Correction of Facial Deformity in Sturge–Weber Syndrome

Abstract

Background: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.Methods: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.Results: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.Conclusions: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.

Comparison of Orthognathic Surgery Outcomes Between Patients With and Without Underlying High-Risk Conditions: A Multidisciplinary Team-Based Approach and Practical Guidelines

“…To expand on these previous reports, the present study compared the OGS-related outcomes of high- and regular-risk groups that were managed by an OGS-specific multidisciplinary approach in the past 14 years. Most of the underlying conditions considered in this study have not been previously reported in the context of OGS procedures, and only a few conditions have been described in some case reports [33,34,35]. As some of these underlying conditions are common worldwide, it is probable that the corresponding patients may not have received OGS due to the potential risk of complications or the treatment providers may not have specified details of high-risk conditions in their publications.…”

A Rare Case of Incidentally Detected Sturge-Weber Syndrome: a Case Report and Review of the Current Surgical Procedures

“…These techniques were adequate, and the results are satisfactory [12]. We further reviewed surgical intervention procedures and summarized the reported studies and cases in Table 1 [13][14][15][16][17][18]. Most of the discussed surgical procedures revealed a significant outcome.…”

Surgical Management of Facial Port-Wine Stain in Sturge Weber Syndrome

“…Manifestations are variable but are usually characterized by involvement of the unilateral skin, brain, and eye resulting in a classic triad including ipsilateral facial capillary malformation known as port-wine stain, intracranial vascular malformation known as leptomeningeal angiomatosis, and ocular vascular malformations causing glaucoma [1,[6][7][8]. The leptomeningeal involvement can result in severe seizure disorder and intellectual disability, while the facial port-wine stain extends across the trigeminal nerve distribution, affecting both bones and soft tissue and potentially causing severe facial disfigurement [9]. The management of the resulting facial disharmony is undertaken by reconstructive plastic surgeons and includes laser treatments and challenging surgical reconstruction of the soft tissue and bony malformation to restore symmetry [9][10][11][12].…”

“…The leptomeningeal involvement can result in severe seizure disorder and intellectual disability, while the facial port-wine stain extends across the trigeminal nerve distribution, affecting both bones and soft tissue and potentially causing severe facial disfigurement [9]. The management of the resulting facial disharmony is undertaken by reconstructive plastic surgeons and includes laser treatments and challenging surgical reconstruction of the soft tissue and bony malformation to restore symmetry [9][10][11][12].…”