IS Case 566: Sprengel's deformity
Imaging Sciences URMC 2010
Publication Date: 2011-11-16
History
Patient is a 9-year-old girl with neck pain.
Findings
AP view of the upper thorax including bilateral scapula demonstrated an elevated right scapula compared to the left scapula, consistent with Sprengel's deformity.
Diagnosis
Sprengel's deformity
Discussion
Sprengel’s deformity is a congenital deformity of the scapula in which the scapula is elevated than its expected location. The scapula differentiates at the level of C4-C6 cervical vertebrae and then descends down to the thorax by the third month of gestation. Sprengel’s deformity is caused by the failure of the scapula to descend to the thorax. The scapula is usually about 2-10cm elevated.
Sprengel’s deformity is commonly associated with other deformities of cervical or thoracic vertebrae-like fused ribs, fused vertebrae, scoliosis, spina bifida and Klippel-Feil syndrome (fused cervical spine, short webbed neck, decreased range of motion of cervical spine and low hairline).
The definitive treatment for Sprengel’s deformity is surgical. In patients with only minimal effect on range of motion, physical therapy is used to help maintain range of motion. Surgery is reserved for patients with marked disability and surgery at a young age (usually before 8 years of age) provides best results.
References
- Thacker MM, Feldman DS. Sprengel deformity. eMedicine, Jul 8, 2009. http://emedicine.medscape.com/article/1242896-overview
- Cho TJ, Choi IH, Chung CY, Hwang JK. The Sprengel deformity. Morphometric analysis using 3D-CT and its clinical relevance. J Bone Joint Surg Br. 2000 Jul;82(5):711-8. PMID: 10963171
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