The scapula lies more superior than it should in relation to the thoracic cage and is usually hypoplastic and
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
• 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
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
Active range of motion may be lost because of motor weakness of deltoid, supraspinatus
and trapezius muscles.
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
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.
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
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.
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
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.