| 引用本文: | 张钊莲,马健,刘达恩,李顺堂.手术治疗36例1型神经纤维瘤病临床分析[J].中国临床新医学,0,():-. |
| Zhangzhaolian,Majian,Liudaen.手术治疗36例1型神经纤维瘤病临床分析[J].中国临床新医学,0,():-. |
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| 摘要: |
| 目的 回顾性分析1型神经纤维瘤病(Neurofibromatosis type 1,NF1)的临床特征，并探讨其手术切除策略和修复重建效果。方法 回顾性分析2012年1月至2026年12月广西医科大学第一附属医院烧伤整形外科收治的36例NF1患者临床资料。统计患者性别、年龄、NF1的类型、病变部位、病灶数量，统计每次术中出血量、切除肿瘤的重量，对瘤体复发、缺损修复方法及手术效果进行观察和随访。结果 本组共36例NF1患者，男性 26例，女性10例， 平均年龄26.1 ± 15.7岁。多发性皮肤型神经纤维瘤28例（巨大NF1 9例），丛状神经纤维瘤肿瘤8例（2例侵犯肋骨）。病变部位发生在躯干18例，四肢10例，面部7例，肛周会阴1例，瘤体体积4cm×5cm×7cm～49cm×50cm×25cm，9例巨大NF1中6例累及躯干背部，1例累及肛周会阴部，2例累及四肢。其中1例有明显的家族性病史。18例切除后直接缝合闭合；10例切除后局部皮瓣转移修复；9例巨大的NF1中2例术前血管外科会诊后行术前栓塞主干供血动脉，8例患者采取分次切除的手术策略，1例躯干背部巨大NF1选择一次性手术完全切除瘤体并反取瘤体皮移植联合负压封闭引流技术，负压值维持在-150～-250mmHg(1 mmHg=0.133kPa)，平均每次手术出血量约为355.15 ± 150.6ml。随访6个月至3年，患者对外观均较为满意，无运动及感觉功能障碍。结论 根据瘤体部位、累及的范围，选择一次或分次切除的手术策略，采用整形外科理念手术设计手术切口，达到切除肿瘤和整形修复的目的，并减少手术出血等相关风险，特殊部位的巨大神经纤维瘤患者联合多学科进行诊治，可以最大程度改善外形及正常功能，提高生活质量，获得较好的效果。 |
| 关键词: 1 型神经纤维瘤病 神经纤维瘤 修复重建 外科手术 整形 |
| DOI： |
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| 基金项目:神经突蛋白在糖尿病创面愈合中的作用及机制研究 |
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| Clinical Analysis of Surgical Treatment for 36 Cases of Neurofibromatosis Type 1 |
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Zhangzhaolian,Majian,Liudaen
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The First Affiliated Hospital of Guangxi Medical University
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| Abstract: |
| Objective:? To retrospectively analyze the clinical characteristics of neurofibromatosis type 1 (NF1) and to explore the surgical resection strategies and the outcomes of repair and reconstruction. Methods:? Clinical data of 36 NF1 patients admitted to the Department of Burn and Plastic Surgery, the First Affiliated Hospital of Guangxi Medical University from January 2012 to December 2026 were retrospectively analyzed. Parameters including gender, age, NF1 type, lesion site, number of lesions, intraoperative blood loss, weight of resected tumors, tumor recurrence, methods of defect repair, and surgical outcomes were recorded and followed up. Results:? The group comprised 36 NF1 patients, including 26 males and 10 females, with an average age of 26.1 ± 15.7 years. Among them, 28 cases were multiple cutaneous neurofibromas (including 9 cases of giant NF1), and 8 cases were plexiform neurofibromas (2 of which involved the ribs). Lesion sites included the trunk (18 cases), limbs (10 cases), face (7 cases), and perianal/perineal region (1 case). Tumor volume ranged from 4 cm × 5 cm × 7 cm to 49 cm × 50 cm × 25 cm. Among the 9 cases of giant NF1, 6 involved the back of the trunk, 1 involved the perianal/perineal region, and 2 involved the limbs. One patient had a significant family history. Wound closure was achieved by direct suturing in 18 cases and by local flap transfer in 10 cases. Among the 9 cases of giant NF1, 2 underwent preoperative embolization of the main feeding arteries after consultation with vascular surgery, and 8 were treated with staged resection. One case of giant NF1 on the trunk underwent one-stage complete resection with reverse dermal grafting combined with vacuum sealing drainage (negative pressure maintained at -150 to -250 mmHg; 1 mmHg = 0.133 kPa). The average intraoperative blood loss per surgery was approximately 355.15 ± 150.6 ml. During a follow-up period of 6 months to 3 years, patients were satisfied with their appearance, and no motor or sensory dysfunction was observed. Conclusion:? Based on the tumor location and extent of involvement, surgical strategies of one-stage or staged resection, combined with plastic surgery principles for incision design, can achieve tumor removal and aesthetic repair while reducing risks such as surgical bleeding. For giant neurofibromas in special locations, multidisciplinary diagnosis and treatment can maximize improvements in appearance and function, enhance quality of life, and yield favorable outcomes. |
| Key words: Neurofibromatosis type 1 Neurofibroma Repair and reconstruction Surgery Plastic surgery |
