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We describe our experience in 2 institutions handling bladder prolapse through a patent urachus (PU), together with a brief review of published literature. Case 1: A term neonate with congenital prolapsed bladder via PU. Ultrasound at 21 weeks gestation revealed a male fetus with a large midline pelvic cyst communicating with the bladder which disappeared on subsequent 27 weeks ultrasound. Case 2: A term female neonate with congenital prolapsed bladder via PU with no prenatal diagnosis. In both cases the bladder closure was undertaken during the newborns' first days of life., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: Urologic substudies of prenatal myelomeningocele (MMC) closure have focused primarily on continence without significant clinical benefit. Fetoscopic MMC repair (FMR) is a newer form of prenatal intervention and touts added benefits to the mother, but urological outcomes have yet to be analyzed. We set out to focus on bladder safety rather than continence and examined bladder outcomes with different prenatal MMC repairs (FMR and prenatal open [POMR]) and compared bladder-risk-categorization to traditional postnatal repair (PSTNR)., Methods: An IRB-approved retrospective analysis of all patients undergoing all forms of MMC repairs with inclusion and exclusion criteria based on the MOMS trial was performed. Bladder safety assessment required initial urodynamic studies (UDS), renal bladder ultrasound (RBUS), and/or voiding cystourethrogram (VCUG) within the 1st year of life. Follow-up analyses within the cohorts required follow-up studies within 18 months after initial evaluations. Outcomes assessed included bladder-risk-categorization based on the CDC UMPIRE study (high, intermediate, and safe), hydronephrosis (HN), and vesicoureteral reflux (VUR). A single reader evaluated each UDS., Results: Initial UDS in 93 patients showed that the prevalence of high-risk bladders were 35% FMR versus 36% PSTNR and 60% POMR. Follow-up UDS showed only 8% of FMR were high-risk compared to 35% POMR and 36% PSTNR. Change from initial to follow-up bladder-risk-category did not reach significance (p = .0659); however, 10% PSTNR worsened to high-risk on follow-up, compared to none in either prenatal group. Subanalysis of follow-up UDS between the prenatal cohorts also was not significant (p = .055). Only 8% of FMR worsened or stayed high-risk compared to 35% with POMR (p = .1). HN was significantly different at initial and subsequent follow up between the groups with the least in the FMR group., Conclusions: Early outcome UDS analyses demonstrated lower incidence of high-risk bladders in FMR patients with a trend toward clinically significant improvement compared to POMR in regard to all evaluated metrics. Larger, prospective, confirmatory studies are needed to further evaluate the potential benefits on FMR on bladder safety and health., (© 2021 Wiley Periodicals LLC.)

Objective: The present study aimed to investigate the molecular mechanism underlying congenital obstructive nephropathy (CON)., Methods: The microarray dataset GSE70879 was downloaded from the Gene Expression Omnibus, including 3 kidney samples of megabladder mice and 4 control kidneys. Using this dataset, differentially expressed miRNAs (DEMs) were identified between the kidney samples from megabladder mice and controls, followed by identification of the target genes for these DEMs and construction of a DEM and target gene interaction network. Additionally, the target genes were subjected to Gene Ontology and pathway enrichment analyses, and were used for construction of a protein-protein interaction (PPI) network. Finally, regulatory networks were constructed to analyze transcription factors for the key miRNAs., Results: From 17 DEMs identified between kidney samples of megabladder mice and controls, 3 key miRNAs were screened, including mmu-miR-150-5p, mmu-miR-374b-5p and mmu-miR-126a-5p. The regulatory networks identified vascular endothelial growth factor A (Vegfa) as the common target gene of mmu-miR-150-5p and five transcription factors, including nuclear receptor subfamily 4, group A, member 2 (Nr4a2), Jun dimerisation protein 2 (Jdp2), Kruppel-like factor 6 (Klf6), Neurexophilin-3 (Nxph3) and RNA binding motif protein 17 (Rbm17). The gene encoding phosphatase and tensin homolog (Pten) was found to be co-regulated by mmu-miR-374b-5p and high mobility group protein A1 (Hmga1), whereas the kirsten rat sarcoma viral oncogene (Kras) was identified as a common target gene of mmu-miR-126a-5p and paired box 6 (Pax6)., Conclusions: In summary, the above-listed key miRNAs, transcription factors and key genes may be involved in the development of CON., (Copyright © 2018. Published by Elsevier B.V.)

Background/purpose: Reconstruction of microbladders is a difficult surgical challenge: How can a neobladder be recreated when >90% of the new bladder is augmented patch, and how can a Mitrofanoff conduit be anastomosed when the native bladder is so tiny? This series describes microbladders secondary to anuria and/or diversion that required augmentation. This was done using a de-tubularized ileal segment, and because of the small size of the native bladder, Mitrofanoff anastomosis was performed to the bowel patch (using a novel "Keel Procedure")., Methods: Our surgical experience in reconstruction of microbladders was reviewed: pre and post augmentation capacity, compliance and maximum detrusor pressure were compared. The success of the Mitrofanoff anti-reflux technique is described. Data are given as median (interquartile range) and compared by Wilcoxon paired rank test., Results: 10 patients, median bladder capacity pre-op 10 (9-20) mls were reconstructed. The follow up time is 2.7years (2.2-4.2). Post-op bladder capacity increased 16-fold to 167 (114-281) mls, P<0.01. Compliance significantly improved from 1.7 (0.3-4.8) to 14.3 (4.1-66.3) mls/cmH 2 O, P<0.05. Maximum detrusor over-activity decreased from 27 (7-120) to 12.5 (8-26) cmH 2 O, (N.S. P=0.3). Videourodynamics confirmed a leak in 2 patients, leading to incorporation into the technique of a non-absorbable seromuscular suture to provide long-term robustness to the antireflux procedure., Conclusion: Bladder augmentation in microbladders is possible, and a functional Mitrofanoff procedure with a continent anti-reflux procedure can be created using the "Birmingham keel technique" implanting the Mitrofanoff into the augment patch, with 80% success (similar to published results for conventional anastomosis to native bladders)., Level of Evidence: Level IV., (Copyright © 2017. Published by Elsevier Inc.)

