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Congenital Urinary Malformations: Diagnostic Management, Treatment and Outcome
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Congenital Urinary Malformations: Diagnostic Management, Treatment and Outcome

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Nephrology & Urology".

Deadline for manuscript submissions: closed (1 July 2026) | Viewed by 4626

Editors

Dr. Maria Sergio

E-Mail Website
Guest Editor
Pediatric Surgical Unit, Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties "G D'Alessandro, University of Palermo, Palermo, Italy
Interests: pediatric surgery; urology; pediatric urology; congenital abnormalities; hydronephrosis; neonatology; enuresis; urodynamics; neonatal surgery
Dr. Marco Pensabene

E-Mail Website
Guest Editor
Pediatric Surgical Unit, Department Health Promotion of Mother and Child Care, Internal Medicine and Medical Specialties, University of Palermo, 90127 Palermo, Italy
Interests: pediatric surgery; urology

Special Issue Information

Dear Colleagues,

Introduction

Congenital malformations of the urinary system and kidneys represent a vast chapter in pediatric nephrourology. Many of these conditions require close collaboration between pediatric specialists in both medical and surgical fields, who, by studying the entire aspect of the abnormality, can achieve the best outcome with the least invasive treatment.

With the aim of exploring fundamental aspects of certain conditions and addressing some still-debated questions, in this Special Issue, we present our experience and recent findings from the literature.

Within the context of renal and urinary tract malformations, vesicoureteral reflux and its many clinical and therapeutic implications must be addressed. Similarly, significant attention should be given to ureteral malformations, which, in their severe forms, still present treatment challenges due to potential short- and long-term complications. The desire to tackle issues such as hypertension and solitary kidney, as well as varicocele, reflects a specific interest in prevention for adult well-being, thereby reducing risk factors which could compromise quality of life.

Dr. Maria Sergio
Dr. Marco Pensabene
Guest Editors

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Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • kidney
  • CAKUT
  • hypospadias
  • vesico-ureteral reflux
  • renal dysplasia
  • hypertension
  • renal function
  • imaging in pediatric urology

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Published Papers (4 papers)

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Research

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16 pages, 1079 KB  
Article
The Role of Vitamin D in Modulating the Innate Immune Response in Children with Vesicoureteral Reflux
by Marius-Cosmin Colceriu, Diana Jecan-Toader, Paul Luchian Aldea, Bogdan Bulată, Dan Delean, Alina Grama, Alexandra Mititelu, Tudor Lucian Pop, Simona Clichici, Teodora Mocan and Andreea-Liana Boț (Răchişan)
Children 2026, 13(6), 811; https://doi.org/10.3390/children13060811 - 12 Jun 2026
Viewed by 375
Abstract
Background: Vitamin D, through its role in antimicrobial peptide (AMP) expression, may influence innate immunity and inflammation in urinary tract infections (UTIs). This study evaluated its role in patients with vesicoureteral reflux (VUR) and its contribution to the pathophysiology of reflux nephropathy [...] Read more.
Background: Vitamin D, through its role in antimicrobial peptide (AMP) expression, may influence innate immunity and inflammation in urinary tract infections (UTIs). This study evaluated its role in patients with vesicoureteral reflux (VUR) and its contribution to the pathophysiology of reflux nephropathy (RN). Methods: We conducted a cross-sectional observational study of 25 pediatric patients with VUR, representing a subgroup analysis of a larger cohort examined in a previous study. We determined patients’ vitamin D status, correlated it with recurrent UTIs and RS, and explored its relationship with urinary LL-37, NGAL, and IL-6 levels as markers of innate immune function. Results: Serum vitamin D levels ranged from 10.7 to 123.2 ng/mL (mean 39.5 ng/mL); 12% had deficiency and 20% had insufficient levels. Low vitamin D levels were detected in patients with more than five acute pyelonephritis (APNs), with a mean value classified as insufficient (27.3 ng/mL). Patients with RS had a lower mean vitamin D level compared to those without (30.51 ng/mL vs. 41.23 ng/mL), though the difference was not statistically significant (p = 0.39). No significant associations were found between vitamin D and urinary IL-6 or NGAL levels. A strong positive correlation was observed between vitamin D and urinary LL-37/creatinine (r = 0.78, r2 = 0.61). Conclusions: Vitamin D appears to influence the frequency of UTIs and the development of RS, primarily by modulating LL-37 secretion, suggesting a possible role in the pathophysiology of RN. Full article
(This article belongs to the Special Issue Congenital Urinary Malformations: Diagnostic Management, Treatment and Outcome)
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Review

