Impact of Spinal Deformity and Surgery on Health-Related Quality of Life in Cerebral Palsy: A Multicenter Prospective Controlled Trial.

BACKGROUND: Spinal fusion for scoliosis associated with cerebral palsy (CP) is challenging to study because specialized outcome measures are needed. Therefore, evidence in favor of the benefits of surgery has not been firmly established. This study aimed to determine if corrective spinal fusion improves health-related quality of life (HRQoL) in children with CP scoliosis at 2 years. METHODS: Children with CP and scoliosis who met the criteria for posterior spinal fusion were offered enrollment at 16 US and Canada centers. Participants' families selected either operative intervention (OP) or nonoperative treatment (NON) in discussion with their surgeon with no influence by the decision to participate in the research study. Demographic, clinical data (function level, magnitude of deformity, comorbidities), and HRQoL (CPCHILD Questionnaire) were collected at baseline and 2 years. Change (from baseline) in total CPCHIL scores was the primary outcome. RESULTS: Three hundred one OP and 34 NON subjects had complete baseline and 2-year data. At baseline, both groups were comparable in function level, comorbid status, and CPCHILD scores (52.1 ±15.3 vs. 53.4 ±14.5; P=0.66). The OP group had a larger spinal deformity magnitude (84.5˚ ± 21.8˚ vs. 66.3˚ ± 18.1˚) (P=0.001). The total CPCHILD score improved in the OP group by 6.6 points (P<0.001). NON scores were unchanged (+1.2; P=0.65) during follow-up. There were also significant score increases in the OP group for 5 of 6 CPCHILD domains. The change in CPCHILD scores from enrollment to 2 years was more significant in the OP group (P=0.05). CONCLUSION: For children with CP who undergo spinal fusion, HRQoL improved over preoperative levels and an unchanged nonoperative control group. LEVEL OF EVIDENCE: Level II.

Rib-on-Pelvis Deformity and Reported Pain in Neuromuscular Early-Onset Scoliosis.

BACKGROUND: Neuromuscular early-onset scoliosis (N-EOS) often presents with a long sweeping thoracolumbar scoliosis and pelvic obliquity. With severe pelvic obliquity, the ribs come into contact with the high side of the pelvis, termed rib-on-pelvis deformity (ROP). The goal of this study is to evaluate whether ROP is associated with reported pain and other health-related quality of life (HRQOL) measures. We hypothesize that ROP is associated with increased pain and negative HRQOL. METHODS: A multicenter international registry was queried for all nonambulatory patients with N-EOS from 2012 to 2022. Both surgical and nonsurgical patients were included. ROP was classified as a binary radiographic assessment of preoperative (surgical patients) and most recent follow-up (nonsurgical patients) upright radiographs. Reported pain and other HRQOL measures were assessed through the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24). Patients with nonupright radiographs or EOSQ-24 questionnaires and corresponding radiographs >4 months apart were excluded. RESULTS: Totally, 225 patients (8.4±3.1 y, 55% female) were included. The median major curve was 63.3 (IQR: 40.6 to 81.2) degrees and median pelvic obliquity was 15.5 degrees (IQR: 8.8 to 26.4). Eighty-three patients (37%) had ROP. ROP was associated with both frequency (P<0.001) and severity (P<0.001) of pain. ROP was associated with worse general health (P=0.01), increased difficulty with vocalization (P=0.02), increased frequency of shortness of breath (P=0.002), and increased difficulty sitting upright (P=0.04). Regarding overall EOSQ-24 domains, ROP was associated with worse general health, pain/discomfort, pulmonary function, and physical function (P<0.01). In a subanalysis of 76 patients who underwent surgical intervention with at least 2 years of follow-up, patients with preoperative ROP experienced significantly greater improvements in both frequency (P=0.004) and severity (P=0.001) of pain than the patients without preoperative ROP at 2 years postoperatively. CONCLUSIONS: The overall incidence of ROP in N-EOS is about 37%. ROP is associated with greater pain and worse HRQOL through the EOSQ-24 questionnaire. Furthermore, these patients experienced a greater reduction in pain after surgery. Clinicians and parents must be aware that ROP is possibly a pain generator, but responds positively to surgical intervention. LEVEL OF EVIDENCE: Level III.

Rib-on-pelvis deformity: a modifiable driver of pain and poor health-related quality of life in cerebral palsy.