A monochorionic-diamniotic twin baby presented with intrauterine growth restriction and anuria. The baby was found to have bladder agenesis, a pelvic dysplastic horseshoe kidney, vertebral anomalies, a ventricular septal defect and facial dysmorphisms. It was surprising to find no abnormalities in amniotic fluid indices prenatally, suggesting the possibility of urine output that declined as the pregnancy proceeded. Some degree of twin-to-twin transfusion of amniotic fluid was also possible, which could have rescued the oligohydramnios known to be associated with kidney and urinary tract abnormalities. It was also notable that there was no abnormality in respiratory function, especially since further investigations revealed close to no kidney function. The intrauterine growth restriction (IUGR) along with the multiple anomalies found made the baby unsuitable for dialysis and transplant, and the decision of transition to palliative care was made., Competing Interests: Conflicts of Interest: None declared., (2016 BMJ Publishing Group Ltd.)

Bladder diverticula in children are mostly congenital, frequently associated with vesicoureteral reflux. Their positive diagnosis is based primarily on retrograde urethrocystography. One complication of this condition is acute urinary retention. We report the case of a 4-month-old infant who presented acute retention of urine secondary to bilateral congenital bladder diverticulum and review the literature on this subject., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

We present a scientific investigation into the pathogenesis of a urinary bladder disease. The disease in question is called urofacial syndrome (UFS), a congenital condition inherited in an autosomal recessive manner. UFS features incomplete urinary bladder emptying and vesicoureteric reflux, with a high risk of recurrent urosepsis and end-stage renal disease. The story starts from a human genomic perspective, then proceeds through experiments that seek to determine the roles of the implicated molecules in embryonic frogs and newborn mice. A future aim would be to use such biological knowledge to intelligently choose novel therapies for UFS. We focus on heparanase proteins and the peripheral nervous system, molecules and tissues that appear to be key players in the pathogenesis of UFS and therefore must also be critical for functional differentiation of healthy bladders. These considerations allow the envisioning of novel biological treatments, although the potential difficulties of targeting the developing bladder in vivo should not be underestimated., (© The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA.)

Patent urachus is one of the least commonly seen of the urachal anomalies. In this report, we present a case of a patent urachus in a15-day-old female who presented with leakage from the umbilical site. The purpose of this article is to discuss the embryology, clinical manifestations, diagnosis, and management of patent urachus.

Background: Urofacial syndrome (UFS) is characterised by congenital bladder dysfunction accompanied by a characteristic abnormal grimace upon smiling and crying. In recent years, biallelic mutations of HPSE2 and LRIG2 have been reported in UFS patients. Non-neurogenic neurogenic bladder (NNNB) has a bladder identical to UFS without typical facial features. The aim of this study was to analyse HPSE2 mutations in patients with UFS and NNNB or severe lower urinary tract dysfunction (LUTD) without abnormal facial expression., Methods: Patients with UFS, NNNB and severe LUTD were enrolled in the study. We examined a total of 35 patients from 33 families. There were seven UFS patients from five different families, 21 patients with NNNB and seven with LUTD. HPSE2 gene mutation analysis was performed using the polymerase chain reaction protocol followed by Sanger sequencing in these patients., Results: A twin pair with UFS was found to be homozygous for c.457C>T (p.Arg153*) mutation. No other pathogenetic variant was detected., Conclusion: HPSE2 mutations were found in one UFS family but not detected in patients with NNNB and severe LUTD. Considering the increasingly recognised cases of NNNB that were diagnosed in early childhood period, genetic factors appear to be responsible. Thus, further genetic studies are needed to discover novel associated gene variants in these bladder anomalies., (© 2015 S. Karger AG, Basel.)

