Microvascular free-flap correction of severe hemifacial atrophy

Romberg's disease is a progressive hemifacial atrophy of unknown etiology. Microsurgical reconstruction, focusing on the correction of facial asymmetry and restoration of contour, has become the "gold standard." We report our experience with 15 patients involving 16 free-tissue transfers with a minimum of 1 year of follow-up who were treated from July of 1989 to January of 1993. All patients were classified as having severe atrophy. There were 7 males and 8 females in the series. Distribution of disease was a coup de sabre type or segmental pattern in 6 patients, whereas 9 patients had a hemifacial distribution. Fourteen patients had unilateral disease (7 right and 7 left), and 1 patient had bilateral atrophy. The average age of onset of disease was 11.9 years. The average duration of atrophy was 6.7 years. No patient was operated on with a quiescent interval of less than 2 years. Average age at operation was 28.7 years, with a range from 6 to 46 years. Follow-up ranged from 1 to 4.5 years. Two patients had facial hematomas as the only complication. No flaps were lost. Flap revisions consisting of minor contour corrections were performed in 10 patients. Limited recurrence of facial atrophy was seen in a single patient 2 years postoperatively. All patients rated their improvement as excellent. The deepithelialized extended parascapular flap with large fascial extensions of dorsal thoracic fascia is our procedure of choice. This fascia can be folded into variable thicknesses to correct subtle contour defects of the upper lip, medial canthus, eyelids, and ear that have reportedly been difficult to reconstruct.(ABSTRACT TRUNCATED AT 250 WORDS)