Cervical Spine Disorder in Children

Transcription

1 Cervical Spine Disorder in Children

2 Embryology of Vertebra Para axial Mesoderm 42~44, 42~44 somite 4 occipital somite 8 cervical somite 12 thoracic somite 5 lumbar somite 10 sacral somite 5 coccyx somite Ventral part sclerotome Dorsomedial myotome Dorsolateral dermatome

3 Atlas, odontoid, axis 4 occiput somite 8 cervical somite 1 st 2 nd 3 rd 4 th st occiput somite nd occiput somite rd occiput somite th occiput somite Occipital bone & Foramen magnum 4 occiput somite proatlas 4 th occiput somite atlas lat mass, ring odontoid tip Odontoid from C1 Axis body from C2

4 Atlas, odontoid, axis C1 (atlas) Lateral mass ring from 4 th occiput somite Atlas ring from C1 somite C2 (axis) Odontoid tip from 4 th occiput somite Odontoid body from C1 somite C2 body from C2 somite

5 Atlas, odontoid, axis 4 th occiput somite 4 th occiput somite C2 somite C1 somite C1 somite C2 somite C2

6 Odontoid Anomalies

7 Odontoid anomaly / Lig instability Spondyloepiphyseal dysplasia Morquio disease(mucopolysaccharidosis) Down syndrome Osteogenesis imperfecta Neurofibromatosis

8 Spondyloepiphyseal dysplasia Extension Flexion

9 Neurofibromatosis

10 Os Odontoideum : Separation of dens from C2 body Frequency : rare, but exact frequency unknown Common in Morquio, MED, Down synd Symptoms Local mechanical neck pain Neurologic symptoms Neurovascular symptoms vertebral a compression cervical & brainstem ischemia gait ataxia, syncope, vertigo, and visual disturbances, brainstem infarcts & seizures

11 Os Odontoideum

12 Etiology: Controversy, vs - Cong failure of fusion of dens & body persistent ossiculum terminale Ossicle much smaller, at the level of C1 Associated with Klippel Feil syndrome, occipitalized atlas, or basilar invagination

13 persistent ossiculum terminale 4 th occiput somite 4~6 Fusion at age 12yrs C2 somite C1 somite C2

14 - Fracture or excessive movement of odontoid synchondrosis before closure age of 5-6 years pulling of fragment by alar lig, Non-union prox part of fragment- circulation(+) by alar lig, distal part- AVN(+) resorbed &rounded 2 types of os odontoideum orthotopic os odontoideum : anatomic position dystopic os odontoideum : non-anatomic position

15 Etiology - Os Odontoideum 4 th occiput somite retraction by apical lig C2 somite C1 somite Circulation(+) Ischemic & resoption Fusion 3~6yrs C2 somite Fx at synchondrosis

16 orthotopic os odontoideum dystopic os odontoideum

17 Odontoid ossification Grows & ossifies until age 18 Tip of dens reach upper margin of arch of C1 at age 9 (easily mistaken for hypoplasia)

18 Indications for surgery Significant Instability Neurologic or neurovascular involvement Persistent and disabling pain Progressive instability, SAC < 13mm

19 Instability index (a-b/a) : 53% Extension A a b Flexion B

20 Cervical decompression and fusion

21 Atlanto-Occipital fusion

22 Atlanto-occipital fusion CVJ 0.14 ~ 2.76%. 38% Chiari malformation Basilar impression : common

23 Atlanto-occipital fusion (Occipitalization of Atlas) Clinical findings : short neck, low hairline, Instability(+) in 50% Sxs: : neck pain, neurology limited neck motion Tx : Myelopathy(-), instability(-) no Tx Myelopathy(+),instability(+) fusion

24 Klippel - Feil syndrome

25 Klippel-Feil syndrome : Cong fusion of cervical vertebra Failure of segmentation of somite Clinical Triad : Short, webbed neck Low hair line Restricted neck motion In practice,, any failure of segmentation in C-spine C is called Klippel-Feil syndrome

