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Diagnose & Behandlung

Congenital deformity of the spine-classification


classification of congenital scoliosis

  • failure of formation-wedge vertebrae (2 pedicles), half vertebrae...

     - unsegmented half vertebra: fused to the vertebral body above and below

     - partially segmented half vertebra

     - fully segmented half vertebra-separated above and below by disc space

  • failure of segmentation-block vertebrae, unilateral bar...
  • combination

 

Bild von half vertebra, contralateral thoracic curve special combinations

     half vertebra, contralateral thoracic curve special combinations

  • hemimetameric shift-hemivertebra counterbalanced by another one on the contralateral side in the same region
  • unilateral bar and contralateral hemivertebra-"worst case scenario", progression per year up to 10° Cobb!

 

 

    Bild von worst case scenario: unilateral bar and contralateral half-vertebra                 Bild von 2 wedge vertebrae severe progression

    worst case scenario:                   2 wedge vertebrae severe progression
    unilateral bar and contral-
    ateral half-vertebra

 

  • hemivertebra thoracolumbar 2-3,5°/ye
  • 2 hemivertebrae 5°
  • unilateral unsegmented bar 6-9°
  • unilateral unsegmented bar with contralateral hemivertebra >10°
  • most severe progression in thoracolumbar region!
  • most severe progression up to 5th year and in adolescence/puberty

 

Bild von combined deformities, spine out of balance classification of congenital kyphosis

combined deformities, spine out of balance classification of congenital kyphosis (Mc Master)

 

  • failure of formation
      - posterolateral quadrant hemivertebra
      - butterfly vertebra
      - posterior hemivertebra
      - wedge vertebra
  • failure of segmentation
      - anterior unsegmented bar
      - block vertebra
  • mixed anormalies
  • unclassifiable anomalies

 

congenital vertebral displacement-sudden sagittal kyphotic displacement, most severe of congenital kyphosis

 

 

Bild von congenital vertebral displacement-sudden sagittal kyphotic displacement, most severe of congenital kyphosis

there exist no known genetic abnormalities associated with the development of congenital kyphosis

 

Bild von Klippel-Feil-syndrome

Klippel-Feil-syndrome

 

  • segmentation defects of the cervical spine
  • plagiocephaly
  • reduced cervical spine mobility
  • anomalies of urinary system in one third cong. Heart anomalies in 15%
  • Sprengel in 20-30%
  • problems in hearing in one third

 

Sprengel deformity

  • congenital failure of descendence of one or both shoulders; mostly left shoulder elevated
  • „omovertebral bone“ (connection scapula-C5 or C6)
  • often Klippel-Feil, rib anomalies…
  • 75% girls
  • scapula small and rotated

 

Bild von Klippel-Feil with Sprengel deformity congenital sacral problems

Klippel-Feil with Sprengel deformity

 

congenital sacral problems

  • lumbosacral abnormalities (partial, total, asymmetric, symmetric)
  • sacral obliquity
  • sacral dysplasias up to sacral agenesis
  • sacral dysraphism (MMC)

congenital sacral obliquity

  • angular deformation of the sacrum from a horizontal line drawn parallel from a line across
  • the femoral heads from congenital origin!
  • it is not a pelvic obliquity!
  • mostly sacral endplate elevated at the right side
  • max.20°, but can cause lumbar scoliosis up to 50°-effect like a lumbar hemivertebra

congenital sacral agenesia (Renshaw 1978)

  • unilateral agenesis, partial or total
  • symmetrical partial with stable articulation iliac bone-S1
  • variable lumbar and total sacral agenesis with articulation iliac bone-lowest lumbar vertebra
  • variable lumbar and total sacral agenesis with either fused iliac bones or amphiarthrosis

 

Bild von symptoms of severe forms

symptoms of severe forms

  • often maternal diabetes
  • in severe cases bowel and bladder dysfunction
  • deformities of hips (flexion, abduction, outward rotation), knees (flexion contracture),
  • feet, atrophy of the legs, motoric palsy („Buddha-like-position“)

congenital rib deformities

  • mostly in combination with congenital scoliosis, rarely with congenital kyphosis
  • 70% had thoracic or thoracolumbar concomitant congenital scoliosis
  • mostly on concave side of unilateral failure of segmentation
  • 40% with Sprengel deformity
  • prognosis without difference to scoliosis without rib fusions

associated anomalies

  • syringomyelia (abnormal fluid collection in the medullary canal of the spine,
  • caused by Arnold-Chiari-syndrome, basilar invagination, cord compression, trauma,
  • arachnoiditis, can cause scoliosis, kyphosis, motor weakness, dyscoordination, neuropathic
  • arthropathy, pain)


Bild von Chiari malformation

Chiari malformation (caudal dislocation of cerebellar tonsils below the foramen magnum), diastematomyelia, diplomeylia-fibrous ligament or osseous bar, mostly in lumbar spine

 

Bild von diastematomyelia

diastematomyelia tethered cord (filum terminale pulls cord down to L4)…MRI brain stem and complete

 

Bild von tethered cord

tethered cord spine; release always before spine surgery, better not at the same operation; seldom spontaneous release of symptoms in scoliosis >40°
congenital heart disease (25%)-septum defects, hypoplastic left heart, transposition…
echocardiogram
genitourinary anomalies (20%)-renal aplasia, duplicate ureters, hypospadia…renal
ultrasound

anamnesis for neurologic infantile symptoms

  • is the child toilet trained ?
  • bed wetting problem ?
  • bowel or bladder „accidents“ ?
  • limping ?
  • shoe size difference ?

clinical evaluation

  • spinal dysraphm ?
  • asymmetric calves, cavus feet, clubfeet, vertical tali ?
  • truncal or pelvic imbalance ?
  • spinal balance frontal and sagittal
  • rib cage deformity ?
  • in-and exspiration capacity
  • neurologic deficit ?
  • standing and sitting size

     - rib hump
     - asymmetry of lumbar height
     - height of shoulders
     - height of pelvic rim
     - vertical centre line

radiologic parameters ap

  • Cobb - angle (main-curve, secondary - curve)
  • rotation (Nash and Moe 0 - 4)
  • deviation of perpendicular line
  • pelvic inclination
  • Risser -sign (0-5)

radiologic parameters sagittal

  • evaluation of perpendicular line S1
  • evaluation of L4- (should be horizontal)

     measurements
     - Cobb-angle thoracic cyphosis
     - Cobb-angle lumbar lordosis
     - Cobb-angle Th11-L1 (should be 0°)

CT in congenital deformities

  • bony details
  • good details of apical hemivertebra (for hemivertebra excision)
  • myelo-CT for complex dysraphic problems
  • CT-models for complex deformities


Bild von posterior hemivertebra in CT

posterior hemivertebra in CT

 

MRI in congenital deformities

  • for detection of neural axis abnormalities
  • necessary MRI from brain stem to sacrum
  • evaluation of disks and growth potential
  • evaluation of kidneys and lungs…


lung function
„of all spinal deformity patients having early death due to cor pulmonale , congenital scoliosis patients probably have the highest frequency“ (Wimter 1983)
lung function analysis (total capacity, vital capacity, Tiffeneau-test…)
blood gas analysis



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