Mandibular Reconstruction in a Patient With Rosai-Dorfman Disease

J Craniofac Surg. 2016 Jan;27(1):e13-7. doi: 10.1097/SCS.0000000000002281.

Abstract

Objective: The Rosai-Dorfman disease (RDD) is a rare disorder known as sinus histiocytosis with massive lymphadenopathy which affects other organs besides the lymphatic nodes. The most frequent clinical presentation is the skin involvement, but the most serious one is when the central nervous system is compromised. There are not clinical cases in the literature reporting the mandibular involvement and its management. The aim of this study is to report the case of a patient with this disease, her treatment, and her follow-up.

Study: A 32-year-old woman with RDD, who underwent a right mandibulectomy to treat osteomyelitis, and who suffered hemolytic anemia, lymphoproliferative syndrome, and bacterial meningitis by meningococcus treated successfully, is presented. She also had osteosynthesis plate exposure in 3 occasions. The authors performed a mandibular reconstruction with a fibula free flap. Vertical osteogenic distraction was done to improve the vertical height of the bone for osteointegrated implants and later oral rehabilitation.

Methods: The senior authors performed mandibular reconstruction with a fibula free flap. Vertical osteogenic distraction was performed to improve the vertical height of the bone for osteointegrated implants and later oral rehabilitation. The patient achieved adequate function with a good mandibular height and occlusion.

Results: The patient had a follow-up of 6 months. She is highly satisfied with her result. Preoperative and postoperative photos are shown.

Discussion: In this rare case, the authors show the difficulties when treating this disease. The follow-up of the patient showed a successful reconstruction with good functional results.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Bone Plates
  • Bone Transplantation / methods
  • Dental Implantation, Endosseous / methods
  • Female
  • Fibula / surgery
  • Follow-Up Studies
  • Free Tissue Flaps / transplantation
  • Histiocytosis, Sinus / complications*
  • Humans
  • Mandibular Diseases / surgery*
  • Mandibular Reconstruction / methods*
  • Osteogenesis, Distraction / methods
  • Osteomyelitis / surgery*
  • Patient Satisfaction
  • Prosthesis-Related Infections / surgery
  • Transplant Donor Site / surgery
  • Treatment Outcome