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A Case Of Short Neck
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  • 1. A CASE OF SHORT NECK
    PROF.S.TITO’S UNIT
  • 2. Perumal a 45 yr old male admitted with
    • c/o difficulty in using both lower limbs -2 years duration
    • 3. c/o giddiness on getting up -1 week
  • Presenting history
    Onset was gradual in onset progressive in nature followed by unable to drive rickshaw
    No h/o motor,sensory,involuntarymovts of upper limb
    h/o suggestive of proximal and distal muscle weakness of lower limb
    h/o tightness of all four limbs
    No h/o wasting or fasciculations
    No h/o involuntary movements
  • 4. No h/o diff in passing through narrow pathways
    No h/o sensory disturbancces
    h/o diff in walking in darkness,&face wash
    No h/o olfactory, visual disturbances
    No h/o motor and sensory abnormalities of face
    h/o change of voice -3months
    No h/o other cranial nerve disturbances
    No h/o bladder,bowel and other autonomic disturbances
  • 5. PAST HISTORY; No h/o DM,HT,TB h/0 trauma falling from height present treated as IP in hospital 2 months records not available at 2 yrs of age.
    Personal history; occ.smoker,alcoholic
    Family history; no h/o any relevant illness
  • 6. GENERAL EXAMINATION
    Pt conscious,oriented,notanaemic not jaundiced,nocyanosis,no clubbing ,no gla.
    Height:neck ratio 19:1
    Webbing of neck
    Low hair line
    sprengel’s anomaly
    Left hemiatrophy
    Restricted neck movts on side to side
    Prominent epiglottis on opening mouth
    Mirror movements-synkinesia
    Pulse 70/mt,BP102/66 mmhg
  • 7. SHORT NECK
    LOW HAIRLINE
  • 8. PROMINENT EPIGLOTTIS
  • 9. LT.HEMIATROPHY
  • 10. synkinesia
  • 11. HMF-NORMAL
    Cranial nerves;1,2,3,4,,6,7-normal
    5th nerve – lt side diminished cor reflex dim. touch sensation with brisk jaw jerk
    8th nerve conductive deafness lt side 9&10 nerves gag reflex diminished 11th nerve normal 12th nerve normal
  • 12. Motor system
    Superficial reflexes –abdominal and cremasteric reflex present
  • 13. Spastic gait
  • 14. Sensory system
    Pain and temperarure-normal
    Joint position and vibration sense diminished in all four limbs incl.vertebral
    Romberg’s sign positive
  • 15. CEREBELLUM
    No nystagmus
    Finger nose,figer-finger-nose defective Lt.side
    No Dysdiaadokinesia,slurring of speech present
    Heel-shin test positive-lt side
    Tandem walking-defective
  • 16. INVESTIGATIONS
  • 17. Basilar Angle
  • 18. CHAMBERLAIN’S LINE
    • joins posterior tip of hard palate to posterior rim of foramen magnum dense 3.6mm below it
    • 19. Basillarinvagination
    Chamberlain`s line
  • 20. 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
    Mcgregor`s Line
  • 21. Height Index Of Klaus
    HEIGHT INDEX OF KLAUS – dense to tuberculam line < 30
    basillarinvagination
  • 22. McRae’s LINE
    Joins anterior and posterior edges of foramen magnum: sagittal diameter of foramen magnum. (Avg – 35mm);dense below the line
    foramen stenosis
    McRae`s Line
  • 23. Clivus Canal Line
  • 24.
  • 25. MRI LS SPINE
  • 26. DIAGNOSIS
    KLIPPEL FEIL SYNDROME
  • 27. KLIPPEL FEIL SYNDROME
    Congenital fusion of cervical vertebrae
    Failure of normal segmentation of the cervical vertebrae/somite between 3rd and 8th 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
  • 28. 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, occipitoatlantoid 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
  • 29. 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
  • 30. FEIL’S TRIAD
    Low posterior hair line
    Short neck
    Limitation of head and neck movements / decreased range of motion in cervical spine
  • 31. CLINICAL FEATURES
    Webbing of soft tissues on each side of the neck (extending from mastoid process to acromion of shoulders)- ‘pterygiumcolli’
    torticollis due to contracture of sternocleidomastoid muscle or bony abnormalities
    Facial asymmetry
    Sprengel deformity/ high scapula
    Scoliosis and/or kyphosis
  • 32. 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
  • 33. 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
  • 34. 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.
  • 35. 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
  • 36. 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
  • 37. THANK YOU