Patient-Reported Outcome Measurement Information System (PROMIS) Scores in Pediatric Patients With Arthrogryposis

Abstract

Background: Arthrogryposis multiplex congenita (AMC) is a clinical term that is used to describe congenital contractures that lead to childhood deformities. Treatment aims are to maximize function while minimizing pain and disability. Few studies have explored patient-reported outcomes in the pediatric arthrogrypotic population, particularly concerning mobility. The aim of this study was to report scores for the Patient-Reported Outcome Measurement Information System (PROMIS) questionnaire for pediatric patients with arthrogryposis with regards to mobility, upper extremity (UE) function, pain interference, and peer relationships. Methods: A retrospective chart review of 76 patients with AMC aged 5 to 17 who completed the PROMIS questionnaire between January 1, 2017 to March 24, 2020 was performed. Results were collected for four domains: mobility, UE function, pain interference, and peer relationships. Outcomes were stratified by type of arthrogryposis: Amyoplasia (Am), Distal Arthrogryposis (DA), and Other Diagnoses (OD). Results of subjects with isolated upper or lower extremity involvement were compared with subjects with involvement of upper and/or lower extremities. Outcomes were correlated with history of surgical intervention. Results: Children with Am and OD demonstrated moderate impairment of mobility (average: 35.2 and 35.9, respectively), while those with DA reported only mild impairment (average: 44.9). UE function was severely impaired for children with Am (average: 23.0), moderately impaired for OD (average: 33.0), and mildly impaired for DA (average: 43.4). All patient groups reported normal ranges of pain interference, as well as good peer relationships. A moderate negative correlation between number of surgical interventions and mobility scores, and a weak negative correlation between number of surgeries and pain interference scores were found. Conclusions: Children with AMC experience limited mobility and UE function, but normal levels of pain interference and good peer relationships. The average values provided in this study will serve as a baseline from which to evaluate the efficacy of both nonoperative and surgical interventions.

PROMIS Utilization in Pediatric Orthopaedics: A Scoping Review

“…A total of 51 studies8–10,17–64 of PROMIS in pediatric orthopaedics were identified in the current review. Study characteristics by year, author, orthopaedic population, topic, and cohort size can be found in Appendix, Supplemental Digital Content 1, http://links.lww.com/BPO/A524.…”

“…For the purposes of this review, patients were grouped by body part or subspecialty. Most studies were included within the “Hand and Upper Extremity”10,23–34 (25.5%, n=13) and “Sports”47–58 (23.5%, n=12) categories followed by “Spine”8,41–46 (13.7%, n=7), “Trauma”9,59–64 (13.7%, n=7), “General Pediatric Orthopaedics”17–22 (11.8%, n=6), “Lower Extremity”35–39 (9.8%, n=5), and “Orthopaedic Oncology”40 (2%, n=1).…”

“…The methodology of the studies included 3 categories: (1) Outcome studies8–10,17,18,23–30,34–38,40–42,47,48,55,57,59–64 in which baseline PROMIS scores were assessed in a specific disease state or to evaluate the outcome of a surgical procedure or treatment (n=31, 60.8%), (2) PROM comparison studies8,19–23,29,31,33,39,41,43–46,49,54,56,58 in which PROMIS was compared with a legacy PROM within a given field or between different PROMIS domains or versions (n=19, 37.3%), and (3) observational studies20,32,50–53 in which PROMIS was utilized to assess for differences associated with patient characteristics (n=6, 12%). Five8,20,23,29,41 of the included studies were considered to fit in 2 categories.…”

“…There were 1961 unique citations included after the removal of 1756 duplicates. After initial screening, 183 studies were screened under full-text review leaving a final number of 51 studies8–10,17–64 included in this scoping review (Fig. 1).…”

“…The methodology of the studies included 3 categories: (1) Outcome studies [8][9][10]17,18,[23][24][25][26][27][28][29][30][34][35][36][37][38][40][41][42]47,48,55,57,[59][60][61][62][63][64] in which baseline PROMIS scores were assessed in a specific disease state or to evaluate the outcome of a surgical procedure or treatment (n = 31, 60.8%), (2) PROM comparison studies 8,[19][20][21][22][23]29,31,33,39,41,[43][44][45][46]49,54,56,58 in which PROMIS was compared with a legacy PROM within a ...…”