History and Physical Examination

The hallmarks of the Sprengel deformity (congenital elevation of the scapula) are shoulder asymmetry and restriction of shoulder abduction. Clinically, the affected scapula usually is elevated 2-10 cm and is adducted, and its inferior pole is rotated medially. Because of this rotation, the glenoid faces inferiorly.

A prominence in the suprascapular region is characteristic because of the upwardly rotated superomedial angle of the scapula, which causes the ipsilateral side of the neck to appear fuller and its normal contour to be lost. The scapula is hypoplastic, and the length of the vertebral border is decreased. Occasionally, some anterior bending of the supraspinous portion is present.

Passive movement of the glenohumeral joint, including abduction and external and internal rotation, may be normal. However, scapulothoracic movements may be severely limited. In 40% of patients with a Sprengel deformity, combined abduction is limited to less than 100º. The omovertebral bone may also limit abduction by affecting scapular mobility, and it can also limit neck movement if it is attached high in the cervical spine. Other causes of limited abduction include abnormal and weakened scapular muscles.

The left side is more commonly affected than the right side is. The condition may sometimes be bilateral, in which case it is functionally more disabling, albeit much more cosmetically acceptable.

Problems that may be associated with Sprengel deformity include syndromes such as the following:

Klippel-Feil syndrome ^{[22, 23, 24, 25, 26]}
Greig syndrome ^[27]
Poland syndrome ^{[28, 29]}
VATER association (ie, vertebral defects, imperforate anus, tracheoesophageal fistula, and radial and renal dysplasia) ^[30]
Velocardiofacial syndrome ^[31]
Floating-harbor syndrome ^[32]
Goldenhar syndrome ^[33]
X-linked dominant hydrocephalus, skeletal anomalies, and mental disturbance syndrome ^[19]

These syndromes are extremely rare, with the possible exception of the Klippel-Feil syndrome (see the image below).

Clinical photograph of a child with Sprengel deformity and Klippel-Feil syndrome.

View Media Gallery

Classification

A Sprengel deformity can be classified by severity according to the Cavendish grading system, as follows^[4]:

Grade 1 (very mild) - The shoulders are almost level, and the deformity cannot be noticed with the clothes on
Grade 2 (mild) - The shoulders are almost level, but the superomedial portion of the high scapula is visible as a lump
Grade 3 (moderate) - The deformity is visible, and the affected shoulder is elevated 2-5 cm higher than the opposite shoulder
Grade 4 (severe) - The scapula is very high, with the superomedial angle at the occiput, with neck webbing and brevicollis

This classification, however, is difficult to apply in bilateral cases.

