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丛状神经纤维瘤属于多发性神经纤维瘤病的一种类型,起源于雪旺氏细胞,局部广泛侵犯,其在头颈部占相当大比例,可累及多数神经,使深部骨质吸收,使邻近软组织及表面皮肤增生、变厚、质韧、色素沉着、造成狮面等颌面部畸形,还可累及鼻道、眼睑,影响视力、咀嚼和唇的活动,但不发生转移。作者们报导1956～1978年的10例;诊断时平均年龄10岁6个月,男女比为3:2,7例有神经纤维瘤家族史,随访期平均8年。6例有眼部症状:睑下垂、睑巨大肿块,3例视力下降,3例眼外肌运动障碍。除明显颈面部畸形外,2例咽侧受累,1例外耳道等受累引起传导性聋。平均都行过3次手术以改善
Plexus neurofibromatosis is a type of multiple neurofibromatosis that originates from Schwann cells and is extensively invaded. It accounts for a significant proportion of the head and neck and can affect most nerves, allowing deep bone mass to be absorbed. Adjacent to soft tissue and surface skin hyperplasia, thickening, tough, pigmentation, resulting in maxillofacial deformity such as lion face, but also involving the nasal passages, eyelids, affecting vision, chewing and lip activities, but no transfer. The authors reported 10 cases from 1956 to 1978; the average age at diagnosis was 10 years and 6 months, the ratio between males and females was 3:2, and 7 cases had a family history of neurofibromatosis. The average follow-up period was 8 years. Six patients had ocular symptoms: drooping ptosis, massive lumps, 3 cases of decreased vision, and 3 cases of extraocular muscle movement. In addition to significant cervical and facial deformities, 2 cases of pharyngeal involvement and 1 of the ear canal involvement caused conductive spasms. On average, three surgeries were performed to improve