Purpose: The lazy bladder syndrome (LBS) is characterized by an infrequent voiding, a large bladder capacity without neurological or urological disorders. In spite of being relatively common, there is little literature about it. The aim of our study was to compare the clinical features and urodynamic findings in asymptomatic or symptomatic patients with lazy bladder syndrome., Patients and Methods: We reviewed the charts of 126 adult patients diagnosed with lazy bladder syndrome. Clinical and radiological features, urodynamic findings and therapeutic management were evaluated. With these data, we divided patients into 2 groups: asymptomatic and symptomatic patients. After, we performed a comparative analysis of the data., Results: The incident of LBS was significantly higher in women (81%). Twenty-one patients were asymptomatic (17%), 105 patients were symptomatic (83%). The patients with symptomatic LBS were significantly older (54.3 years ± 14.7). Voiding dysfunction (53%) and urinary retention (27%) were the most common symptoms in symptomatic group. In the uroflowmetry test, maximum and mean uroflow were significantly higher in asymptomatic group (P=0.0074). Reduced bladder sensation revealed no difference in the 2 groups, but in the symptomatic group, detrusor has also a poor contractility (P=0.0001). Nineteen patients (18%) had uro-nephrological complications., Conclusion: LBS is certainly underestimated. Voiding dysfunction, urinary retention in infrequent voiders or uro-nephrological complication (urinary tract infection with fever, ureteral reflux…) should recall LBS diagnosis. The hypothesis of bladder structural failure or autonomic nervous system dysfunction may be discussed (suggested)., Level of Evidence: 5., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Lower urinary tract and/or kidney malformations are collectively the most common cause of end-stage renal disease in children, and they are also likely to account for a major subset of young adults requiring renal replacement therapy. Advances have been made regarding the discovery of the genetic causes of human kidney malformations. Indeed, testing for mutations of key nephrogenesis genes is now feasible for patients seen in nephrology clinics. Unfortunately, less is known about defined genetic bases of human lower urinary tract anomalies. The focus of this review is the genetic bases of congenital structural and functional disorders of the urinary bladder. Three are highlighted. First, prune belly syndrome, where mutations of CHRM3, encoding an acetylcholine receptor, HNF1B, encoding a transcription factor, and ACTA2, encoding a cytoskeletal protein, have been reported. Second, the urofacial syndrome, where mutations of LRIG2 and HPSE2, encoding proteins localised in nerves invading the fetal bladder, have been defined. Finally, we review emerging evidence that bladder exstrophy may have genetic bases, including variants in the TP63 promoter. These genetic discoveries provide a new perspective on a group of otherwise poorly understood diseases.

Contrast fistulogram (FG) and distal pressure colostogram (DPCG) are standard diagnostic methods for the assessment of anorectal malformations. Pelvic magnetic resonance imaging (MRI) earned a place among essential diagnostic methods in preoperative investigations after the Currarino syndrome and a high incidence of associated spinal dysraphism were described. The aim of our study was to evaluate the possibility of substituting FG and DPCG by a modified pelvic MRI, e.g. MRI fistulogram (MRI-FG) and MRI colostogram (MRI-DPCG). The prospective study involved 29 patients with anorectal malformations who underwent a modified pelvic MRI. The length and course of fistulas and rectum, and the presence of sacral anomalies were studied on MRI images and compared with images obtained by radiologic examinations. Modified MRI brought identical results as contrast studies in 25 patients when related to the fistula and rectum length and course. MRI was more accurate for the detection of sacral anomalies. MRI-FG was the only imaging method used in the four most recent patients. The results support the assumption that conventional contrast examinations for the assessment of anorectal malformations can be replaced by MRI, thus reducing the radiation dose., (© 2013.)

Purpose: Congenital primary bladder diverticulum is a rare cause of infra-vesical obstruction. We present a series of 12 cases who presented with urinary retention secondary to a large primary bladder diverticulum. The aim is to high light the diagnostic difficulties and management issues of congenital bladder diverticulae., Methods: We reviewed the case sheets of 12 patients with congenital bladder diverticulae who presented as lower tract obstruction with or without infection at a tertiary care centre in the last 10 years. All patients had urine examinations (complete microscopic with culture), serum creatinine, ultrasonography, intravenous urogram, voiding cystourethrogram with or without cystoscopy. The cases were managed by extravesical mobilization of diverticulae, diverticulectomy, and ureteric re-implantation. All patients (aged 1-36 months, mean 16.8 months) presented with retention of urine but 4 of them had symptoms of fever, tachycardia, abdominal distension and vomiting. Serum creatinine was normal in 10 out of 12 cases, but was high in two. Urine cultures grew Escherichia coli in 5 cases. Ultrasonography showed moderate to severe hydronephrosis on the left side in 5 cases, on the right side in 4 cases and bilateral in 3 cases but diverticulae could be located in only 8 cases. Intravenous urogram revealed hydrouretero-nephrosis in 9 cases, a poor functioning kidney with hydrouretero-nephrosis in 3 cases and bilateral hydrouretero-nephrosis in 3 cases. A Voiding Cystourethrogram confirmed the diagnosis in all cases. Vesicoureteric reflux (Grade 4-5) into the ipsilateral ureter was seen in 9 children of which it was bilateral in three cases., Results: All children underwent extravesical diverticulectomy with re-implantation of the ipsilateral ureter in 9 cases, bilateral in 3 cases. All had an uneventful recovery except for one who had a suprapubic leak which healed on conservative treatment. Postoperative ultrasonograms showed mild hydronephrosis in 2 cases and micturating cystourethrograms revealed a grade III V-U reflux in one case which was managed conservatively. Patients are maintaining a sterile urine culture after a follow up of 6-36 months., Conclusion: Primary bladder diverticulum should be kept as a differential diagnosis in cases of bladder outlet obstruction in infants and children. A carefully done voiding cystourethrogram is the hallmark of diagnosis. Good results can be achieved by diverticulectomy and primary definitive repair with ureteric re-implantation even in infants., (Copyright © 2011 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Objective: We present the case of an adult patient diagnosed with Hutch diverticula after examination. Indications were of a type of congenital bladder diverticula very rare in children and unusual in adults, as very few cases in adults are addressed in the literature., Methods: Intravenous Urography and Voiding Cystourethrogram (VCUG) were realized, Results: The intravenous urography revealed ureterohydronephrosis in the excretory phase that did not have repletion defects suggesting lithiasis at distal urethra. On the other hand, a narrowing of the urethra was observed at the point of bladder entry due to the presence of a juxtameatal bladder diverticulum. Next a retrograde cystography was performed which ruled out vesicourethral reflux and revealed that the diverticulum presented elevated residuals after urination. As a result of our patient's clinical characteristics and the absence of vesico-urethral reflux, we decided to perform an endoscopic surgical opening of the diverticula's neck. CONCLUSIONS Hutch paraurethral diverticula is an very uncommon entity, even in infancy. The Voiding Cystourethrogram (VCUG) is especially useful in diagnosing these patients. In symptomatic cases surgical correction of the diverticula is required.

