Musculoskeletal manifestations of Russell-Silver syndrome.
The musculoskeletal manifestations of Russell-Silver syndrome were studied in 25 patients. The most common manifestations were short stature (25 patients), limb-length discrepancy (23 patients), clinodactyly (19 patients) metacarpal bone and phalangeal abnormalities (13 patients), scoliosis (9 patients), foot syndactylism (5 patients), and developmental dysplasia of the hips (3 patients). Five patients underwent lower extremity limb-lengthening procedures for discrepancies greater than 3 cm and three patients had successful pelvic and/or femoral osteotomies for hip dysplasia. All 18 patients studied had retardation of bone age, which peaked at age 7 years.
Abraham
Edward
E
Department of Orthopaedics, The University of Illinois College of Medicine at Chicago, Chicago, Illinois 60612, USA. eda@uic.edu
Altiok
Haluk
H
Lubicky
John P
JP
D000015:Abnormalities, Multiple / Q000473:pathology*; D000293:Adolescent; D001827:Body Height; D001848:Bone Diseases, Developmental / Q000473:pathology*; D019080:Cafe-au-Lait Spots / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D004550:Elbow / Q000002:abnormalities; D005260:Female; D005385:Fingers / Q000002:abnormalities; D005532:Foot Deformities, Congenital / Q000473:pathology; D005865:Gestational Age; D006130:Growth Disorders / Q000473:pathology*; D006228:Hand Deformities, Congenital / Q000473:pathology; D006618:Hip Dislocation, Congenital / Q000473:pathology; D006801:Humans; D007223:Infant; D007719:Knee Joint / Q000002:abnormalities; D007870:Leg Length Inequality / Q000473:pathology; D008297:Male; D008663:Metacarpus / Q000002:abnormalities; D012189:Retrospective Studies; D012600:Scoliosis / Q000473:pathology; D013576:Syndactyly / Q000473:pathology; D013577:Syndrome; D014034:Toes / Q000002:abnormalities