PURPOSE: Cerebral Palsy (CP) often presents with a sweeping thoracolumbar scoliosis and pelvic obliquity. With severe pelvic obliquity, the ribs come into contact with the high side of the oblique pelvis, termed rib-on-pelvis deformity (ROP). ROP can result in costo-iliac impingement, or pain associated with ROP, and can also adversely affect breathing and sitting balance. The goal of this study was to evaluate whether CP patients with ROP have worse health-related quality of life (HRQOL) before surgery and a greater improvement in HRQOL after surgery. METHODS: A retrospective analysis of a prospectively collected, multicenter, international registry was performed for all nonambulatory patients with CP treated with spinal fusion with at least two-year follow-up. HRQOL was measured via the Caregiver Priorities & Child Health Index of Life with Disabilities (CPCHILD) questionnaire domains (0 = most disability, 100 = least disability). ROP was defined as having a rib distal to the superior portion of the iliac crest on preop upright radiographs. The ROP group and control group without ROP were compared regarding six domain scores and total score of CPCHILD. Multiple linear regression was used to control for curve apex location, major coronal Cobb angle, type of tone, and pelvic obliquity. RESULTS: 340 patients met inclusion criteria (52% female, mean age 14.0 years). The mean major coronal Cobb angle was 81 degrees and mean pelvic obliquity was 22 degrees. 176 patients (51.8%) had ROP while 164 patients (48.2%) did not. ROP was independently associated with worse preoperative Positioning/Transfers/Mobility (PTM), Comfort & Emotions (C&E), and total CPCHILD score via the CPCHILD questionnaire (p < 0.05). Patients with preoperative ROP experienced a greater improvement in the C&E and PTM domains as well as total CPCHILD score than patients without ROP (p < 0.05). CONCLUSION: CP patients with rib-on-pelvis deformity experience more pain and worse HRQOL than patients without this deformity. These patients experienced a greater improvement in HRQOL after spinal fusion measured via the CPCHILD questionnaire.

Pressure-specified sensory device versus electrodiagnostic testing in brachial plexus upper trunk injury.

Brachial plexus upper trunk injury is associated with winged scapula owing to the close anatomical course of the long thoracic nerve and upper trunk. Needle electromyography is a common diagnostic test for this injury; however, it does not detect injury in most patients with upper trunk damage. The pressure-specified sensory device may be an alternative to needle electromyography. Thirty patients with winged scapula and upper trunk injury were evaluated with needle electromyography (EMG) and pressure-specified sensory device (PSSD) tests. EMG testing of the biceps muscle was compared with PSSD testing of the dorsal hand skin (C6 damage), and EMG testing of the deltoid and spinati muscles was compared with PSSD testing of the deltoid skin (C5 damage). PSSD pressure values were significantly higher on the affected arm. On the basis of published and calculated threshold values the PSSD was found to be significantly more sensitive than EMG. The PSSD tests consistently identified injuries that were not detected by needle EMG tests. These findings provide strong evidence that the PSSD is more effective than needle EMG in the detection of brachial plexus upper trunk injury.

Engaging clinical nurses in quality and performance improvement activities.

Nursing performance measures are an integral part of quality initiatives in acute care; however, organizations face numerous challenges in developing infrastructures to support quality improvement processes and timely dissemination of outcomes data. At the Hospital of the University of Pennsylvania, a Magnet-designated organization, extensive work has been conducted to incorporate nursing-related outcomes in the organization's quality plan and to integrate roles for clinical nurses into the Department of Nursing and organization's core performance-based programs. Content and strategies that promote active involvement of nurses and prepare them to be competent and confident stakeholders in quality initiatives are presented. Engaging clinical nurses in the work of quality and performance improvement is essential to achieving excellence in clinical care. It is important to have structures and processes in place to bring meaningful data to the bedside; however, it is equally important to incorporate outcomes into practice. When nurses are educated about performance and quality measures, are engaged in identifying outcomes and collecting meaningful data, are active participants in disseminating quality reports, and are able to recognize the value of these activities, data become one with practice.

Complex palliative care and transition: a case review.

This case study assesses the effect that health and social care had on the chronic life-limiting condition of a young man going through transition from children's to adult services. He was taken into foster care at 16 years old and placed with his maternal uncle and aunt. Relevant literature is introduced to consider the impact that placement had on the foster family and the young person as he was transitioned over a two-year period to adult services, not only in terms of their adaptations, but also on his health and prognosis. Close liaison between social services and hospital and community teams meant that medical and social outcomes were markedly improved for this young man.