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治疗结节性硬化症面部血管纤维瘤的不同谱系:两例报告

Managing Different Spectrums of Tuberous Sclerosis Facial Angiofibroma: A Report of Two Cases.

作者信息

Mustaqim Khairul, Mohd Ghazi Ahmad Faiz Najmuddin, Harun Nor Rashid Shawaltul Akhma, Mohamad Syafiq

机构信息

Surgery, Universiti Sains Malaysia School of Medical Sciences, Kota Bharu, MYS.

Plastic and Reconstructive Surgery, Hospital Raja Perempuan Zainab II, Kota Bharu, MYS.

出版信息

Cureus. 2023 Feb 19;15(2):e35200. doi: 10.7759/cureus.35200. eCollection 2023 Feb.

DOI:10.7759/cureus.35200
PMID:36960246
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10031289/
Abstract

Tuberous sclerosis is one of the rarest genetically linked disorders that can affect a multitude of body systems in various forms. Patients with facial angiofibroma may face issues arising from the various modalities and approaches that can be applied. Moreover, surgeons also face challenges in preparing patients for specific interventions. Here, we are reporting the two spectrums of this condition that may present, along with how we managed patients in our center. The first case is a severe form of tuberous sclerosis involving the skin as well as neurological manifestation, while the second case is a milder form. Both were treated with serial excision and electrosurgery, respectively. Facial angiofibroma in tuberous sclerosis can present in various spectrums. Serial excision and electrosurgery are deemed acceptable. Few advancements have been made in the management of this condition, and combination therapies have shown favorable outcomes. Optimizing patient comorbidities is imperative before intervention and multidisciplinary team involvement would ensure patient safety.

摘要

结节性硬化症是最罕见的基因相关疾病之一,可通过多种形式影响多个身体系统。患有面部血管纤维瘤的患者可能会面临因可应用的各种方式和方法而产生的问题。此外,外科医生在为患者准备特定干预措施时也面临挑战。在此,我们报告这种疾病可能出现的两种情况,以及我们在本中心如何治疗患者。第一例是结节性硬化症的严重形式,累及皮肤以及神经系统表现,而第二例是较轻的形式。两例分别采用连续切除和电外科手术治疗。结节性硬化症中的面部血管纤维瘤可呈现多种情况。连续切除和电外科手术被认为是可接受的。在这种疾病的治疗方面进展甚微,联合治疗已显示出良好的效果。在干预前优化患者的合并症至关重要,多学科团队的参与将确保患者安全。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/4c7fbcb5babe/cureus-0015-00000035200-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/9a3e2c298aca/cureus-0015-00000035200-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/757d6c1401f8/cureus-0015-00000035200-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/bcf41ed90a74/cureus-0015-00000035200-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/38180f8eb03d/cureus-0015-00000035200-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/4c7fbcb5babe/cureus-0015-00000035200-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/9a3e2c298aca/cureus-0015-00000035200-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/757d6c1401f8/cureus-0015-00000035200-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/bcf41ed90a74/cureus-0015-00000035200-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/38180f8eb03d/cureus-0015-00000035200-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238b/10031289/4c7fbcb5babe/cureus-0015-00000035200-i05.jpg

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