Scoliosis in Rett syndrome: a comparative analysis of postoperative complications

Rett syndrome, a neurodevelopmental disorder primarily affecting females, presents unique challenges in managing associated scoliosis. This study aims to evaluate the efficacy and challenges of posterior spinal fusion (PSF) in Rett syndrome patients by analyzing postoperative complications. A retrospective cohort study was conducted using a large national database. We included Rett syndrome patients aged 10-18 years who underwent PSF between 2010 and 2020. Outcomes such as medical and surgical complications, emergency department visits, readmissions, mortality, and reoperation rates up to 5 years were compared with a matched neuromuscular scoliosis (NMS) group. The study identified 195 Rett syndrome patients and 973 NMS patients. Post-surgery, Rett syndrome patients showed a significantly higher incidence of pneumothorax (56.9%, P < 0.001), respiratory failure (24.6%, P = 0.013), and pneumonia (26.2%, P < 0.001). Additionally, ileus (7.2%, P = 0.041), acute kidney injury (14.9%, P = 0.029), and urinary tract infections (14.9%, P < 0.001) were also significantly more frequent in the Rett syndrome group. Rett syndrome group also had higher rates of transfusion (11.3%, P = 0.004). Interestingly, the incidence of pseudarthrosis, implant complications, junctional failures, and the necessity for reoperation did not significantly differ at postoperative year 2. Mid-term follow-up showed that the reoperation rates over a 5-year period did not significantly differ between the Rett syndrome and NMS groups. Rett syndrome is associated with increased immediate postoperative complications, necessitating tailored preoperative planning, and intensive postoperative care. Despite these challenges, the mid-term surgical outcomes are comparable to those in NMS patients.