The hip in Stickler syndrome

Citation
Ps. Rose et al., The hip in Stickler syndrome, J PED ORTH, 21(5), 2001, pp. 657-663
Citations number
47
Categorie Soggetti
Ortopedics, Rehabilitation & Sport Medicine
Journal title
JOURNAL OF PEDIATRIC ORTHOPAEDICS
ISSN journal
02716798 → ACNP
Volume
21
Issue
5
Year of publication
2001
Pages
657 - 663
Database
ISI
SICI code
0271-6798(200109/10)21:5<657:THISS>2.0.ZU;2-O
Abstract
Stickler syndrome is an autosomal dominant connective tissue disorder with a prevalence similar to that of Marfan syndrome. No previous study has exam ined hip pain or abnormalities in a large series of patients with Stickler syndrome. The purpose of this study was to describe hip abnormalities and t heir correlation with age and chronic hip pain in a cohort of 51 patients f ollowed at the National Institutes of Health. Ten percent of patients had p rotrusio acetabuli, 21% coxa valga, and 34% of adults had hip osteoarthriti s. Sixty-three percent of all patients and 79% of adults had chronic hip pa in. In additions 16% of adult patients had a history of femoral head failur e during youth. Arthritic changes and adult age were associated with hip pa in. In summary, hip abnormalities are commonly observed in Stickler syndrom e. Young patients require careful evaluation of hip pain, and regular scree ning of children with Stickler syndrome may be indicated for early detectio n of hip complications.