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后尿道瓣膜:产前诊断及膀胱功能长期管理的作用;单中心观点及文献综述

Posterior urethral valves: Role of prenatal diagnosis and long-term management of bladder function; a single center point of view and review of literature.

作者信息

Pellegrino Chiara, Capitanucci Maria Luisa, Forlini Valentina, Zaccara Antonio, Lena Federica, Sollini Maria Laura, Castelli Enrico, Mosiello Giovanni

机构信息

ERN EUROGEN Affiliated Center, Division of Neuro-Urology and Surgery for Continence, Bambino Gesù Childrens' Hospital, IRCCS, Rome, Italy.

Pediatric Surgery Division, University of Genova, Genoa, Italy.

出版信息

Front Pediatr. 2023 Jan 6;10:1057092. doi: 10.3389/fped.2022.1057092. eCollection 2022.

Abstract

Posterior Urethral Valves (PUV) are the most common cause of lower urinary tract obstruction. More severe forms are detected early in pregnancy (mainly type I), while other forms are usually discovered later in childhood when investigating lower urinary tract symptoms. Bladder dysfunction is common and is associated with urinary incontinence in about 55% (0%-72%). Despite the removal of the obstruction by urethral valve ablation, pathological changes of the urinary tract can occur with progressive bladder dysfunction, which can cause deterioration of the upper urinary tract as well. For this reason, all children with PUV require long-term follow-up, always until puberty, and in many cases life-long. Therefore, management of PUV is not only limited to obstruction relief, but prevention and treatment of bladder dysfunction, based on urodynamic observations, is paramount. During time, urodynamic patterns may change from detrusor overactivity to decreased compliance/small capacity bladder, to myogenic failure (valve bladder). In the past, an aggressive surgical approach was performed in all patients, and valve resection was considered an emergency procedure. With the development of fetal surgery, vesico-amniotic shunting has been performed as well. Due to improvements of prenatal ultrasound, the presence of PUV is usually already suspected during pregnancy, and subsequent treatment should be performed in high-volume centers, with a multidisciplinary, more conservative approach. This is considered to be more effective and safer. Primary valve ablation is performed after clinical stability and is no longer considered an emergency procedure after birth. During childhood, a multidisciplinary approach (pediatric urologist, nephrologist, urotherapist) is recommended as well in all patients, to improve toilet training, using an advanced urotherapy program with medical treatments and urodynamic evaluations. The aim of this paper is to present our single center experience over 30 years.

摘要

后尿道瓣膜(PUV)是下尿路梗阻最常见的原因。更严重的类型在孕期早期被发现(主要是I型),而其他类型通常在儿童期出现下尿路症状时才被发现。膀胱功能障碍很常见,约55%(0%-72%)的患者伴有尿失禁。尽管通过尿道瓣膜切除术解除了梗阻,但随着膀胱功能障碍的进展,尿路仍可能出现病理变化,这也可能导致上尿路恶化。因此,所有患有PUV的儿童都需要长期随访,一直到青春期,在很多情况下甚至需要终身随访。所以,PUV的治疗不仅限于解除梗阻,基于尿动力学观察,预防和治疗膀胱功能障碍至关重要。随着时间推移,尿动力学模式可能从逼尿肌过度活动转变为顺应性降低/膀胱容量减小,再到肌源性衰竭(瓣膜性膀胱)。过去,对所有患者都采取积极的手术方法,瓣膜切除术被视为紧急手术。随着胎儿手术的发展,也开展了羊膜腔内分流术。由于产前超声技术的改进,孕期通常已怀疑PUV的存在,后续治疗应在大型中心采用多学科、更保守的方法进行。这被认为更有效且更安全。在临床稳定后进行一期瓣膜切除术,出生后不再将其视为紧急手术。在儿童期,也建议对所有患者采用多学科方法(小儿泌尿科医生、肾病科医生、尿疗师),通过先进的尿疗计划结合药物治疗和尿动力学评估来改善如厕训练。本文的目的是介绍我们单中心30年的经验。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad34/9853300/525117c603dc/fped-10-1057092-g001.jpg

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