Paediatric Cervical spine Flashcards
What is Klippel- feil Syndrome?
Multiple abnormal segments of cervical spine
What is the pathophysiology of Klippel- Feil syndrome?
- Failure of normal formation & segementation of cervical somites at 3-8 week gestation
Name any associated conditions with Klippel- feil Syndrome?
- Congential scoliosis
- Sprengle’s deformity
- Renal disease
- basiliar invagination
- atantloaxial instability
- congential heart disease
What are the signs and symptoms of Klippel- Feil Syndrome?
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
What imaging is useful in Klippel- Feil syndrome?
Xrays
- Ap, lateral & odontoid
- lateral - basilar invagination
- Atantolaxial instability- ADI >5mm
- Degenerative changes of cervical spine
- calcification within intervertebral space- resoluton 6/12
What is the tx of Klippel- feil syndrome?
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
what is this
- Congential muscular torticollis **
- Rotatory atantloaxial instability
- Klippel - feil Syndrome
- Infection
- Tumour
- Trauma
Define congential muscular torticollis?
- A congential packaging disorder typically caused by contraction of Sternocleidomastoid
What is the aetiology of congential muscular torticollis?
- True aetiology unclear
- Some suggest intrauterine compartment syndrome of SCM
- non SCM causes include
- trauma
- infection
- tumour
- ophthalmology
- vestibular
What other disorders is congential muscular torticollis associated with?
- other packaging disorders
- Devlepmental dysplasis of hips - DDH = 20%
- Metatarsus adductus
- traumatic delivery
- plagiocephaly- head asymmetry
- congential altano-occiptial abnormalities
What is seen on examination of a child with congential muscular torticollis?
- 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
What are the indications for imaging?
what imaging modality is useful?
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
What is the tx of congential muscular torticollis?
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 30o
What is atlantoaxial rotatory instablity?
- C1-C2 rotatory instability caused by subluxation/facet dislocation
- common cause of childhood torticollis
- spectrum of disease from subluxation to full facet dislocation
What is the cause of atlantoaxial rotatory stability?
- trauma
- Retropharyngeal abscess- Grisel’s disease
- Less common
- Down’s syndrome
- RA
Tumours - congential anomalies