46. Surgical outcomes in Rett syndrome patients are comparable to cerebral palsy patients

Abstract

BACKGROUND CONTEXT Rett syndrome (RS) patients often present with scoliosis, which requires surgical correction. However, literature is limited and reports major surgical complications including respiratory failure, prolonged intubation, infections, significant blood loss, and prolonged hospital stay. Cerebral palsy (CP) patients undergoing scoliosis surgery may be comparable to RS patients. They are often nonverbal, nonambulatory with frequent respiratory and surgical complications. Most surgeons have comparatively more experience treating CP patients than RS. PURPOSE The objective of this study is to compare perioperative outcomes of RS and CP patients. STUDY DESIGN/SETTING Retrospective review. PATIENT SAMPLE A total of 27 RS and 78 CP patients undergoing PSF from 2005-2018. OUTCOME MEASURES Cobb, kyphosis, pelvic obliquity, coronal decompensation, extubation rate, complication rate, EBL, surgical time, anesthesia time, levels fused, fixation points, METHODS Patient demographics, perioperative parameters, and X-ray measurements were collected at pre, post, and follow-up. Furthermore, a subanalysis was performed comparing the nonambulatory patients. Wilcoxon rank sums test and chi-square tests were utilized. RESULTS No significant difference for preop Cobb angle (p=0.16), kyphosis (p=0.20), and pelvic obliquity (p=0.09). No significant difference for fixation points (p=0.42), levels fused (p=0.79), time to extubation (p=0.53), complication rate (p=0.66), length of ICU (p=0.07) or hospital stay (p=0.71). EBL was significantly higher in CP patients (p=0.006) as was duration of anesthesia (p=0.04), and transfusion volume (p=0.01). However, surgical time (p=0.07) was similar. Postop Cobb (p=0.008) was significantly higher for CP, but kyphosis (p=0.18) and pelvic obliquity (p=0.09) were similar. Significant difference was found between both preop (p=0.02) and postop (p=0.03) coronal decompensation. Subanalysis of nonambulatory Rett and CP patients showed no significant difference in outcomes. CONCLUSIONS RS patients are comparable to CP patients in terms of surgical, periop, and radiographic outcomes. CP patients had significantly more EBL, intraop transfusions, and longer anesthesia time. The subjects did not differ in their ICU or hospital stays, nor their complication rate. Outcomes and periop expectations of RS patients are similar to CP. FDA DEVICE/DRUG STATUS This abstract does not discuss or include any applicable devices or drugs.