· congenital elevation of shoulder
· autosomal dominant
· associated with neighbouring congenital anomalies
· failure of descent of arm bud
· arm bud appears in 3rd wk
· at level of C5 to T1
· scapula develops in arm bud in 5th wk
· descends over next 3 months
· to level of T2 to T7
· scapula elevated
· superior angle rotated upwards and forwards
· shoulder musculature deficient
· limited shoulder abduction and external rotation
· scapula may be
· smaller than other side
· joined to cervical spine by fibrous or bony (omovertebral) bar
· associated abnormalities
· cervical ribs
· fused or absent thoracic ribs
· Klippel-Feil syndrome
· thoracic vertebral anomalies
· hypoplastic humerus or clavicle
· various procedures described
· soft tissue release
· osteotomy
· risk of
· hypertrophic scar
· brachial plexus injury
· simplest procedure is
· excision of prominent angle
· excision of omovertebral bar
· rare condition
· theories
· failure of fusion of 2 ossific centres of clavicle
· pressure from subclavian artery
· painless swelling over mid clavicle
· presents soon after birth
· almost always on right side
· bulbous ends overlying each other
· birth fracture of clavicle
· pain
· traumatic delivery
· callus on x-ray
· cleidocranial dysostosis
· bilateral
· associated facial and pelvic bone abnormalities
· options
· excision of prominent lateral end of sternal part
· bone graft and internal fixation