A case-controlled comparison of postoperative analgesic dosing between girls with Rett syndrome and girls with and without developmental disability undergoing spinal fusion surgery.
BACKGROUND
Rett syndrome is associated with severe motor and communicative impairment making optimal postoperative pain management a challenge. There are case reports documenting reduced postoperative analgesic requirement in Rett syndrome.


OBJECTIVE
The goal of this preliminary investigation was to compare postoperative analgesic management among a sample of girls with Rett syndrome compared to girls with and without developmental disability undergoing spinal fusion surgery.


METHODS
The medical records of eight girls with Rett syndrome (mean age = 13.2 years, sd = 1.9), eight girls with developmental disability (cerebral palsy; mean age = 13.1 years, sd = 2.0), and eight girls without developmental disability (adolescent idiopathic scoliosis; mean age = 13.4, sd = 1.8) were reviewed. Data related to demographics, medications, and route of drug administration were recorded.


RESULTS
Girls with Rett syndrome received significantly fewer morphine equivalent opioids postoperatively (M = 0.26 mg·kg-1·day-1, sd = 0.10) compared to girls with adolescent idiopathic scoliosis (M = 0.47mg·kg-1·day-1, sd = 0.13; 95% CI -0.34 to -0.08; P = 0.001) and girls with CP (M = 0.40 mg·kg-1per day, sd = 0.14; 95% CI -0.27 to -0.02; P = 0.01). Girls with Rett syndrome received significantly fewer opioid patient-controlled analgesic (PCA) bolus doses (given by proxy; M = 42.63, sd = 17.84) compared to girls with adolescent idiopathic scoliosis (M = 98.25, sd = 52.77; 95% CI -96.42 to -14.83; P = 0.01). There was also some evidence indicating girls with Rett syndrome received fewer bolus doses compared to girls with CP (M = 80.88, sd = 38.93; 95% CI -79.05 to 2.55; P = 0.06). On average, girls with Rett syndrome also received smaller total doses of acetaminophen, diazepam, and hydroxyzine.


CONCLUSIONS
This study highlights possible discrepancies in postoperative pain management specific to girls with Rett syndrome and suggests further investigation is warranted to determine best practice for postoperative analgesic management for this vulnerable patient population.
Gillette Children's Specialty Healthcare, St. Paul, MN, USA.
Chantel C
CC
Barney
Department of Educational Psychology, University of Minnesota, Minneapolis, MN, USA.
Alyssa M
AM
Merbler
Department of Educational Psychology, University of Minnesota, Minneapolis, MN, USA.
Kelsey
K
Quest
Department of Educational Psychology, University of Minnesota, Minneapolis, MN, USA.
Breanne J
BJ
Byiers
Department of Neuroscience, Pharmacology and Dermatology, University of Minnesota Medical School, Minneapolis, MN, USA.
George L
GL
Wilcox
Gillette Children's Specialty Healthcare, St. Paul, MN, USA.
Scott
S
Schwantes
Gillette Children's Specialty Healthcare, St. Paul, MN, USA.
Samuel A
SA
Roiko
Rett Syndrome Clinic, Gillette Children's Specialty Healthcare, St. Paul, MN, USA.
Timothy
T
Feyma
Rett Syndrome Clinic, Gillette Children's Specialty Healthcare, St. Paul, MN, USA.
Arthur
A
Beisang
Department of Educational Psychology, University of Minnesota, Minneapolis, MN, USA.
Frank J
FJ
Symons
D000293:Adolescent; D016058:Analgesia, Patient-Controlled / Q000706:statistics & numerical data; D000701:Analgesics, Opioid / Q000627:therapeutic use*; D016022:Case-Control Studies; D002658:Developmental Disabilities / Q000150:complications*; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D010149:Postoperative Pain / Q000188:drug therapy*; D015518:Rett Syndrome / Q000150:complications* / Q000601:surgery*; D013123:Spinal Fusion
D000701:Analgesics, Opioid
adolescent; communication disorders; opioid; pain; pediatric; scoliosis