Romberg's disease, also known as progressive hemifacial atrophy or Parry-Romberg syndrome is an acquired disease causing atrophy of soft tissues and even the bone and cartilage in the face. This leads to severe deformity of the facial contour, but still its etiology is unclear. There have been various attempts to surgically manage Romberg's disease. Artificial implants like silicone or hydroxyapatite were used to correct the defect on the face. Autologous fat injection or dermofat graft, bone graft are also commonly used surgical methods. Recently, free flap transfer is being widely applied to correct the asymmetry of the face. But due to the bulkiness of the flap, gravitational effects leading to ptosis and remaining focal defects, it is hard to have an aesthetically satisfied result by just a single operation.

Three patients with Romberg's disease have received a secondary operation followed by perforator flap transfer to reconstruct the facial contour. In two cases, deep inferior epigastric artery perforator free flap was transferred previously and in another case, thoracodorsal artery perforator free flap was used. Secondary operations were done to further correct the facial disfigurations.

All three patients were females and the average age was 31.7 years. Each patient had one to two additional operations after the free flap transfer (average 1.67 times). Fat injection was the most frequently performed operation followed by liposuction. The average amount of injected fat was 30.4cc and the suctioned amount of fat was 18.0cc. Intervals between each operation were an average 185.4 days. No post-operative complications were reported and all patients were satisfied with the results.

The important matter in reconstructing the facial contour in Romberg's disease is to restore the soft tissue defects and to maintain the results permanently. Free flap transfer is commonly used nowadays because it can reconstruct a considerable amount of soft tissue in a single operation, and its effects last continuously. The introduction of perforator flaps allowed the flaps to be more customized, still there are limitations in restoring the facial contour in just a single stage. Liposuction is a reliable method to correct the bulky or ptotic flap and fat injection can be used to correct focal defects. The authors were able to achieve satisfactory results without any complications by performing secondary operations with an interval about six months.

Table I. Patient Summary

 

	Sex	Age at 1st op.	Type of flap*	Additional operations	Op. intervals (days)
1	F	16	DIEP	1. Liposuction & fat injection	164
				2. Liposuction & fat injection	162
2	F	55	TDAP	1. Fat injection	106
				2. Fat injection	176
3	F	24	DIEP	1. Liposuction	319

*: DIEP=deep inferior epigastric artery perforator free flap, TDAP=thoracodorsal artery perforator free flap

Fig. 1. 16-year old female patient.

Fig. 2. 55-year old female patient.

Fig. 3. 24-year old female patient.