Perioperative management and outcome of patients with Rett syndrome undergoing scoliosis surgery: a retrospective review

Karmaniolou, Iosifina; Krishnan, Ramprabu; Galtrey, Eleanor; Cleland, Stuart; Ramesh, Vijayaraghavan

doi:10.1007/s00540-015-1974-3

Cited by 11 publications

(15 citation statements)

References 22 publications

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“…Similarly, as many as 40% of people with Rett syndrome have been reported to undergo corrective spinal surgery (Anderson, Wong, Jacoby, Downs, & Leonard, ) and children with Rett syndrome are very likely to undergo spinal fusion (Karmaniolou et al., ). Whilst positive outcomes of spinal surgery have been reported (Downs et al., ; Kerr et al., ; Larsson et al., ), it is associated with a high rate of post‐operative major medical complications (Gabos, Inan, Thacker, & Borkhu, ).…”

Section: Discussionmentioning

confidence: 99%

“…<25%; Cans et al, 1999) of those with intellectual disabilities requiring postural care will have cerebral palsy. Indeed, a number of specific conditions related to intellectual disabilities may result in the need for postural care (Ager et al, 2006;Burns, Powell, Hsia, & Reinker, 2003;Butler et al, 2002;Damasceno, Cristante, Marcon, & Barros Filho, 2014;Detweiler, Thacker, Hopkins, Conway, & Gripp, 2013;Imagama et al, 2013;Karmaniolou, Krishnan, Galtrey, Cleland, & Vijayaraghavan, 2015;Kerr, Webb, Prescott, & Milne, 2003;Laurier et al, 2015;Li, Shen, Liang, & Sheng, 2015;de Lind van Wijngaarden, de Klerk, Festen, & Hokken-Koelega, 2008;Takebayashi et al, 2006).…”

Section: Introductionmentioning

confidence: 99%

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Postural care for people with intellectual disabilities and severely impaired motor function: A scoping review

Robertson

Baines

Emerson

et al. 2016

Research Intellect Disabil

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Background: Poor postural care can have severe and life-threatening complications.This scoping review aims to map and summarize existing evidence regarding postural care for people with intellectual disabilities and severely impaired motor function.Method: Studies were identified via electronic database searches (MEDLINE, CINAHL, PsycINFO and Web of Science) covering January 1990 to March 2016, and email requests to researcher networks. Results were collated via descriptive numerical summary of studies and thematic analysis. Results:Twenty-three studies were identified and summarized narratively in relation to three themes: characteristics and prevalence, interventions and service related issues. The evidence base is small with significant gaps. Lack of evidence for nighttime positioning equipment and 24-hr postural care needs to be addressed urgently. Conclusion:Future research should be clearly directed towards ascertaining how best postural care interventions can be employed to help improve the health and quality of life of people with intellectual disabilities. K E Y W O R D Sintellectual disabilities, postural care, scoping review |Published for the British Institute of Learning Disabilities ROBERTSON ET al. posture also underpin the ability to function and access the environment (Crawford & Stinson, 2015).Over the past 15 years, there has been increased awareness of the need to provide postural care over the entire 24-hr period. This usually involves adaptive seating and/or moulded wheelchairs; night-time positioning equipment (NTPE); moving and handling techniques; and advice and training across all settings that the person accesses (Crawford & Stinson, 2015). NTPE can be used during the substantial proportion of the day when the person is in bed and has been reported to be beneficial even for those with established body shape distortion (Goldsmith, 2000).It has been proposed that most people who lack the ability to change their position would benefit from 24-hr body positioning (Crawford & Stinson, 2015) and that 24-hr postural care strategies should be considered in physical therapy programmes to prevent or delay the development of contractures or skeletal deformities in children and young people at risk of developing these (National Institute for Health & Care Excellence, 2012). However, disabled children generally are not being provided with the specialist equipment they require (such as seating and postural support systems), with provision of equipment being patchy at best (Centre for Economics and Business Research, 2014). It is widely accepted that the current systems that provide equipment and related services to children fail (CSED Consultant's Report, 2010).The importance of the issue of postural care in relation to people with intellectual disabilities has been highlighted in a number of reports noting gaps in services and the need to provide postural care interventions to prevent postural deformities from developing (Heslop et al., 2013;Mansell, 2010;Michael, 2008, RCGP, 2012. No systematic...

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Postural care for people with intellectual disabilities and severely impaired motor function: A scoping review

Robertson

Baines

Emerson

et al. 2016

Research Intellect Disabil

View full text Add to dashboard Cite

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“…In their postoperative study of Rett syndrome, Karmaniolou et al . did not detect differences in postoperative pain management of girls with Rett syndrome undergoing scoliosis surgery when comparing the experiences of 24 girls to existing pediatric postoperative guidelines. While this is an encouraging finding, the current sample, although smaller, used matched control groups (developmentally typical and developmentally disabled) on age, gender, and surgical procedure—perhaps increasing the ability to detect differences specific to the surgical procedure and its postoperative analgesic management.…”

Section: Discussionmentioning

confidence: 85%

“…The purpose of the current study was to further compare postoperative pain assessment scores and management practices in girls with Rett syndrome and extend the Karmaniolou et al . study by comparing a Rett syndrome sample against age‐ and gender‐matched controls across the same surgical procedure. Because previous studies have shown a discrepancy in pain management based on cognitive impairment associated with developmental delay , two comparison groups were included in this study.…”

Section: Introductionmentioning

confidence: 99%

“…A recent retrospective review of 24 girls with Rett syndrome undergoing 29 surgical procedures did not confirm the case study reports of reduced analgesic requirements following surgery. Although a control group was not included, the type and dosing of opioids delivered did not differ from standard practice for pediatric populations (2). Additionally, when eight girls with Rett syndrome were compared to large samples of girls with idiopathic scoliosis and girls with neurologic scoliosis, Rumbak et al (15) found that girls with Rett syndrome actually received significantly more morphine equivalent opioids postoperatively compared to the typically developing girls with idiopathic scoliosis.…”

Section: Introductionmentioning

confidence: 99%

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

Barney

Merbler

Quest

et al. 2017

Pediatric Anesthesia

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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.

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Neuromuscular Scoliosis: An Overview

2022

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Perioperative management and outcome of patients with Rett syndrome undergoing scoliosis surgery: a retrospective review

Abstract: Our case series demonstrates a high incidence of complications in this subpopulation, mainly postoperative. Extreme postoperative vigilance is required and recovery in a high dependency unit is highly recommended.

Cited by 11 publications

References 22 publications

Postural care for people with intellectual disabilities and severely impaired motor function: A scoping review

Postural care for people with intellectual disabilities and severely impaired motor function: A scoping review

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

Neuromuscular Scoliosis: An Overview

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