A case‐controlled comparison of postoperative analgesic dosing between girls with Rett syndrome and girls with and without developmental disability undergoing spinal fusion surgery

Abstract

Background Rett syndrome (RTT) 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 RTT. Aim The goal of this preliminary investigation was to compare postoperative analgesic management among a sample of girls with RTT compared to girls with and without developmental disability undergoing spinal fusion surgery. Method The medical records of 8 girls with Rett syndrome (mean age=13.2 years, SD=1.9), 8 girls with developmental disability (cerebral palsy [CP]; mean age= 13.1 years, SD= 2.0), and 8 girls without developmental disability (adolescent idiopathic scoliosis [AIS]; mean age=13.4, SD=1.8) were reviewed. Data related to demographics, medications, and route of drug administration were recorded. Results Girls with RTT received significantly fewer morphine equivalent opioids postoperatively (M = 0.26 mg/kg/day, SD = 0.10) compared to girls with AIS (M = 0.47 mg/kg/day, SD= 0.13; 95% CI −0.34 to −0.08; p=.001) and girls with CP (M = 0.40 mg/kg/day, SD = 0.14; 95% CI −0.27 to −0.02; p = .01). Girls with RTT received significantly fewer opioid patient controlled analgesic (PCA) bolus doses (given by proxy; M=42.63, SD=17.84) compared to girls with AIS (M=98.25, SD=52.77; 95% CI −96.42 to −14.83; p = .01). There was also some evidence indicating girls with RTT received fewer bolus doses compared to girls with CP (M=80.88, SD=38.93; 95% CI −79.05 to 2.55; p=.06). On average, girls with RTT also received smaller total doses of acetaminophen, diazepam and hydroxyzine. Conclusion This study highlights possible discrepancies in postoperative pain management specific to girls with RTT and suggests further investigation is warranted to determine best practice for postoperative analgesic management for this vulnerable patient population.

Timeline of diagnosed pain causes in children with severe neurological impairment

“…Venipuncture is a distressing procedure in children with SNI compared to healthy peers ( 31 , 32 ), and these data highlighted the need for pain management strategies in this population, such as the mandatory use of topical anaesthesia. Likewise, the literature highlights the risk of undertreatment of pain after surgery in children with SNI ( 9 , 10 ). This series’ high number of surgical procedures reminds paediatricians of the need for careful pain evaluation and treatment after surgery.…”

“…Various studies offered hints and lists of features that indicate pain in non-verbal children and highlighted possible expressions of pain ( 1 ). This assessment is critical in acute pain episodes and in the context of procedural or post-operative pain where there is still a higher risk of undertreatment ( 9 , 10 ). Gross Motor Function Classification System (GMFCS) is a pragmatic system that further delineates the severity of motor and functional limitations mostly of children with cerebral palsy, but it is also broadly used in other patients with SNI and CMC ( 11 ).…”

Intensive Postural and Motor Activity Program Reduces Scoliosis Progression in People with Rett Syndrome

“…Surgery should not be delayed until skeletal maturity has been achieved. Barney and colleagues suggested the need for RTT-specific post-operative pain management [22]. The authors reported that, after spinal fusion, patients with RTT received fewer total doses of opioids compared to girls with cerebral palsy and idiopathic scoliosis.…”