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11 pages, 213 KB  
Review
Electrical and Functional Magnetic Stimulation in the Management of Children’s Lower Urinary Tract Dysfunction—A Current Literature Review
by Edva Anna Frunda, András Kiss, Árpád Olivér Vida, Tibor Lóránd Reman, Raul-Dumitru Gherasim, Daniel Porav-Hodade, Virgil Gheorghe Osan, Carmen Viorica Muntean, Orsolya Katalin Ilona Mártha and Lorena Meliț
Children 2026, 13(3), 322; https://doi.org/10.3390/children13030322 - 25 Feb 2026
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Abstract
Background and Objective: Children’s lower urinary tract dysfunction (LUTD) is a frequently diagnosed condition. Initial management measures, such as parental education, urotherapy, and constipation management, followed by drug-based and surgical therapies, are well known. In recent years, different electrical, transcutaneous nerve stimulation methods [...] Read more.
Background and Objective: Children’s lower urinary tract dysfunction (LUTD) is a frequently diagnosed condition. Initial management measures, such as parental education, urotherapy, and constipation management, followed by drug-based and surgical therapies, are well known. In recent years, different electrical, transcutaneous nerve stimulation methods (sacral, tibial, and intravesical) were studied, and magnetic stimulation has gained importance in the treatment of lower urinary tract dysfunctions. This review aimed to examine the efficacy of functional magnetic stimulation in treating children’s LUTD. Materials and Methods: Our review was carried out in the following databases: PubMed/Medline and Google Scholar. Results: We limited our search to articles published in the last 8 years (2018–2025). Our used keywords were: “functional magnetic stimulation, electrical stimulation, children’s lower urinary tract dysfunction, children’s overactive bladder, enuresis”. The final search revealed a total of 132 results; articles written in languages other than English were excluded. In the end, a total of 16 articles presenting ES (12 articles) and FMS (four articles) in the treatment of children’s lower urinary tract dysfunction were included in our narrative review. Conclusions: In spite of a few clinical controlled trials, our review concerning functional magnetic stimulation (FMS) provides acceptable evidence to support the efficacy of these methods among the pediatric population. In the future, large sample, randomized controlled trials are needed in this field. Full article
(This article belongs to the Special Issue Congenital Urinary Malformations: Diagnostic Management, Treatment and Outcome)

