • If atlas is fused, posterior arch is anterior to the line; posterior compression of spinal cord may occur
    • SCHMIDT-FISCHER ANGLE
    • Angle of axes of atlanto-occipital joints
    • Normally 124-127 degrees
    • Angle is wider in condylar hypoplasia
  • X RAY SKULL LATERAL VIEW OPEN MOUTH X RAY ATLANTO OCCIPITAL FUSION C5 C6 PARTIAL BLOCK VERTEBRA OS ODONTOIDEUM
  • MRI BRAIN AND CERVICAL SPINE
    • Os odontoideum is noted
    • No fluid collection is noted
    • Wackenheim’s clivus canal line is transected by odontoid process- BASILAR INVAGINATION
    • PLATYBASIA
  • MRI BRAIN AND CERVICAL SPINE
    • Chamberlain’s line bisected by odontoid process –
    • BASILAR INVAGINATION
    • Gross narrowing of spinal canal at C1-C2 level, causing spinal cord compression. Mid sagittal AP diameter of spinal canal at this level 6-7 mm
  • Anterior subluxation of C 1 over C 2 vertebra. ATLANTO- DENTAL interspace is 8-10 mm
  •  
  • MRI REPORT
    • Gross atlanto-axial subluxation causing significant compression of upper cervical spinal cord
    • Atlanto-occipital assimilation
    • Basilar invagination/ platybasia
    • Os odontoideum
  • TREATMENT
    • Medical therapy depends on the congenital anomalies present in the syndrome.
    • Referrals to
    • Nephrology
    • Urology
    • Cardiology
    • ENT may be needed because of the associated anomalies
    • NEUROSURGEON
  • TREATMENT
    • Minimally involved patients lead normal lives with only minor restrictions.
    • Should avoid contact sports that place neck at risk.
    • For mechanical symptoms, cervical collar, analgesics, NSAIDS, or careful traction can be used.
    • For neurologic compromise a thorough work-up to find the exact area of irritation, then fusion of the appropriate segments posteriorly. Decompression may be employed based on the site of the stenosis.
    • Dislocations and basilar invagination are treated by careful traction followed by posterior fusion.
    • Neurologic deficits and persistent pain are indications for surgery
  • TREATMENT OF CV JUNCTION ANOMALIES
    • MANAGEMENT OF REDUCIBLE DEFORMITIES
    • Primary goal is stabilization
    • reducing the compression on brainstem and spinal cord
    • MANAGEMENT OF IRREDUCIBLE DEFORMITIES
    • Decompression
    • VENTRAL AND DORSAL APPROACHES WITH FIXATION IF NEEDED
  • THANK YOU