J Korean Orthop Assoc.  2013 Aug;48(4):319-323.

Sprengel Deformity with Bilateral Huge Omovertebra

• Yoon GW
• Chung SH

Abstract

Sprengel's deformity is characterized by the congenital migration of the scapula superiorly in relation to the thoracic cage. Other congenital anomalies, such as Klippel-Feil syndrome, may occur in combination with Sprengel's deformity. We report on a case of Sprengel's deformity with a huge bilateral omovertebrae, which was combined with the clinical features of Klippel-Feil syndrome, including cervical fusion, short neck, low posterior hairline, and limitation of neck motion. However, no other deformities or functional defects were observed.

Keyword

Sprengel deformity; Klippel-Feil syndrome

MeSH Terms

Congenital Abnormalities
Klippel-Feil Syndrome
Neck
Scapula
Shoulder Joint
Congenital Abnormalities
Scapula
Shoulder Joint

Figure

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  Figure 1 Sprengel's deformity can be seen on both sides. Abduction of the left shoulder is limited.

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  Figure 2 Computed tomography of the upper chest showed cervical fusion and bilateral Sprengel's deformity with omovertebra.

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  Figure 3 The size of the pathologic specimens were: (A) 10×6 cm; (B) 9×4 cm.

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  Figure 4 Postoperative result 15 months later showed stationary level of both scapulae.

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  Figure 5 Postoperative result 15 months later showed an improved abduction of the left shoulder.

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