Sprengel deformity

 

Sprengel

 

Definition

 

Rare congenital abnormality

Failure of descent of shoulder

 

Epidemiology

 

AD & Sporadic 

 

Tends to be in girls & on left

 

Associated with other congenital abnormalities

- scoliosis

- diastematomyelia 

- klippel-Feil Syndrome (usually bilateral)

- cervical ribs / fused or absent thoracic ribs

- thoracic vertebral anomalies

 

Etiology

 

Failure of descent of arm bud by 3rd fetal month

 

Clinical 

 

Scapula small and elevated

- superior angle rotated upwards & forwards

- shoulder musculature deficient

 

Limited shoulder abduction

- scapula joined to cervical spine by fibrous or bony bar

- Omovertebral bar

 

X-ray 

 

Small high scapula

 

Omovertebral bone - bone linking scapula and hyoid

 

Operative management

 

Indications

 

Cosmesis

Improve abduction range

 

Options

 

Scapula resection

Shoulder relocation

 

Scapula resection

 

Cosmetic procedure

- excision of prominent scapula above spine)

- excision of omovertebral bar

 

Scapula relocation

 

Woodward procedure

- clavicular osteotomy to protect brachial plexus

- excise omovertebral bar

- mobilise scapula caudally

- detach trapezius & rhomboids from spinous process insertion

- reattach more inferiorly to create dynamic inferior force

 

Green procedure

- clavicular osteotomy

- medial scapula release

 

Naik et al Int Orthop 2020

- 40 cases Sprengel treated with modified Green procedure

- mean age 5 years

- mean improvement abduction 36 degrees

- one transient brachial plexus injury

- complications:  wound gaping / pleural tear / scapula winging

 

Trisolino et al Children 2024

- 42 cases Sprengel treated with modified Green procedure

- +/- clavicular osteotomy

- clavicular osteotomy improved abduction by mean 25 degrees 

- complications 7%, 2 reoperations

- 70% high satisfaction