Steel syndrome: dislocated hips and radial heads, carpal coalition, scoliosis, short stature, and characteristic facial features.
BACKGROUND
A syndrome of children with short stature, bilateral hip dislocations, radial head dislocations, carpal coalitions, scoliosis, and cavus feet in Puerto Rican children, was reported by Steel et al in 1993. The syndrome was described as a unique entity with dismal results after conventional treatment of dislocated hips. The purpose of this study is to reevaluate this patient population with a longer follow-up and delineate the clinical and radiologic features, treatment outcomes, and the genetic characteristics.


METHODS
This is a retrospective cohort study of 32 patients in whom we evaluated the clinical, imaging data, and genetic characteristics. We compare the findings and quality of life in patients with this syndrome who have had attempts at reduction of the hips versus those who did not have the treatment.


RESULTS
Congenital hip dislocations were present in 100% of the patients. There was no attempt at reduction in 39% (25/64) of the hips. In the remaining 61% (39/64), the hips were treated with a variety of modalities fraught with complications. Of those treated, 85% (33/39) remain dislocated, the rest of the hips continue subluxated with acetabular dysplasia and pain. The group of hips that were not treated reported fewer complaints and limitation in daily activities compared with the hips that had attempts at reduction.


CONCLUSIONS
Steel syndrome is a distinct clinical entity characterized by short stature, bilateral hip and radial head dislocation, carpal coalition, scoliosis, cavus feet, and characteristic facial features with dismal results for attempts at reduction of the hips.


METHODS
Prognostic Study Level II.
Flynn
John M
JM
Department of Pediatric Orthopeadic Surgery, Hospital La Concepción, San German, Puerto Rico. jmflynnmd@aol.com
Ramirez
Norman
N
Betz
Randal
R
Mulcahey
Mary Jane
MJ
Pino
Franz
F
Herrera-Soto
Jose A
JA
Carlo
Simon
S
Cornier
Alberto S
AS
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000503:physiopathology*; D000293:Adolescent; D000328:Adult; D001827:Body Height; D002348:Carpal Bones / Q000002:abnormalities / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006618:Hip Dislocation, Congenital / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000628:therapy; D006801:Humans; D004204:Joint Dislocations / Q000151:congenital / Q000000981:diagnostic imaging; D008297:Male; D008875:Middle Aged; D011647:Puerto Rico; D011788:Quality of Life; D011859:Radiography; D011884:Radius / Q000000981:diagnostic imaging / Q000503:physiopathology; D012189:Retrospective Studies; D012600:Scoliosis / Q000000981:diagnostic imaging / Q000503:physiopathology*; D013577:Syndrome; D013997:Time Factors; D055815:Young Adult