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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. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information, Author(s): Chun-Chen Lin [sup.1] [sup.2] [sup.3] , Jin-Cherng Sheu [sup.4] , Pei-Shan Tsai [sup.2] [sup.3] [sup.5] [sup.6] , Ming-Dar Lee [sup.7] , Tsu-Hua Lin [sup.1] , Jeng-Daw Tsai [sup.1] [sup.2] [...]

OBJECTIVE. We sought to assess the clinical and imaging findings in intermittent hydronephrosis secondary to ureteropelvic junction obstruction, with particular emphasis on the characteristic ultrasonographic findings. METHODS. This prospective, longitudinal, observational study included all children who had intermittent ureteropelvic junction obstruction and presented with abdominal pain over 6 years. Renal ultrasound was used as an initial screening tool to detect intermittent hydronephrosis. Renal ultrasonography was repeated every 1 to 2 days to record serial changes from the symptomatic to the asymptomatic stage. Their clinical manifestations and imaging findings were studied. RESULTS. Eighteen patients (14 boys, 4 girls) were studied. Most had sharp pain that began acutely and typically lasted for CONCLUSIONS. The keys to diagnosis are awareness of the syndrome, a detailed history, and immediate and serial imaging studies during painful crises. A thickened renal pelvic wall during convalescence is an important ultrasonic sign of intermittent hydronephrosis. www.pediatrics.org/cgi/doi/10.1542/ peds.2005-0583 doi:10.1542/peds.2005-0583 Key Words intermittent hydronephrosis, ureteropelvic junction obstruction, prospective studies, renal ultrasonography, renal pelvic wall thickening, convalescence, intravenous pyelogram, diuretic renal scan, computed tomography, dismembered pyeloplasty, follow up Abbreviations UPJO--ureteropelvic junction obstruction IVP--intravenous pyelogram VCUG--voiding cystourethrography, CT--computed tomography DTPA--diethylene triamine pentaacetic acid, URETEROPELVIC JUNCTION OBSTRUCTION (UPJO) is attributed to a functional or anatomic narrowing of the junction between the renal pelvis and the ureter. It is 1 of the most enigmatic clinical [...]