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A Case Of Short Neck


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A Case Of Short Neck

  2. 2. Perumal a 45 yr old male admitted with <br /><ul><li> c/o difficulty in using both lower limbs -2 years duration
  3. 3. c/o giddiness on getting up -1 week</li></li></ul><li>Presenting history<br /> Onset was gradual in onset progressive in nature followed by unable to drive rickshaw<br />No h/o motor,sensory,involuntarymovts of upper limb<br />h/o suggestive of proximal and distal muscle weakness of lower limb<br />h/o tightness of all four limbs<br />No h/o wasting or fasciculations<br />No h/o involuntary movements<br />
  4. 4. No h/o diff in passing through narrow pathways<br />No h/o sensory disturbancces<br />h/o diff in walking in darkness,&face wash<br />No h/o olfactory, visual disturbances<br />No h/o motor and sensory abnormalities of face<br />h/o change of voice -3months<br />No h/o other cranial nerve disturbances<br />No h/o bladder,bowel and other autonomic disturbances<br />
  5. 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.<br />Personal history; occ.smoker,alcoholic<br />Family history; no h/o any relevant illness<br />
  6. 6. GENERAL EXAMINATION<br />Pt conscious,oriented,notanaemic not jaundiced,nocyanosis,no clubbing ,no gla.<br />Height:neck ratio 19:1<br />Webbing of neck<br />Low hair line<br />sprengel’s anomaly<br />Left hemiatrophy<br />Restricted neck movts on side to side<br />Prominent epiglottis on opening mouth<br />Mirror movements-synkinesia<br />Pulse 70/mt,BP102/66 mmhg<br />
  7. 7. SHORT NECK<br />LOW HAIRLINE<br />
  9. 9. LT.HEMIATROPHY<br />
  10. 10. synkinesia<br />
  11. 11. HMF-NORMAL<br />Cranial nerves;1,2,3,4,,6,7-normal <br /> 5th nerve – lt side diminished cor reflex dim. touch sensation with brisk jaw jerk<br /> 8th nerve conductive deafness lt side 9&10 nerves gag reflex diminished 11th nerve normal 12th nerve normal<br />
  12. 12. Motor system<br />Superficial reflexes –abdominal and cremasteric reflex present<br />
  13. 13. Spastic gait<br />
  14. 14. Sensory system<br />Pain and temperarure-normal<br />Joint position and vibration sense diminished in all four limbs incl.vertebral<br />Romberg’s sign positive<br />
  15. 15. CEREBELLUM<br />No nystagmus<br />Finger nose,figer-finger-nose defective Lt.side<br />No Dysdiaadokinesia,slurring of speech present<br />Heel-shin test positive-lt side<br />Tandem walking-defective<br />
  16. 16. INVESTIGATIONS<br />
  17. 17. Basilar Angle<br />
  18. 18. CHAMBERLAIN’S LINE<br /><ul><li>joins posterior tip of hard palate to posterior rim of foramen magnum dense 3.6mm below it
  19. 19. Basillarinvagination</li></ul>Chamberlain`s line<br />
  20. 20. MCGREGOR’S LINE (Basal line)-<br />Joins hard palate to lowest point of occipital bone<br />Tip of dens should not exceed 5 mm above this line<br />Mcgregor`s Line<br />
  21. 21. Height Index Of Klaus<br />HEIGHT INDEX OF KLAUS – dense to tuberculam line &lt; 30<br />basillarinvagination<br />
  22. 22. McRae’s LINE<br />Joins anterior and posterior edges of foramen magnum: sagittal diameter of foramen magnum. (Avg – 35mm);dense below the line<br /> foramen stenosis<br />McRae`s Line<br />
  23. 23. Clivus Canal Line<br />
  24. 24.
  25. 25. MRI LS SPINE<br />
  27. 27. KLIPPEL FEIL SYNDROME<br />Congenital fusion of cervical vertebrae<br />Failure of normal segmentation of the cervical vertebrae/somite between 3rd and 8th weeks of fetal development (rather than a secondary fusion) <br />Maurice Klippel and Andre Feil – 1912<br />Incidence – 1 in 42,000 births ; more in females<br />Autosomal dominant inheritance – C2-C3 fusion. Autosomal recessive – C5- C6 fusion<br />
  28. 28. CLASSIFICATION<br />Feil’s classification<br />Type I – massive fusion of many cervical and upper thoracic vertebrae with synostosis<br />Type II – fusion of only 1 or 2 vertebrae (with hemivertebrae , scoliosis, occipitoatlantoid fusion)<br />Type III – presence of lower thoracic and upper lumbar spine anomalies with I/II<br />Type IV – sacral agenesis<br />Samartzis’s classification (2006)<br />To clarify prognosis<br />Type I – single congenitally fused cervical segment<br />Type II – multiple non-contiguous fused segments<br />Type III – multiple contiguous fused segments<br />
  29. 29. CLINICAL FEATURES<br />Patients with upper cervical spine involvement tend to present at an earlier age than those whose with lower cervical spine involvement<br />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<br />Scoliosis – some patients congenital due to involvement of thoracic spine , others scoliosis compensatory to cervical scoliosis<br />
  30. 30. FEIL’S TRIAD<br />Low posterior hair line<br />Short neck<br />Limitation of head and neck movements / decreased range of motion in cervical spine<br />
  31. 31. CLINICAL FEATURES<br />Webbing of soft tissues on each side of the neck (extending from mastoid process to acromion of shoulders)- ‘pterygiumcolli’<br />torticollis due to contracture of sternocleidomastoid muscle or bony abnormalities<br />Facial asymmetry<br />Sprengel deformity/ high scapula<br />Scoliosis and/or kyphosis<br />
  32. 32. CLINICAL FEATURES CONTD..<br />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<br />Urinary tract abnormalities – agenesis of kidney, horseshoe kidney, hydronephrosis, tubular ectasia, renal ectopia, double collecting system<br />Cardiovascular- VSD, PDA, coarctation of aorta, patent foramen ovale<br />
  33. 33. CLINICAL FEATURES CONTD..<br />Deafness (absence of auditory canal and microtia)<br />Synkinesia- involuntary paired movements of the hand ( mirror movements)<br />Neurologic deficit- facial nerve Palsy, rectus muscle palsy, ptosis of eye, cleft palate, etc<br />
  34. 34. RADIOLOGICAL FINDINGS<br />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. <br />MRI with head flexed and extended will most accurately access subluxation and cord compression along with cord anomalies. <br />Wasp-waist sign- anterior concave indentation at the site of the absent or fused interspace between the fused vertebrae. <br />In the young child (&lt;5y) the fusion is more apparent in the posterior elements. <br />X-rays of the T-spine because of extension of synostoses below the neck. <br />
  35. 35. TREATMENT<br />Medical therapy depends on the congenital anomalies present in the syndrome. <br />Referrals to<br />Nephrology<br />Urology<br />Cardiology<br />ENT may be needed because of the associated anomalies<br />NEUROSURGEON<br />
  36. 36. TREATMENT<br />Minimally involved patients lead normal lives with only minor restrictions. <br />Should avoid contact sports that place neck at risk. <br />For mechanical symptoms, cervical collar, analgesics, NSAIDS, or careful traction can be used. <br />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. <br />Dislocations and basilar invagination are treated by careful traction followed by posterior fusion. <br />Neurologic deficits and persistent pain are indications for surgery <br />
  37. 37. THANK YOU<br />