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Sprengel's deformity
Also known as:
Sprengel's anomaly
Sprengel’s phenotype
Sprengel's shoulder
Sprengel's syndrome
Associated persons:
Renzo Corno
Otto Gerhard Karl Sprengel
Description:
Congenital elevation of the scapula with rotation of its lower angle toward the spine. Scoliosis is frequently present, and torticollis may occasionally be associated. The abnormality may be unilateral or bilateral and occur in isolation or as a syndromic component. Abduction of shoulder beyond 90 degrees is impossible. It is due to failure of descent of the scapula during embryonic development from its position in the neck to its normal position in the posterior thorax. It happens usually in girls, and may have a genetic basis in some instances. Frequently associated with Klippel-Feil syndrome.
A familial form of the Sprengel deformity is known as Corno's disease.
Sprengel's deformity was first probably first described by Moritz Michael Eulenburg (1811-1877) in 1863. Sprengel in 1891 extended the spectre. Further comprehensive working over in 1908 by Vittorio Putti (1880-1940).
Bibliography:
- M. M. Eulenburg:
Hochgradige Dislocation der Scapula, bedingt durch Retraction des M. levator anguli und des oberen Teiles des M. cucullaris. Heilung mittels subcutaner Durchschneidung beider Muskeln und entsprechender Nachbehandlung.
Archiv für klinische Chirurgie, Berlin, 1863, 4: 304.
- O. K. Sprengel:
Die angeborene Verschiebung des Schulterblattes nach oben.
Archiv für klinische Chirurgie, Berlin, 1891, 42: 545-549.
- V. Putti:
Beitrag zur Aetiologie, Pathogenese und Behandlung des angeborenen Hochstandes des Schulterblattes.
Fortschritte auf dem Gebiete der Röntgenstrahlen, 1908, 12: 328.
- R. Corno:
Scapolo alta congenita a carattere familiare.
Clinca Pediatrica, Bologna, 1956, 38: 948-952.
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