Ⅰ型神经纤维瘤病的多学科计划性手术治疗

目的探讨Ⅰ型神经纤维瘤病（NF1）的手术治疗原则。方法 1998年5月至2006年8月,共收治NF1患者15例,分为局限型和巨大型两类,均行多学科计划性手术。结果每位患者手术1～5次,平均2.6次。15例病变4例近全切除、5例次全切除、6例部分切除,除暂时性面瘫2例,涎瘘、视力下降各1例外,其余未发生明显术后并发症。15例患者术后随访1.5～6.5年,4例近全切除者未发现残留病变明显再生长,其余11例发现残留病变有不同程度再生长,但目前均尚未引起新的功能和美观问题。结论 NF1是一种神经源性疾病,病变通常较难完全切除,而次全或部分切除又常导致残留病变再生长。我们建议进行多学科计划性手术治疗,对局限型病变力求近全切除,巨大型病变若不能近全切除,则以改善功能障碍和畸形为主。长期随访和术后定期影像学检查监控残留病变是必须的。

Surgical Treatment of Neurofibromatosis Type 1 by Multidisciplinary Schedule

Objective To explore the principles of surgical treatment in neurofibromatosis type 1（NF1）.Methods From May 1998 to August 2006,15 patients with NF1 were divided into small type and massive type.All patients were treated by multidisciplinary schedule.Results Each patient underwent operation for 1 to 5 times,and the average operation time was 2.6.Among 15 patients,4 patients underwent near-total resection,5 patients underwent subtotal resection,and 6 patients underwent partial resection.Except temporal facial nerve paralysis was found in 2 patients,salivary fistula and decreased vision was respectively found in 1 patient,other patients healed without obvious complications.During the post-operative follow-up periods of 1.5 to 6.5 years,no recurrences were developed in 4 patients underwent near-total resection.Recurrences in different degrees were found in other patients,but new cosmetic and functional disturbances have not been developed at present.Conclusion NF1 is a neurogenic disease.Complete resection for NF1 usually is impossible,but recurrence after subtotal or partial resection is common.We suggest NF1 should be treated by multidisciplinary schedule.Small type should be performed near-total resection.Massive type should be tried to improve functional and cosmetic disturbances when near-total resection could not be performed.Long-term follow-up and postoperative regular imaging are necessary to monitor the residual lesion.