Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Sprengel Deformity Workup

  • Author: Mihir M Thacker, MBBS, MS(Orth), DNB(Orth), FCPS(Orth), D'Ortho; Chief Editor: Harris Gellman, MD  more...
 
Updated: Aug 04, 2016
 

Imaging Studies

Radiography

The Sprengel deformity is best visualized on an anteroposterior (AP) view of the chest and both shoulders (see the image below). A lateral view of the cervical and thoracic spine must also be obtained to rule out associated spinal anomalies.

Posteroanterior chest radiograph. This image depic Posteroanterior chest radiograph. This image depicts bilateral Sprengel deformities.

The scapular displacement can be measured by using the method described by Leibovic et al (see the image below).[32] On an AP radiograph of the chest, draw three lines as follows:

  • Line 1 – From the midpoint of the acromioclavicular joint to the midpoint of the sternoclavicular joint
  • Line 2 – From the midpoint of the acromioclavicular joint to the inferior angle of the scapula
  • Line 3 – A vertical line along the spinous processes of the vertebrae
Photo illustrating the Leibovic method for determi 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.

The superior scapular angle (SSA) is the angle between lines 1 and 2. The inferior scapular angle (ISA) is the angle between lines 2 and 3. These angles give the clinician an idea about the scapular rotation.

As the scapula is derotated back toward normal, the SSA increases and the ISA decreases. The caudad displacement of the scapula is measured by a line drawn from the center of the acromioclavicular joint perpendicular to line 3. The vertebral body at which this intersects provides an idea regarding the level of the scapula. Because this result is not a numeric value, it is not affected by growth. However, the SSA, ISA, and the level of the scapula are measured preoperatively and compared with the postoperative follow-up values.

CT and MRI

Computed tomography (CT) with three-dimensional (3D) reconstruction may be performed to visualize the pathoanatomy of the affected region and to visualize the omovertebral bar.[33] CT may also help in planning surgery (eg, if the scan shows that the height-to-width ratio is markedly decreased, then the prominent convexity of the vertebral border along with the supraspinous portion of the scapula should be resected).[17]

Magnetic resonance imaging is currently employed in this setting as well.[34]

Appropriate imaging studies should also be performed for any associated anomalies.[35]

 
 
Contributor Information and Disclosures
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 Chief, Pediatric Orthopedic Surgery, Professor of Orthopedic Surgery and Pediatrics, Director, Center for Children, Hospital for Joint Diseases, New York University Langone 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, Scoliosis Research Society, Pediatric Orthopaedic Society of North America

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.

References
  1. Hamner DL, Hall JE. Sprengel's deformity associated with multidirectional shoulder instability. J Pediatr Orthop. 1995 Sep-Oct. 15(5):641-3. [Medline].

  2. Tachdjian MO. Pediatric Orthopedics. 2nd ed. Philadelphia, Pa: WB Saunders; 1990. Vol 1: 136-8.

  3. Ogden JA, Conlogue GJ, Phillips MS, Bronson ML. Sprengel's deformity. Radiology of the pathologic deformation. Skeletal Radiol. 1979. 4(4):204-11. [Medline].

  4. Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg Br. 1972 Aug. 54(3):395-408. [Medline]. [Full Text].

  5. Mooney JF 3rd, White DR, Glazier S. Previously unreported structure associated with Sprengel deformity. J Pediatr Orthop. 2009 Jan-Feb. 29(1):26-8. [Medline].

  6. 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. [Medline]. [Full Text].

  7. Eulenberg M. Casuistische mittelheilungen aus dem gembeite der orthopadie. Arch Klin Chir. 1863. 4:301-11.

  8. 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].

  9. Sprengel OK. Die angeborene verschiebung des schulterblattes nach oben. Archiv Fur Klinische Chirurgie, Berlin. 1891. 42:545-9.

  10. 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.

  11. 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. [Medline].

  12. Engel D. The etiology of the undescended scapula and related syndromes. J Bone Joint Surg. 1943. 25:613-25. [Full Text].

  13. Oxnard CE. Evolution of the human shoulder: some possible pathways. Am J Phys Anthropol. 1969 May. 30(3):319-31. [Medline].

  14. Ogden JA, Phillips SB. Radiology of postnatal skeletal development. VII. The scapula. Skeletal Radiol. 1983. 9(3):157-69. [Medline].

  15. 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. [Medline].

  16. Jeannopoulos CL. Congenital elevation of the scapula. J Bone Joint Surg Am. 1952 Oct. 34 A(4):883-92. [Medline]. [Full Text].

  17. 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. [Medline]. [Full Text].

  18. 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. [Medline]. [Full Text].

  19. 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. 1995 Dec 4. 59(4):506-11. [Medline].

  20. 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. [Medline]. [Full Text].

  21. 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. 2007 Nov 16. epub ahead of print. [Medline].

  22. Samartzis D, Herman J, Lubicky JP, Shen FH. Sprengel's deformity in Klippel-Feil syndrome. Spine. 2007 Aug 15. 32(18):E512-6. [Medline].

  23. Hensinger RN. Orthopedic problems of the shoulder and neck. Pediatr Clin North Am. 1977 Nov. 24(4):889-902. [Medline].

  24. 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. [Medline]. [Full Text].

  25. 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. [Medline].

  26. Hadley MD. Carpal coalition and Sprengel's shoulder in Poland's syndrome. J Hand Surg [Br]. 1985 Jun. 10(2):253-5. [Medline].

  27. Yiyit N, Işıtmangil T, Öksüz S. Clinical analysis of 113 patients with Poland syndrome. Ann Thorac Surg. 2015 Mar. 99 (3):999-1004. [Medline].

  28. Fernbach SK, Glass RB. The expanded spectrum of limb anomalies in the VATER association. Pediatr Radiol. 1988. 18(3):215-20. [Medline].

  29. Pollard ME, Cushing MV, Ogden JA. Musculoskeletal abnormalities in velocardiofacial syndrome. J Pediatr Orthop. 1999 Sep-Oct. 19(5):607-12. [Medline].

  30. 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. [Medline].

  31. Avon SW, Shively JL. Orthopaedic manifestations of Goldenhar syndrome. J Pediatr Orthop. 1988 Nov-Dec. 8(6):683-6. [Medline].

  32. Leibovic SJ, Ehrlich MG, Zaleske DJ. Sprengel deformity. J Bone Joint Surg Am. 1990 Feb. 72(2):192-7. [Medline]. [Full Text].

  33. 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. [Medline].

  34. Bindoudi A, Kariki EP, Vasiliadis K, Tsitouridis I. The rare sprengel deformity: our experience with three cases. J Clin Imaging Sci. 2014. 4:55. [Medline]. [Full Text].

  35. 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. [Medline]. [Full Text].

  36. 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. [Medline].

  37. 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. [Medline].

  38. 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. [Medline].

  39. 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].

  40. Green WT. The surgical correction of congenital elevation of the scapula (Sprengel's deformity). J Bone Joint Surg Am. 1957. 39-A:1439-48.

  41. 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. [Medline]. [Full Text].

  42. 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. [Medline].

  43. 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. [Medline].

  44. 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. [Medline].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.