Sprengel deformity

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Classification according to ICD-10
Q74.0 Sprengel deformity
ICD-10 online (WHO version 2019)

The Sprengelsche deformity or Sprengel deformity is a dysostosis with axial participation, as a one-sided shoulder high state expressed with too small a scapula. The deformity , named after the German surgeon Otto Sprengel (1852–1915), can occur in the context of Klippel-Feil syndrome , but also together with other changes such as scoliosis , rib malformations or spina bifida.

The cause is an inhibition of the downward migration of the shoulder blade system from the level of the 5th cervical vertebrae in the caudal direction. The time of damage is assumed to be in the 3rd month of pregnancy; familial accumulation is described.

In addition to a shortening of the neck muscles, there is often a connective tissue or cartilaginous cord between the upper edge of the scapular and the vertebral appendages of the lower cervical spine. If this is bony, it is referred to as the omovertebral bone, os omo-vertebrale , and can be seen on x-rays. In the other cases, this strand can be shown in the magnetic resonance imaging (MRI).

Os omo-vertebrale in Sprengel deformity, lateral projection of the cervical spine

Girls are preferably affected, also the left side somewhat more frequently, although the disease can also occur on both sides. Clinically, the fixed shoulder blade elevation with restricted mobility and the accompanying kyphoscoliosis are in the foreground.

Individual evidence

  1. ↑ OK Sprengel. The congenital upward displacement of the shoulder blade . In: Archive for Clinical Surgery , Berlin, 1891, 42: 545-549.
  2. ^ Pschyrembel: Clinical Dictionary
  3. ↑ H. Neuhof: "Congenital elevated shoulder (Sprengel's deformity). Family type." in: Journal of Orthopedics and Trauma Surgery 1913, 31, pages 519-525

literature

  • Hans Zilch: Textbook Orthopedics. De Gruyter, Berlin 1989, ISBN 3-11-010766-X .
  • Wolfgang Dihlmann: Joints - vertebral connections. Thieme, Stuttgart 1987, ISBN 3-13-471203-2 .
  • F. Hefti: Children's orthopedics in practice. Springer 1998, ISBN 3-540-61480-X , page 494f.

Web links