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

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

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Background: Scoliosis and limb length discrepancy are the major orthopaedic abnormalities in patients with Silver-Russel syndrome (SRS).In this paper, Boxes 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, Switch Tray functions and the biomechanics.