Spinal Deformity in Russell-Silver Syndrome

Kent T Yamaguchi Jr; Jennifer B Salem; Karen S Myung; Antonio N Romero Jr; David L Skaggs

doi:10.1016/j.jspd.2014.06.003

Spinal Deformity in Russell-Silver Syndrome

Spine Deform. 2015 Jan;3(1):95-97. doi: 10.1016/j.jspd.2014.06.003. Epub 2014 Dec 18.

Authors

Kent T Yamaguchi Jr¹, Jennifer B Salem¹, Karen S Myung¹, Antonio N Romero Jr², David L Skaggs³

Affiliations

¹ Children's Orthopaedic Center, Children's Hospital of Los Angeles, 4650 W Sunset Boulevard, Mailstop #69, Los Angeles, CA 90027, USA.
² Department of Orthopaedics, LAC/USC Medical Center, 1200 N. State Street, GNH 3900, Los Angeles, CA 90033, USA.
³ Children's Orthopaedic Center, Children's Hospital of Los Angeles, 4650 W Sunset Boulevard, Mailstop #69, Los Angeles, CA 90027, USA. Electronic address: DSkaggs@chla.usc.edu.

PMID: 27927458
DOI: 10.1016/j.jspd.2014.06.003

Abstract

Study design: Cross-sectional analysis.

Objectives: To evaluate the prevalence of scoliosis and kyphosis in patients with Russell-Silver syndrome (RSS).

Summary of background data: Russell-Silver syndrome was described by Silver and Russell in the 1950s and 1960s and is characterized by body asymmetry and other growth abnormalities. To the authors' knowledge, this is the first study to evaluate the prevalence of scoliosis and kyphosis in patients with Russell Silver Syndrome.

Methods: The authors performed a cross-sectional analysis of 163 persons, identified through a national RSS foundation, who consented to be included in the study and responded to a general survey questionnaire. Subjects who reported a diagnosis of scoliosis and/or kyphosis were subsequently asked to submit copies of prior spinal radiographs for evaluation at the authors' center. For evaluation of scoliosis and kyphosis on the radiographs, the researchers reviewed posteroanterior and lateral standing radiographs to measure coronal and sagittal Cobb angles.

Results: Of 163 respondents, 24 (14%) reported scoliosis, 5 (3.1%) reported kyphosis, and 6 (3.8%) reported both kyphosis and scoliosis, with average age of diagnosis of 8 years (range, 1-43 years). Of these respondents, 6 reported a history of bracing for scoliosis and/or kyphosis and 3 braced respondents reported having had surgery for scoliosis and/or kyphosis. An additional 3 respondents reported that corrective spinal surgery was "planned for the future."

Conclusions: Persons with RSS have a high prevalence of spinal deformity (21%) and a significant number of these patients will undergo corrective surgery (6 of 34; 18%).

Keywords: Kyphosis; Prevalence; Russell–Silver syndrome; Scoliosis; Spinal deformity.