Workup

Hamner DL, Hall JE. Sprengel's deformity associated with multidirectional shoulder instability. J Pediatr Orthop. 1995 Sep-Oct. 15 (5):641-3. [QxMD MEDLINE Link].
Congenital high scapula (Sprengel deformity). Herring JA, ed. Tachdjian's Pediatric Orthopedics. 6th ed. Philadelphia: Elsevier; 2022. Vol 1: 343-4.
Ogden JA, Conlogue GJ, Phillips MS, Bronson ML. Sprengel's deformity. Radiology of the pathologic deformation. Skeletal Radiol. 1979. 4 (4):204-11. [QxMD MEDLINE Link].
Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg Br. 1972 Aug. 54 (3):395-408. [QxMD MEDLINE Link]. [Full Text].
Mooney JF 3rd, White DR, Glazier S. Previously unreported structure associated with Sprengel deformity. J Pediatr Orthop. 2009 Jan-Feb. 29 (1):26-8. [QxMD MEDLINE Link].
Grogan DP, Stanley EA, Bobechko WP. The congenital undescended scapula. Surgical correction by the woodward procedure. J Bone Joint Surg Br. 1983 Nov. 65 (5):598-605. [QxMD MEDLINE Link]. [Full Text].
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.
Boon JM, Potgieter D, Van Jaarsveld Z, Frantzen DJ. Congenital undescended scapula (Sprengel deformity): a case study. Clin Anat. 2002 Mar. 15 (2):139-42. [QxMD MEDLINE Link].
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. 1969 May. 30 (3):319-31. [QxMD MEDLINE Link].
Ogden JA, Phillips SB. Radiology of postnatal skeletal development. VII. The scapula. Skeletal Radiol. 1983. 9 (3):157-69. [QxMD MEDLINE Link].
Doita M, Iio H, Mizuno K. Surgical management of Sprengel's deformity in adults. A report of two cases. Clin Orthop Relat Res. 2000 Feb. 371:119-24. [QxMD MEDLINE Link].
JEANNOPOULOS CL. Congenital elevation of the scapula. J Bone Joint Surg Am. 1952 Oct. 34 A (4):883-92. [QxMD MEDLINE Link]. [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. 2000 Jul. 82 (5):711-8. [QxMD MEDLINE Link]. [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. 2007 Jun. 46 (2):111-20. [QxMD MEDLINE Link]. [Full Text].
Ferlini A, Ragno M, Gobbi P, Marinucci C, Rossi R, Zanetti A, et al. Hydrocephalus, skeletal anomalies, and mental disturbances in a mother and three daughters: a new syndrome. Am J Med Genet. 1995 Dec 4. 59 (4):506-11. [QxMD MEDLINE Link].
Pargas C, Santana A, Czoch WL, Rogers KJ, Mackenzie WG. Sprengel Deformity in Biological Sisters. J Am Acad Orthop Surg Glob Res Rev. 2020 Apr. 4 (4):[QxMD MEDLINE Link]. [Full Text].
Matsuoka T, Ahlberg PE, Kessaris N, Iannarelli P, Dennehy U, Richardson WD, et al. Neural crest origins of the neck and shoulder. Nature. 2005 Jul 21. 436 (7049):347-55. [QxMD MEDLINE Link]. [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. 2008 Feb. 29 (2):306-7. [QxMD MEDLINE Link].
Samartzis D, Herman J, Lubicky JP, Shen FH. Sprengel's deformity in Klippel-Feil syndrome. Spine (Phila Pa 1976). 2007 Aug 15. 32 (18):E512-6. [QxMD MEDLINE Link].
Hensinger RN. Orthopedic problems of the shoulder and neck. Pediatr Clin North Am. 1977 Nov. 24 (4):889-902. [QxMD MEDLINE Link].
Hensinger RN, Lang JE, MacEwen GD. Klippel-Feil syndrome; a constellation of associated anomalies. J Bone Joint Surg Am. 1974 Sep. 56 (6):1246-53. [QxMD MEDLINE Link]. [Full Text].
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. [QxMD MEDLINE Link]. [Full Text].
Keats TE. Ocular hypertelorism (Greig's syndrome) associated with Sprengel's deformity. Am J Roentgenol Radium Ther Nucl Med. 1970 Sep. 110 (1):119-22. [QxMD MEDLINE Link].
Hadley MD. Carpal coalition and Sprengel's shoulder in Poland's syndrome. J Hand Surg Br. 1985 Jun. 10 (2):253-5. [QxMD MEDLINE Link].
Yiyit N, Işıtmangil T, Öksüz S. Clinical analysis of 113 patients with Poland syndrome. Ann Thorac Surg. 2015 Mar. 99 (3):999-1004. [QxMD MEDLINE Link].
Fernbach SK, Glass RB. The expanded spectrum of limb anomalies in the VATER association. Pediatr Radiol. 1988. 18 (3):215-20. [QxMD MEDLINE Link].
Pollard ME, Cushing MV, Ogden JA. Musculoskeletal abnormalities in velocardiofacial syndrome. J Pediatr Orthop. 1999 Sep-Oct. 19 (5):607-12. [QxMD MEDLINE Link].
