Surgical treatment of patients with Parry-Romberg syndrome.

INTRODUCTION

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.

MATERIAL AND METHOD: 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.

RESULTS

The appearance was improved in all the patients. There was only 1 free flap loss reported during postoperative management.

CONCLUSIONS

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.