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先天性面部浸润性脂肪瘤病:婴儿面部不对称的罕见原因。

Congenital Facial Infiltrating Lipomatosis: A Rare Cause of Facial Asymmetry in Infants.

作者信息

El Omri Malika, Mouna Belakhdher, Safa Jemlii, Zeineb Nfikha, Monia Ghammem, Jihene Houas, Wassim Kermani

机构信息

Department of Ear, Nose, Throat and Head and Neck Surgery, Farhat Hached University Hospital, Sousse, Tunisia.

Pathology Department, Farhat Hached University Hospital, Sousse, Tunisia.

出版信息

Ear Nose Throat J. 2024 Apr 1:1455613241241869. doi: 10.1177/01455613241241869.

Abstract

Congenital facial infiltrating lipomatosis (CFIL) is a rare condition that typically affects the trunk and limbs, but can also occur in the face. Managing CFIL is a significant challenge due to its diffuse involvement in important facial structures. This case report aims to describe a rare form of lipomatosis and discuss the clinical and therapeutic aspects of this entity. The patient was a 5-year-old girl who was admitted to our department due to a right paralateronasal swelling that had been present for 5 months. During the physical examination, a swelling of the right nasolabial was observed. A computed tomography scan of the facial bone revealed an extra bony soft tissue mass near the vestibule of the right nostril in contact with the frontal process of the maxillary bone and the anterior wall of the right maxillary sinus. The patient underwent excision under general anesthesia. The histological report indicated congenital focal lipomatosis. There was no recurrence of swelling a year after initial surgery. Facial infiltrating lipomatosis is a rare congenital disorder in which mature lipocytes invade adjacent tissue. Due to its diffuse infiltration and involvement of important facial structures, complete surgical excision is often impossible. Specific management of this condition requires insight into its pathogenesis as surgical removal of the mass is usually unsuccessful.

摘要

先天性面部浸润性脂肪瘤病(CFIL)是一种罕见疾病,通常累及躯干和四肢,但也可发生于面部。由于其广泛累及重要面部结构,CFIL的治疗是一项重大挑战。本病例报告旨在描述一种罕见的脂肪瘤病形式,并讨论该疾病的临床和治疗方面。患者为一名5岁女孩,因右侧鼻旁肿胀5个月入院。体格检查时,发现右侧鼻唇沟肿胀。面部骨骼计算机断层扫描显示,右侧鼻孔前庭附近有一个骨外软组织肿块,与上颌骨额突和右侧上颌窦前壁相连。患者在全身麻醉下接受了切除术。组织学报告显示为先天性局灶性脂肪瘤病。初次手术后一年,肿胀未复发。面部浸润性脂肪瘤病是一种罕见的先天性疾病,成熟脂肪细胞侵入相邻组织。由于其弥漫性浸润和累及重要面部结构,通常无法进行完全手术切除。这种疾病的具体治疗需要深入了解其发病机制,因为手术切除肿块通常不成功。

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