Sprengel’s shoulder

sprengel's shoulder, etiology, pathology, features, treatment

1. Sprengel’s shoulder by- kajal sansoya

2. Introduction It is developmental condition leading to a high or descended scapula ,is rare but most common congenital deformity of shoulder complex. Mainly shoulder abduction decreases, and shoulder function also decreases. In this condition, scapular muscles are poorly developed or are replaced by fibrous band.

3. Definition Rare congenital abnormality which arises from interruption of normal caudal migration of scapula and is characterized by elevation and medial rotation of scapula.

4. Incidence Most common congenital malformation of the shoulder girdle. Mostly noticed at birth. Equally affected in both gender. Left side is most common than right, bilateral only in 10%.

5. Etiology This may be due to failure of scapular descent from the cervical spine. The high scapula may be attached to the spine by a tough fibrous band or a cartilaginous bar( omovertebral bar)

6. Patho -physiology Occur between 9 th &12 th week of gestation. An arrest in the development of bone, cartilage, muscles also occur. The trapezius is absent , rhomboids and thin band represents levator scapulae. Serratus anterior muscle may be weak, leading to wringing of scapula.

7. Changes seen in scapula Dysplastic Smaller in the vertical plane and larger horizontally Inferior angle is rotated medially, causing the glenoid fossa to face inferiorly Convexity of the upper portion of the scapula is increased and curvature of the clavicular shaft is decreased, forming a narrower scapuloclavicular space, may contribute to brachial plexus compression.

8. Clinical features Deformity is the only symptom & it may be noticed at birth. The shoulder on the affected side is elevated, the scapula looks& feels abnormally high, smaller than usual & somewhat prominent. Occasionally both scapulae are affected The neck appears shorter than usual & there may be kyphosis or scoliosis of the thoracic spine.

9. Shoulder movements are painless but abduction and elevation may be limited by fixation of the scapula. Torticollis Facial asymmetry Crania bifida or spina bifida may present

10. Cavendish Grading Grade 1 - very mild Grade 2 - mild, shoulder , slightly unaligned Grade 3 - moderate, shoulder high Grade 4 – severe, with superior angle of scapula near the occiput .

11. Examination Assessment of the shoulder may include an assessment of the cervical spine, examination can be an intensive one. Examiner must remember that the arm, of which the shoulder is an integral part, may act as an open kinetic chain when the hand is free to move, or as closed kinetic chain when the hand is fixed to some relatively immovable object.

12. Knowledge of muscle balance and muscle force couples becomes imperative in determining a diagnosis. Eating, reaching, dressing are considered open kinetic chain activities, whereas crutch walking and pushing up from a chair are considered closed kinetic chain movements. As with any assessment, the examiner is comparing one side of body with the other. This comparison is necessary because of individual differences among normal people.

13. Examiner will access ……? Active movements of shoulder Scapulo -humeral rhythm Scapular control Passive movements Resisted isometric movements Functional assessment Special test-DRST , Apley’s scratch test, rowe sign, gagey hyperabduction test

14. Causes of scapular imbalance pattern Increased protraction - tight pectoralis minor, weak/lightened lower trapezius , serratus ant. Increased depression - weak upper trapezius Loss of scapular stabilization - early/excessive protraction, lateral rotation of scapula, elevation of scapula, tight lateral rotators, secondary impingment .

15. Investigations CT scan Dxca scan MRI X-ray

16. elevated scapula With short vertebral border. The scapula resembles equilateral triangle Rotation of inferior angle Either towards the spine or less commonly to the opposite direction Omovertebral bone Connecting the superior angle to the cervical spine Radiological criteria

17. Surgical Treatment Factors to be assessed- severity of the deformity functional impairment age , associated contributed condition Surgery is best advisable foe patient – between 3& 8 years of age, with moderate or severe cosmetic/ functional deformity. Surgical intervention before the age of 2 years is extensive & is technically more difficult. Best result are obtained if surgery is performed below the age of 5

18. Surgical options Putti’s procedure- detachment of the scapula insertion of the rhomboids & trapezius , omovertebral bar resection, followed by lowering the scapula& fixing its inferior angle to a rib at the corrected level.

19. Shrock modified putt’s procedure- subperiosteal dissection of the musculature and adding an osteomy of the supraspinous scapular region and the acromial base to fcailitate scapular descent.

20. Green scapuloplasty – resection of the prominent superior scapular border and extra periosteal division of muscular attachment of the scapula to allow the scapula to be displaced inferiorly and muscular reattachments at the newer corrected level at the scapula.

21. Modified woodward’s procedure- detachment of attachment of trapezius and rhomboids from spinous process Release of omovertebral band Excision of superomedial angle of scapula Relocation of scapula to new position Suturing of trapezius to inferior spinous process closure in layers.

22. Post-operative complications Wringing of scapula Brachial plexus injury Keloid formation

23. Physiotherapy Gradual relaxed passive mobilization of the shoulder & scapula Suitable pain relief modality TENS, IFT, hot packs used to induce relaxation Special attention is given to achieve early mobility of the scapula and the shoulder abduction, elevation. Overall mobilization and strengthening of shoulder girdle muscles Emphasize maximum possible correction of the posture and its maintenance is important.

24. Exercises include Shoulder shrugs Functional reach Horizontal and vertical reaching exercises Pendulum exercises Shoulder wheel Wall ladder Wall cleaning Shoulder pulley

25. Short term goal Pain relief- TENS Stiffness- hot pack Enhance movement- electrical stimulation Adhesion break- US Mobility- paraffin wax, mobilization Exercises for 1 week- passive/active assisted shoulder movements

26. Long term goals Strength –strengthening exercises include resistance exercises, gripping exercises, shoulder wheel, pulley, wall ladder Gross movements - wall cleaning, functional reach, gripping exercises, stretching exercises Fine skills - dressing up, picking up the small things, use of pen or pencil, reaching exercises.

27. Reference:- Essential orthopedics, maheshwari and mhaskar , essential orthopedics including clinical methods(6 th edition, page no-382-385)chapter-congenital deformities Essential orthopedics & applied physiotherapy, jayent joshi , parksh kotwal (5 th edition, page no- 352-354)chapter-deformities. Orthopedics physical assesment , David j Magee, Robert C. Manske ( 7 th edition, page no-488-500)chapter-

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