Section of Dermatology, Division of Paediatric Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.
J Rheumatol. 2010 Oct;37(10):2174-9. doi: 10.3899/jrheum.100062. Epub 2010 Sep 15.
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.
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.
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.
Surgical treatment is a potential useful intervention in children with facial disfigurement. Prospective data are needed.
有关面部局限性硬皮病患者的手术选择和结果的信息很少。我们评估了小儿面线性硬皮病患者的手术结果；并评估了手术干预对面部受影响儿童的心理社会影响。
对接受手术干预的面部 En Coup De Sabre 硬皮病（ECDS）和 Parry-Romberg 综合征（PRS）患儿进行回顾性图表审查；包括人口统计学数据、临床特征和手术干预类型。向同意参加调查的患者发送了一份涵盖 4 个领域（身体、情感、社会和症状）的 13 个问题的问卷。回顾性分析手术治疗和结果。
17 名患者接受了手术干预（自体脂肪注射、Medpor 植入物、骨泥颅骨成形术和游离腹股沟皮瓣）以矫正面部不对称。10 名患者回答了问卷（58.8%的应答率）。对自己的外表不满意、失去信心和被欺负是手术的原因。与其他 3 个分量表相比，调查的外观分量表显示出最低的标准化分数和对患者生活质量的最大负面影响。所有受试者都愿意接受另一次手术，并会向其他患有 ECDS 和 PRS 的患者推荐手术。
手术治疗是面部畸形儿童的一种潜在有用的干预措施。需要前瞻性数据。
