Wójcicki Piotr, Zachara Mateusz
Department of Plastic Surgery, Wroclaw Medical University, ul. Jana Pawla II 2, Polanica-Zdrój, Poland.
Ann Plast Surg. 2011 Mar;66(3):267-72. doi: 10.1097/SAP.0b013e3181ea1eb4.
Parry-Romberg syndrome is an extremely rare disease characterized by the slow atrophy of the face involving the skin, subcutaneous tissue, fatty tissue, muscles, and even bones. The cause of the disease is unknown. The onset is slow and it begins usually during the first 2 decades of life, more often between the ages of 5 and 15. The purpose of the study was to present the principles of management and treatment outcomes in patients with Parry-Romberg syndrome.
A total of 14 patients (12 females and 2 males) aged between 14 and 36 years (mean, 21.5 years), were treated at the Hospital of Plastic Surgery in Polanica Zdrój between 1970 and 2005. Hemifacial atrophy affected the left side in 7 patients, and the right side in the remaining 7. Altogether 45 operations were performed. Less severe changes were reconstructed by means of dermal grafts, galeal flaps; patients with more severe facial deformities were treated with free tissue transfer: parascapular and latissimus dorsi flaps (6 cases). The treatment was completed with facial modeling, dermal regrafting, and scars correction.
The appearance was improved in all the patients. There was only 1 free flap loss reported during postoperative management.
Dermal grafts are used in the treatment of milder forms of Romberg disease to improve facial symmetry. In severe cases of facial atrophy, the surgical management is based on free-tissue transfer.
帕里-罗姆伯格综合征是一种极为罕见的疾病，其特征为面部缓慢萎缩，累及皮肤、皮下组织、脂肪组织、肌肉，甚至骨骼。病因不明。起病缓慢，通常始于生命的前20年，多见于5至15岁之间。本研究的目的是介绍帕里-罗姆伯格综合征患者的治疗原则及治疗效果。
1970年至2005年期间，在波兰扎德鲁伊的整形外科医院对14例患者（12例女性，2例男性）进行了治疗，年龄在14至36岁之间（平均21.5岁）。7例患者左侧出现半侧面部萎缩，其余7例右侧出现萎缩。共进行了45次手术。较轻的改变通过真皮移植、帽状腱膜瓣进行修复；面部畸形较严重的患者采用游离组织移植治疗：肩胛旁皮瓣和背阔肌皮瓣（6例）。治疗以面部塑形、真皮再次移植和瘢痕矫正完成。
所有患者的外观均得到改善。术后管理期间仅报告1例游离皮瓣丢失。
真皮移植用于治疗较轻形式的罗姆伯格病，以改善面部对称性。在严重面部萎缩病例中，手术治疗基于游离组织移植。
