How is progressive unilateral facial atrophy treated?

  Progressive unilateral facial atrophy, also known as Romberg’s disease, is a progressive atrophy of the skin and soft tissues of one side of the face or also of the bone and cartilage tissues, and is a tissue dystrophy. It is common in women.  The etiology is unknown and may be related to infection, trauma, sympathetic nerve dysfunction, and trigeminal peripheral neuritis. A genetic component has not been confirmed.  Onset The disease usually starts in adolescence and progresses very slowly. The disease usually starts on the cheek and lips or forehead, and gradually expands in all directions, eventually extending to all the skin, subcutaneous tissues, nose, lips and red lips of one side of the face, or along the area where the trigeminal nerve branches travel. The skin atrophy is weak, often accompanied by flaking. There is hyperpigmentation in the significant areas of atrophy. In severe cases, facial bones and nasal cartilage are also atrophied. The atrophy stops at the midline and is clearly demarcated from the healthy side, which is a diagnostic feature, and is occasionally associated with other neurological disorders such as Horner’s syndrome, contralateral cortical epilepsy, and trigeminal neuralgia. In most cases, after 2-12 years, it gradually stabilizes and does not progress.  Treatment options For unilateral facial atrophy, the following surgical approaches are available (1) fat, fascia-fat, and dermal-fat tissue free graft filling, which can be performed in one or subdivisions depending on the severity of the condition. When the filled tissues need to overlap to increase the thickness, the number of layers should not be too many, otherwise necrosis and absorption will occur in the central part due to blood circulation disorders. The interval between each operation should be 6 months.  (2) The skin tube implantation with the epidermis removed, the pre-prepared skin tube is transferred so that the tip is combined with the normal tissue of the temporal region, and later the skin tube is dissected and spread along the original suture line, and the epidermis is removed and implanted under the skin of the cheek. This method can avoid the absorption of implanted tissues and is expected to improve the nutritional effect of facial atrophy.  (3) Bone or cartilage implantation is used in cases with severe bone tissue atrophy.  (4) Implantation or injection of tissue substitutes, such as the implantation of silicone rubber blocks, but because of skin atrophy, the implant blocks are easily dislodged.  (5) Free tissue transplantation with anastomosis of blood vessels, such as free skin flaps or myocutaneous flaps with the epidermis removed, and implantation of the greater omentum. The surgical technique is more complicated, but can be completed in one visit, with stable efficacy and a strong effect of improving the nutritional status of atrophic tissues.