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Diseases

Genetic and Rare Diseases Information Center (GARD)

Sprengel deformity


Other Names for this Disease
  • Congenital upward displacement of the scapula
  • High scapula
  • Sprengel's shoulder
  • Congenital elevation of the scapula
  • Sprengel's deformity
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Treatment

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How might Sprengel deformity be treated?

Treatment of Sprengel deformity depends on the degree of functional impairment and/or cosmetic disfigurement. Many people with Sprengel deformity do not need surgery and may have physical therapy to maintain range of motion and strengthen weak muscles.[1]

For those who do require surgery, the goals are to release the binding of the scapula and relocate the scapula.[1] Surgery can improve the cosmetic appearance and contour of the neck, and improve the scapular function when it is severely impaired. However, the ability to increase shoulder abduction is limited.[1]

For surgical indication, many experts refer to the Cavendish classification - one method used for grading the severity of Sprengel deformity.[2] This method classifies the condition into grades I through IV, with grade I being the most mild (almost invisible when covered with clothes) and grade IV being the most severe (with over 5 centimeters of elevation of the shoulder, and neck webbing).[3] Although no improvement or worsening has been reported in untreated grade I and II cases, surgery is recommended in grade III and IV deformities. However, the Cavendish classification may be subjective and inaccurate since it is based on the structure of the deformity (rather than function) and aesthetic criteria.[2]

The optimal age for surgery is controversial, but most experts recommend that it be done before age 8 to obtain the best surgical result. There are several surgical options that may be considered depending on each person's situation. Many of the surgical procedures for Sprengel deformity leave unsightly scars, so the cosmetic improvement must be carefully considered.[1]
Last updated: 1/6/2016

References
  1. Mihir M Thacker. Sprengel Deformity. Medscape Reference. November 7, 2014; http://emedicine.medscape.com/article/1242896-overview.
  2. Wada A, et. al. Sprengel deformity: morphometric assessment and surgical treatment by the modified green procedure. Journal of pediatric orthopedics. January, 2014; 34(1):55-62.
  3. Elena Trajcevska et. al. Sprengel Deformity. Radiopaedia. 2016; http://radiopaedia.org/articles/sprengel-deformity.


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Clinical Trials & Research for this Disease

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
Other Names for this Disease
  • Congenital upward displacement of the scapula
  • High scapula
  • Sprengel's shoulder
  • Congenital elevation of the scapula
  • Sprengel's deformity
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.