Paediatric Cervical spine

Question 1

What is Klippel- feil Syndrome?

Answer 1

Multiple abnormal segments of cervical spine

Question 2

What is the pathophysiology of Klippel- Feil syndrome?

Answer 2

• Failure of normal formation & segementation of cervical somites at 3-8 week gestation

Question 3

Name any associated conditions with Klippel- feil Syndrome?

Answer 3

• Congential scoliosis
• Sprengle’s deformity
• Renal disease
• basiliar invagination
• atantloaxial instability
• congential heart disease

Question 4

What are the signs and symptoms of Klippel- Feil Syndrome?

Answer 4

Symptoms

• Stiff neck

Signs

• Low posterior hair line
• short webbed neck
• Limited cervical ROM
• high scapula- Sprengel’s
• jaw anormalies
• partial loss of hearing
• torticolis
• scoliosis

Question 5

What imaging is useful in Klippel- Feil syndrome?

Answer 5

Xrays

• Ap, lateral & odontoid
• lateral - basilar invagination
  
  • Atantolaxial instability- ADI >5mm
  • Degenerative changes of cervical spine
  • calcification within intervertebral space- resoluton 6/12

Question 6

What is the tx of Klippel- feil syndrome?

Answer 6

non operative

• Observation with activity modification
  
  • asymptomatic patients
  • most common presentation
  • councelling to avoid putting the neck at risk during certain activties

Surgical

• Surgical decompression and fusion
  
  • for basilar invagination
  • chronic pain
  • myelopathy
  • assoc atlantoaxial instability
  • adj level disease if symptomatic

Question 7

what is this

Answer 7

• Congential muscular torticollis **
• Rotatory atantloaxial instability
• Klippel - feil Syndrome
• Infection
• Tumour
• Trauma

Question 8

Define congential muscular torticollis?

Answer 8

• A congential packaging disorder typically caused by contraction of Sternocleidomastoid

Question 9

What is the aetiology of congential muscular torticollis?

Answer 9

• True aetiology unclear
• Some suggest intrauterine compartment syndrome of SCM
• non SCM causes include
  
  • trauma
  • infection
  • tumour
  • ophthalmology
  • vestibular

Question 10

What other disorders is congential muscular torticollis associated with?

Answer 10

• other packaging disorders
• Devlepmental dysplasis of hips - DDH = 20%
• Metatarsus adductus
• traumatic delivery
• plagiocephaly- head asymmetry
• congential altano-occiptial abnormalities

Question 11

What is seen on examination of a child with congential muscular torticollis?

Answer 11

• Head tilted - laterally to side of SCM tightness
• Chin rotated away from side of SCM
• Paplable neck mass & fibrosis noted within forst 4 weeks of life

Question 12

What are the indications for imaging?

what imaging modality is useful?

Answer 12

Xrays

• If no palpable mass if felt so that other conditions of torticollis can be ruled out
  
  • rotatory atlanto-axial instabilty
  • Klippel- Feil syndrome

USS

• If palpable mass is felt
• can help distinguish from congential muscular torticollis for more serious neurological or osseous abnormalities

Question 13

What is the tx of congential muscular torticollis?

Answer 13

Non operative

• Passive stretch
• conditions for <1 year
• limitations <30 degrees
• stretch include stretch lateral away from affected side and chin towards affected side
• 90% respond to passive stretch

Operative

• z plasty lengthening or distal biploar release of SCM
• conditions > 1 year
• limitations greater in 30^o

Question 14

What is atlantoaxial rotatory instablity?

Answer 14

• C1-C2 rotatory instability caused by subluxation/facet dislocation
• common cause of childhood torticollis
• spectrum of disease from subluxation to full facet dislocation

Question 15

What is the cause of atlantoaxial rotatory stability?

Answer 15

• trauma
• Retropharyngeal abscess- Grisel’s disease
• Less common
  
  • Down’s syndrome
  • RA
    Tumours
  • congential anomalies

Question 16

What is the pathology of atlantoaxial rotatory instability?

Answer 16

• thought to be due to ligaments intact
• transverse ligament intact
  
  • spinal stenosis can only happen with severe rotation & facet dislocation
• Transverse ligament is disrupted
  
  • a component of atherolithesis >5mm then spinal canal stenosis can occur with less rotation- 45 degrees
  • vertebral arteries maybe at risk

Question 17

Can you describe the classification of atlantoaxial rotatory instability?

Answer 17

• Type 1- unifacet dislocation- transverse lig intact
• Type 2- unifacet dislocation w ant displacment 3-5mm
  
  • transverse ligament torn
• Type 3- bilateral anterior facet displacment >5mm
• type 4- posterior displacement of atlas

Question 18

What are the symptoms of atlantoaxial rotatory instability?

Answer 18

Symptoms

• Tilted head
• neck pain
• headache

Signs

• ispilsateral rotation and contralateral tilt of head in relation of C1
• controlateral sternocleidmoastoid may be spastic
• reduced cervical rotation

Question 19

What imaging is useful in dx of atlantoaxial rotatory instability?

Answer 19

Xray

• AP
• Odontoid peg
• lateral
• flexion/extension

Dynamic CT

• gold standard
• take ct stright forward then in max rotation to the right/left
  
  • you will see fixed rotation of C1/C2 which does not change with dynamic rotation

Question 20

What is the tx of atlantoaxial rotatory instability?

Answer 20

Non operative

• Soft collar, anti-inflammatory medication, exercise programme
  
  • subluxation <1 week
  • many pts spontaneously reduce before seeking medical team
• Head halter traction & bracing
  
  • subluxation pesistent >1 week
  • small amounts 5lbs
  • either hospital/at home for up to 3 weeks
  • muscle relaxants/analgesics maybe required
• Halo Traction & bracing
  
  • subluxation presists >1 month

Operative

• Posterior C1-2 Fusion
  
  • ​if subluxation presists >3months
  • Neurological deficit present