Congenital clavicle osteoarthritis
Classification according to ICD-10 | |
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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 .
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:
- Traumatic birth clavicular fracture
- Cleidocranial Dysostosis
- Post-traumatic pseudarthrosis
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
- ↑ a b c Congenital clavicle osteoarthritis. In: Orphanet (Rare Disease Database).
- ↑ a b c F. Hefti: Pediatric orthopedics in practice. Springer, 1998, ISBN 3-540-61480-X , p. 478.
- ↑ 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 .
- ^ L. Saint-Pierre: Pseudarthrosis congénitale de la clavicule droite. In: Ann Anat Pathol (Paris) . Vol. 7, 1930, pp. 625-627.