eMedicine Specialties > Orthopedic Surgery > Hand & Upper Extremity
Sprengel Deformity: Follow-up
Updated: Jul 8, 2009
Outcome and Prognosis
Prognostic factors include the following:
- Severity of the deformity
- Age at surgery: Generally, results of surgery in children older than age 6 years are not as good.
- Type of procedure: Relocation surgeries have better functional outcomes.
- Associated anomalies: Anomalies such as Klippel-Feil syndrome compromise the eventual result.
Future and Controversies
Future
In a report by Mears, the author described a novel approach that included an oblique plane osteotomy of the scapular body, along with release of the long head of triceps from the scapula.36 He reported a significant improvement in function following this procedure.
Controversies
Surgical correction in older patients (>8 y) is controversial, and the results of surgery are not as good. However, in a study by Doita et al, the authors had good results after surgical correction in 2 adults, and they recommended surgery even in older patients.17
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References
Eulenberg M. Casuistische mittelheilungen aus dem gembeite der orthopadie. Arch Klin Chir. 1863;4:301-11.
Willet A, Walsham WJ. A second case of malformation of the left shoulder-girdle; removal of the abnormal portion of bone; with remarks on the probable nature of the deformity. Med Chir Trans. 66;1883:145-58. [Full Text].
Sprengel OK. Die angeborene verschiebung des schulterblattes nach oben. Archiv Fur Klinische Chirurgie, Berlin. 1891;42:545-9.
Kolliker T. Mittheilungen aus der chirurgischen casuistik und kleinere mittheilungen. Bemerkungen zum aufsatze von Dr. Sprengel. Die angeborene verschiebung des schulterblattes nach oben. Arch Klin Chir. 1891;42:925.
Hamner DL, Hall JE. Sprengel's deformity associated with multidirectional shoulder instability. J Pediatr Orthop. Sep-Oct 1995;15(5):641-3. [Medline].
Tachdjian MO. Pediatric Orthopedics. Vol 1. 2nd ed. Philadelphia, Pa: WB Saunders; 1990:136-8.
Ogden JA, Conlogue GJ, Phillips MS, Bronson ML. Sprengel's deformity. Radiology of the pathologic deformation. Skeletal Radiol. 1979;4(4):204-11. [Medline].
Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg Br. Aug 1972;54(3):395-408. [Medline]. [Full Text].
Mooney JF 3rd, White DR, Glazier S. Previously unreported structure associated with Sprengel deformity. J Pediatr Orthop. Jan-Feb 2009;29(1):26-8. [Medline].
Grogan DP, Stanley EA, Bobechko WP. The congenital undescended scapula. Surgical correction by the Woodward procedure. J Bone Joint Surg Br. Nov 1983;65(5):598-605. [Medline]. [Full Text].
Chen CP. Syndromes and disorders associated with omphalocele (III): single gene disorders, neural tube defects, diaphragmatic defects and others. Taiwan J Obstet Gynecol. Jun 2007;46(2):111-20. [Medline]. [Full Text].
Ferlini A, Ragno M, Gobbi P, et al. Hydrocephalus, skeletal anomalies, and mental disturbances in a mother and three daughters: a new syndrome. Am J Med Genet. Dec 4 1995;59(4):506-11. [Medline].
Matsuoka T, Ahlberg PE, Kessaris N, et al. Neural crest origins of the neck and shoulder. Nature. Jul 21 2005;436(7049):347-55. [Medline]. [Full Text].
Engel D. The etiology of the undescended scapula and related syndromes. J Bone Joint Surg. 1943;25:613-25. [Full Text].
Oxnard CE. Evolution of the human shoulder: some possible pathways. Am J Phys Anthropol. May 1969;30(3):319-31. [Medline].
Ogden JA, Phillips SB. Radiology of postnatal skeletal development. VII. The scapula. Skeletal Radiol. 1983;9(3):157-69. [Medline].
Doita M, Iio H, Mizuno K. Surgical management of Sprengel's deformity in adults. A report of two cases. Clin Orthop Relat Res. Feb 2000;371:119-24. [Medline].
Jeannopoulos CL. Congenital elevation of the scapula. J Bone Joint Surg Am. Oct 1952;34 A(4):883-92. [Medline]. [Full Text].
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. Jul 2000;82(5):711-8. [Medline]. [Full Text].
