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Bilateral Sprengel's Syndrome with Situs Inversus Totalis

Bilateral Sprengel's Syndrome with Situs Inversus Totalis


Acta orthop. Scandinav. 41, 403-410, 1970 Department of Surgery, Free University, Amsterdam, The Netherlands BILATERAL SPRENGEL’S SYNDROME WITH SITUS INVERSUS TOTALIS G.DEN OTTER Received 16.11.70 The main abnormality in the syndrome that carries the name of Sprengel (1891) is a congenital abnormally high location of the shoulder blade. The condition occurs equally in males and females and both sides are evenly involved, In only 10 per cent of cases is the deformity bilateral. In this syndrome the shoulder blade itself is hypoplastic and its cranial part, containing the supraspinate fossa, is elongated and protrudes ventrally. The scapula is rotated so that the spina scapulae runs upwards towards the midline, whereas the distal angulus scapulae points away from the thoracic cage. This results in a slight endorotation of the upper arm and some loss of abduction, flexion, and extension in the shoulder joint, The loss of function may be aggravated by shortening and contracture of the shoulder muscles. Many of these patients therefore prefer to wear the arm on their back. The high location of the shoulder forces them to keep the head forward and slightly rotated towards the normal side, which may cause the false impression of a
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