Definition
Congenital elevation of shoulder
Epidemiology
AD & Sporadic
Tends to be in girls & on left
- like CDH
Associated with other congenital abnormalities
Aetiology
Failure of descent of arm bud
- arm bud appears in week 3 (level of C5 to T1)
- scapula develops in arm bud in week 5 (Opposite C5)
- descends over next 3/12
Usually by 3rd fetal month to level of T2 to T7
Clinical Features
Scapula small
- scapula elevated
- superior angle rotated upwards & forwards
- shoulder musculature deficient
Limited shoulder abduction
- scapula joined to cervical spine by fibrous or bony bar
- Omovertebral bar
Usually presents as Neonate, but if mild presents later
Associated Abnormalities
Scoliosis
Diastematomyelia
Klippel-Feil Syndrome (usually bilateral)
Cervical ribs
Fused or absent Thoracic ribs
Thoracic Vertebral anomalies
Hypoplastic Humerus or Clavicle
X-ray
Small high scapula
Omovertebral bone
- can see bone linking scapula and hyoid
Bilateral abduction xray reveals lack of ST motion
Operative Management
Indications
Cosmesis
Attempt to improve abduction range
Complications
Hypertrophic scar due to high strain on scar
Brachial Plexus Injury
Options
1. Scapula Resection
Simplest procedures
- only cosmetic
- excision of prominent angle (excise scapula above spine)
- excision of Omovertebral bar
2. Woodward Procedure
Concept
- principle concern is brachial plexus palsy
- best outcome in children 3 - 8
Technique
A. Midline incision
- clavicular osteotomy to protect brachial plexus
- excise omovertebral bar
B. Mobilise scapula caudally
C. Detach Trapezius & Rhomboids from spinous process insertion
- reattach to supraspinous ligaments more inferiorly
- dynamic force inferiorly on scapula
3. Green Procedure
Osteotomy clavicle first
- avoiding plexus injury
Scapula release
- released from medial border of scapula
- reattach after scapula reduced