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1 型神经纤维瘤病皮肤神经纤维瘤的坚固手术方法。

Robust surgical approach for cutaneous neurofibroma in neurofibromatosis type 1.

机构信息

Department of Dermatology.

Comprehensive Neurofibromatosis Clinic, and.

出版信息

JCI Insight. 2019 Apr 30;5(11):128881. doi: 10.1172/jci.insight.128881.

Abstract

BACKGROUND

Cutaneous neurofibromas (cNF) are physically disfiguring, painful, and cause extensive psychologic harm in patients with neurofibromatosis type 1 (NF1). There is currently no effective medical treatment and surgical procedures are inaccessible to most NF1 patients globally.

OBJECTIVE

While research is underway to find an effective medical treatment for cNF, there is an urgent need to develop surgical approach that is accessible to all NF1 patients in the world with the skill set and equipment found in most general medical office settings. Here, we present a robust surgical approach to remove cNF that does not require sterile surgical field, utilizes accessible clinical equipment, and can be performed by any health care providers including family practitioners, and physician assistants.

METHODS

In a prospective case-series, patients with NF1 underwent this surgical procedure which removes multiple cutaneous neurofibromas. The Dermatology Life Quality Index was given to subjects before and after the procedure as surrogate for patient satisfaction.

RESULTS

83 tumors were removed throughout the body from twelve individuals. Examination at follow-up visits revealed well-healed scars without infection or adverse events including aberrant scarring. Patient satisfaction with the procedure was high with significant improvements in symptoms, daily activities, leisure, personal relationships, and treatment experience (P = 0.00062).

CONCLUSION

This study demonstrates a robust surgical approach to management cutaneous neurofibromas which can be accessed world-wide to individuals with NF1 and performed by a wide-variety of medical specialists with high clinical efficacy and patient satisfaction.

摘要

背景

皮肤神经纤维瘤(cNF)在外观上具有破坏性,会引起疼痛,并给 1 型神经纤维瘤病(NF1)患者造成广泛的心理伤害。目前尚无有效的医学治疗方法,而且全球大多数 NF1 患者都无法进行手术。

目的

虽然正在研究针对 cNF 的有效医学治疗方法,但目前迫切需要开发一种手术方法,使全球所有 NF1 患者都能获得,并且该方法所需的技能和设备在大多数普通医疗办公室都能找到。在这里,我们提出了一种可靠的去除 cNF 的手术方法,该方法不需要无菌手术环境,可利用易得的临床设备,并且可以由任何医疗保健提供者(包括家庭医生和医师助理)进行。

方法

在一项前瞻性病例系列研究中,患有 NF1 的患者接受了这种手术,以去除多个皮肤神经纤维瘤。在手术前后,使用皮肤病生活质量指数(Dermatology Life Quality Index)作为患者满意度的替代指标。

结果

从 12 名个体的全身共切除了 83 个肿瘤。随访时的检查显示,愈合良好的疤痕没有感染或其他不良事件,包括异常瘢痕形成。患者对该手术的满意度很高,症状、日常活动、休闲、人际关系和治疗体验均有显著改善(P = 0.00062)。

结论

本研究证明了一种针对皮肤神经纤维瘤的可靠手术方法,该方法可在全球范围内供 NF1 患者使用,并且可以由各种医疗专家进行,具有较高的临床疗效和患者满意度。

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