عيوب لوحة الكتف الخلقيه- sprengel's deformity - البروفيسور...

11/1/2014

Professor Freih Abuhassan -

University of Jordan 1

Sprengel's deformity


Freih Odeh Abu Hassan

FRCS (Eng.), FRCS (Tr. & Orth.)

Professor of Orthopedics

University of Jordan - Amman

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University of Jordan

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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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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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Other terms

1-Sprengel's anomaly,

2-Sprengel's shoulder,

3-Congenital high scapula,

4-Undescended scapula.

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ProblemComplex 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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Associated anomalies1-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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The gross pathology1-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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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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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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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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Associated Syndromes•Klippel-Feil syndrome

•Greig syndrome

•Poland syndrome

•VACTERL

•Velocardiofacial syndrome

•Floating-harbor syndrome

•Goldenhar syndrome

•Mental disturbance syndrome

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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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Preoperative investigations1-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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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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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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2-Woodward procedure

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3-Mears procedurePartial resection of the scapula and a release

of the long head of triceps.

4-Scapular Osteotomy

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Complications of surgical treatment of


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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5=Neurovascular complications

resulting from compression between the

clavicle and first rib when the scapula was

displaced inferiorly.

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JournalSurgery NoPeriod Author

JPO-A, 1996Modified Woodward 1612YearBorges etal

2001JPO-A, Partial Resection 815yearMears

JBJS-B, 1983Woodward1213yearGrogan etal

Acta Orthop Scand 1993 Woodward2320 yearGreitemann etal

Acta Ortho. Belgica, 2005Modified Green1214yearAydinli etal

JBJS-B, 2005Osteotomy1216yearMcMurtry etal

JPO-B, 2002Modified Woodward 1915yearKhairouni etal

JBJS-A, 1981Woodward1112yearCarson etal

JPO-A, 1981Modified Green2820yearKlisić etal

JBJS-B, 1980Osteotomy1610yearWilkinson

JBJS-A, 1990Modified Green1816yearLeibovic etal

Clin Orthop Relat Res. 2007 Partial scapulectomy26year18Zhang etal

209 cases =1980-200711/1/2014 27



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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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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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6 CASES

7 CASES

NoCavendish

0Grade 1

0Grade 2

6Grade 3

7Grade 4 11/1/2014 32



Indications of surgery=Marked deformity

=Restriction of ROM

=Both

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Surgical Technique

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Trapezius reflection

Trapezius reflection

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Supraspinatus release

Rhomboids, Levator release

Supraspinatus release

Rhomboids, Levator release

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11/1/2014 49Professor Freih Abuhassan -


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Associated KF Synd.

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11/1/2014 55Professor Freih Abuhassan -


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Conclusion

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عيوب لوحة الكتف الخلقيه- sprengel's deformity - البروفيسور...

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