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小儿双侧先天性肾积水的复杂管理:多学科方法

Complex Management of Bilateral Congenital Hydronephrosis in a Pediatric Patient: A Multidisciplinary Approach.

作者信息

Motofelea Nadica, Tamasan Ionela Florica, Tanasescu Sonia Aniela, Hoinoiu Teodora, Ioana Jabri Tabrizi Madalina, Pop Gheorghe Nicusor, Motofelea Alexandru Catalin

机构信息

Doctoral School, "Victor Babes" University of Medicine and Pharmacy Timisoara, 300041 Timisoara, Romania.

Department of Obstetrics and Gynecology, "Victor Babes" University of Medicine and Pharmacy Timisoara, Eftimie Murgu Sq. No. 2, 300041 Timisoara, Romania.

出版信息

Healthcare (Basel). 2025 Apr 25;13(9):998. doi: 10.3390/healthcare13090998.

Abstract

Congenital anomalies of the kidney and urinary tract (CAKUT) are common developmental malformations and a leading cause of pediatric renal dysfunction. Severe hydronephrosis, especially when accompanied by ureteral duplication, ureterocele, or neurogenic bladder, poses significant diagnostic and therapeutic challenges. This case report presents a 7-year-old male with prenatally diagnosed bilateral grade IV/V hydronephrosis (according to the radiology hydronephrosis grading system), complicated by the right pyeloureteral duplication, the left ureterocele, and the neurogenic bladder. The patient's clinical course was marked by recurrent urinary tract infections (UTIs), progressive renal dysfunction, and multiple surgical interventions. Initial decompression via bilateral ureterostomy and stenting led to significant improvements in renal function. However, the patient experienced recurrent febrile UTIs caused by multidrug-resistant pathogens, necessitating repeated hospitalizations and intravenous antibiotic therapy. Serial imaging studies documented persistent hydronephrosis, a neurogenic bladder, and vesicoureteral reflux. Subsequent surgical interventions included bilateral ureteral reimplantation, excision of the left ureterocele, and removal of a fibroepithelial polyp from the bladder wall. Despite these interventions, residual left hydronephrosis and right kidney hypoplasia persisted, underscoring the need for long-term surveillance. This case highlights the diagnostic and therapeutic challenges of managing CAKUT and emphasizes the importance of a multidisciplinary approach integrating imaging, functional assessment, and surgical planning. Early diagnosis and timely intervention can stabilize renal function, but ongoing monitoring and individualized treatment remain crucial for optimizing long-term outcomes in children with complex CAKUT.

摘要

先天性肾脏和尿路畸形(CAKUT)是常见的发育性畸形,也是小儿肾功能不全的主要原因。严重肾积水,尤其是伴有输尿管重复、输尿管囊肿或神经源性膀胱时,会带来重大的诊断和治疗挑战。本病例报告介绍了一名7岁男性,产前诊断为双侧IV/V级肾积水(根据放射学肾积水分级系统),并发右侧肾盂输尿管重复、左侧输尿管囊肿和神经源性膀胱。患者的临床病程以反复尿路感染(UTI)、进行性肾功能不全和多次手术干预为特征。通过双侧输尿管造口术和支架置入进行初始减压后,肾功能有显著改善。然而,患者因多重耐药病原体引起反复发热性UTI,需要反复住院和静脉抗生素治疗。系列影像学检查记录了持续性肾积水、神经源性膀胱和膀胱输尿管反流。随后的手术干预包括双侧输尿管再植术、切除左侧输尿管囊肿以及切除膀胱壁上的纤维上皮息肉。尽管进行了这些干预,左侧残余肾积水和右侧肾发育不全仍然存在,这突出了长期监测的必要性。本病例强调了管理CAKUT的诊断和治疗挑战,并强调了整合影像学、功能评估和手术规划的多学科方法的重要性。早期诊断和及时干预可以稳定肾功能,但持续监测和个体化治疗对于优化复杂CAKUT患儿的长期预后仍然至关重要。

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