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Tytuł pozycji:

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

Tytuł:
Zinner syndrome in children: clinical presentation, imaging findings, diagnosis, and outcome.
Autorzy:
Lin CC; Department of Pediatrics, MacKay Children's Hospital, No 92, Section 2, Chungshan North Road, Taipei, Taiwan.; Department of Medicine, MacKay Medical College, New Taipei City, Taiwan.; MacKay Junior College of Medicine, Nursing and Management, Taipei, Taiwan.
Sheu JC; Department of Pediatric Surgery, MacKay Children's Hospital, Taipei, Taiwan.
Tsai PS; Department of Medicine, MacKay Medical College, New Taipei City, Taiwan.; MacKay Junior College of Medicine, Nursing and Management, Taipei, Taiwan.; Department of Biomedical Imaging and Radiological Sciences, National Yang Ming Chiao Tung University, Taipei, Taiwan.; Department of Radiology, MacKay Memorial Hospital, Taipei, Taiwan.
Lee MD; Department of Pediatrics, Hsinchu MacKay Memorial Hospital, Hsinchu City, Taiwan.
Lin TH; Department of Pediatrics, MacKay Children's Hospital, No 92, Section 2, Chungshan North Road, Taipei, Taiwan.
Tsai JD; Department of Pediatrics, MacKay Children's Hospital, No 92, Section 2, Chungshan North Road, Taipei, Taiwan. .; Department of Medicine, MacKay Medical College, New Taipei City, Taiwan. .; Department of Medicine, Taipei Medical University, Taipei, Taiwan. .
Źródło:
Pediatric nephrology (Berlin, Germany) [Pediatr Nephrol] 2022 Dec; Vol. 37 (12), pp. 3075-3084. Date of Electronic Publication: 2022 Mar 25.
Typ publikacji:
Review; Journal Article
Język:
English
Imprint Name(s):
Publication: Berlin : Springer International
Original Publication: Berlin : Springer International, c1987-
MeSH Terms:
Multicystic Dysplastic Kidney*/complications
Kidney Diseases*/diagnosis
Urogenital Abnormalities*/diagnosis
Urogenital Abnormalities*/diagnostic imaging
Genital Diseases, Male*/complications
Cysts*
Urinary Tract Infections*/etiology
Urinary Tract Infections*/complications
Infant ; Male ; Humans ; Child ; Kidney/diagnostic imaging ; Kidney/abnormalities ; Kidney Pelvis ; Syndrome
References:
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Contributed Indexing:
Keywords: Kidney agenesis; Multicystic dysplastic kidney; Pediatric; Seminal vesicle cyst; Zinner syndrome
Entry Date(s):
Date Created: 20220325 Date Completed: 20221025 Latest Revision: 20230108
Update Code:
20240105
DOI:
10.1007/s00467-022-05516-2
PMID:
35332378
Czasopismo naukowe
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. A higher resolution version of the Graphical abstract is available as Supplementary information.
(© 2022. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)
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