Sprengel Shoulder

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