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 depicts bilateral Sprengel deformities.

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The scapular displacement can be measured by using the method described by Leibovic et al (see the image below).^[34] On an AP radiograph of the chest, three lines are drawn 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 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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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.^[35] 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 (MRI) is currently employed in this setting as well.^[36]

Appropriate imaging studies should also be performed for any associated anomalies.^[37]

Treatment & Management

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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) Workup

Radiography

CT and MRI

References

Tables

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