Hersh JH, Groom KR, Yen FF, Verdi GD. Changing phenotype in Floating-Harbor syndrome. Am J Med Genet. 1998 Feb 26. 76 (1):58-61. [QxMD MEDLINE Link].
Avon SW, Shively JL. Orthopaedic manifestations of Goldenhar syndrome. J Pediatr Orthop. 1988 Nov-Dec. 8 (6):683-6. [QxMD MEDLINE Link].
Leibovic SJ, Ehrlich MG, Zaleske DJ. Sprengel deformity. J Bone Joint Surg Am. 1990 Feb. 72 (2):192-7. [QxMD MEDLINE Link]. [Full Text].
Yamada K, Suenaga N, Iwasaki N, Oizumi N, Minami A, Funakoshi T. Correction in malrotation of the scapula and muscle transfer for the management of severe Sprengel deformity: static and dynamic evaluation using 3-dimensional computed tomography. J Pediatr Orthop. 2013 Mar. 33 (2):205-11. [QxMD MEDLINE Link].
Bindoudi A, Kariki EP, Vasiliadis K, Tsitouridis I. The rare sprengel deformity: our experience with three cases. J Clin Imaging Sci. 2014. 4:55. [QxMD MEDLINE Link]. [Full Text].
Dilli A, Ayaz UY, Damar C, Ersan O, Hekimoglu B. Sprengel deformity: magnetic resonance imaging findings in two pediatric cases. J Clin Imaging Sci. 2011. 1:13. [QxMD MEDLINE Link]. [Full Text].
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. 2001 Mar-Apr. 21 (2):242-5. [QxMD MEDLINE Link].
McMurtry I, Bennet GC, Bradish C. Osteotomy for congenital elevation of the scapula (Sprengel's deformity). J Bone Joint Surg Br. 2005 Jul. 87 (7):986-9. [QxMD MEDLINE Link].
Ross DM, Cruess RL. The surgical correction of congenital elevation of the scapula. A review of seventy-seven cases. Clin Orthop Relat Res. 1977 Jun. 125:17-23. [QxMD MEDLINE Link].
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.
Gonen E, Simsek U, Solak S, Bektaser B, Ates Y, Aydin E. Long-Term Results of Modified Green Method in Sprengel's Deformity. J Child Orthop. 2010 Aug. 4 (4):309-14. [QxMD MEDLINE Link]. [Full Text].
Wada A, Nakamura T, Fujii T, Takamura K, Yanagida H, Yamaguchi T, et al. Sprengel deformity: morphometric assessment and surgical treatment by the modified green procedure. J Pediatr Orthop. 2014 Jan. 34 (1):55-62. [QxMD MEDLINE Link].
Jiang Y, Guo Y, Zhu Z, Wang Y. Surgical management of Sprengel's deformity by a modification of Green's procedure : A single center experience. Orthopade. 2020 Mar. 49 (3):255-259. [QxMD MEDLINE Link].
Siu KK, Ko JY, Huang CC, Wang FS, Chen JM, Wong T. Woodward procedure improves shoulder function in Sprengel deformity. Chang Gung Med J. 2011 Jul-Aug. 34 (4):403-9. [QxMD MEDLINE Link].
Walstra FE, Alta TD, van der Eijken JW, Willems WJ, Ham SJ. Long-term follow-up of Sprengel's deformity treated with the Woodward procedure. J Shoulder Elbow Surg. 2013 Jun. 22 (6):752-9. [QxMD MEDLINE Link].
Nakamura N, Inaba Y, Machida J, Saito T. Use of glenoid inclination angle for the assessment of unilateral congenital high scapula. J Pediatr Orthop B. 2016 Jan. 25 (1):54-61. [QxMD MEDLINE Link].
Alsiddiky AM, Rafiq Z, Bakarman KA, Alhuzaimi FS, Asif M. A Novel Modification of Woodward Procedure for Correction of Sprengel Deformity by Application of Anchoring Sutures. Indian J Orthop. 2021 May. 55 (Suppl 1):189-198. [QxMD MEDLINE Link]. [Full Text].
Öner A, Köksal A, Çimen O, Kargin D, Albayrak A, Akman YE. Modified Woodward Technique for Sprengel Deformity and a Modification of the Cavendish Classification. J Pediatr Orthop. 2020 Sep. 40 (8):401-407. [QxMD MEDLINE Link].
Soldado F, Barrera-Ochoa S, Diaz-Gallardo P, Nguyen TQ, Nguyen DH, Knörr J. Outcomes following endoscopic-assisted Woodward procedure for Sprengel deformity. J Child Orthop. 2021 Dec 1. 15 (6):583-588. [QxMD MEDLINE Link]. [Full Text].
Bin Majid O, Alzaid SZ, Al-Zayed Z, Almonaie S, Albekairi AA, Ahmed M. Outcomes of Woodward's Procedure for Sprengel's Shoulder Using Neurophysiological Monitoring of the Brachial Plexus Without Clavicular Osteotomy: A Retrospective Study. Cureus. 2021 Nov. 13 (11):e19797. [QxMD MEDLINE Link]. [Full Text].
Feng L, Zhang X, Guo D, Li C, Qi X, Bai Y, et al. Utilization of intraoperative neuromonitoring during the Woodward procedure for treatment of Sprengel deformity. J Shoulder Elbow Surg. 2022 Aug. 31 (8):e405-e412. [QxMD MEDLINE Link].