Other

Jump to: Research, Review

15 pages, 2826 KB  
Case Report
Robot-Assisted Anderson–Hynes Pyeloplasty for Lower-Moiety Ureteropelvic Junction Obstruction in an Incomplete Duplex Collecting System Presenting as Dietl’s Crisis: A Case Report
by Dimitrios Deligiannis, Panagiotis Mitsos, Anna Papakonstantinou, Spyridon Skoufias and Aris Kaltsas
Children 2026, 13(7), 934; https://doi.org/10.3390/children13070934 - 16 Jul 2026
Viewed by 183
Abstract
Background/Objectives: Intermittent ureteropelvic junction obstruction (UPJO), classically termed Dietl’s crisis, may be missed when imaging is obtained outside symptomatic periods. The challenge is amplified in duplicated collecting systems, where the obstructed moiety may not be recognized on screening ultrasonography. Case Presentation: A 14-year-old [...] Read more.
Background/Objectives: Intermittent ureteropelvic junction obstruction (UPJO), classically termed Dietl’s crisis, may be missed when imaging is obtained outside symptomatic periods. The challenge is amplified in duplicated collecting systems, where the obstructed moiety may not be recognized on screening ultrasonography. Case Presentation: A 14-year-old boy presented with recurrent severe right flank pain triggered by heavy fluid intake, associated with nausea and vomiting and separated by symptom-free intervals. An initial renal ultrasound performed between attacks was normal. One week later, the same pain pattern recurred together with a febrile urinary tract infection. Computed tomography urography (CTU) demonstrated an incomplete right duplex collecting system with a bifid ureter, marked hydronephrosis of the lower moiety, delayed contrast excretion, and focal narrowing at the lower-moiety ureteropelvic junction adjacent to one crossing artery and one crossing vein. Diuretic 99mTc-mercaptoacetyltriglycine (MAG3) renography documented obstructive drainage, with a post-furosemide drainage half-time (T1/2) > 20 min, differential renal function of 51% on the right, and split moiety function of 31% (upper) and 20% (lower). The patient underwent transperitoneal robot-assisted dismembered Anderson–Hynes pyeloplasty of the lower moiety with the reconstructed ureteropelvic junction repositioned anterior to the crossing vessels; a 6 Fr × 26 cm double-J stent was placed and subsequently removed at 4 weeks postoperatively. Total skin-to-skin operative time was 75 min, estimated blood loss < 100 mL, and the postoperative course was uneventful. At early 3-month follow-up, the patient remained free of Dietl-type episodes, and ultrasonography showed marked reduction in lower-moiety hydronephrosis. Selective postoperative CTU, obtained because of the unusual bifid anatomy, demonstrated patent drainage. Conclusions: A normal interval ultrasound should not exclude intermittent UPJO when the history is stereotypical, and cross-sectional plus functional imaging is decisive when duplex anatomy and crossing vessels coexist. Full article
(This article belongs to the Special Issue Congenital Urinary Malformations: Diagnostic Management, Treatment and Outcome)
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13 pages, 732 KB  
Systematic Review
Application of Ultrasound in Primary Vesicoureteral Reflux: From Diagnosis to Follow Up
by Marco Pensabene, Benedetto Spataro, Fabio Baldanza, Francesco Grasso, Gregorio Serra, Veronica Notarbartolo, Mario Giuffrè, Giovanni Corsello, Elisa Zambaiti, Maria Rita Di Pace and Maria Sergio
Children 2025, 12(10), 1363; https://doi.org/10.3390/children12101363 - 9 Oct 2025
Cited by 3 | Viewed by 2155
Abstract
Background and Objectives: Primary vesicoureteral reflux (VUR) is a common pediatric urological disorder that can lead to significant renal morbidity if undetected or improperly managed. Ultrasound (US) plays a pivotal role in its assessment, providing a radiation-free tool to prenatal assessment, diagnosis, treatment, [...] Read more.
Background and Objectives: Primary vesicoureteral reflux (VUR) is a common pediatric urological disorder that can lead to significant renal morbidity if undetected or improperly managed. Ultrasound (US) plays a pivotal role in its assessment, providing a radiation-free tool to prenatal assessment, diagnosis, treatment, and long-term follow-up. This study aims to systematically review the literature on the use of US in pediatric primary VUR, emphasizing its applications in prenatal and postnatal diagnosis, intraoperative guidance, and follow-up monitoring. Methods: A systematic review of the literature was performed on PubMed in accordance with PRISMA guidelines. The research strategy used the following keywords: Ultrasound Vesicoureteral reflux, VUR Ultrasound, and VUR Sonography. A total of 2222 records were initially identified. After screening titles and abstracts for relevance, 2165 studies were excluded because they did not focus on ultrasound procedures, did not specify age limits, were redundant, involved non-homogeneous populations, or were unavailable in full text. Results: Prenatal US enables early identification of urinary tract anomalies suggestive of VUR, facilitating targeted postnatal evaluation. Postnatally, contrast-enhanced voiding ultrasound (CEVUS) offers a non-ionizing method for VUR confirmation or exclusion. Intraoperatively, US improves the accuracy and efficacy of bulking agent placement, potentially enhancing surgical outcomes. In follow-up, US remains essential for both conservatively managed and surgically treated patients, enabling timely detection of complications or recurrence. Conclusions: Ultrasound represents a useful tool in the management of pediatric primary VUR, applicable across all clinical stages, avoiding radiation exposure, and improving surgical effectiveness and follow-up management. Full article
(This article belongs to the Special Issue Congenital Urinary Malformations: Diagnostic Management, Treatment and Outcome)
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