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Zinner syndrome in children: clinical presentation, imaging findings, diagnosis, and outcome

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Abstract

Background

Zinner syndrome (ZS), the association of congenital seminal vesicle cyst (SVC) and ipsilateral kidney anomalies, is rarely diagnosed in childhood. This study aimed to assess presentation, imaging findings, management, and outcome of pediatric ZS.

Methods

Sixteen children with ZS were diagnosed and managed at our hospital from 2003 to 2021. We reviewed the medical records to collect data on initial symptoms, results of imaging studies, complications, operation, and follow-up.

Results

Ultrasound was used in all 16 cases as initial diagnostic tool. Fourteen patients were asymptomatic at diagnosis: these were transferred from obstetricians or pediatricians for evaluation of the prenatally or postnatally detected ultrasonic kidney anomalies. SVCs were incidentally noted on ultrasonography. The other two cases initially presented with urinary tract infection (UTI). Kidney anomalies included multicystic dysplastic kidney in 3 and kidney agenesis in 13 patients. Eleven (68.7%) patients had ipsilateral ectopic ureters entering SVC. Four (36.4%) patients had a reflux from urethra into SVC (urethro-cystic reflux) on voiding cystourethrography. Ten (62.5%) patients remained asymptomatic over a mean of 58 months (range, 7–216 months), two patients developed lower urinary tract dysfunction, and five patients had UTIs. Two boys needed SVC removal, and SVC had disappeared in two patients after 2.5–4 years of follow-up.

Conclusions

Unilateral kidney hypodysplasia with ectopic ureter inserting into the ipsilateral SVC is a characteristic sign for diagnosis of ZS. In our case series, ZS was mainly asymptomatic. Urethro-cystic reflux was associated with UTIs in young infants. SVC removal was rarely required.

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Author information

Authors and Affiliations

  1. Department of Pediatrics, MacKay Children’s Hospital, No 92, Section 2, Chungshan North Road, Taipei, Taiwan

    Chun-Chen Lin, Tsu-Hua Lin & Jeng-Daw Tsai

  2. Department of Medicine, MacKay Medical College, New Taipei City, Taiwan

    Chun-Chen Lin, Pei-Shan Tsai & Jeng-Daw Tsai

  3. MacKay Junior College of Medicine, Nursing and Management, Taipei, Taiwan

    Chun-Chen Lin & Pei-Shan Tsai

  4. Department of Pediatric Surgery, MacKay Children’s Hospital, Taipei, Taiwan

    Jin-Cherng Sheu

  5. Department of Biomedical Imaging and Radiological Sciences, National Yang Ming Chiao Tung University, Taipei, Taiwan

    Pei-Shan Tsai

  6. Department of Radiology, MacKay Memorial Hospital, Taipei, Taiwan

    Pei-Shan Tsai

  7. Department of Pediatrics, Hsinchu MacKay Memorial Hospital, Hsinchu City, Taiwan

    Ming-Dar Lee

  8. Department of Medicine, Taipei Medical University, Taipei, Taiwan

    Jeng-Daw Tsai

Authors
  1. Chun-Chen Lin
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  2. Jin-Cherng Sheu
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  3. Pei-Shan Tsai
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  4. Ming-Dar Lee
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  5. Tsu-Hua Lin
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  6. Jeng-Daw Tsai
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Contributions

Study conception and design: Chun-Chen Lin and Jeng-Daw Tsai. Material preparation, data collection and analysis: Pei-Shan Tsai, Ming-Dar Lee and Tsu-Hua Lin. Writing—original draft preparation: Chun-Chen Lin. Writing—review and editing: Jeng-Daw Tsai. Supervision: Jin-Cherng Sheu.

All authors read and approved the final manuscript.

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Correspondence to Jeng-Daw Tsai.

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Lin, CC., Sheu, JC., Tsai, PS. et al. Zinner syndrome in children: clinical presentation, imaging findings, diagnosis, and outcome. Pediatr Nephrol 37, 3075–3084 (2022). https://doi.org/10.1007/s00467-022-05516-2

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  • DOI: https://doi.org/10.1007/s00467-022-05516-2

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