Floemer F, Magerkurth O, Jauckus C, Lütschg 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. Nov 16 2007;epub ahead of print. [Medline].
Samartzis D, Herman J, Lubicky JP, Shen FH. Sprengel's deformity in Klippel-Feil syndrome. Spine. Aug 15 2007;32(18):E512-6. [Medline].
Hensinger RN. Orthopedic problems of the shoulder and neck. Pediatr Clin North Am. Nov 1977;24(4):889-902. [Medline].
Hensinger RN, Lang JE, MacEwen GD. Klippel-Feil syndrome; a constellation of associated anomalies. J Bone Joint Surg Am. Sep 1974;56(6):1246-53. [Medline]. [Full Text].
Keats TE. Ocular hypertelorism (Greig's syndrome) associated with Sprengel's deformity. Am J Roentgenol Radium Ther Nucl Med. Sep 1970;110(1):119-22. [Medline].
Hadley MD. Carpal coalition and Sprengel's shoulder in Poland's syndrome. J Hand Surg [Br]. Jun 1985;10(2):253-5. [Medline].
Fernbach SK, Glass RB. The expanded spectrum of limb anomalies in the VATER association. Pediatr Radiol. 1988;18(3):215-20. [Medline].
Pollard ME, Cushing MV, Ogden JA. Musculoskeletal abnormalities in velocardiofacial syndrome. J Pediatr Orthop. Sep-Oct 1999;19(5):607-12. [Medline].
Hersh JH, Groom KR, Yen FF, Verdi GD. Changing phenotype in floating-harbor syndrome. Am J Med Genet. Feb 26 1998;76(1):58-61. [Medline].
Avon SW, Shively JL. Orthopaedic manifestations of Goldenhar syndrome. J Pediatr Orthop. Nov-Dec 1988;8(6):683-6. [Medline].
Boon JM, Potgieter D, Van Jaarsveld Z, Frantzen DJ. Congenital undescended scapula (Sprengel deformity): a case study. Clin Anat. Mar 2002;15(2):139-42. [Medline].
Leibovic SJ, Ehrlich MG, Zaleske DJ. Sprengel deformity. J Bone Joint Surg Am. Feb 1990;72(2):192-7. [Medline]. [Full Text].
McMurtry I, Bennet GC, Bradish C. Osteotomy for congenital elevation of the scapula (Sprengel's deformity). J Bone Joint Surg Br. Jul 2005;87(7):986-9. [Medline].
Ross DM, Cruess RL. The surgical correction of congenital elevation of the scapula. A review of seventy-seven cases. Clin Orthop Relat Res. Jun 1977;125:17-23. [Medline].
Woodward JW. Congenital elevation of the scapula: correction by release and transplantation of muscle origins. J Bone Joint Surg Am. 1961;43:219-28. [Full Text].
Green WT. The surgical correction of congenital elevation of the scapula (Sprengel's deformity). J Bone Joint Surg Am. 1957;39-A:1439-48.
Mears DC. Partial resection of the scapula and a release of the long head of triceps for the management of Sprengel's deformity. J Pediatr Orthop. Mar-Apr 2001;21(2):242-5. [Medline].
Further Reading
Related eMedicine topics
Klippel-Feil Syndrome
Velocardiofacial Syndrome
Scapulothoracic Joint Pathology
Keywords
Sprengel deformity, Sprengel's deformity, Sprengel anomaly, Sprengel's anomaly, congenital high scapula, congenital elevation of the scapula, failure of scapular descent, Sprengel's shoulder, Sprengel shoulder, hochgradige dislocation der scapula, high-grade dislocation of the scapula, hypoplastic scapula, elevated scapula, absent ribs, fused ribs, chest wall asymmetry, Klippel-Feil syndrome, cervical ribs, congenital scoliosis, cervical spina bifida, diastematomyelia, omovertebral bone, high shoulder, shoulder deformity, Greig syndrome, Greig cephalopolysyndactyly syndrome, GCPS, Poland syndrome, Poland anomaly, VATER association, velocardiofacial / velo-cardio-facial syndrome, VCFS, DiGeorge syndrome, floating harbor syndrome, floating-harbor syndrome, Goldenhar syndrome, oculo-auriculo-vertebral/oculoauriculovertebral syndrome, OAV syndrome, X-linked dominant hydrocephalus, skeletal anomalies, mental disturbance syndrome
Follow-up: Sprengel Deformity