Sprengel deformity, or congenital elevation of the scapula, is a complex deformity of the shoulder and is the most common congenital shoulder abnormality. An initial diagnosis can often be made on radiography, but CT or MRI is often necessary to evaluate the details of the abnormality.
Sprengel deformity is usually noticed at birth and has both cosmetic and functional implications. The elevated scapula is visually noticeable and there is an associated restriction in the motion of the scapula and glenohumeral joint.
The Cavendish classification 2,6 is one method used for grading:
- very mild deformity is observed
- when covered with clothes the deformity is almost invisible
- the deformity is still mild but appears as a bump
- the superomedial portion of the high scapula is convex, forming a bump
- moderate deformity with 2-5 cm of visible elevation of the affected shoulder
- severe deformity with >5 cm elevation of the affected shoulder, accompanied by neck webbing
The abnormality results from failure of caudal migration of the scapula during early fetal development.
Sprengel deformities usually coexist with other congenital abnormalities, particularly those involving the vertebrae and ribs. An omovertebral bar (fibrous, cartilaginous and/or osseous connection between the scapula and cervical spine) is often present.
It is also commonly associated with hypoplasia or atrophy of regional muscles, and these associated features can cause further misshaping of the shoulder and limitation of shoulder movement.
Patients with Sprengel deformity often have one or more of the following abnormalities and conditions:
- Klippel-Feil syndrome
- spina bifida
- underdevelopment of clavicle or humerus
These possible co-existing anomalies need to be looked for in any patient presenting with Sprengel deformity.
The affected scapula is elevated and rotated, with the inferior angle directed laterally.
The radiographic Rigault classification 3,7:
- grade I: superomedial angle lower than T2 but above T4 transverse process
- grade II: superomedial angle located between C5 and T2 transverse process
- grade III: superomedial angle above C5 transverse process
CT with 3D reconstruction is being used to evaluate omovertebral connection and scapula dysplasia and malpositioning. It can be used in preoperative planning.
There may be a role in MRI to assess omovertebral connection.
Treatment and prognosis
Surgery is performed to improve cosmetic and functional disability. It is generally considered for patients between 3 and 8 years of age who have moderate to severe disability (or a Cavendish score of 3-4) 1.
Two of the most used surgical methods are the “Woodward” procedure and the “modified-Green” procedure with good functional and cosmetic outcome.
History and etymology
It is named after Otto Gerhard Karl Sprengel (1852-1915), a German surgeon who described four cases in 1891.
Possible differential diagnosis on presentation:
- 1. Kadavkolan AS, Bhatia DN, Dasgupta B et-al. Sprengel's deformity of the shoulder: Current perspectives in management. Int J Shoulder Surg. 2011;5 (1): 1-8. doi:10.4103/0973-6042.80459 - Free text at pubmed - Pubmed citation
- 2. Dilli A, Ayaz UY, Damar C et-al. Sprengel deformity: magnetic resonance imaging findings in two pediatric cases. J Clin Imaging Sci. 2011;1 : 13. doi:10.4103/2156-7514.76691 - Free text at pubmed - Pubmed citation
- 3. Andrault G, Salmeron F, Laville JM. Green's surgical procedure in Sprengel's deformity: cosmetic and functional results. Orthop Traumatol Surg Res. 2009;95 (5): 330-5. doi:10.1016/j.otsr.2009.04.015 - Pubmed citation
- 4. Cho TJ, Choi IH, Chung CY et-al. The Sprengel deformity. Morphometric analysis using 3D-CT and its clinical relevance. J Bone Joint Surg Br. 2000;82 (5): 711-8. J Bone Joint Surg Br (link) - Pubmed citation
- 5. Sprengel's Deformity. Radiology. 1941;36 (5): 624-626. Radiology (citation) - doi:10.1148/36.5.624
- 6. Cavendish ME. Congenital elevation of the scapula. J Bone Joint Surg Br. 1972;54 (3): 395-408. J Bone Joint Surg Br (link) - Pubmed citation
- 7. Rigault P, Pouliquen JC, Guyonvarch G et-al. [Congenital elevation of the scapula in children. Anatomo-pathological and therapeutic study apropos of 27 cases]. Rev Chir Orthop Reparatrice Appar Mot. 62 (1): 5-26. - Pubmed citation
Upper limb anatomy
skeleton of the upper limb
- carpal bones (mnemonic)
- accessory ossicles of the upper limb
- accessory ossicles of the shoulder
- accessory ossicles of the elbow
- accessory ossicles of the wrist (mnemonic)
- joints of the upper limb
- sternoclavicular joint
- acromioclavicular joint
- glenohumeral joint
- scapulocostal joint (scapulothoracic joint)
- suprahumeral joint
- associated structures
- blood supply - scapular anastomosis
- ossification centres
- elbow joint
- wrist joint
- hand joints
- shoulder joint
- spaces of the upper limb
- pectoral region
- rotator cuff interval
- quadrangular space
- lateral triangular space
- medial triangular space
- cubital tunnel
- cubital fossa
- anatomical snuff box
- carpal tunnel
- Guyon's canal
- space of Poirier
- extensor compartments of the wrist
- muscles of the upper limb
- shoulder girdle
- anterior compartment of the arm
- posterior compartment of the arm
- anterior compartment of the forearm
- posterior compartment of the forearm (extensors)
- thenar (lateral)
- hypothenar (medial)
- accessory muscles
- blood supply to the upper limb
- subclavian artery (mnemonic)
- axillary artery
- brachial artery (proximal portion)
- common interosseous artery
- posterior ulnar recurrent artery
- anterior ulnar recurrent artery
- proper dorsal digital artery
- deep palmar brach of the ulnar artery
- princeps pollicis artery
- persistent median artery of the forearm
- radial artery
- innervation of the upper limb
- intercostobrachial nerve
brachial plexus (mnemonic)
- branches from the roots
- branches from the trunks
- branches from the cords
- terminal branches
- lymphatic drainage of the upper limb