Zinner syndrome in pediatric age group: An underdiagnosed entity.

Autor: Shashi KK; Department of Radiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA. Electronic address: drkumargowda@gmail.com., 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., Yu RN; Department of Urology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA., Chow JS; Department of Radiology, Boston Children's Hospital, 300 Longwood Avenue, Boston, MA 02115, USA.
Jazyk: angličtina
Zdroj: Journal of pediatric urology [J Pediatr Urol] 2024 Aug; Vol. 20 (4), pp. 705.e1-705.e7. Date of Electronic Publication: 2024 Jun 19.
DOI: 10.1016/j.jpurol.2024.06.014
Abstrakt: Introduction: 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.
Materials and 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.
Discussion: 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.
Conclusion: 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.
Competing Interests: Conflict of interest None.
(Copyright © 2024 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)
Databáze: MEDLINE
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