En coup de sabre scleroderma and Parry-Romberg syndrome in adolescents: Surgical options and patient-related outcomes

Abstract

Objective. There is little information regarding surgical options and outcomes in patients with facial localized scleroderma. We evaluated the surgical outcomes of procedures performed for linear scleroderma of the face in the pediatric age group; and assessed psychosocial effects of surgical interventions on the affected children. Methods. A retrospective chart review was performed of children with en coup de sabre scleroderma (ECDS) and Parry-Romberg syndrome (PRS) who underwent surgical intervention; this included demographic data, clinical features, and type of surgical interventions. A questionnaire of 13 questions covering 4 domains (physical, emotional, social, and symptoms) was sent to patients who consented to take part in the survey. Surgical treatments and outcomes were analyzed retrospectively. Results. Seventeen patients underwent surgical intervention (autologous fat injections, Medpor implants, bone paste cranioplasty, and free groin flap) to correct facial asymmetry. Ten patients answered the questionnaire (58.8% response rate). Unhappiness with their appearance, loss of confidence, and bullying were cited as reasons for surgery. The appearance subscale of the survey demonstrated the lowest standardized scores and greatest negative effect on the patients' quality of life compared to the 3 other subscales. All subjects would consider another surgery and would recommend surgery to other patients with ECDS and PRS. Conclusion. Surgical treatment is a potential useful intervention in children with facial disfigurement. Prospective data are needed. The Journal of Rheumatology Copyright © 2010. All rights reserved.