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Sprengel’s Deformity

 

synonyms:

Sprengel’s Deformity ICD-9

  • 755.52 (congenital elevation of the scapula; Sprengel's deformity)

Sprengel’s Deformity Etiology / Epidemiology / Natural History

  • Failure of scapular descent during embryologic development.
  • Right = left
  • @ 20% bilateral

Sprengel’s Deformity Anatomy

  • scapula lies at level of C4-5 at 5thweek of gestation and migrates caudally below T3 by 12 wks
  • failure of descent results in high, small and wide scapula that is malrotated and limits scapulthoracic motion most notable in abduction(90-100 degrees)
  • 50% of pts have a fibrous band, cartilaginous bar, or omovertebral bone extending from vertebral border of superior angle of scapula to a posterior element of 4th-7th cervical vertebrae

Sprengel’s Deformity Clinical Evaluation

  • Hypoplastic, high riding scapula +/- decreased scapulothoracic motion.
  • may cause neck pain or cosmetic concerns

Sprengel’s Deformity Xray / Diagnositc Tests

Sprengel’s Deformity Classification / Treatment

  • Cavendish Classification (Cavendish ME, JBJS 1092;54Br:395).
  • Grade 1: glenohumeral joints lefel, no deformity visible with patient is dressed.
    Treatment: none generally needed.
  • Grade 2: gelnohumeral joints leel, prominence in the neck visible when dressed.
    Treatment: consider resection of scapular prominence and omovertebral bar mainly for cosmesis.
  • Grade 3: shoulder elevation 2-5cm above contralateral side
    Treatment: resection of supierior margin of the scapula and omovertebral bar +/- derotation and caudal repositioning of the scapular.
  • Grade 4: scapula lies at level of occiput
    Treatment: resection of supierior margin of the scapula and omovertebral bar with derotation and caudal repositioning of the scapular.
  • Modified Green procedure=excision of supraspinous portion of scapula and omovertral bone vs relocation of scapula. Average 75 degree improvement in abduction (Bellemans M, JPO 8:194;1999).
  • Woodward procedure: detachment and relocation of the parascdapular musculature.
  • Vertical osteotomy (McMurtry I, JBJS 2005;87Br:986).
  • Older children may require claivicular osteotomy to prevent iatrogenic nerve injury

Sprengel’s Deformity Associated Deformities

  • Klippel-Feil syndrome
  • Scoliosis
  • Torticolllis
  • Facial asymmentry
  • Hemivertebrae
  • Rib synostosis
  • Clavicular abnormalities
  • Renal anomalies
  • Pulmonary disorders
  • Shoulder musculature hypoplasia

Sprengel’s Deformity Complications

Sprengel’s Deformity Follow-up Care

Sprengel’s Deformity Review References

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