/Steel syndrome

Steel syndrome

Rare Disease

MONDO:0014061

Also Known As

bilateral hip and radial head dislocations-short stature-scoliosis-carpal coalitions-pes cavus-facial dysmorphism syndromesteel syndromeSTLSdislocated hips and radial heads, carpal coalition, scoliosis, and short stature

Definition

A rare genetic bone disease characterized by short stature, bilateral congenital hip dislocation, radial head dislocation, carpal coalition, scoliosis, pes cavus, and atlantoaxial subluxation. Dysmorphic facial features include broad forehead, broad nasal bridge, hypertelorism, and mild midface hypoplasia. Association with bilateral sensorineural hearing loss has also been described.