Perioperative Considerations in Patients With Rett Syndrome as Compared With Those With Cerebral Palsy.

Area of Science:

• Neurosurgery
• Orthopedic Surgery
• Pediatric Spine Surgery

Background:

• Rett syndrome (RS) and Cerebral Palsy (CP) patients often present with neuromuscular scoliosis.
• Surgical correction of scoliosis in RS patients has been linked to improved survival rates.
• Both RS and CP patients frequently experience surgical complications, and are often nonverbal and nonambulatory.

Purpose of the Study:

• To compare perioperative outcomes of posterior spinal fusion (PSF) for neuromuscular scoliosis in patients with Rett syndrome (RS) versus Cerebral Palsy (CP).
• To identify risk factors for complications in this patient population.

Main Methods:

• Retrospective cohort study of 36 RS and 80 CP patients undergoing PSF between 2005 and 2023.
• Collected and analyzed pre- and post-operative radiographic data, including Cobb angle and coronal decompensation.
• Compared complication rates, estimated blood loss (EBL), surgical time, and length of stay (LOS); sub-analysis focused on non-ambulatory patients (GMFCS IV-V).

Main Results:

• Complication rates were similar between RS and CP groups (P=0.09).
• CP patients had significantly higher EBL (P=0.001), transfusion rates (P=0.001), and surgical time (P=0.001).
• Postoperative Cobb angle was significantly worse in CP patients (P=0.002); nonambulatory status increased odds of complications (OR=6.17).

Conclusions:

• Rett syndrome patients demonstrated superior surgical, perioperative, and radiographic outcomes compared to Cerebral Palsy patients undergoing PSF.
• Ambulatory status was identified as a significant independent risk factor for postoperative complications.
• These findings suggest potential differences in surgical management strategies for neuromuscular scoliosis in RS versus CP patients.