African Journal of Paediatric Surgery About APSON | PAPSA  
Home About us Editorial Board Current issue Search Archives Ahead Of Print Subscribe Instructions Submission Contact Login 
Users Online: 829Print this page  Email this page Bookmark this page Small font size Default font size Increase font size 
 
ORIGINAL ARTICLE
Year : 2015  |  Volume : 12  |  Issue : 1  |  Page : 36-40

Correction of the axial and appendicular deformities in a patient with Silver-Russel syndrome


1 Department of First Medical, Ludwig Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, Hanusch Hospital; Department of Paediatric, Orthopaedic Hospital of Speising, Vienna, Austria
2 Department of Paediatric, Orthopaedic Hospital of Speising, Vienna, Austria
3 Department of First Medical, Ludwig Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, Hanusch Hospital, Vienna, Austria

Correspondence Address:
Dr. Ali Al Kaissi
Department of First Medical, Ludwig-Boltzmann Institute of Osteology, Hanusch Hospital of WGKK and AUVA Trauma Center Meidling, Hanusch Hospital and Orthopaedic Hospital of Speising, Vienna
Austria
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.150969

Clinical trial registration EK Nr. 921/2009

Rights and Permissions

Background: Scoliosis and limb length discrepancy are the major orthopaedic abnormalities in patients with Silver-Russel syndrome (SRS). In this paper, we describe a series of orthopaedic interventions in an attempt to overcome the progressive pathologic mechanism in a 7-year-old girl who manifested the full phenotypic features of SRS. Materials and Methods: Unilateral hip dislocation, progressive scoliosis and limb length discrepancy have been dealt with through Pemberton osteotomy, spinal fusion and Taylor-Spatial-Frame respectively. Results: In order to correct the axial and the appendicular deformities a sum of seven operations were performed (between the age of 7 years and 13 years). Pemberton osteotomy was performed to treat dislocation of her right hip because of developmental dysplasia of the hip. Spinal fusion (spondylodesis) of segments Th3-L5 was done to correct her scoliosis. And, to overcome the limb length discrepancy of 15-cm we used Taylor-Spatial-Frame with percutaneous distal corticotomy of the femur, and the proximal tibia, as well as the foot, were performed. We were able to minimize the limb length discrepancy to 5 cm. The girl became able to walk with the aid of a below knee orthosis and through lifting the left limb with 5-cm height shoe. Conclusion: Limb lengthening surgery in patients with multiple malformation complex as in SRS is associated with high recurrence risk because of; muscular hypotonia, overtubulation of the long bones, and the poor bone regenerative quality. Our interventions were principally directed towards improving the cosmetic outlook, functions and the biomechanics.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed4651    
    Printed71    
    Emailed0    
    PDF Downloaded132    
    Comments [Add]    

Recommend this journal