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鳃瘘在鳃耳畸形综合征中的表现:诊断与治疗的挑战。

A true branchial fistula in the context of branchiootic syndrome: challenges of diagnosis and management.

机构信息

University of Cambridge, Cambridge, UK.

Department of Plastic and Reconstructive Surgery, Addenbrooke's Hospital, CB2 0QQ, UK.

出版信息

J Plast Reconstr Aesthet Surg. 2014 Sep;67(9):1288-90. doi: 10.1016/j.bjps.2014.04.012. Epub 2014 May 14.

DOI:10.1016/j.bjps.2014.04.012
PMID:24933236
Abstract

BACKGROUND

The presence of a branchial fistula with communication both internally and externally: a 'true' branchial fistula is rare, and may arise in the context of autosomal dominant conditions such as branchiootic syndrome and branchiootorenal syndrome.

STUDY

We discuss the case of a true branchial fistula, which recurred after initial surgical excision, in a patient with branchiootic syndrome. The residual tract was dissected in a second operation through stepladder neck incisions and removed in toto via an intraoral approach. No renal abnormalities were detected on investigation with ultrasound.

DISCUSSION

Incomplete excision of a branchial sinus is likely to cause recurrence however intraoperative visualisation of the tract can can sometimes prove challenging. An combined intraoral and external approach aids delineation and tract definition when there is a true branchial fistula and can therefore facilitate a complete excision. Suspicion of an hereditary aetiology should be raised in patients with bilateral or preauricular features, or a positive family history, which may then prompt additional renal and genetic investigation.

摘要

背景

存在与内部和外部都相通的鳃裂瘘管:真正的鳃裂瘘管很少见,可能发生在常染色体显性条件下,如鳃耳肾综合征和鳃耳肾畸形综合征。

研究

我们讨论了一例鳃耳肾综合征患者的真正鳃裂瘘管,该瘘管在初次手术切除后复发。在第二次手术中,通过梯形颈切口解剖残留窦道,并通过口腔内途径完全切除。超声检查未发现肾脏异常。

讨论

不完全切除鳃裂窦可能导致复发,但是术中窦道的可视化有时可能具有挑战性。当存在真正的鳃裂瘘管时,口腔内和外部联合入路有助于窦道的描绘和定义,因此可以促进完全切除。对于双侧或耳前特征、阳性家族史的患者,应怀疑遗传性病因,这可能会促使进行额外的肾脏和遗传检查。

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A true branchial fistula in the context of branchiootic syndrome: challenges of diagnosis and management.鳃瘘在鳃耳畸形综合征中的表现:诊断与治疗的挑战。
J Plast Reconstr Aesthet Surg. 2014 Sep;67(9):1288-90. doi: 10.1016/j.bjps.2014.04.012. Epub 2014 May 14.
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