Sprengel shoulder

1. Dr. Gokul Dev C
JR 1, Dept. Of Orthopaedics
KMCT Medical College

2.  Congenital Elevation Of Scapula
 Congenital High Scapula
 Scapula Elevata
 Earliest description : Eulenberg (1863)
 Named after Otto Gerhard Karl Sprengel
(1852-1915) , a German surgeon who
described four cases in 1891.

3.  Rare - Most common congenital
shoulder anomaly in children
 Typically congenital
 May not become apparent until grows older
 Girls > Boys (3:1)
 Unilateral > Bilateral
 Bilateral in 10–30%
 Right scapulae = left scapulae
 Sporadic (Rarely – run in families : AD Trait)

6.  Develops embryologically with the upper limb
 Appears - 5th week of gestation along with arm
bud (opposite to C4,C5,C6)
 Descends to the final anatomical position (T2-T8
vertebrae) by 12 weeks of gestation
 Develop from pleuripotent mesenchymal cells
 Cellular signaling pathways guide growth and
development of scapula, surrounding muscles,
bones, and nerves.

7.  Results from failure of scapular descent.
 Usually during the 9-12 th week
 The scapula remains
 Hypoplastic
 High-riding
 Medially rotated
 With varying degrees of
reduced scapulo-thoracic movement.
 Bone, Cartilage and Scapular muscles poorly
developed and are replaced by fibrous bands.

8.  The exact, underlying cause - unknown.
 ? Interruption of embryonic subclavian blood
supply
 At level of subclavian, internal thoracic or
suprascapular artery

9.  Deformity is cardinal symptom - Painless
 Variable loss of ROM of Arm, Shoulder Blade and
Cervical Spine
 Affected scapula appears
 Hypoplastic/Dysplastic and high riding into the C spine
 Increased width to height ratio
 Inferior pole is rotated medially with glenoid pointing
inferiorly - thus restricting gleno-humeral abduction
(≤ 90°)
 Convexity of upper part of scapula increased, curvature
of clavicle shaft reduced – narrow scapulo-clavicular
space – leads to brachial plexus injury
 Other associated abnormalities

10. • Omovertebral bar/bone
• Omoclavicular bars
• Klippel-Feil syndrome (35% with Sprengel deformity)
• Kyphosis, Scoliosis
• Spina bifida
• Torticollis , Brevicollis
• Scapular winging
• Rib anomalies (fused or absent ribs)
• Facial asymmetry
• VACTERL association
• Foot deformities

11.  In about 50% of patients
 An abnormal bridge between the superior
scapular angle and the cervical spine (spinous
process/lamina/transverse process)
 Omovertebral Bar: fibrous/cartilaginous bridge
 Omovertebral Bone: trapezoid/rhomboidal bone

12.  Brachial plexus palsy
 Rickets
 Osteomalacia
 Paralysis (long thoracic nerve – Winging of Scapula)
 Malunited scapular fractures
 Scoliosis

13. Examine both sides of patient
 Active movements of shoulder
 Passive movements
 Scapulo - humeral rhythm
 Scapular control
 Resisted isometric movements
 Functional assessment
 Special tests - DRST (Dynamic Rotatory Stability
Test), Apley's scratch test, Rowe’s sign, Gagey
hyperabduction test

14.  X-ray : Shoulder AP
 Confirm diagnosis
 Omovertebral bone – may be seen
 Presence of associated abnormalities.
 MRI or CT Scan
 Identify omovertebral connection,
 Scapula dysplasia/ Position
 Used in preoperative planning
 Diagnosed prenatally - USG

15.  The Radiographic RIGAULT CLASSIFICATION
 Grade I: Superomedial angle lower than T2 but
above T4 transverse process
 Grade II: Superomedial angle located between
C5 and T2 transverse process
 Grade III: Superomedial angle above C5
transverse process

18.  Non operative – Observation + Physiotherapy
 Mild cases (no shoulder dysfunction)
 No severe cosmetic concerns
 Cavendish score of 1-2
 Annual observation till skeletal maturity, to
assess for progression of deformities
 Operative – Surgical fixation
 For conspicuous deformities with disability
(abduction < 110-120)
 Severe cosmetic concerns
 Cavendish score of 3-4
 To decrease deformity and improve shoulder
function

19.  Based on Cavendish classification
 Watch for spinal abnormalities prior
 Age of surgical intervention - often debated.
 Early intervention - maximal correction during
further growth.
 But child may not bear extensive nature of
intervention.
 Age of at least 3 years - the optimal time for
intervention (3 to 6 yrs).

