Walz Benjamin, Pfefferle Vanessa, Häfner Hans-Martin, Kofler Lukas
Universitätshautklinik Tübingen, Liebermeisterstr. 25, 72076, Tübingen, Deutschland.
Zentrum für Seltene Hauterkrankungen, Liebermeisterstr. 25, 72076, Tübingen, Deutschland.
Dermatologie (Heidelb). 2022 Dec;74(12):961-968. doi: 10.1007/s00105-023-05220-8. Epub 2023 Sep 20.
Neurofibromatosis type 1, also known as Recklinghausen's disease, is a neurocutaneous tumor syndrome that is genetically determined and is associated with infestation of the integument with neurofibromas (nerve sheath tumors). The occurrence of neurofibromas can be very stressful for patients and often contributes to a reduced quality of life for patients, especially if externally visible body parts are affected. The aim of this study is to show to what extent the resection of cutaneous neurofibromas can improve patients' quality of life.
For this study, we conducted a retrospective data collection via questionnaire on the quality of life before and after the surgical removal of cutaneous neurofibromas at the Department of Dermatology, University Hospital of Tübingen. An adapted dermatological quality of life index and a postoperative questionnaire on patient satisfaction were used. In addition, patient data were taken from doctor's notes, surgical reports, and outpatient documentation. A total of 30 patients with neurofibromatosis type 1 who underwent inpatient or outpatient surgery for cutaneous neurofibromas at the Tübingen dermatology hospital between 2016 and 2020 were surveyed. The survey results were statistically analyzed and represented as absolute and relative frequencies.
Our study indicates an improved quality of life after surgery for cutaneous neurofibromas, especially regarding limitations in everyday life, self-consciousness, the choice of clothing, and leisure activities. The majority of our patients showed no new occurrence of neurofibromas in the surgical area and postoperative bleeding or wound infections were rare.
In relation to the high level of satisfaction with the surgical and cosmetic results and also the positive influence on quality of life, our study indicates a favorable risk-benefit ratio for the resection of cutaneous neurofibromas in neurofibromatosis I.
1型神经纤维瘤病,也称为雷克林豪森病,是一种由基因决定的神经皮肤肿瘤综合征,与皮肤神经纤维瘤(神经鞘瘤)的侵袭有关。神经纤维瘤的出现对患者来说可能非常有压力,并且常常导致患者生活质量下降,尤其是当身体外部可见部位受到影响时。本研究的目的是表明切除皮肤神经纤维瘤能在多大程度上改善患者的生活质量。
在本研究中,我们通过问卷调查对图宾根大学医院皮肤科手术切除皮肤神经纤维瘤前后的生活质量进行了回顾性数据收集。使用了经过调整的皮肤病生活质量指数和术后患者满意度问卷。此外,患者数据来自医生记录、手术报告和门诊病历。对2016年至2020年间在图宾根皮肤科医院接受住院或门诊手术切除皮肤神经纤维瘤的30例1型神经纤维瘤病患者进行了调查。对调查结果进行了统计分析,并以绝对频率和相对频率表示。
我们的研究表明,切除皮肤神经纤维瘤后生活质量得到改善,尤其是在日常生活限制、自我意识、服装选择和休闲活动方面。我们的大多数患者在手术区域没有出现新的神经纤维瘤,术后出血或伤口感染很少见。
鉴于对手术和美容效果的高度满意度以及对生活质量的积极影响,我们的研究表明,对于I型神经纤维瘤病患者切除皮肤神经纤维瘤,风险效益比良好。