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

Abstract

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.

Limb Lengthening in Russell-Silver Syndrome: An Update Confirming Safe and Speedy Healing

“…We previously published what is, to our knowledge, the only case series in the literature reporting the outcomes of patients with RSS treated with limb lengthening for limb equalization purposes, with other prior studies in the form of case reports. 4,5 This 2013 series of 7 lengthened segments in 5 patients with RSS established that not only did this small group of patients have adequate bone healing after lengthening surgery; they in fact had statistically significantly faster bone healing than their counterparts with discrepancies attributable to tumor or congenital etiologies as determined by bone healing index (BHI), and similar healing rates to those with posttraumatic LLD. 5 However, due to small numbers in the primary cohort, trends within the RSS group with respect to which surgical and patient characteristics optimize bone healing after lengthening surgery could not be established, and the pace of healing has not been validated with larger patient numbers.…”