Steel syndrome

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.

Disease data

Classification

Disease

Synonyms

Bilateral hip and radial head dislocations-short stature-scoliosis-carpal coalitions-pes cavus-facial dysmorphism syndrome

Zespół obustronnych zwichnięć bioder i głowy kości promieniowej, niskiego wzrostu, skoliozy, zrostów kości nadgarstka, stopy wydrążonej i dysmorfii twarzy

ORPHA code

438117

OMIM code

615155

ICD10 code

Q87.5

ICD11 code

*Soruce