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

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.