Congenital Muscular Torticollis

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