Purpose: In this article we highlight the difference, from established adult urology, in required approach to the care of adolescents and young adults presenting with the long-term consequences of the major congenital anomalies of the genitourinary tract. We review some abnormalities of the kidneys, progressive renal failure and disorders of bladder function from which general conclusions can be drawn., Materials and Methods: The published literature was reviewed and augmented with material from our institutional databases. For renal function the CAKUT (congenital abnormalities of the kidney and urinary tract) database at University College London Hospitals was used, which includes 101 young adult patients with CAKUT in whom the urinary tract has not been diverted or augmented. For bladder function some data are from patient records at Boston Children's Hospital., Results: Adolescents who grow up with the burden of a major congenital anomaly have an overwhelming desire to be normal. Many achieve high levels of education and occupy a wide range of employment scenarios. Babies born with damaged kidneys will usually experience improvement in renal function in the first 3 years of life. Approximately 50% of these cases will remain stable until puberty, after which half of them will experience deterioration. Any urologist who treats such patients needs to test for proteinuria as this is a significant indicator of such deterioration. In its absence, the urologist must have a reasonable strategy for seeking a urological cause. The most effective management for nephrological renal deterioration is with angiotensin converting enzyme inhibitors, which slow but do not prevent end stage renal failure. Renal deterioration is generally slower in these patients than in those with other forms of progressive renal disease. The bladder is damaged by obstruction or by functional abnormalities such as myelomeningocele. Every effort should be made to stabilize or reconstruct the bladder in childhood. A dysfunctional bladder is associated with or causes renal damage in utero, but continued dysfunction will cause further renal damage. Bladder function often changes in puberty, especially in boys with posterior urethral valves who may experience high pressure chronic retention. Dysfunction is managed with antimuscarinic drugs, clean intermittent self-catheterization and intestinal augmentation. Adult urologists must be able to manage the long-term problems associated with these treatments., Conclusions: Pediatric conditions requiring management in adolescence are rare but have major, lifelong implications. Their management requires a broad knowledge of pediatric and adult urology, and could well be a specialty in its own right. Therefore, adult urologists must remain aware of the conditions, the problems that they may encounter and the special management required for these patients to live normal lives., (Copyright © 2012 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.)

Congenital bladder diverticula (CBD) are unusual but not rare in childhood. Report of acute urinary retention due to congenital bladder diverticula are rarely found in the literature. Here we report a case of congenital bladder diverticulum with acute urinary retention in a ten months old male infant. Urinary tract infection and urinary retention are the most frequent presentation forms. In this case CBD produce urinary obstruction as a result of compression of the urethra which causes urinary retention. Voiding cystourethrogram revealed a large bladder diverticulum. Bladder diverticulum should be a differential diagnosis in male children, presenting with urinary retention, fever and an abdominal mass. Ultrasonography, voiding cystourethrogram & cystoscopy confirm the diagnosis and diverticulectomy was successfully done for the child.

A 24-year-old man was referred to our department for urinary retention and urinary tract infection. He was pointed out a bladder diverticulum in childhood. Computerized tomography (CT) scanning and magnetic resonance imaging (MRI) showed a bladder diverticulum, 10 by 8 cm in size, which was located posteriorly in the bladder. He underwent resection of the bladder diverticulum and left ureterovesiconeostomy. The histopathologic finding showed a bladder wall with thin muscular layer. The operation made urination possible with successful resolution of voiding symptoms. Herein we report this rare case of congenital bladder diverticulum which was treated in adulthood, and present a review the literature.

3 cases of fetal megacystis have been described in the article. In each clinical situation the pathology was diagnosed in the first half of the pregnancy. The authors have attempted to present clinical management in congenital megacystis. Key elements in the further diagnostics of megacystis in a fetus include invasive procedures to exclude or confirm chromosomal aberrations in fetus and detailed ultrasound anatomy scan of the fetus. The possibility of prenatal treatment of megacystis (placement of vesico-amaniotic shunt) is the source of considerable controversy. Biochemical assessment of fetal kidney function seems to be the most important element in evaluation of patients before introducing the intrauterine therapy. Contraindication for the prenatal treatment may be not only fetal aneuploidy but also serious maternal pregnancy complications, such as severe preeclampsia accompanied by deterioration of renal function and increasing ascites. The decision to place the shunt in a fetus should be made bearing in mind that although it significantly reduces the risk of perinatal death, it may unfortunately fail to improve renal function of the affected child.

Aim: In this article, we report our experience with diverticulectomies of symptomatic congenital bladder diverticula in children, which was performed by utilizing pneumovesicoscopy., Materials and Methods: Six boys, 4-8 years of age (mean, 5.6) underwent pneumovesicoscopic diverticulectomy at our institution from June 2007 to June 2008. There were 4 right-single diverticula and 2 double-left diverticula. Under cystoscopic control, after the cystopexy to the abdominal wall, a midline 5-mm trocar for a 0-degree telescope and two lateral 3-mm reusable trocars through the anterolateral wall of the bladder in the midclavicular line were introduced, insufflating the bladder with carbondioxide to 10-12 mm Hg pressure. The diverticulum/a were inverted into the bladder and the mucosa around the neck was circumcized by using scissors and a monopolar hook. The defect was sutured with interrupted sutures and the bladder was drained with a Foley catheter, which was introduced at the site of the 5-mm port, and a urethral catheter., Results: Mean operative time was 110 minutes. No major peri- or postoperative complications were recorded, except that the displacement of one of the lateral trocars that resulted in gas leakage in 1 case. The trocar was replaced and the procedure was completed. An ultrasound and a voiding cystourethrogram, performed from 3 to 6 months after the operation, showed the disappearance of the diverticulum/a., Conclusions: In our experience, pneumovesicoscopic diverticulectomy is an easy, safe procedure and can be considered a valid alternative to the open or laparoscopic procedures. It also provides no postoperative discomfort and a good cosmetic result.

