Treatment of Progressive Hemifacial Atropht (Romberg's Desease) with Microvascuar Free Flap

ABSTRACT

Romberg's disease is an uncommon condition manifested by progressive hemifacial atrophy of the skin, soft tissue and bone. Facial asymmetry with soft tissue deficiency in Romberg's disease causes a significant disability affecting the social life and can bring about many psychological problems. The aim of surgical treatment is cosmetic amelioration of the defect. Several conventional reconstructive procedures have been used for correcting facial asymmetry. They include fat injections, dermal fat grafts, silicone injections, cartilage and bone grafts, pedicled flaps and free flaps. We report our experience with 6 patients involving 6 free flaps with a minimun of 1 year follow-up who were treated from October 1989 to March 1998. All patients were classified as having moderate to severe atrophy. The average age of disease onset was 4.5 years. The average duration of atrophy was 5.2 years. No patient was operated on with a quiescent interval of less than 1 year. The average age at operation was 14.1 years, ranging from 10 to 24 years. Follow-up ranged from 1 to 9 years. Reconstruction was performed using 2 groin dermofat free flaps and 4 latissimus dorsi muscular free flaps. To achieve the finest symmetrical and aesthetic results, several ancillary procedures were performed in 4 patients. These procedures included Le-Fore 1 1/2 leveling osteotomy, sagittal split ramus osteotomy, reduction malarplasty and angleplasty, rib and calvarial bone graft, correction of alopecia and additional dermofat graft. All patients were satisfied with the results. We believe that free flap is one of the best choices for contoured restoration of facial asymmetry in Romberg's disease.