Media Gallery

Clinical photograph of a child with Sprengel deformity and Klippel-Feil syndrome.
Posteroanterior chest radiograph. This image depicts bilateral Sprengel deformities.
Photo illustrating the Leibovic method for determining scapular rotation and position. ISA = inferior scapular angle; Line 1 = line drawn from the midpoint of the acromioclavicular joint to the midpoint of the sternoclavicular joint; Line 2 = line drawn from the midpoint of the acromioclavicular joint to the ISA; Line 3 = vertical line drawn along the spinous processes of the vertebrae; SSA = superior scapular angle.

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Author

Mihir M Thacker, MBBS, MS(Orth), DNB(Orth), FCPS(Orth), D'Ortho Associate Professor of Orthopedic Surgery and Pediatrics, Jefferson Medical College of Thomas Jefferson University; Consulting Staff, Department of Pediatric Orthopedic Surgery, Alfred I duPont Hospital for Children; Orthopedic Oncologist, Helen F Graham Cancer Center and Christiana Care Health Services

Mihir M Thacker, MBBS, MS(Orth), DNB(Orth), FCPS(Orth), D'Ortho is a member of the following medical societies: Children's Oncology Group, Medical Council of India, Musculoskeletal Tumor Society, Pediatric Orthopaedic Society of North America, Limb Lengthening and Reconstruction Society

Disclosure: Nothing to disclose.

Coauthor(s)

David S Feldman, MD Clinical Professor of Orthopedic Surgery and Pediatrics, NYU Langone Medical Center and NYU Hospital for Joint Diseases; Director, Feldman Spinal Deformity Correction and Hip Preservation Center, The Paley Institute, St Mary’s Medical Center

David S Feldman, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, American Orthopaedic Association, Association for the Study and Application of the Method of Ilizarov International and External Fixation, International Perthes Study Group, Medical Society of the State of New York, New York State Society of Orthopaedic Surgeons, Palm Beach Pediatric Society, Pediatric Orthopaedic Society of North America, Scoliosis Research Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Robert J Nowinski, DO Clinical Assistant Professor of Orthopaedic Surgery, Ohio State University College of Medicine and Public Health, Ohio University College of Osteopathic Medicine; Private Practice, Orthopedic and Neurological Consultants, Inc, Columbus, Ohio

Robert J Nowinski, DO is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Medical Association, Ohio State Medical Association, Ohio Osteopathic Association, American College of Osteopathic Surgeons, American Osteopathic Association

Disclosure: Received grant/research funds from Tornier for other; Received honoraria from Tornier for speaking and teaching.

Chief Editor

Harris Gellman, MD Consulting Surgeon, Broward Hand Center; Voluntary Clinical Professor of Orthopedic Surgery and Plastic Surgery, Departments of Orthopedic Surgery and Surgery, University of Miami, Leonard M Miller School of Medicine; Clinical Professor of Surgery, Nova Southeastern School of Medicine

Harris Gellman, MD is a member of the following medical societies: American Academy of Medical Acupuncture, American Academy of Orthopaedic Surgeons, American Orthopaedic Association, American Society for Surgery of the Hand, Arkansas Medical Society, Florida Medical Association, Florida Orthopaedic Society

Disclosure: Nothing to disclose.

Additional Contributors

Michael S Clarke, MD Clinical Associate Professor, Department of Orthopedic Surgery, University of Missouri-Columbia School of Medicine

Michael S Clarke, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Arthroscopy Association of North America, American Academy of Pediatrics, American Association for Hand Surgery, American College of Surgeons, American Medical Association, Clinical Orthopaedic Society, Mid-Central States Orthopaedic Society, Missouri State Medical Association

Disclosure: Nothing to disclose.

Sections Sprengel Deformity (Congenital Elevation of Scapula)
Overview
Presentation
Workup
Treatment
Media Gallery
References

Sprengel Deformity (Congenital Elevation of Scapula) Clinical Presentation

History and Physical Examination

Classification

References

Tables

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