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

Abstract

Prader-Willi syndrome (PWS) patients can present with scoliosis which can be treated with serial cast correction (SCC) or with growth friendly surgery (GFS). This study's purpose was to describe the results of SCC as well as GFS for PWS patients with early-onset scoliosis (EOS). PWS patients were identified from 2 international multicenter EOS databases. Scoliosis, kyphosis, spine height (T1-S1), right/left hemithoracic heights/widths (RHTH, LHTH, RHTW, LHTW) were measured pretreatment, postoperation, and at 2-year follow-up. Complications were recorded. Overall, 23 patients with 2-year follow-up were identified. Pretreatment; patients treated with SCC (n=10) had mean age of 1.8±0.6 years; body mass index (BMI), 16±1.5 kg/m; scoliosis, 45±18 degrees; kyphosis, 56±9 degrees; T1-S1, 22.4±2.4 cm; RHTH, 8.0±2.0 cm; LHTH, 8.5±1.7 cm; RHTW, 6.6±1.3 cm; and LHTW, 8.0±1.0 cm. Patients treated with GFS (n=13) had mean age of 5.8±2.6 years; BMI, 21±5.4 kg/m; scoliosis, 76±14 degrees; kyphosis, 59±25 degrees; T1-S1, 24.1±3.6 cm; RHTH, 10.0±1.6 cm; LHTH, 10.6±1.6 cm; RHTW, 9.4±2.5 cm; and LHTW, 8.1±2.8 cm. At 2-year follow-up, patients treated with SCC had mean scoliosis 37±11 degrees (18% correction, P=0.06); kyphosis, 42±6 degrees (NS); T1-S1, 26.4±2.1 cm (P<0.01); RHTH, 9.0±1.1 cm (13%; P=0.30); LHTH, 10.0±1.5 cm (18%, P<0.01); RHTW, 7.4±1.1 cm (12%, P<0.01); and LHTW, 8.0±1.0 cm (0%, P=0.34). At 2-year follow-up, patients treated with GFS had mean scoliosis 42±13 degrees (45% correction, P<0.000001); kyphosis, 53±13 degrees (10%, P=0.19); T1-S1, 31.5±5.4 cm (P<0.00001); RHTH, 12.0±2.4 cm (20%; P<0.01); LHTH, 12.0±1.7 cm (13%; P<0.01); RHTW, 9.8±1.3 cm (4%; P=0.27); and LHTW, 7.9±2.3 cm (3%;P=0.11). As an entire group, patients with a BMI>17 kg/m² had more device-related than disease-related complications (P=0.09). Patients treated with SCC had 0.9 complications per patient. Patients treated with GFS had 2.2 complications per patient [≤5 y more often had ≥2 complications (P=0.05)]. At 2-year follow-up, SCC and GFS were both effective in treating EOS in PWS patients. Patients treated with SCC had significant improvements in spine height and LHTH. Patients treated with GFS had significant improvements in scoliosis magnitude, spine height, RHTH, and LHTH. Level IV-therapeutic study.