Definition
Twisted / Wry neck secondary to fibromatosis in sternocleidomastoid
Epidemiology
Packaging defect
- commonest first born
- 75% on right
Associations
CDH 20%
Metatarsus adductus 15%
Breech presentation
Klippel - Feil Syndrome
Arthrogryposis
Aetiology
Fibrosis of SCM on one side
Fails to grow & causes progressive deformity
Pathogenesis
Unknown
Theories
1. Ischaemia secondary to position in utero
- compartment syndrome SCM
2. Birth injury with haemorrhage
Natural History
Many resolve spontaneously
However if untreated get permanent facial asymmetry
Clinically
Lump may be noticed in first few weeks of life
- often disappears
Head tilted to one side so ear approaches shoulder
Head turned towards other shoulder
Associated facial asymmetry
DDx
Primary
Congenital fibrosis SCM
Congenital vertebral anomaly
- Klippel Feil
- os ondontoid
- C1-2 fusion
- unilateral C1 deficiency
- many others
Secondary
Trauma
- atlantoaxial rotatory subluxation
- # C1 /2
Grisel's syndrome
Ocular dysfunction
Infection / Discitis
SCM scar / tumour
HNP
X-ray
To exclude congenital vertebral anomaly
- 17 cases of unilateral C1 deficiency with wry neck in literature
Indicated with failure non operative management
Management
Non operative
Stretching exercises
90% successful
Techniques
1. Parents taught to carry child with their arm under flexed side of neck
- stretches SCM whilst carrying
2. Passive stretching exercises
- lateral head bend away from affected side
- head rotation towards affected side
- 90% success
Operative
Indications
If persists past 1 year age chance of resolution very poor
- operate especially if > 30o limitation of movement
Timing
Ling et al Clin Orthop 1976
- 103 operations
- high rate scar tethering if released <1 year old
Options
1. Distal release first
- if not successful proximal release also
2. Distal and proximal release
- often at age 4-6
- expose both and mark with sling (more difficult to isolate after one end divided)
- complete release both
Technique
Distal release
- 5cm transverse incision 1cm above medial end clavicle
- incise tendon sheath
- draw tendons out (NB sternal and clavicular heads)
- divide / Z plasty / excise 2 cm of both tendons
- explore wound digitally for any fibrous bands and divide these
- if release incomplete perform proximal release
Proximal release
- incision immediately behind & below ear
- divide SCM transversely just distal to tip of mastoid process
- NB spinal accessory nerve at risk
Post operative
- manual stretching 3x /d starts at 1 week post-op