From the Division of Plastic and Reconstructive Surgery, University of Wisconsin School of Medicine and Public Health; and Summit Medical Group.
Plast Reconstr Surg. 2018 Nov;142(5):1275-1283. doi: 10.1097/PRS.0000000000004922.
Parry Romberg disease (hemifacial atrophy) and linear scleroderma (coup de sabre) are progressive, usually unilateral facial atrophies of unknown cause. The gold standard treatment for these patients has been microsurgical reconstruction following the "burning out" of the facial atrophy and stable contour for 2 years.
The authors report their experience treating patients with hemifacial atrophy and linear scleroderma with free tissue transfers between 1989 and 2016. A modified parascapular flap based on the circumflex scapular artery was most commonly used.
A total of 177 patients were included. The most common complication was hematoma, occurring in 12 patients (7 percent). Follow-up ranged from 1 to 26 years. All patients subjectively experienced improved facial symmetry and aesthetics. No disease process has recurred to date, even in cases of severe, progressive disease.
In the authors' experience, patients treated early in their disease course have immediate and sustained correction of their deformity, with slowing or in most cases cessation of the disease process following free tissue transfer. The authors now advocate for immediate reconstruction for active disease, especially in young children.
CLINICAL QUESTIONS/LEVEL OF EVIDENCE: Therapeutic, IV.
Parry Romberg 病（半侧颜面萎缩）和线状硬皮病（横断性偏侧面萎缩症）是进行性的，通常为原因不明的单侧面部萎缩。这些患者的金标准治疗方法是在面部萎缩和稳定轮廓“燃烧殆尽”并稳定 2 年后进行显微外科重建。
作者报告了他们在 1989 年至 2016 年间使用游离组织转移治疗半侧颜面萎缩和线状硬皮病患者的经验。最常使用基于旋肩胛动脉的改良肩胛旁皮瓣。
共纳入 177 例患者。最常见的并发症是血肿，发生在 12 例患者（7%）。随访时间为 1 至 26 年。所有患者均主观上体验到面部对称性和美观度的改善。迄今为止，即使是严重的进行性疾病，也没有疾病过程复发。
在作者的经验中，在疾病早期接受治疗的患者可立即获得并维持其畸形的矫正，并且在游离组织转移后，疾病进程会减缓或在大多数情况下停止。作者现在提倡对活动性疾病进行即刻重建，特别是在年幼的儿童中。
临床问题/证据等级：治疗，IV 级。
