Skip to Main Content

We have a new app!

Take the Access library with you wherever you go—easy access to books, videos, images, podcasts, personalized features, and more.

Download the Access App here: iOS and Android. Learn more here!

Asymmetrical long bone bowing with a “beaten-metal” appearance of the skull, dolichomacrocephaly, and ocular hypertelorism.

Bowing of Long Bones, Asymmetrical and Symmetrical.

Very rare; only a few cases described.

Two sibs with congenital asymmetrical long bone bowing and one with congenital symmetrical long bone bowing were described. Other abnormalities included beaten-metal appearance of the skull, dolichomacrocephaly, ocular hypertelorism, and bone-within-bone appearance of vertebrae. Differential diagnosis must be made with camptomelic dysplasia, kyphomelic dysplasia, hypophosphatasia, Grant syndrome, and osteogenesis imperfecta.

This illness is a special mild form of the other bone dysplasia genetic syndromes and has no special repercussion on anesthesia technique, with the potential exception of positioning and access for locoregional anesthesia.

Grant Syndrome: Persistent wormian bones, blue sclerae, mandibular hypoplasia, shallow glenoid fossae, camptomelia, and hypotonia. Apparently, it is an autosomal dominant trait.

Osteogenesis Imperfecta: Inherited disease responsible for varying degrees of skeletal fragility. Minimal trauma is sufficient to cause fractures and bone deformities.

Moore LA, Moore CA, Smith JA, et al: Asymmetric and symmetric long bone bowing in two sibs: An apparently new bone dysplasia. Am J Med Genet 47:1072, 1993.  [PubMed: 8291526]

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.