Department of Burn and Plastic surgery, Cho Ray Hospital, Ho Chi Minh City, Viet Nam.
Department of Vascular Surgery, Cho Ray Hospital, Ho Chi Minh City , Viet Nam.
J Med Case Rep. 2024 Nov 21;18(1):564. doi: 10.1186/s13256-024-04916-2.
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.
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.
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.
神经纤维瘤病被认为是一种罕见的遗传性疾病，主要影响神经组织。最常见的类型是神经纤维瘤病 1 型，其特征是丛状神经纤维瘤。
我们报告了一例 30 岁的拉格莱族女性，患有面部丛状神经纤维瘤，不仅累及左侧眼眶，还导致颞骨破坏和面部畸形。该疾病始于她的童年时期，入院时她的躯干和四肢有许多神经纤维瘤。根据临床症状、计算机断层扫描图像和组织病理学结果，该患者被诊断为神经纤维瘤病 1 型。为了安全起见，我们将治疗分为两个阶段。第一阶段处理了肿瘤的供血和脑膜缝合，然后进行了手术切除和皮瓣重建手术。
丛状神经纤维瘤的主要治疗方法是手术，旨在切除肿瘤和处理并发症。然而，基于肿瘤的性质和范围，每个病例的治疗方案都不同，需要更多的验证和临床实践。
