Zinner syndrome in pediatric age group: An underdiagnosed entity.

Multicystic dysplastic kidney Pediatrics Renal agenesis Seminal vesical cyst Zinner

Journal

Journal of pediatric urology
ISSN: 1873-4898
Titre abrégé: J Pediatr Urol
Pays: England
ID NLM: 101233150

Informations de publication

Date de publication:
19 Jun 2024
Historique:
received: 19 12 2023
revised: 12 06 2024
accepted: 13 06 2024
medline: 1 7 2024
pubmed: 1 7 2024
entrez: 30 6 2024
Statut: aheadofprint

Résumé

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. 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. 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. 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. 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.

Identifiants

pubmed: 38945789
pii: S1477-5131(24)00313-9
doi: 10.1016/j.jpurol.2024.06.014
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

Déclaration de conflit d'intérêts

Conflict of interest None.

Auteurs

Kumar K Shashi (KK)

Department of Radiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address: drkumargowda@gmail.com.

Harsha Garg (H)

Department of Diagnostic, Molecular and Interventional Radiology, The Mount Sinai Hospital, Icahn School of Medicine, 1 Gustave Levy Place, New York, NY 10029, USA.

Richard N Yu (RN)

Department of Urology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.

Jeanne S Chow (JS)

Department of Radiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.

Classifications MeSH