Abstract
Sprengel’s deformity is a congenital anomaly characterized by a hypoplastic and elevated scapula that results in cosmetic and functional impairment. In this study, we report the results of 3 severe cases of bilateral Sprengel’s deformity with associated Klippel-Feil syndrome treated by the modified Woodward procedure. Between 2013 and 2015, 3 patients of a mean age of 5.4 years were diagnosed with bilateral Sprengel’s deformity and associated Klippel-Feil syndrome. All the shoulders were graded as Cavendish 4 and had a bar connecting the scapula with the cervical vertebrae. Patients were managed surgically with the modified Woodward procedure and a clavicular osteotomy. The mean follow-up time was 62 months. No intraoperative or postoperative complications were recorded and all patients remained neurologically intact. The average improvement of the abduction of the shoulders was 30° and the mean lowering of the scapula was 2.6 cm. All patients improved by at least 1 Cavendish grade. The modified Woodward procedure is a reliable and effective technique to improve shoulder cosmesis and function even in severe cases of bilateral Sprengel’s deformity with associated Klippel-Feil syndrome.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Eulenberg H. Beitrag zur dislocation der scapula. Amtl Ber Deutscher Naturforsch Aertze Karlstad. 1863;37:291–4.
Sprengel O. Die Angeborene Verschiebung des Schullterblaters nach oben. Arch Klin Chir. 1891;42:545–9.
Samartzis D, Herman J, Lubicky JP, Shen FH. Sprengel’s deformity in Klippel-Feil syndrome. Spine. 2007;32(18):E512–6.
Harvey EJ, Bernstein M, Desy NM, Saran N, Ouellet JA. Sprengel deformity: pathogenesis and management. J Am Acad Orthop Surg. 2012;20(3):177–86.
Bavinck JN, Weaver DD. Subclavian artery supply disruption sequence: hypothesis of a vascular etiology for Poland, Klippel-Feil, and Mobius anomalies. Am J Med Genet. 1986;23(4):903–18.
Staheli LT. Fundamentals of Pediatric Orthopedics. Wolters Kluwer: Fifth Edition; 2015.
Kadavkolan AS, Bhatia DN, Dasgupta B, Bhosale PB. Sprengel’s deformity of the shoulder: current perspectives in management. Int J Shoulder Surg. 2011;5(1):1–8.
Carson WG, Lovell WW, Whitesides TE Jr. Congenital elevation of the scapula. Surgical correction by the Woodward procedure. J Bone Joint Surg Am. 1981;63(8):1199–207.
Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg Br. 1972;54(3):395–408.
Woodward JW. Congenital elevation of the scapula: correction by release and transplantation of muscle origins: a preliminary report. J Bone Joint Surg Am. 1961;43:219–28.
Stelzer JW, Flores MA, Mohammad W, Esplin N, Mayl JJ, Wasyliw C. Klippel-Feil Syndrome with Sprengel deformity and extensive upper extremity deformity: a case report and literature review. Case Rep Orthop. 2018;2018:5796730.
Mooney JF 3rd, White DR, Glazier S. Previously unreported structure associated with Sprengel deformity. J Pediatric Orthop. 2009;29(1):26–8.
Farsetti P, Weinstein SL, Caterini R, De Maio F, Ippolito E. Sprengel’s deformity: long-term follow-up study of 22 cases. J Pediatric Orthop B. 2003;12(3):202–10.
Jeannopoulos CL. Congenital elevation of the scapula. J Bone Joint Surg Am. 1952;34 A(4):883–92.
Borges JL, Shah A, Torres BC, Bowen JR. Modified Woodward procedure for Sprengel deformity of the shoulder: long-term results. J Pediatric Orthop. 1996;16(4):508–13.
Grogan DP, Stanley EA, Bobechko WP. The congenital undescended scapula. Surgical correction by the woodward procedure. J Bone Joint Surg Br. 1983;65(5):598–605.
Availability of Data and Material
The data that support the findings of this study are available from the corresponding author (Frideriki Poutoglidou) upon reasonable request.
Code Availability
Not applicable.
Author information
Authors and Affiliations
Contributions
Frideriki Poutoglidou assisted with the surgical operations, collected the data, drafted the manuscript, provided final approval of the version to be published, and has agreed to be accountable for all the aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Dimitrios Metaxiotis performed the surgical procedures, drafted the manuscript, and provided final approval of the version to be published.
Dimitrios Alvanos assisted with the surgical operations, helped shape the final manuscript, and provided final approval of the version to be published.
Anastasios Mpeletsiotis participated in the drafting of the manuscript and provided critical feedback and final approval of the version to be published.
Corresponding author
Ethics declarations
Ethical Approval
This retrospective study was approved by our hospital’s Institutional Review Board.
Consent to Participate
Not applicable.
Consent for Publication
The patient’s parents have given their consent to use the patients’ medical data in this publication.
Conflict of Interest
The authors declare no conflict of interest.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Poutoglidou, F., Metaxiotis, D., Alvanos, D. et al. Bilateral Sprengel’s Deformity Treated with the Modified Woodward Procedure: Technique and Outcome of 3 Consecutive Case Reports. SN Compr. Clin. Med. 3, 754–758 (2021). https://doi.org/10.1007/s42399-021-00781-y
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s42399-021-00781-y