关键词: Multicystic dysplastic kidney Pediatrics Renal agenesis Seminal vesical cyst Zinner

Mesh : Humans Retrospective Studies Male Child Seminal Vesicles / abnormalities diagnostic imaging Child, Preschool Syndrome Female Adolescent Abnormalities, Multiple / diagnosis Wolffian Ducts / abnormalities Cysts / diagnosis surgery Infant

来  源:   DOI:10.1016/j.jpurol.2024.06.014

Abstract:
BACKGROUND: Zinner Syndrome (ZS), a rare congenital malformation of the mesonephric duct, combines seminal vesicle cyst (SVC) with ipsilateral upper urinary tract abnormalities. Typically asymptomatic in childhood, ZS manifests between 2nd to 4th decades with bladder symptoms, perineal pain and infertility. Diagnostic confirmation with additional imaging is needed when either renal or seminal abnormalities are identified.
METHODS: A retrospective study spanning 22 years identified 20 pediatric ZS cases through clinical analytics. Demographic, clinical, and radiological data were analyzed, including presenting complaints, imaging modalities (ultrasound, CT, MRI), and surgical findings. The study was HIPAA-compliant and IRB-approved.
RESULTS: Among 20 cases (mean age: 7.3 years), clinical presentations included asymptomatic cases, urinary symptoms, and abdominal pain. Imaging revealed renal anomalies (agenesis, multicystic dysplastic kidney) and seminal vesicle abnormalities. Surgical interventions (n = 12) addressed symptomatic cases, often involving robotic or laparoscopic procedures.
CONCLUSIONS: ZS, though rare, presents with varied clinical features, necessitating a multidisciplinary approach. Early diagnosis is facilitated by prenatal identification of renal abnormalities. Surgical intervention is reserved for symptomatic cases, with techniques such as vesiculectomy and resection of remnant structures employed.
CONCLUSIONS: This study highlights ZS\'s diverse clinical and radiological spectrum, emphasizing the need for vigilance in detecting overlapping entities. Timely identification, utilizing advanced imaging techniques, is crucial for accurate diagnosis and appropriate management of Zinner Syndrome in the pediatric population.
摘要:
背景:Zinner综合征(ZS),罕见的先天性中肾管畸形,合并精囊囊肿(SVC)与同侧上尿路异常。通常在儿童时期无症状,ZS在第2到第4个十年之间出现膀胱症状,会阴疼痛和不孕症。当确定肾脏或精液异常时,需要进行额外的影像学检查的诊断确认。
方法:一项历时22年的回顾性研究通过临床分析确定了20例小儿ZS病例。人口统计,临床,并分析了放射学数据,包括提出投诉,成像模式(超声,CT,MRI),和手术发现。该研究符合HIPAA和IRB批准。
结果:在20例(平均年龄:7.3岁)中,临床表现包括无症状病例,泌尿症状,和腹痛。影像学显示肾脏异常(发育不全,多囊性发育不良肾)和精囊异常。手术干预(n=12)解决了有症状的病例,通常涉及机器人或腹腔镜手术。
结论:ZS,虽然罕见,呈现不同的临床特征,需要多学科方法。肾脏异常的产前鉴定有助于早期诊断。手术干预保留用于有症状的病例,采用囊泡切除术和残存结构切除术等技术。
结论:这项研究突出了ZS的不同临床和放射学谱,强调在发现重叠实体时需要保持警惕。及时识别,利用先进的成像技术,对于儿科人群中Zinner综合征的准确诊断和适当管理至关重要。
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