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

Abstract

Study design: Retrospective cohort study.

Objective: This study aimed to compare perioperative outcomes of Rett syndrome (RS) and cerebral palsy (CP) patients undergoing posterior spinal fusion for neuromuscular scoliosis.

Summary of background data: Surgical correction in the treatment of scoliosis for patients with RS has been shown to increase the survival rate. CP patients, like RS patients, are often nonverbal and nonambulatory, with frequent surgical complications.

Materials and methods: Retrospective review of 36 RS and 80 CP patients undergoing posterior spinal fusion from 2005 to 2023. Data and x-ray measurements were collected preoperatively and postoperatively. A subanalysis was performed comparing nonambulatory patients (GMFCS IV-V). The Wilcoxon-Rank Sum, Fisher exact, and χ 2 tests were utilized.

Results: The primary outcome measure, complication rates, was similar between the groups ( P =0.09). Preoperative Cobb angle, levels fused, fixation points, and LOS were similar ( P >0.05). EBL was significantly higher in CP patients, as was the rate of transfusion ( P =0.001) and surgical time ( P =0.001). Postoperative Cobb angle ( P =0.002) was significantly higher for CP patients. There was no significant difference between CP and RS patients in both preoperative ( P =0.383) and postoperative ( P =0.051) coronal decompensation. Nonambulatory status was associated with increased odds of having a postoperative complication (OR=6.17, 95% CI=1.36-28.04). Subanalysis of nonambulatory RS and CP patients revealed significantly higher postoperative Cobb ( P =0.008), EBL ( P =0.019), and surgical time ( P =0.017) in CP patients compared with RS patients. There were no significant differences in preoperative Cobb, levels fused, fixation points, hospital stay, or complication rate ( P >0.05).

Conclusion: RS patients are shown to have better outcomes than CP patients in terms of surgical, perioperative, and radiographic variables. Ambulatory status was identified as an independent risk factor for complications.