Congenital clavicle osteoarthritis

from Wikipedia, the free encyclopedia
Classification according to ICD-10
Q74.0 Other congenital malformations of the upper extremity (s) and shoulder girdle - Congenital pseudoarthrosis of the clavicle
ICD-10 online (WHO version 2019)

The congenital clavicle osteoarthritis is a rare, congenital pseudarthrosis of the collarbone .

Congenital clavicle osteoarthritis in a 3-year-old girl

frequency

The disease is rare, with around 200 patients reported to date. The right side is affected much more frequently, the left side mainly in dextrocardia and situs inversus . Both sides are affected in 10%, and additional cervical ribs are present in 15% .

root cause

The cause is not trauma related, there is no association with neurofibromatosis or fibrous dysplasia .

This is due to the failure of the two primary ossification nuclei of the clavicle to fuse .

Clinical manifestations

Clinical criteria are painless swelling occurring after birth or in the first few years of life and shoulder asymmetry.

diagnosis

The diagnosis can be made using a simple x-ray . Features are:

  • Missing callus
  • Defect in the middle of the collarbone
  • rounded edges

Differential diagnosis

The following are to be distinguished:

therapy

Treatment is limited to symptomatic pseudarthroses, for which surgical correction at the age of 5 with resection , spongiosaplasty and stabilization is recommended.

history

The first known description comes from L. Saint-Pierre in 1930.

literature

  • B. Schmidt-Rohlfing, C. Niedhart, EH Schwer, FU Niethard: The clavicle osteoarthritis in childhood: differential diagnosis, clinic, therapy and results. In: Journal for orthopedics and their border areas. Vol. 139, No. 5, 2001, pp. 447-451, doi: 10.1055 / s-2001-17989 , PMID 11605298 .
  • G. Köster, E. Kunze, M. von Knoch: The operative treatment of the congenital clavicle osteoarthritis. In: Journal for orthopedics and their border areas. Vol. 137, No. 5, 1999, pp. 414-418, doi: 10.1055 / s-2008-1037383 , PMID 10549118 .

Web links

Individual evidence

  1. a b c Congenital clavicle osteoarthritis. In: Orphanet (Rare Disease Database).
  2. a b c F. Hefti: Pediatric orthopedics in practice. Springer, 1998, ISBN 3-540-61480-X , p. 478.
  3. A. Gomez-Brouchet, J. Sales de Gauzy, F. Accadbled, A. Abid, MB Delisle, JP Cahuzac: Congenital pseudarthrosis of the clavicle: a histopathological study in five patients. In: Journal of pediatric orthopedics. Part B. Vol. 13, No. 6, 2004, pp. 399-401, PMID 15599233 .
  4. ^ L. Saint-Pierre: Pseudarthrosis congénitale de la clavicule droite. In: Ann Anat Pathol (Paris) . Vol. 7, 1930, pp. 625-627.