Growth Friendly Surgery and Serial Cast Correction in the Treatment of Early-onset Scoliosis for Patients With Prader-Willi Syndrome

Abstract

Level IV-therapeutic study.

Clinical Observations and Treatment Approaches for Scoliosis in Prader–Willi Syndrome

“…Severe curves are casted until the child is old enough to undergo surgery for an expandable spine implant (5-8 years of age) ( Figure 4). Oore et al reported the only published series of serial casting for children with PWS, noting a reduction of curve size in their ten patients, from 45° to 37° [40]. We reviewed our results from 34 children with PWS with more than 24 months' follow-up after their initial spine cast.…”

“…In addition, up to 62% of patients with PWS have low bone mineral density and other musculoskeletal manifestations [11,[42][43][44][45][46]. The combination of the PJK/DJK and bone weakness leads to a rate of hardware pull out/failure/rod fracture between 17% and 31% [9,40,41,47,48]. The consequences can be catastrophic [49].…”

“…Patients with PWS exhibit "skin picking", which can lead to invasion or injury of their incisions. Combined with potential poor quality of the soft tissues, the rate of wound dehiscence and related deep infections are reported between 19% and 38% of cases [40,41,48]. To avoid these issues, a negative pressure wound therapy bandage is placed over the incision for the first five days, both to help with any seepage and to act as an "early warning" system, as the pump will alarm if the patient breaks the pressure seal by scratching their incision.…”

“…The rods are lengthened every three months, using an external spinning magnet, which causes the magnet within the rod to rotate thereby lengthening the rod (Figure 4). Oore et al found that the average initial curve was reduced from 76 • to 42 • at time of implant surgery with no change in curve size over the subsequent two years of rod elongation [40]. Expandable implants, as a technique, have a life span of approximately five years, after which the ongoing process of spontaneous fusion along the section of the instrumented spine leads to stiffness and an inability to lengthen further [50,51].…”