Surgical Orthodontic Treatment for Skeletal Maxillary Protrusion in Sturge-Weber Syndrome: A Case Report and Review of the Literature

Abstract

Sturge-Weber syndrome (SWS) is characterized by hemangiomas, glaucoma, and central nervous system disorders. Here, we report the case of a 15-year-old boy with SWS and upper-lip hypertrophy who underwent surgical orthodontic treatment for correction of a large overjet and deep overbite. In addition to the a large overjet and deep overbite, interdental spacing was observed in both the arches. The mandible was retrognathic and deviated to the right side. No maxillary occlusal canting or temporomandibular joint symptoms were observed. The patient was diagnosed with skeletal maxillary protrusion with spaced dentition and mandibular deviation to the right due to SWS. After presurgical orthodontic treatment using a multibracket appliance, we performed a sagittal split ramus osteotomy (SSRO) alone due to the presence of a hemangioma around the maxilla. No abnormal bleeding or cerebral hemorrhage due to increased blood pressure was observed during the SSRO. Postoperatively, the maxillary and mandibular arches were well-aligned, the deep overbite and excessive overjet improved, and bilateral angle class I molar and canine relationships were established. Furthermore, mandibular deviation improved, and the midlines of both arches approximately coincided with the facial midline. In conclusion, orthognathic surgery is feasible in patients with SWS after carefully evaluating the sites and sizes of the hemangiomas.

Keywords: hemangioma; orthodontic treatment; sagittal split ramus osteotomy; skeletal maxillary protrusion; sturge-weber syndrome.

Figure 1. Pretreatment evaluation

(A) Facial photographs. (B) Intraoral photographs.

Figure 2. Pretreatment evaluation, Plaster models.

Figure 3. Pretreatment evaluation.

(A) Lateral cephalometric radiograph. (B) Original tracing of lateral view. (C) Frontal Cephalometric radiograph.

Figure 4. Pretreatment evaluation, Panoramic radiograph.

Figure 5. Pretreatment evaluation.

Schuller’s radiographs. LO, left open; LC, left close; RO, right open; RC, right close.

Figure 6. Presurgical evaluation.

(A) Facial photographs. (B) Intraoral photographs.

Figure 7. Presurgical evaluation.

Plaster models.

Figure 8. Presurgical evaluation.

Panoramic radiograph.

Figure 9. Presurgical evaluation.

(A) Lateral cephalometric radiograph. (B) Original tracing of lateral view. (C) Frontal cephalometric radiograph.

Figure 10. Presurgical evaluation.

Schuller’s radiographs. LO, left open; LC, left close; RO, right open; RC, right close.

Figure 11. Presurgical evaluation.

(A) Fat-suppressed T2 weighted magnetic resonance image. The arrowhead shows the atrophy of the left cerebral hemisphere which is typical symptom of SWS. (B) Computed tomography image. The arrowhead shows that the inferior turbinate was thickened and the nasal septum had deviated. This makes nasal intubation difficult.

Figure 12. Post-treatment evaluation.

(A) Facial photograph. (B) Intraoral photograph.

Figure 13. Post-treatment evaluation.

Plaster cast model.

Figure 14. Post-treatment evaluation.

Panoramic Radiograph.

Figure 15. Post-treatment evaluation.

(A) Lateral cephalometric radiograph. (B) Original tracing of lateral view. (C) Frontal cephalometric radiograph.

Figure 16. Post-treatment evaluation.

Schuller’s Radiograph. LO, left open; LC, left close; RO, right open; RC, right close.

Figure 17. Post-treatment evaluation.

Cephalometric superimposition: solid line, pretreatment; dotted line, post-treatment.

Figure 18. Treatment algorithm for SWS patients.

OGS, Orthognathic Surgery; SSRO, Sagittal Split Ramus Osteotomy.