Purpose: Congenital primary bladder diverticulum is a rare condition and may present with urinary infection; other forms of presentation are rare. We present a series of infants who presented with urinary retention secondary to large primary bladder diverticulum., Methods: Seven infants were evaluated for symptoms of lower urinary tract obstruction. All seven were infant boys; three were neonates. Investigations included ultrasonogram, voiding cystourethrogram (VCUG) and cystoscopy., Results: Six infants had single large diverticulum while one had bilateral diverticula. VCUG was diagnostic in all cases demonstrating the mechanism of obstruction clearly except one where bilateral diverticula was diagnosed only on cystoscopy prior to definitive surgery. Five children (including one neonate) underwent successful definitive repair consisting of diverticulectomy and ureteral re-implant while two neonates were planned for a staged correction. One neonate later in the series underwent definitive primary repair as bladder was good sized. All five children have done well after definitive repair at a follow-up of 6-72 months., Conclusion: Primary bladder diverticulum in infants may present with lower urinary obstructive symptoms indistinguishable from posterior urethral valves. A carefully done VCUG can help in diagnosis. Primary definitive repair can be undertaken even in first few months of life with good results.

Objective: To determine the prenatal evolution/natural history and postnatal outcome of fetuses diagnosed with a neural tube defect (NTD) lacking the Arnold-Chiari-II malformation (ACM II)., Material and Methods: This retrospective study reviewed 16 fetuses evaluated with ultrasound (US) and MRI at a single referral center from 1/2000 to 8/2007. Follow-up studies and available postnatal outcomes were reviewed., Results: Postpartum diagnosis was terminal myelocystoceles 7/16 (44%); myelomeningoceles (MMCs) 3/16 (19%); lipomyelomeningoceles 2/16(13%); and thoracic myelocystocele 1/16 (6%). Three patients (19%) were lost to follow-up or termination of pregnancy. Two prenatally diagnosed 'closed' NTD were postnatally found to be MMCs. Three of the myelocystoceles had additional omphalocele, bladder extrophy, imperforate anus and spinal defect (OEIS complex). For the total cohort, impaired lower extremity function was seen in 38%, impaired bladder function in 64%, and ventriculoperitoneal shunting in 8%. Four fetuses with a myelocystocele developed hindbrain herniation in the third trimester of pregnancy. The preterm delivery rate was 38%. Five of eight (63%) neonates with postnatally diagnosed myelocystoceles had mothers with a body mass index over 30., Conclusion: Prenatal differentiation between closed and open NTD is not always possible. Postnatal outcome of isolated myelocystocele and MMC seems to be more favorable than for an NTD with ACM II (shunt requirement). Incontinence is the major childhood morbidity. Maternal obesity may be a risk factor for closed NTDs.

Congenital H-type rectovaginal fistulas and single ectopic bilateral ureters are each rare malformations. We describe a baby girl with a congenital rectovaginal fistula diagnosed 2 years after correction of single ectopic bilateral ureters. To our knowledge, this is the first association of these entities. Repair of fistula was complicated by recurrence, requiring a second procedure. The recommended operation for this anomaly requires separating the suture lines on the vagina and rectum. The practice of simply oversewing and then buttressing the suture lines is probably not sufficient.

Urine bladder diverticula are usually asymptomatic, therefore they are incidentally diagnosed during examinations for other purposes. A Chinese patient, with a history of pulmonary adenocarcinoma first underwent a technetium-99m methylene diphosphonate whole-body bone scan. In addition to multiple skeletal metastases, abnormal shape of the urine bladder was also noted, which overlapped the superior ramus of the right pubic bone. In order to: a) better delineate the shape and structure of the urine bladder; b) visualize the pubic bone and c) detect the exact location and structural changes of skeletal lesions in the lumbosacral region, single photon emission tomography/computerized tomography (SPET/CT) scan of this region was performed immediately, and demonstrated: a) a big urine bladder diverticulum in the right hemipelvis; b) structural changes of skeletal metastases while metastases in the pubic bone were ruled out. An overview of the etiology, histopathology, complications, management and imaging of urine bladder diverticulum is also presented. We consider our case the first to be diagnosed by SPET/CT and differentiated from metastases of the overlapping bone in a 69 years old female.

The case of intrauterine idiopathic bladder rupture with consecutive urinary ascites is presented. Conservative management resulted in complete resolution of the lesion.

Congenital bladder diverticula are rare anomalies of the bladder. Patients present with infection, hematuria, and/or urinary obstruction. We report on the case of a 12-year-old boy who developed gross hematuria and recurrent infection owing to a 12-cm bladder diverticulum. Robotic-assisted laparoscopic diverticulectomy was performed. We describe the first reported robotic-assisted laparoscopic diverticulectomy in a pediatric patient.

