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Brachyolmia-amelogenesis imperfecta syndrome

ORPHA:2899· ICD-10 Q76.3

Definition

An exceedingly rare form of brachyolmia, characterized by mild platyspondyly, broad ilia, elongated femoral necks with coxa valga, scoliosis, and short trunked short stature associated with amelogenesis imperfecta of both primary and permanent dentition.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Childhood