Question
A 4 year old boy has an asymmetric neck and limited shoulder abduction on the left.
- Describe the deformity and its embryology.
- What associated conditions would you look for?
- Outline the surgical options.
Answer
Deformity
- High, small and medially rotated scapula
- Sprengel deformity, failed descent of the scapula in the embryo
- Scapula normally descends from C4 to T2 level in weeks 9 to 12
- Omovertebral bone or band links the superomedial scapula to the cervical spine in about a third
- Abduction limited by the scapula failing to rotate
Associations
- Klippel-Feil syndrome
- Congenital scoliosis and rib anomalies
- Renal anomalies, request renal ultrasound
- Spina bifida
Cavendish grading
| Grade | Appearance |
|---|---|
| 1 | Very mild, shoulders level, not visible when dressed |
| 2 | Mild, shoulders level, lump in the neck web when dressed |
| 3 | Moderate, shoulder 2 to 5 cm higher |
| 4 | Severe, superior angle near the occiput |
Surgery
- Best between 3 and 8 years for cosmesis and function
- Excise the omovertebral bone and superomedial scapula
- Woodward procedure, release and inferior transfer of trapezius and rhomboid origins
- Green procedure, extraperiosteal release and scapular transfer
- Clavicle osteotomy reduces brachial plexus traction
Related pages
Author Contributions
Orthofracs team
Reviewed by Professor Phong Tran, Head of Orthopaedic Surgery, Western Health. Last updated 10 October 2026.