Correction of Facial Deformity in Sturge-Weber Syndrome

Although previous studies have reported soft-tissue management in surgical treatment of Sturge-Weber syndrome (SWS), there are few reports describing facial bone surgery in this patient group. The purpose of this study is to examine the validity of our multidisciplinary algorithm for correcting facial deformities associated with SWS. To the best of our knowledge, this is the first study on orthognathic surgery for SWS patients.

A retrospective chart review included 2 SWS patients who completed the surgical treatment algorithm. Radiographic and clinical data were recorded, and a treatment algorithm was derived.

According to the Roach classification, the first patient was classified as type I presenting with both facial and leptomeningeal vascular anomalies without glaucoma and the second patient as type II presenting only with a hemifacial capillary malformation. Considering positive findings in seizure history and intracranial vascular anomalies in the first case, the anesthetic management was modified to omit hypotensive anesthesia because of the potential risk of intracranial pressure elevation. Primarily, both patients underwent 2-jaw orthognathic surgery and facial bone contouring including genioplasty, zygomatic reduction, buccal fat pad removal, and masseter reduction without major complications. In the second step, the volume and distribution of facial soft tissues were altered by surgical resection and reposition. Both patients were satisfied with the surgical result.

Our multidisciplinary algorithm can systematically detect potential risk factors. Correction of the asymmetric face by successive bone and soft-tissue surgery enables the patients to reduce their psychosocial burden and increase their quality of life.