Objectives: The purpose of the study is to present the authors' clinical and surgical experience with congenital bladder diverticula in nine pediatric patients at a developing world tertiary care center., Patients and Methods: Records of nine patients diagnosed and treated as congenital bladder diverticula from 2000 to 2007 were retrospectively reviewed for age, sex, chief complaints, associated anomalies, investigative work-up, operative notes, and postoperative follow-up., Results: All were males. Age at presentation ranged from six months to eight years (mean three years). All were diagnosed postnatally by ultrasound and/or voiding cystourethrography (VCUG) and confirmed on urethrocystoscopy. Open surgical excision of diverticulum was done in all the patients. Ureteral reimplantation was simultaneously done only in three patients with VCUG-documented high-grade vesicoureteral reflux (VUR). With an average follow-up of four years, gradual resolution of symptoms was seen in seven of nine patients whose postoperative follow up records were available. There was no diverticulum recurrence at the defined mean follow-up., Conclusion: Pediatric patients with recurrent urinary tract infections and voiding dysfunction should always be evaluated for congenital bladder diverticulum. Although investigations such as intravenous urography (IVU), urodynamic studies, nuclear renal scanning, and, sometimes, CT scan and MRI, form an important part of preoperative diagnostic work-up and postoperative follow up, USG and VCUG may be enough when availability and cost are the constraints. Diverticulectomy, with ureteral reimplantation for high-grade reflux, provides good results without recurrence.

Objectives: Surgical treatment of a congenital bladder diverticulum is indicated in symptomatic children. Diverticulectomy can be performed by an open or a laparoscopic approach. We report our recent experience in using the pneumovesicoscopic approach for accomplishing vesical diverticulectomy., Methods: We operated on three boys with a mean age of 11.6 years (10-14 years) during August 2006 to February 2007. In all children, a ureteric catheter was introduced first by cystoscopy followed by intravesical CO(2) insufflation at a pressure of 12-15 mmHg. Three trocars were inserted under visual control in the bladder. Diverticulectomy was performed. The defect was closed by interrupted sutures. Bladder drainage was achieved using a urethral catheter for 2 days., Results: The mean operative time was 133.3 min (100-180 min). Oral intake began after a mean of 5.3h (4-6h). Minimal blood loss was encountered. Non-steroidal analgesics were used only during the 1st day postoperatively with no need for morphine. All patients were discharged on the 2nd day postoperatively after removal of the urethral catheter and tube drain. The mean follow-up period was 5 months (3-6 months)., Conclusion: Pneumovesicoscopic diverticulectomy is a feasible procedure. It does not require a long learning curve, and is associated with shorter hospital stay and rapid recovery with good cosmetic aspect. Pneumovesicoscopy has the potential to be used in the treatment of other conditions such as vesicoureteral reflux, and may replace open surgery.

Objectives: To analyze our series of primary congenital diverticula (PCD) and their association with vesicoureteral reflux., Methods: We have taken care of 23 children with PCD. Eleven of them had big diverticula (> 2 cm) and twelve small. In the first group, 4 children had vesicoureteral reflux and 5 in the second group. In group A, ureteral reimplantation was performed at the time of diverticulum excision. Nor diverticula neither refluxes were operated in group B., Results: We analyze separately results in both groups. Group A: Patients were operated including diverticulum excision. There were not recurrences except in one case with Ehler-Danlos Syndrome. No reimplanted ureter showed postoperative reflux. Nevertheless, one case with multiple bladder diverticula without reflux presented reflux after the excision of several diverticula without reimplantation. Group B: Small diverticula did not undergo surgery Spontaneous outcome of reflux was similar to that of the general population without diverticula., Conclusions: Bladder diverticula are frequently associated with vesicoureteral reflux. The presence of reflux is not an absolute condition for surgical or endoscopic treatment. When diverticula are big in size (Group A) the indication for surgery comes from recurrent infection or voiding disorders, not from reflux. If they undergo surgery, ureteral reimplantation is performed in the case they had reflux or for technical reasons like bladder wall weakness. When diverticula are small (Group B) the presence of reflux does not condition treatment, being the rate of spontaneous resolution similar to the general population.

We report 2 cases of patent urachus with bladder prolapse, which has a characteristic finding of "disappearance of cyst" antenatally. In the first case, a 34-year-old woman presented at 14 weeks gestation for evaluation of a cyst. Prenatal ultrasonography revealed a cystic mass at the base of the umbilical cord, communicating with the bladder. At 26 weeks gestation, the cyst had disappeared, and a solid mass bulged out inferior to the umbilical cord. At term, the patient delivered a male infant. Examination of the neonate demonstrated mucosal protrusion inferior to the umbilical cord. Catheterization confirmed communication with the bladder. We diagnosed patent urachus with bladder prolapse. In the second case, a 36-year-old woman presented at 19 weeks gestation for evaluation of moderate bilateral hydronephrosis and an abdominal cyst. Prenatal ultrasonography revealed a cystic mass at the base of the umbilical cord, communicating with the bladder. At 26 weeks gestation, the cyst had disappeared. At term, the patient delivered a male infant displaying patent urachus with bladder prolapse. Understanding of the development of urachus is important for prenatal diagnosis, which in turn allows surgery immediately after birth. We showed bladder function after surgery remains good at school age.