26 Associated Anomalies Facial abnomaly, webbing, torticollis (26%) Sprengel s deformity (50%) Scoliosis (60%) Renal abnormalities (35%) cause of Death Deafness (15~35%) Congenital heart disease (14~29%) Synkinesia, mirror movement (20%)

27 Klippel-Feil syndrome Renal anomalies Spinal cord neurology MRI Hypermobility degeneration canal stenosis Basilar impression from occiput-cervical anomay Instability & Neurology

28 Klippel-Feil synd Head tilt or rotation Halo immobilzation Surgical fusion progression of deformity rigid head tilt or rotation severe neck pain neurologic deficit occipitocervical instability with neurology

29 Cervical Spine in Down syndrome

30 Cervical anomalies in Down synd (Trisomy 21) Atlantoaxial instability due to lig laxity Occipitalization of atlas Hypoplasia of post arch of C1 Os Odontoideum

31 Incidence of instability : 10~25% But, most children are asymptomatic Symptom(+) among instabilities : variable (0~18%),. :.

32 High failure rates of fusion & resorption (d/t collagen defect & T-cell immune deficiency) Less likelihood of improvement of neurology Low rates of neurology with variable Sxs (tingling sense ~ Sudden death in normal pts) Development of neurology with increasing AGE Preventive fusion need? All needs fusion? When to fuse?

33 Treatment guidelines Myelopathy(+) needs fusion Marked instability(+), myelopathy(-) fusion Instability(+), neurology(-) no surgery (segal,1991)

34 Guidelines for Down s syndrome American Academy of Pediatrics, Competitive high risk sports instability. 2.

35 Atlantoaxial rotatory subluxation

36 Instability Developmental anomaly Trauma Inflammation of adjacent neck tissues URI, Grisel synd, Adenitis, Ear infection X-ray evaluation AP, Lat view Open mouth view Flexion & Extension lat view (!)

37 Fielding & Hawkins classification

38 Tx of atlantoaxial rotatory sublux (Philips, Hensinger 1989) -, < 1 wk, : soft collar & rest < 1~4wks : Halo traction, firm color 6wks > 4 wks : Halo traction, Halo vest 6wks Failed reduction CR under G/A or Arthrodesis C1-C2 dislocation on visit Arthrodesis

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40 Atlantoaxial rotary subluxation (type II M / 17 C.C. : Torticollis (for 3mo.) Trauma(-), URI(+) Type II

41 Atlantoaxial rotary subluxation (type III) Atlantoaxial rotary subluxation for 3 months Fielding & Hawkins : Type III Traction & fusion

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43 (, X-ray) (SCIWORA- spinal cord injury without radiologic abnormalities) Occiput-C2 (8~10 ) 70% of cervical injuries Ligament laxity, hypermobility, more horizontal facets Large head size fulcrum at C3 Breech delivery, Child abuse, shaking Motor vehicle, fall down, athetic injury

44 SCIWORA (spinal cord injury without radiologic abnormalities) Unique to children under 8yrs 7-66% of pts. with c-spine inj. Sp. column elastic limit : 2 inches Sp. cord elastic limit : 1/4 inches

45 Facial abrasions, head trauma, clavicle fx, High speed motor vehicle injury fall from height X-ray Cervical AP, Lat, both oblique, open mouth view flexion-extension lat view

46 (x) (o) (o).

47 X-ray evaluation - cervical alignment Spinolaminar line of Swischuk C mm. Ant vertebral body line pseudosubluxation 8, 4mm Prevertebral soft tissue swelling 5~6mm at C2 level Post interspinous process distance Cervical lordosis

48 Pseudosubluxation Ligamentous laxity Luscha jt underdevelopment Shallow, horizontal facet jt 4mm

49 X-ray evaluation Atlantoaxial instability C1/C2 10 ADI (Atlanto-dens interval) -4mm SAC(Space available for cord) -13mm Power s ratio- 1.0 BC / AO=1.0

