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Sprengel's Deformity

Aetiology

  • Scapulae normally complete their decent from C5 in the neck to T5, by the third month of fetal life
  • Arrest of decent may occur due to either fibrous or bony tissue, which causes congenital undescended scapula

Classification

Grade 1: (very mild) shoulders are level and deformity is minimal when the patient is dressed
Grade 2: (mild) shoulders are almost level and deformity is a lump in the web of the neck
Grade 3: (moderate) shoulders are elevated 2 - 5 cm and the deformity is easily visible
Grade 4: (severe) shoulders are quite elevated, superior angle scapula is near the occiput

Clinically

  • Associated deformities of the cervical spine are common
  • May be a family history of scapular deformity
  • Deformity is the only symptom and may be noticed at birth
  • Scapula is hypoplastic and usually shaped like an equilateral triangle in a malrotated position, with the glenoid facing down, decreasing the range of abduction
  • Gleno-humeral motion is not restricted, but scapulo-thoracic motion may be severely affected
  • 1o of upward rotation of the scapula occurs with 2o of abduction of the humerus
  • 1/3 cases have full range of abduction, 1/3 have ranges from 135 - 180o and 1/3 have ranges from 90-135o
  • 30% are associated with a omovertebral bone
    • Extends from the superior angle of the scapula to the spinous process, lamina or transverse process of the lower cervical vertebrae
    • If the omovertebral bone is attached high in the cervical spine, cervical movement will be limited
  • Shoulder on the affected side is elevated usually between 2 - 10 cm 
  • Scapula looks and feels abnormally high. It is also smaller than normal and more prominent
  • Occasionally a bilateral condition (15%) leads to less obvious deformity
  • Associations
    • Scoliosis 40%
    • Rib abnormalities 25%; including cervical ribs, torticollis
    • Facial asymmetry
    • Renal anomalies
    • Abnormal musculature; atrophy or complete absence commonly occurs, esp. pectoralis major, rhomboids, serratus anterior and latisimus dorsi
    • Cervical spine anomalies 20%
    • Klippel-Feil syndrome (there is bilateral failure of scapular descent, marked anomalies of the cervical spine and failure of fusion of the occipital bones)
    • Diastomatomyelia

X-Rays

  • Associated deformities such as fusion of cervical vertebrae, kyphosis, scoliosis
  • May show a bony bridge between the scapula and the cervical spine (omovertebral bone)

Treatment

  • Concerns of the patient are usually cosmetic
  • In some cases restriction of the ROM is an indication for surgery
  • Ideal age for surgery is between 3 years (muscles developed enough to enable dissection) and 6 years (before the scapula becomes too fixed in its abnormal position)
  • Mild cases require no treatment
  • Excision of the omovertebral bone, when present, and the supra-spinous part of the scapula improves appearance and function

Operations

  • Excision of the omovertebral bone alone has not been an effective treatment of Sprengel's shoulder
  • Green procedure
    • Reflection or division of the trapezius, and muscles inserted into the vertebral border of the scapula
    • Excision of the omovertebral bone
    • Scapula is pulled down and the muscles reattached, with the scapula in its new position
    • Should be done in association with morcelisation of the middle 1/3 of the clavicle to avoid complications
  • Woodward procedure
    • Trapezius, rhomboids and levator scapulae are divided from their insertion into the spine and reattached at a lower level
    • Excision of the omovertebral bone. Subperiosteal resection of the omovertebral bone causes no recurrence
    • Should be done in association with morcelisation of the clavicle. Surgical relocation of the scapula may lead to scissoring of the brachial plexus and / or subclavian artery between the clavicle and first rib, therefore must morcelise the middle 1/3 of the clavicle prior to commencing the posterior surgery

Prognosis

  • Average increase in motion following Woodward procedure was 50o
  • Average scapular lowering was 1.6 cm 
  • Cosmetic result rated good or excellent in 82%
  • Younger patients obtain the best range of motion 
  • 60% of patients developed unsightly surgical scars
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