Objectives: To present the symptoms, treatment, evolution, and pathologic findings of primary (nonobstructive) diverticula in childhood. Primary (nonobstructive) diverticula are not frequent in childhood. Bladder diverticula have classically been defined as "hernias of the mucosa through muscular fibers of the bladder wall.", Methods: From September 1985 through September 2005, we treated 11 children categorized has having primary congenital bladder diverticula. At diagnosis, their ages ranged between 7 months and 9 years (mean 4.6 years). Ten were boys and one was a girl. The main reason for consultation was febrile urinary infection (n = 5), recurrent urinary infections (n = 3), enuretic syndrome (n = 1), gross hematuria (n = 1), and pain in the right iliac fossa (n = 1). One child had Ehlers-Danlos syndrome. All the diverticula were larger than 2 cm in diameter. Of the 11 children, 10 underwent surgery. Clinical observation was chosen for the 9-month-old infant., Results: All 10 operated children had developed favorably at a follow-up of 2 to 20 years (mean 9.3). The 9-month-old child continued under observation for 5 years. We observed no diverticulum recurrence and no postoperative vesicoureteral reflux in the reimplanted children. Pathologic examination was performed of the excised diverticula in 7 cases, and muscular fibers were found in all of them; however, most of the fibers were very fine, particularly at the dome of the diverticulum., Conclusions: The results of our study have shown that urinary tract infection is the most common symptom of bladder diverticula. Surgical treatment provided good results, without any recurrence or morbidity. The bladder diverticula were lined with fine muscular fibers.

Purpose: Paraureteral or Hutch diverticula are congenital bladder diverticula that occur at or adjacent to the ureteral hiatus and are associated with vesicoureteral reflux in the majority of cases. Surgical treatment has traditionally been ureteral reimplantation with or without diverticulectomy. We present our experience with endoscopic treatment of vesicoureteral reflux associated with paraureteral diverticula., Materials and Methods: Of 745 patients undergoing endoscopic treatment for vesicoureteral reflux between 2002 and 2006, 17 (2.3%) had paraureteral diverticula located at the refluxing ureter. The hydrodistention implantation technique was used and dextranomer/hyaluronic acid copolymer was used as bulking material. Success was defined as vesicoureteral reflux grade 0 on postoperative voiding cystourethrogram at 1 to 3 months after a single treatment., Results: A total of 20 refluxing ureters with associated paraureteral diverticula were treated in 17 patients. Of the cases 14 were unilateral and 3 were bilateral. Reflux was grade I in 6 patients, grade II in 4, grade III in 8, grade IV in 1 and grade V in 1. A mean of 1.2 ml bulking agent was injected per ureter. Overall success was 81% (13 of 16 patients) after a single injection. Success per vesicoureteral reflux grade was 100% (6 of 6 patients) for grade I, 100% (3 of 3) for grade II, 63% (5 of 8) for grade III, 100% (1 of 1) for grade IV and 100% (1 of 1) for grade V. Endoscopic treatment failed in 3 patients. Multivariate analysis identified large diverticular size and high bulking agent volume as predictors of treatment failure. Age, reflux grade and the presence of unilateral vs bilateral paraureteral diverticula did not impact outcome., Conclusions: Endoscopic injection of dextranomer/hyaluronic acid copolymer is an excellent choice for the treatment of vesicoureteral reflux associated with paraureteral diverticula because it has a high success rate and avoids open surgery.

Kidney transplantation (KT) in children with end-stage renal disease and an abnormal bladder poses a complex management challenge. Ureterocystoplasty (UC) has been previously reported in older children with non-compliant bladders, but the timing and technique of repair are controversial. This case reports the youngest patient, a 20-month-old boy to undergo successful single-stage UC and living-related KT. UC was performed because of a fibrotic, non-compliant bladder. A temporary vesicostomy was placed to provide adequate drainage in the presence of urethral stenosis. The patient developed a single episode of pyelonephritis within the first six months post-operatively, but there were no other urologic complications. At 13 months, the renal function is excellent with a mean glomerular filtration rate of 100 mL/min/1.73 m(2) and no clinical evidence of rejection. This case demonstrates that simultaneous UC and KT can be safely performed even in infants with non-compliant bladders and renal failure.

The underlying mechanisms of urinary-tract infections (UTI) in renal transplant recipients are still not fully understood. In otherwise healthy children, bladder dysfunction increases the susceptibility to UTI. The aim of this study was to evaluate lower-urinary-tract function in children and adolescents after renal transplantation. Sixty-eight recipients of renal transplants, 5-20 years of age and 1-15 years after transplantation, were evaluated for their bladder function with a questionnaire, uroflowmetry and bladder ultrasound, and for renal function (glomerular filtration rate) by measuring clearance of inulin or iohexol. Forty-nine patients (72%) had some type of abnormality of bladder function. Abnormal bladder capacity was found in 26%, abnormal urinary flow in 50% and residual urine in 32% of the patients. There was no significant difference in bladder or renal function in children with urinary-tract malformations compared with those with normal urinary tract. Furthermore, there was no significant difference in renal function in patients with bladder dysfunction compared with those without. The incidence of bladder dysfunction is high in children and adolescents after renal transplantation, but the clinical significance of this finding and whether there is a correlation between bladder dysfunction and UTI in these patients need to be clarified further.

We report on a successful treatment of hypoplastic bladder in a 5-month-old girl with single ectopic ureter in a solitary kidney. Side-to-side anastomosis was performed first between the urethral ectopic ureter and side wall of the bladder. Nine months later when the bladder capacity increased to 70 ml, ureterocystoneostomy was done through psoas hitch procedure. She retains urine of about 270 ml and evacuate completely at 14 years of age.

A 3-day-old male alpaca cria was presented for lack of vigor and failure to urinate since birth. Based on the history, laboratory data, ultrasonographs, surgical findings, and postmortem examination, the cria was diagnosed with bilateral renal agenesis and hypoplastic bladder, a congenital condition rarely seen in veterinary medicine.

