- Discussion:
- autosomal dominant proportionate dwarfism that affects bones formed
intramembranously (eg. facial bones, cranium, & clavicles);
- dislocations of hips, knees, and the radial heads may occur;
- clavicle:
- most commlonly, absence of the central portion of the clavicle with
rudimentary medial and lateral portions remaining;
- may be confused w/
pseurdoarthrosis of the clavicle
-
coxa vara:
- consider intertrochanteric osteotomy if varus is < 100 deg;
- reference:
- Management of developmental coxa vara in cleidocranial dysostosis.
MF Richie. et al.
Orthopaedics Vol 12. 1989. p 1001-1004.
- pubis: shows delayed ossification (hence, it may appear to be widened);
Medical Progress: Epiphyseal Disorders.
The epiphyseal dysplasias.
Sprenger J: CORR 1976;114:46.
Avascular necrosis of the hip in multiple epiphyseal dysplasia.
Mackenzie W et al.
J Pediatr Orthop
1989;9:666-671.
Development of the hip in multiple epiphyseal dysplasia. Natural history
and susceptibility to premature osteoarthritis.