20.  Putti’s Procedure
 The Woodward Procedure
 The Green Procedure
 Wikinson’s Osteotomy
 Mear’s Procedure

21. Surgical Principles
 Release the scapular tethers by resection of the
superior angle (superomedial) of scapula and
omovertebral bar.
 Scapula is derotated and relocated to a more
caudal position by shift of either the origin or
insertion of the scapular muscles.
 To relieve compression of brachial plexus and
subclavian artery
 Morcellation of the clavicle (mid clavicular
resection osteotomy ) is performed
 In older patients ( > 8 years)

22.  To prevent brachial plexopathy when mobilizing
the scapula
 The surgeon must take reference from
contralateral scapular spine and not from
inferior margin on affected side

23. Additional Procedures
 Bony Resection
 Extraperiosteal resection of proximal scapular
prominence
 For cosmetic concerns
 May be done with other procedures or alone
 Clavicular Osteotomy / Morcellization.
 To reduce risk of neurovascular injury and to
provide anterior release.
 Helps in additional scapular descent.
 Usually done in older children (>8 years)
 Done along with other procedures

24.  Detatchment of scapular insertion of the para-
scapular muscles (Rhomboids and Trapezius)
 Omovertebral bar resection
 Lowering the scapula and fixing its inferior angle
to a rib at the corrected level
 Not done now
 Shrock Modified Putti’s Procedure
 Subperiosteal resection of the musculature
 Adding an osteotomy of supraspinous scapular
region and the acromial base
 To facilitate scapular descent.

25.  Most commonly used
 For correction of a moderate or severe Sprengel’s
deformity
 Relocates scapula by detachment and caudal
relocation of the midline origin of the para-
scapular muscles (Trapezius and Rhomboids)
 The spine of scapula kept at the same level as that
on the opposite side
 Clavicular osteotomy allows
additional scapular descent.
 Cervical spine anomalies – Negative affect on
outcome

27.  Borges Modification of the Woodward Procedure
 The superomedial scapular prominence resected
 Better abduction and correction of glenoid tilt/
vara.
 Scapula anchored to lower dorsal vertebrae by a
stout absorbable suture
 Good cosmetic and functional improvement (can
improve abduction by 40-50 degrees).
 Better results
 The muscles are incised farther from scapula
 lowers risk of formation of scar keloid.
 A larger mobilization is possible.
 The postoperative scar is not as thick as with Green’s
procedure.

29.  Scapula is derotated by extraperiosteal
detachment of scapular muscles at scapular
insertion
 Resection of the supraspinous part of scapula and
omovertebral bone (if present)
 Scapula is laid free
 Caudal relocation of scapula and fixation using
scapular traction cables done
 The parascapular detached muscles are
reattached.
 The traction wire is removed after 3 weeks
 Can improve abduction by 40-50 degrees

30.  Leibovic Modification of The Green Technique
- The reduced scapula is secured in a pocket
developed in the latissimus dorsi muscle
 Belleman’s and Lamoureux Modification of The
Green Technique
- Serratus anterior muscle is not released
- Immediate postoperative mobilization
encouraged
 Andrault Modification of The Green Technique
- Dis-insertion of supraspinatus
- Clavicular osteotomy
- A limited release of serratus anterior to facilitate
the descent of scapula.

32.  Vertical Scapular Osteotomy
 Lateral half of scapula is brought down caudally
 With concomitant Clavicular Osteotomy or
Morcellization.
 Performed before movement of scapula
 The correction achieved is limited (compared to
other procedures).

34.  Subperiosteal release of muscle insertions on the
superomedial aspect of scapula
 Extraperiosteal resection of the omovertebral
bone
 Osteotomy of the supraspinatus fossa
 Release of long head of triceps and part of the
origin of teres minor
 Inferomedial resection of scapula
to 160° of shoulder abduction
 The gain in shoulder abduction is
more as compared to others.

35.  Straight incision over clavicle extending from 1.5
cm lateral to sternoclavicular joint to 1.5 cm medial
to acromioclavicular joint.
 Expose clavicle subperiosteally.
 Divide the bone 2 cm from each end, remove it,
and cut it into small pieces (morcellate).
 Replace the pieces in the periosteal tube and close
the tube with interrupted sutures.

36.  Brachial plexus injury
 Injury to blood vessels
 Regrowth of the superior pole of the scapula
 Hypertrophic scar / Keloid
 Winging of scapula due to incomplete
reattachment of the serratus anterior

37.  Not favorable – even after surgical management
 Literatures indicate – mobility of the shoulder may
be improved, but the asymmetry almost always
persists.

38.  The arm is supported with a immobilizer
postoperatively
 Gentle passive range of motion of the shoulder &
scapula exercises are started.
 Suitable pain relief modality TENS, IFT, hot packs
used to induce relaxation
 A sling is used after 3 weeks.
 Gradually, active ROM and strengthening exercises
are instituted

39.  Special attention is given to achieve early mobility
of scapula and the shoulder abduction, elevation.
 Physiotherapy is continued for up to 6 months

40. 1. Apley’s System Of Orthopaedics & Fractures Tenth
Edition.
2. Campbell's Operative Orthopaedics - 14th Edition
3. Essential Orthopedics Principles & Practice By
Manish Kumar Varshney.
4. Pubmed: Kadavkolan et. Al. Sprengel's deformity of
the shoulder: Current perspectives in management.
Int J Shoulder Surg. 2011 Jan;5(1):1-8. doi:
10.4103/0973-6042.80459.