4例2型双侧听神经纤维瘤病的临床诊疗

Clinical diagnosis and treatment of bitaleral acoustic neurofibromatosis type 2:a retrospective analysis of 4 patients

目的探讨2型神经纤维瘤病(NF2)的诊断和治疗方法。方法回顾性分析和总结经手术以及病理证实的4例NF2相关双侧听神经瘤患者的临床表现、MRI信号特点及治疗经过,并复习相关文献。结果FN2患者MRI主要表现为双侧听神经瘤,但也可表现为单侧听神经瘤合并脑膜瘤、胶质瘤等。治疗以显微手术为主。结论NF2的临床表现及MRI有一定的特征性,MRI是诊断本病的主要检查手段。目前治疗以外科手术为主,但手术时机需个体化,尽量保护一侧的听力。

Objective To investigate the diagnosis and treatment of neurofibromatosis type2(NF2).Methods The clinical manifestations,magnetic resonance imaging(MRI)findings,and treatment of 4 patients with surgically and pathologically-confirmed NF2-associated bilateral acoustic neufibroma were retrospectively analyzed,along with literature review.Results Bilateral acoustic neuroma was the mainly clinical manifestation in these four NF2 patients,although unilateral acoustic neuroma and schwannoma have also been described in literature.Microsurgery remains the mainstream treatment for NF2.Conclusions NF2 has unique clinical manifestations and MRI findings.It is mainly diagnosed by MRI and treated by surgery.However,the timing of surgery should be individualized and pay attention to the protection of the hearing at one side.