Sprengel's deformity is a congenital anomaly where the scapula is abnormally high. The document discusses the embryology, presentation, associated anomalies, evaluation and surgical treatment of Sprengel's deformity. It focuses on the modified Woodward procedure used to surgically treat 13 patients with Sprengel's deformity at a hospital in Jordan between 1999-2006. The procedure aims to release muscles and reposition the scapula, with the goal of improving shoulder mobility.

1. Sprengel's deformity
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Professor Freih Abuhassan -
University of Jordan 1

2. Sprengel's deformity
Freih Odeh Abu Hassan
FRCS (Eng.), FRCS (Tr. & Orth.)
Professor of Orthopedics
University of Jordan -Amman
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Professor Freih Abuhassan -
University of Jordan

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Professor Freih Abuhassan -
University of Jordan 3

4. Cong. failure of descent of the scapula.
Eulenburg first described it in 1863.
Willet & Walsham described the omovertebral
bone in 1883
Sprengel described 4 cases in 1891.
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Professor Freih Abuhassan -
University of Jordan

5. Embryology:
=The scapula formed from paracervical
mesoderm at level of C4-5.
= Normal location post migration at 9th -
12th week between 2nd-8th ribs posteriorly.
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University of Jordan

6. Other terms
1-Sprengel's anomaly,
2-Sprengel's shoulder,
3-Congenital high scapula,
4-Undescended scapula.
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Professor Freih Abuhassan -
University of Jordan

7. Problem
Complex anomaly associated with.
1-Malposition and dysplasic scapula.
2-Regional muscle hypoplasia or atrophy,
3-Disfigurement
4-Limitation of shoulder movement.
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Professor Freih Abuhassan -
University of Jordan

8. Associated anomalies
1-Absent or fused ribs
2-Klippel-Feil syndrome
3-Congenital scoliosis.
4-Syringomyelia
5-Diastematomyelia 20%
6-Kidney anomalies.
7-Omovertebral bone 25%
(connecting the superomedial scapula to the
post. elements of the cervical spine).
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Professor Freih Abuhassan -
University of Jordan

9. The gross pathology
1-The scapula is small, dysplastic and located
higher than normal
2-Inferior angle is medially rotated
3-Limited rotation of the scapula
4-Convex supraspinous portion of the scapula
5-Curvature of the clavicular shaft is decreased,
forming a narrower scapuloclavicular space,
which may contribute to brachial plexus
compression postoperatively.
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Professor Freih Abuhassan -
University of Jordan

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Professor Freih Abuhassan -
University of Jordan

11. 6-Omovertebral connection
Fibrous, cartilaginous, or bony connection extends
from the superomedial border of the scapula to the
spinous processes, lamina, or transverse processes
of the cervical spine,most commonly the C4-C7.
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University of Jordan

12. 7-Associated abnormal muscles.
1-Trapezius, rhomboid, levator scapulae muscle
2-The serratus anterior muscle may be weak,
3-Pectoralis major, LD, or Sternocleidomastoid.
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University of Jordan

13. Clinically
=Minimal, with no restriction of shoulder motion
=Severe, with the superior angle of the scapula near the
occiput, and marked restriction of the scapulothoracic
motion.
=The omovertebral bone is associated with greater
restriction of motion.
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University of Jordan

14. Associated Syndromes
•Klippel-Feil syndrome
•Greig syndrome
•Poland syndrome
•VACTERL
•Velocardiofacial syndrome
•Floating-harbor syndrome
•Goldenhar syndrome
•Mental disturbance syndrome
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University of Jordan

15. Cavendish classification
•Grade 1: The deformity is very mild.
•Grade 2: The deformity is mild.
superomedial portion visible as a lump.
•Grade 3: The deformity is moderate.
shoulder is elevated 2-5 cm .
•Grade 4: The deformity is severe.
superomedial angle at the occiput,
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University of Jordan

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University of Jordan

17. Preoperative investigations
1-Radiographs of both shoulders, cervical and
thoracic spine
2-CT scan for omovertebral bone.
3-MRI for spina bifida occulta or an intraspinous
lesion.
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Professor Freih Abuhassan -
University of Jordan

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University of Jordan

19. Operation indicated in
=Marked deformity
=Restriction of motion is severe
=Cosmesis
= Age <6-8 years
= Can be done for older age group
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Professor Freih Abuhassan -
University of Jordan

20. Surgical procedures
1- Modified Green scapuloplasty
= Clavicle osteotomy
=Reflection of trapezius from spine of the
scapula
= Supraspinatus detached extraperiosteally
= The omovertebral bar is then excised
= All attached muscles are extraperiosteally
released.
= Reduction to scapula then suture muscles.
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University of Jordan

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22. 2-Woodward procedure
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23. 3-Mears procedure
Partial resection of the scapula and a release
of the long head of triceps.
4-Scapular Osteotomy
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University of Jordan

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25. Complications of surgical treatment of
Sprengel's deformity
1= Loss of correction.
2= Winging of the scapula.
3= Regeneration of the excised
portion of the scapula.
4= Prominent scars.
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University of Jordan

26. 5=Neurovascular complications
resulting from compression between the
clavicle and first rib when the scapula was
displaced inferiorly.
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Professor Freih Abuhassan -
University of Jordan

27. Author Period No Surgery Journal
Borges etal 12Year 16 Modified Woodward JPO-A, 1996
Mears 15year 8 Partial Resection JPO-A, 2001
Grogan etal 13year 21 Woodward JBJS-B, 1983
Greitemann etal 20 year 23 Woodward Acta Orthop Scand 1993
Aydinli etal 14year 12 Modified Green Acta Ortho. Belgica, 2005
McMurtry etal 16year 12 Osteotomy JBJS-B, 2005
Khairouni etal 15year 19 Modified Woodward JPO-B, 2002
Carson etal 12year 11 Woodward JBJS-A, 1981
Klisić etal 20year 28 Modified Green JPO-A, 1981
Wilkinson 10year 16 Osteotomy JBJS-B, 1980
Leibovic etal 16year 18 Modified Green JBJS-A, 1990
Zhang etal 18year 26 Partial scapulectomy Clin Orthop Relat Res. 2007
1980-2007 = 209 cases
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University of Jordan

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29. Between 1999 – 2006
15 modified woodward procedures were
performed on 13 patients at the JUH.
10 Girls and 3 Boys.
Age range at the time of operation,
(3.3–10 years) mean: 6.11 years
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31. 12 of the 13 patients had an associated congenital
anomaly.
7 of these 12 patients had more than one associated
abnormality.
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University of Jordan

32. 7 CASES
6 CASES
Cavendish No
Grade 1 0
Grade 2 0
Grade 3 6
Grade 4 7 Professor Freih Abuhassan -
University of Jordan
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33. Indications of surgery
=Marked deformity
=Restriction of ROM
=Both
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University of Jordan

34. Surgical Technique
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37. Trapezius reflection
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38. Trapezius reflection
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39. Supraspinatus release
Rhomboids,
Levator release
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40. Supraspinatus release
Rhomboids,
Levator release
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51. Associated KF Synd.
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60. Conclusion
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University of Jordan