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第一鳃裂畸形酷似胆脂瘤:儿科病例研究及手术方法。

First Branchial Cleft Anomaly Mimicking Cholesteatoma: A Pediatric Case Study and Surgical Approach.

机构信息

Department of Otorhinolaryngology-Head and Neck Surgery, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.

出版信息

Am J Case Rep. 2024 Nov 29;25:e945421. doi: 10.12659/AJCR.945421.

Abstract

BACKGROUND First branchial cleft anomalies (FBCAs) are rare congenital disorders with clinical manifestations and anatomy that are variable and complex. Due to the anatomical proximity to the facial nerve and association with infection, FBCAs require accurate diagnosis and careful management. The purpose of this report was to share the diagnosis and treatment of an extremely rare case of a 4-year-old girl with an FBCA presenting as a painless mass of the left external auditory canal and an inconspicuous depression in the left mandibular angle, a presentation that can easily be misdiagnosed as cholesteatoma of the middle ear. CASE REPORT A 4-year-old girl without any symptoms presented to our department due to "finding a mass in the left ear canal more than 2 months ago". Otoscopy, computed tomography, magnetic resonance imaging, and physical examinations supported the diagnosis of FBCA. The patient underwent surgery, which included resection of the first branchial fistula and canalplasty. Intraoperative findings revealed that the facial nerve straddles the fistula surface and that the fistula extends from the bottom oblique to the inferior wall of the external auditory canal. The postoperative diagnosis was "first branchial cleft fistula (Work type II)". No facial palsy or recurrence was noted during the follow-up period. CONCLUSIONS This report has highlighted the importance of the correct diagnosis and treatment of FBCAs and awareness of anatomical associations and complications. Careful otologic evaluation can help clinicians make a correct diagnosis. If preoperative examination reveals a connection between the superficial skin and external auditory canal, concurrent canalplasty should be performed.

摘要

背景

第一鳃裂畸形(FBCA)是一种罕见的先天性疾病,其临床表现和解剖结构复杂多样。由于与面神经解剖位置临近,且易并发感染,因此 FBCA 需要准确的诊断和谨慎的处理。本报告旨在分享一例极其罕见的 4 岁女孩 FBCA 的诊断和治疗经验,该患者以左侧外耳道无痛性肿块和左侧下颌角不明显凹陷为表现,容易误诊为中耳胆脂瘤。

病例报告

一名 4 岁女孩无任何症状,因“2 个多月前发现左耳道内有一肿块”就诊于我科。耳镜、计算机断层扫描、磁共振成像和体格检查均支持 FBCA 的诊断。患者接受了手术,包括第一鳃裂瘘管和耳道成形术切除。术中发现面神经跨过瘘管表面,瘘管从底部斜向外耳道下壁延伸。术后诊断为“第一鳃裂瘘管(Work Ⅱ型)”。随访期间未出现面瘫或复发。

结论

本报告强调了正确诊断和治疗 FBCA 的重要性,以及对解剖关系和并发症的认识。仔细的耳科学评估有助于临床医生做出正确诊断。如果术前检查发现浅表皮肤与外耳道之间存在联系,应同时进行耳道成形术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/45e8/11622328/f0f002d16c36/amjcaserep-25-e945421-g001.jpg

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