Congenital Infiltrating Lipomatosis of the Face: Case Report With Presentation of a New Multistep Surgical Approach.

ABSTRACT

PURPOSE: This report describes the first case of congenital infiltrating lipomatosis of the face (CIL-F) that was successfully managed with 2-jaw orthognathic surgery. MATERIALS AND METHODS: The patient was followed from 4 to 18 years of age. The multistep approach used consisted of a facelift-type procedure at 12 years to improve the soft tissue profile. At 13 years, the transverse maxillary deficiency was treated with transpalatal distraction. At 14 years, the patient underwent bimaxillary orthognathic surgery with a genioplasty. RESULTS: The patient's satisfaction level was very high and remained constant during the 4-year follow up. Extraoral clinical examination showed nearly complete harmonization at the soft and hard tissue levels. There was no recurrence of hypertrophy of the bones or facial soft tissues during the 4-year follow-up. CONCLUSION: The results of this study indicate that a surgical approach for CIL-F at the soft and bony levels is possible if indicated. Orthognathic surgery can be performed and good esthetic and functional results can be achieved. To minimize the risk of recurrence, it is important to perform the treatment after the onset of puberty. This harmonization at the osseous level can greatly benefit the psychological well-being of the patient.