DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20151457

The sprengel deformity

Mohd E. Rasul, Annu V. Reddy

Abstract


Sprengel shoulder is a rare congenital deformity of one or both scapulae that is usually detected at birth. It occurs due to failure of the scapula to descend during intrauterine development. Although the deformity appears randomly most of the time, familial cases have been reported. Sprengel shoulder is often associated with Klippel-Feil syndrome and other congenital skeletal deformities. Anteroposterior X-ray imaging can accurately diagnose Sprengel deformity. However, computed tomography and magnetic resonance scans with three-dimensional reconstruction are nowadays used in everyday practice in order to diagnose concomitant abnormalities, study in detail the anatomy of the affected shoulder(s), and plan appropriate management. We present here our imaging experience from one pediatric case with Sprengel shoulder and take the opportunity to discuss this rare entity, which is, nevertheless, the commonest congenital defect of the scapula.


Keywords


Birth defects, High scapula, shoulder, Sprengel deformity, Undescended scapula

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References


Ozsahin M, Uslu M, Inanmaz E, Okur M. Bilateral congenital undescended scapula (sprengel deformity). Am J Phys Med Rehabil. 2012;91:374.

Pellegrin K, Kolkman KA, Waldemar KE. Sprengel deformity presenting as a post-traumatic injury in an afgan boy; A case report. Mil Med. 2013;178:e1379-83.

O’Brien SJ, Voos JE, Nevasier AS, Drakos MC. Developmental anatomy of the shoulder and anatomy of the glenohumeral joint. In: Rockwood CA, Matsen FA. The shoulder. 4th ed. Philadelphia, PA: Saunders/Elsevier; 2009.P 1-12.

Harvey EJ, Bernstein M, Desy NM, Saran N, Ouellet JA. Sprengel deformity: Pathogenesis and management. J Am Acad Orthop Surt. 2012;20:177-86.

Gonen E, Simsek U, Solak S, Bektaser B, Ates Y, Aydin E. Long term results of modified green method in Sprengel deformity. J Child Orthop. 2010;4:309-14.

Sprengel. Die angeborene Verschiebung des Schulterblattes nach oben. Archiv fur klin Chir Von Langenbeck. 1891;42:545-9.

Mallet JF, Bronfen C. Malformations de la ceinture scapulaire chez l’enfant et l’adolescent. EMC-Appareil locomoteur. 2002:7p[15-202-A-10].

Floemer F, Magerkurth O, Jauckus C, Lutschg J, Schneider JF. Klippel-Feil syndrome and Sprengel deformity combined with an intraspinal course of the left subclavian artery and a bovine aortic arch variant. AJNR Am J Neuroradiol. 2008;29:306-7.

Genin G. Anomalies osseuses constitutionnelles. Paris: Sauramps Medical. 2008.

Cho TJ, Choi IH, Chung CY, Hwang JK. The Sprengel deformity. Morphometric analysis usin 3D-CT and its clinical relevance. J Bone Joint Surg Br. 2000;82:711-8.

Fulbier L, Tanner P, Henkes H, Hopf NJ. Omovertebral bone associated with Sprengel deformity and Klippel-Feil syndrome leading to cervical myelopathie. J Neurosurg Spine. 2010;13(2):224-8.

Williams MS. Developmental anomalies of the scapula-the “omo” st forgotten bone. Am J Med Genet A. 2003;120A:583-7.

Mooney 3rd JF, White DR, Glazier S. Previously unreported structure associated with Sprengel deformity. J Pediatr Orthop. 2009;29:26-8.

Rigault P, Pouliquen JC, Guyonvarch G, Zujovic J. Surelevation congenital de l’omoplate chez l’enfant. Etude anatomopathologique et therapeutique a propos de 27 observations. Rev Chir Orthop Reparatrice Appar Mot. 1976;62:5-26.