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Müller-Ribbing-Clément syndrome (Walther Müller)
Also known as:
Fairbank's disease
Fairbank's syndrome II
Ribbing's disease
Ribbing-Müller disease
Associated persons:
Robert Clément
Harold Arthur Thomas Fairbank
Walther Müller
Seved Ribbing
Description:
A rare bone disturbance characterised by stunted stature, obliteration of the hollow bones, premature degenerative atropathy, in particular of the hip joints. Dwarfism is moderate, with an average body height of 152 cm, and body proportions are normal. Other features include prominent and painful joints with limited mobility, waddling gait, thoracic kyphosis, and back pain. Evident at 5 to 10 years of age, with early signs of dysplasia at age 2-3 years. Sometimes pain and difficulty in walking (coxa vera; genu valgum). Absence of disability. Aetiology unknown.
In the Ribbing form the epiphyses are flat and involvement of the hands bones is mild. The Fairbank form is marked by small epiphyses, late ossification of irregular carpal bones, and changes of the metacarpal bones and phalanges.
The correct eponymic term for this condition is open for discussion. Since Harold Arthur Thomas Fairbank (1876-1961) described it first, Fairbank's disease seems the logical choice. The term Müller-Ribbing-Clément is common.
Bibliography:
- H. A. T. Fairbank:
Generalized disease of the skeleton.
Proceedings of the Royal Society of Medicine, 1935, 28: 1611-1619.
An Atlas of General Affections of the Skeleton.
Edinburgh, E. S. Livingstone, 1951, pp. 91-105.
Dysplasia epiphysialis multiplex.
British Journal of Surgery, London, 1947, 34: 225-232.
- S. Ribbing:
Studien über hereditäre multiple epiphysenstörungen.
Acta Radiologica. Supplement, 1937, 34: 1-105.
Hereditary multiple diaphyseal sclerosis.
Acta Radiologica, Stockholm, 1949, 31: 522-536.
- W. Müller:
Das Bild der multiplen erblichen Störungen der Epiphysenverknoecherung.
Zeitschrift für Orthopädie und ihre Grenzgebiete, Stuttgart, 1939, 69: 257.
- J. Spranger, L. O. Langer, H.-R. Wiedemann:
Bone Dysplasias. Fischer-Saunder, Stuttgart. Philadelphia, 1974. Pp. 10-15.
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