Sprengel's Shoulder dnbid

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Sprengel’s shoulder :

D. N. Bid Sprengel’s shoulder

Sprengel’s shoulder:

Sprengel’s shoulder also called as congenital high scapula characterized by an abnormally raised scapula, on one or both the sides The scapular muscles are poorly developed and may be represented by fibrous bands It may be accompanied by scoliosis with convexity on the involved side. 2/13/2011 2


AETIOLOGY imperfect descent of the shoulder girdle The muscles which suffer in their normal development will not fulfill their later function. In this respect the complete or partial defect of the muscles, their fibrous appearance and interruption of the normal differentiation of muscle fibers at the myoblastic stage are more significant. They represent the end results of the muscles which have undergone degeneration and necrosis at an early embryonic stage, and account for secondary contractions. 2/13/2011 3

Picture 1. Clinical photograph of a child with Sprengel deformity and Klippel-Feil syndrome.:

Picture 1. Clinical photograph of a child with Sprengel deformity and Klippel-Feil syndrome. 2/13/2011 4

Picture 2. Radiograph, chest posteroanterior view, depicting bilateral Sprengel deformities.:

Picture 2. Radiograph, chest posteroanterior view, depicting bilateral Sprengel deformities. 2/13/2011 5


PATHOLOGY Changes are found in the bones and muscles. 2/13/2011 6

Sprengel’s shoulder:

Sprengel’s shoulder Pathology continued….. The bones : The scapula may be of normal shape, or may be broadened at the expense of its length. It lies at an unusually high level, and may be attached to the vertebral column or the occipital bone by a band of imperfect muscle tissues, or by fibrous tissues, or even by a bar of a cartilage called the omovertebral mass . Among the associated errors in segmentation of the cervical spine are included hemivertebrae and wedging of the vertebrae, both of which produce congenital scoliosis. The atlas may be in two halves, one or both of which may be fused to the occipital condyles . 2/13/2011 7

Sprengel’s shoulder:

Sprengel’s shoulder Pathology continued….. The muscles : Constant alterations in the musculature of the shoulder girdle are found. The trapezius may be largely absent, the rhomboid and levator scapulae muscles being represented by the muscular fibrosis or cartilaginous band which passes up to the vertebral column or the occipital bone. Occasionally this anomalous band of tissue may be ossified in whole or in part. 2/13/2011 8


CLINICAL FEATURES The scapula on one or both sides is 2-10 cm higher than usual. It is also tilted forwards so that the shoulder appears to be displaced upwards and forwards. When the arm is raised the scapula does not move laterally, nor does its lower angle rotate when the arm is raised above the horizontal. The deformity of the shoulder rather than any functional disability of the arm attract the notice of parents. Only occasionally is there weakness of, or disinclination to use, the limb. All movements of the arm are complete except abduction and elevation to vertical position. Torticollis , cranium bifidum and spina bifida are often coexisting. 2/13/2011 9

Sprengel’s shoulder:

Sprengel’s shoulder Clinically the severity of the elevation of scapula has been described by Cavendish (1972) as: Group 1 – very mild, with the deformity almost unobservable and the shoulder joints level. Group 2 – mild, with the shoulder joints slightly unaligned. Group 3 – moderate, with the shoulder joint obviously higher. Group 4 – severe, with the superior angle of the scapula near the occiput, and webbing may be present. 2/13/2011 10


DIAGNOSIS The x-ray appearances are characteristics, the films showing the unduly high situation of the scapula. 2/13/2011 11


PROGNOSIS Even if operation is undertaken, the prognosis is not very favorable. Literatures indicate that while the mobility of the shoulder may be improved, asymmetry almost always persists. 2/13/2011 12


SURGICAL TREATMENT Many operations have been performed—the omovertebral bone has been removed, the band of fascia has been tenotomized or excised, and a portion of the scapula has been excised – but usually without great improvement. Usually in this deformity operation is not suggested if the functional and cosmetic defect is slight because results are disappointing for improving the function of the shoulder joint. 2/13/2011 13

Slide 14:

Green (1957) described ‘satisfying’ results by dissecting all the muscles attaching the scapula to the spine and trunk- including the omovertebral muscle if present- and reattaching them at a lower level . The supraclavicular portion of the scapula can be excised and the realigned scapula is held in position by wires within a body cast for three weeks. Great care is needed as there is a risk of injury to the accessory nerve or the brachial plexus


PHYSIOTHERAPY AFTER SURGERY Gradual relaxed passive mobilization of the shoulder and scapula. Suitable pain relieving modality like TENS, IFT and hydrocollator packs may be used to induce relaxation. Special attention is given to achieve early mobility of the scapula and the shoulder abduction and elevation. Overall mobilization and strengthening of the shoulder girdle muscles. Emphasize maximum possible correction of the posture of shoulder and maintain it. 2/13/2011 15

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THE END 2/13/2011 16

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