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面部先天性浸润性脂肪瘤病的治疗进展:PIK3CA 突变及微血管重建的作用

Advances in the Treatment of Congenital Infiltrating Lipomatosis of the Face: The Role of PIK3CA Mutations and Microvascular Reconstruction.

作者信息

Moreno Stephen D, Liu Robert H, Robbins Quinn, Burkes Jason

机构信息

Department of Oral and Maxillofacial Surgery, Naval Medical Center San Diego, San Diego, CA.

Department of Oral and Maxillofacial Surgery, National Capital Consortium, Walter Reed National Military Medical Center.

出版信息

J Craniofac Surg. 2025;36(1):229-233. doi: 10.1097/SCS.0000000000010751. Epub 2024 Oct 1.

Abstract

Congenital infiltrating lipomatosis of the face is a rare aggressive-benign disorder characterized by progressive hemifacial overgrowth and complex, often asymmetrical, facial differences. Recently linked with the PIK3CA-Related Overgrowth Spectrum, it arises from mosaic mutations in the PIK3CA gene. Treatment, largely supportive and tailored to individual clinical presentations, requires a multidisciplinary approach. This article reviews the longitudinal care of a young adult patient from 8 years of age, detailing his journey through combined orthognathic and microvascular reconstructive surgery and the outcomes thereof. In addition, it explores the treatment's implications in the context of the PIK3CA mutation, offering insights into potential prognostic and therapeutic considerations.

摘要

先天性面部浸润性脂肪瘤病是一种罕见的侵袭性良性疾病,其特征为进行性半侧面部过度生长以及复杂且常不对称的面部差异。该疾病最近与PIK3CA相关过度生长谱系相关联,由PIK3CA基因的镶嵌突变引起。治疗主要是支持性的,并根据个体临床表现进行调整,需要多学科方法。本文回顾了一名年轻成年患者从8岁起的长期治疗过程,详细介绍了他接受正颌手术和微血管重建手术的历程及其结果。此外,本文还探讨了在PIK3CA突变背景下该治疗的意义,为潜在的预后和治疗考虑提供见解。

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