50 Atlantoccipital Instability Down syndrome Juvenile rheumatoid arthritis Larsen s synd Spondyloepiphyseal dysplasia Os odontoideum Hypochondroplasia Achondroplasia

51 X-ray evaluation Basilar Impression Primary Basilar impression Klippel-Feil synd Atlas occipitalization Odontoid or atlas anomaly Secondary Basilar impression Skull base softening dz RA, OI, Paget dz, Morquio Renal dystrophy

52 33 mm 28 mm Best screening line with high reproducerbility

53 Acknowledgement

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61 Cervical spine problems in Down s syndrome Due to generalized lig. Laxity C0-C1 instability : 15/33 C1-C2 instability : 22/33 Most children are asymptomatic Symptomatic in 2.6% (UMN signs)

62 CVJ problems in Down Lig. Laxity bony anomalies CVJ. C0-C1 & C1-C2 instabilities, rotary luxations, Os odontoideum(10/33) C1-C2 up to 40% < 1%, C0-C1 instability 66%. High rates of nonunion & infection with long fusion (d/t collagen defect & deficient T-cell immune system)

63 Basilar impression Most comm. anomaly of upper c-spine Sx. onset : 3rd to 5th decade Neck pain, Myelopathy Platybasia : basal angle > 140 Midsagittal diameter of F. Magnum < 19 mm

64 Cervical spine affections Several anomalies coexist Coexisting other organ anomalies Upper(C0-C2) & lower C-lesions Craniovertebral junction anomalies

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67 Clinical manifestations Pain Torticollis Weakness Sensory disturbance Gait difficulties Incoordination

68 D.Dx of Torticollis Nonosseus - congenital muscular - Sandifer s syndrome - neurogenic - inflammatory Osseus - basilar impression - atlanto-occipital anomalies - unilateral absence of C1 - familial cervical dysplasia - atlantoaxial rotary displacement - neoplasms

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70 Clinical manifestations Mechanical (direct compression, traction) Vascular (ischemia, infarction) Alterations in CSF (hydroceph., syrinx) Instability, deformity, myelopathy

71 Odontoid anomalies Neurol. involvement (even transient) Flex-Ext view instability > 10 mm SAC < 13 mm Progressive instability Persistent neck complaints

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73 Os odontoideum Neurocentral sychondrosis failure Nonunion d/t unrecognized Fx. Retraction by alar, apical ligament Orthotopic or dystopic position Pain, torticollis, N. compromise

74 Klippel - Feil syndrome C-spine fusion abnormality : Tip of Iceberg Prognostic factors : Associated anomalies Instability of adjacent segments

75 Klippel - Feil syndrome Require the skills of a detective to unravel the full syndrome Hidden features may jeopardize the pt s health more than the anomalous fusion itself

76 Nagib s at-risk groups (J. Neurosurg., 1984) 1 : 2 : 3 : 2 sets of block vertebrae with open intervening disc space Craniocervical anomalies with fusion below C2 Associated with canal stenosis

77 occiput C1 C2 C3 C4 C5 C6 C7 T1 T2

78 Atlantoaxial instability C1-C2 is the most mobile joint in Cx. spine (50% of rotation occurs) > 10 > 4.5mm ADI > 4 mm SAC < 13 mm

79 Apex erosion Routine screening Abnormal : 33 mm, 28 mm

80 Chamberlain s line McGregor s line Wackenheim s line : Easiest method

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83 E : Bimastoid line (within 3mm ~ +10 mm) F : Digastric line (mid-oa joint 10mm )

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87 M/5, L/E paraparesis, minor fall Os odontoideum Neutral Flexion Extension

88 Atlanto-occipital instability More than 1mm translation on flexion-extension x-ray

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90 Odontoid anomalies Mechanical & neurological Sx Down s syndrome Spondyloepiphyseal dysplasia Mucopolysaccharidoses

91 Os odotoideum in 3/11patients -- atlantoaxial instability during cervical motion -- Instability index 36%, 46%, 53%(cervical myelopathy) Extension Flexion