Outcomes of Growth-Friendly Surgery and Posterior Spinal Fusion in Children With Rett Syndrome and Early Onset Scoliosis.

Abstract

Rett syndrome (RS) is a rare neurodevelopmental disorder often associated with progressive scoliosis requiring treatment. Surgical interventions include magnetically controlled growing rods (MCGR) to allow spinal growth in younger patients or posterior spinal fusion (PSF) for definitive curve correction and stability. There is limited literature on outcomes of growth-friendly surgical treatment in RS patients with scoliosis. This multicenter retrospective review included RS patients with scoliosis treated with MCGR or primary PSF with minimum 2-year follow-up. Demographics, radiographic measures, surgical data, complications, unplanned returns to the operating room, and EOSQ-24 scores were recorded pre- and postindex surgery. The study included 15 females with Rett syndrome. Five patients had MCGR and 10 underwent PSF. The mean age of the MCGR group was 8.5 years (range: 7.6 to 9.2) and the PSF group was 12.8 years (range: 10.1 to 17.7). Mean preindex major curve magnitude was similar between the MCGR and PSF patients [75 (range: 67 to 91) vs. 80 degrees (range: 53 to 116)]. At final follow-up, the PSF group achieved greater major curve correction than the MCGR group [35 (range: 21 to 58) vs. 42 degrees (range: 26 to 59)]. The MCGR group had greater mean preindex maximum kyphosis [71 (range: 53 to 89) vs. 57 degrees (range: 18 to 96)] but mean maximum kyphosis at final follow-up was similar between the MCGR and PSF patients [54 (range: 42 to 86) vs. 50 degrees (range 18 to 78)]. Mean preindex T1-T12 and T1-S1 lengths were shorter in the MCGR patients [T1-T12: 16.6 cm (range 13.8 to 19.7) vs. 19.4 cm (range: 14.8 to 23.2); T1-S1: 28.6 cm (range: 25.2 to 33.0) vs. 31.0 cm (range: 22.3 to 37.7)]. Final mean T1-T12 lengths were 22.2 cm (range: 19.8 to 25.4) for the MCGR group and 23.8 cm (range: 20.3 to 26.9) for the PSF group; mean T1-S1 lengths were 35.3 cm (range 31.9 to 40.8) and 36.9 cm (range: 32.3 to 42.6), respectively. There was one complication per group. One MCGR patient had acute respiratory failure requiring prolonged hospitalization, and one PSF patient had a wound infection requiring surgical intervention and antibiotics. MCGR patients showed declines in more EOSQ-24 domains than PSF patients between preindex surgery and last follow-up. Both MCGR and PSF effectively control scoliosis in RS patients. PSF provides superior coronal curve correction, while MCGR promotes spinal growth. The impact of spinal surgery on quality of life warrants further investigation. Larger studies are needed to validate these results and guide individualized treatment for this unique population. Therapeutic level IV.