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Orthopaedic Clinic

Sprengels Shoulder
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The scapula lies more superior than it should in relation to the thoracic cage and is usually hypoplastic and mis-shapened.

 

Etiology : The deformity is the result of failure of embryonic descent of the scapula from its fetal position in the neck to the normal position in the upper posterior thorax.

 

On Examination: The deformity is usually present at the birth but the severity varies. Involvement is usually unilateral and only very rarely bilateral in unilateral cases:

• Shoulder is asymmetric with high scapula.

• Trapezius & neckline is short.

• Scapula is hypoplastic with decreased vertical length.

• It’s shape is distorted with the supraspinous portion of the scapula tilted forwards.

• The extent of involvement of scapula should be determined, normal scapula extends from the second to the seventh or eight thoracic vertebrae, In Sprengl’s, the supero-medial tip may be as high as at C4 and inferior angle at D2.

 

In Bilateral cases:

Neck is very short and thick. The cervical lordosis is exaggerated and abduction of both the shoulders is limited. The Mobility of the scapula on thoracic cage is restricted because of :-

a) Fibrous adhesion between the scapula and the posterior wall.

b) Presence of omovertebral bone - usually palpated in the supraclavicular area

c) Due to forward filting of the spinous process restricting the scapulo-thoracic

motion.

Passive range of movement i.e combined abduction is limited because of loss of

Scapulo-costal motion, the range of motion at the gleno-humeral joint is however normal.

Active range of motion may be lost because of motor weakness of deltoid, supraspinatus and trapezius muscles.

 

Associated Anomalies:

 

1) Muscle: aplasia / fibrosis / contracture of trapezius, rhomboidus, levator scapulae, serratus anterior and deltoid.

Vertebral column: Klippel-Feil syndrome,

Hemivertebrae - congenital scoliosis, spina bifida, cervical spine, diastematomyelia.

 

2) Upper limb:

Clavicle :- Tillted upwards, hypoplastic

Humerus : Shortened.

Longitudinal deficency of tibia, absence of the rays.

Thoracic Cage - fusion of ribs, clavicle to ribs.

Viscera :- kidney - abscence, hypoplasia, ectopia, polycystic disease

Heart – situs inversus.

X-Rays: 1) A-P projection of both shoulders including the cervical and thoracic spine  with the arms at the side and normal abduction.

2) Oblique lateral and 3) Lateral view to demonstrate the omo-vertebral bone.

 

Alternatively CT Scan can be done – for demonstration of Omo-vertebral bar.

MRI for evaluation of spinal pathology

USG Abdomen for renal anomalies.

 

Cavendish Grading:

I - Scapula is almost level and the deformity is not detectable when the

patient is fully clothed.

II - The shoulders are uneven with affected scapula 1-2 cm higher than normal opposite scapula.

III – 2 - 5 cm higher, deformity is unsightly and easily detectable.

IV - > 5cm, superior angle is near the occiput with marked webbing of the neck.

 

Surgeries

— Woodward’s

— Green

 

Green -Described the release of the muscle from the scapula and excision of the supraspinates portion of the scapula and any omo-vertebral bone that is present is excised extra-periosteally.

Klisic’s Modification – osteotomy of the clavicle which lower the scapula and prevents traction to the neurovascular structure (brachial plexus)

 

Woodward - Trapezius and rhomboids are detached from their origin and transferred

distally, omo-vertebral bar if present is excised.

Most common complication is brachial plexus injury which can be prevented by

morsellization of clavicle as first step.

 

Methods of maintaining reduction:-

— Anchored to the rib cage with sutures

— Leibovic modification - suturing the scapula into a pocket in Lattismus dorsi.

 

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