Interdisciplinary care of facial plexiform neurofibroma: a case report

Abstract

Abstract Background Neurofibromatosis is considered a rare genetic disorder primarily affecting neural tissues. The most common type is neurofibromatosis type 1, which is characterized by plexiform neurofibromas. Case presentation We report a case of a 30-year-old Rag-lay ethnic woman with facial plexiform neurofibroma, which not only infiltrated the left eye socket but also caused destruction of temporal bone and facial deformity. The disease started in her childhood, and she had numerous neurofibromas on her trunk and limb at the time of the hospital admission. The patient was diagnosed with neurofibromatosis 1 based on clinical symptoms, computed tomography images, and histopathologic result. For safety, we divided treatment into two phases. For the first phase, we dealt with the blood supply and meningeal suture, and then we performed surgical resection and skin flap reconstruction surgery. Conclusion The main therapy for plexiform neurofibroma is surgery, which aims at tumor removal and management of complications. However, the treatment plan is different in each case based on the nature and extent of tumor, which requires more verification and clinical practice.

Keywords

• Neurofibromatosis type 1
• Facial neurofibroma
• Endovascular intervention
• Skin flap reconstruction surgery