Optimizing care for patients with maxillomandibular Gorham-Stout disease

OBJECTIVE: Gorham Stout disease (GSD) is an osteolysis caused by abnormal vessels, the etiology of which is not fully understood. Maxillary and/or mandibular destruction is found in nearly 20% of cases. We aimed to propose a management algorithm to ensure a successful surgical reconstruction. STUDY DESIGN: We conducted a systematic literature review of all maxillo-mandibular cases of GSD, with data on treatments and follow-up. We have added our two cases of patients with GSD to the published case series. We then performed a logistic regression analysis, using response to treatment as the dependent variable, i.e., resolution of clinical symptoms and radiological osteolysis. RESULTS: A total of 100 patients (including the two cases we managed) with GSD and primary maxillary and/or mandibular involvement were included. Only radiotherapy was significantly associated with disease remission (OR = 7.363 [1.140-47.54], P = .044). The reconstructive surgery performed on the patient without osteolysis stabilization was not stabilized, showed a recurrence of the disease, and resorption of the fibula flap. CONCLUSIONS: This study highlights the importance of strict control of the disease before any major reconstructive surgery for the patient. Radiotherapy appeared to be the only significant factor in our model for disease control.