A traumatic rupture of the bladder in a healthy child is extremely rare and is usually not considered during evaluation of abdominal pain or sepsis. However, a delay in the diagnosis of bladder perforation can result in morbid and sometimes catastrophic outcomes. We report a delayed diagnosis of spontaneous rupture of a congenital bladder diverticulum in a previously healthy child. This case demonstrates that spontaneous bladder rupture may be a rare cause of abdominal complaints and sepsis when the etiology at first seems unclear.

Neonatal infravesical obstruction in females is less common than in boys and rarely reported. In this article, we report on 3 cases of infravesical obstruction in female neonates with different etiologies. We also reviewed the literature on this topic. Including our cases, 17 cases of neonatal infravesical obstruction in girls have been reported. Neonatal infravesical obstruction in females has different underlying pathology compared to boys with congenital tumors being more common in girls. A high index of suspicion is essential for early recognition and management.

The Authors report a case of congenital megacystis without other anomalies, diagnosed in a 27GW female fetus. At prenatal counselling the diagnostic hypothesis formulated were obstructive uropathy (cloacal malformation, uro-genital sinus, urethral atresia), functional alteration (neurogenic bladder, primitive myopathies) or Prune Belly Syndrome (rare in females). The outcome following treatment suppose a visceral myopathy, for this condition, however definitive etiology is obscure.

A 38-year-old female with a history of psychiatric illness presents with irritative urological symptoms and is diagnosed as having a large congenital vesical diverticulum (Hutch diverticulum). An overview of the aetiology, classification and complications of diverticula together with indications for and management of bladder diverticula is discussed. This is the first documented case of a very large symptomatic congenital diverticulum in a female presenting in the third decade of life and emphasizes the importance of ultrasound in irritative urological symptoms resistant to medication.

Purpose: Congenital giant diverticulum of the bladder is a consequence of deficiency in the detrusor musculature and has been reported in male infants as a rare cause of bladder outlet obstruction., Materials and Methods: A 10-year retrospective review revealed 4 patients (3 boys and 1 girl) with bladder outlet obstruction due to a giant bladder diverticulum. Prenatal and postnatal clinical and imaging records were reviewed., Results: Prenatal sonography was unremarkable in all patients. Three males (ages 4 months, 10 months and 3 years) had no medical history of voiding dysfunction, and 1 female (11 years) had the Ehlers-Danlos syndrome. While the girl presented with urinary tract infection, all patients presented with progressively decreasing urinary stream and urinary retention. Each patient underwent voiding cystourethrography (VCUG) and ultrasound. In each patient VCUG showed a giant (greater than 7 cm) bladder diverticulum that descended below the bladder neck and compressed the urethra during voiding. Vesicoureteral reflux was seen in 2 patients. Ultrasonography demonstrated moderate unilateral hydronephrosis in 2 patients. Bladder diverticulectomy was successfully performed in all patients, with ureteral reimplantation in 3., Conclusions: A giant congenital bladder diverticulum, when noted on VCUG to descend below the bladder neck, may lead to bladder outlet obstruction. To our knowledge we report the first case of a female presenting with bladder outlet obstruction due to a giant bladder diverticulum. Children with connective tissue disorders may be predisposed to this disorder, which must be excluded, regardless of gender, in all patients presenting with voiding abnormalities. Surgical diverticulectomy, often with ureteral reimplantation, is the preferred treatment, with excellent long-term results.

Posterior urethral valve is a condition that leads to characteristic changes in the bladder and upper tracts. The bladder develops hypertrophic changes including muscular hypertrophy, dilatation of the prostatic urethra (keyhole appearance), and progressive hydroureteronephrosis. The voiding cystourethrogram confirms the diagnosis and documents vesicoureteral reflux and accompanying bladder changes. The follow-up of the serum creatinine level is a parameter for renal recovery. In our opinion, primary endoscopic ablation of the valves followed by a wait-and-see attitude is the most efficacious management of posterior urethral valves. The development of the bladder function is controlled by ultrasound and voiding cystourethrogram. Urodynamics provide a formal and objective means of assessing bladder function, but should be carefully applied in infants. Valve ablation in a neonate with significant reflux and a markedly trabeculated bladder can remodel itself remarkably within the 1st year of life. The persistence of hydronephrosis, bladder wall thickening and trabeculation, and persistent elevation of serum creatinine can all be harbingers that a degree of bladder outlet obstruction persists and one needs to rule out a persistent anatomic obstruction. At what point a functional obstruction occurs and which management is reasonable are still issues of debate and require the vigilance of a pediatric urologist to sort out. Dysfunctions of the bladder such as hyperreflexia, hypertonic, small capacity bladder, sphincter incompetence and/or myogenic failure should be adequately treated.

Prenatal sonographic diagnosis of fetal obstructive uropathy is relatively common. However, the complication of spontaneous rupture of the obstructed bladder in utero causing fetal urinary ascites is extremely rare. This case report describes an unusual case of congenital bladder perforation and urinary ascites diagnosed in utero. The pertinent literature is reviewed., (Copyright 2003 S. Karger AG, Basel)

The differential diagnosis for antenatal bladder dilation and hydronephrosis is extensive, ranging from posterior urethral valves to neurogenic bladder. We present a case of a female fetus diagnosed antenatally with severe megacystis and bilateral hydroureteronephrosis. The patient was found to have megacystis-microcolon-intestinal hypoperistalsis syndrome, a rare, congenital, and generally fatal disorder in which there is functional obstruction of the bladder and intestine.