Primary non-refluxing megaureter: Natural history, follow-up and treatment.

Primary non-refluxing megaureter (PMU) is a congenital dilation of the ureter which is not related to vesicoureteral reflux, duplicated collecting systems, ureterocele, ectopic ureter, or posterior urethral valves and accounts for 5 to 10% of all prenatal hydronephrosis (HN) cases. The etiology is a dysfunction or stenosis of the distal ureter. Most often PMU remains asymptomatic with spontaneous resolution allowing for non-operative management. Nevertheless, in selective cases such as the development of febrile urinary tract infections, worsening of the ureteral dilatation, or reduction in relative renal function, surgery should be considered. CONCLUSION: Ureteral replantation with excision of the dysfunctional ureteral segment and often ureteral tapering is the gold-standard procedure for PMU, although endoscopic treatment has been shown to have a fair success rate in many studies. In this review, we discuss the natural history, follow-up, and treatment of PMU. WHAT IS KNOWN: • PMU is the result of an atonic or stenotic segment of the distal ureter, resulting in congenital dilation of the ureter, and is frequently diagnosed on routine antenatal ultrasound. WHAT IS NEW: • Most often, PMU remains asymptomatic and clinically stable, allowing for non-operative management. • Nevertheless, since symptoms can appear even after years of observation, long-term ultrasound follow-up is recommended, even up to young adulthood, if hydroureteronephrosis persists. • Ureteral replantation is the gold standard in case surgery is needed. In selected cases, however, HPBD could be a reasonable alternative.

Congenital Giant Megaureter in a 16-Year-Old Female Presenting With Abdominal Pain: A Case Report and Review of Literature.

We describe a case of congenital giant megaureter in a 16-year-old female. She presented with a 5-day history of abdominal distention, right flank pain and tenderness. Right pyelonephritis was suspected. Computerized tomography showed a large cystic abdominal mass with no appreciably functioning left kidney causing secondary compression of the contralateral right ureter. A left upper nephroureterectomy was performed, draining over 3.5 L of fluid. Our experience suggests that congenital giant megaureter should be considered in the differential for pediatric patients presenting with a cystic abdominal mass.

Congenital midureteral stenosis in children: a 13-year retrospective study based on data from a large pediatric medical center.

BACKGROUND: Midureteral stenosis is very rare in children and can cause congenital hydronephrosis. We report our experience treating children with congenital midureteral stenosis at our center, focusing on the differences in preoperative diagnosis and treatment compared with other congenital obstructive uropathies. METHODS: We retrospectively reviewed the medical records of 26 children diagnosed with congenital midureteral stenosis at our center between January 2007 and December 2020, such as preoperative examination methods, intraoperative conditions, and postoperative follow-up results. RESULTS: Of the 1625 children treated surgically for ureteral narrowing, only 26 (1.6%) were diagnosed with midureteral stenosis, including 15 infants and 11 children. Eighteen (69.2%) were boys, 13 (50%) were affected on the left side, and 23 (88.5%) had isolated ureteral stenosis. Overall, 13 (50%) of the children presented with prenatal hydronephrosis, and 13 (50%) presented with abdominal pain or a mass. All the children had undergone urinary ultrasound and intravenous urography preoperatively; the diagnostic rate of ultrasound was 92.3%. Only 7 (26.9%) children had undergone pyelography. All the children had undergone surgery. The ureteral stenotic segment was less than 1 cm long in 25 (96.2)% of the children. The mean follow-up duration was 22 months (range: 6-50 months). One child developed anastomotic strictures. Urinary tract obstruction was relieved in the other children without long-term complications. CONCLUSIONS: Congenital midureteral stenosis is rare, accounting for 1.6% of all ureteral obstructions, and its diagnosis is crucial. Urinary ultrasound has a high diagnostic rate and should be the first choice for midureteral stenosis. Retrograde pyelography can be used when the diagnosis is difficult, but routine retrograde pyelography is not recommended. Congenital ureteral stenosis has a relatively short lesion range, largely within 1 cm. The treatment is mainly resection of the stenotic segment and end-to-end ureteral anastomosis, with a good prognosis.

Bilateral Ureteropelvic Junction Disruptions in a 17-Year-Old With Bilateral Ureteral Duplications: Impact of Unrecognized Duplications and Unique Management of Nephrocutaneous Fistula.

A case of bilateral ureteropelvic junction (UPJ) disruptions in a patient with bilateral duplication anomalies is presented. A UPJ disruption with a duplication anomaly has not been reported let alone bilateral duplication anomalies. The unrecognized duplication anomalies led to isolated and obstructed upper pole segments that were managed by bilateral heminephrectomies. A nephrocutaneous fistula developed after one of the heminephrectomies that was successfully managed using a method of percutaneous fulguration and fibrin sealant. In UPJ disruption cases, the possibility of a duplicated collecting system should be considered as an unrecognized duplication may complicate management and prolong recovery.

Ureteral Sextuplication With a Blind-ending Ureter Originating From the Upper Pole in a Boy: A Case Report.

We experienced an extremely rare case of ureteral sextuplication with a blind-ending ureter originating from the upper pole. The patient had 6 separate ureters and 6 renal pelvises with 1 ureteral orifice on the left. The left kidney was hypoplastic and its differential function was 6.5%. The patient underwent definitive surgical treatment to repair the anomaly (ureteroureterostomy and reimplantation of the formed ureter). The surgery was successful and the postoperative course was uneventful. We herein report the first such case in the English-language literature and discuss the etiology of this ureteral anomaly, the method of diagnostic imaging, and the treatment.

Delayed onset of smooth muscle cell differentiation leads to hydroureter formation in mice with conditional loss of the zinc finger transcription factor gene Gata2 in the ureteric mesenchyme.

The establishment of the peristaltic machinery of the ureter is precisely controlled to cope with the onset of urine production in the fetal kidney. Retinoic acid (RA) has been identified as a signal that maintains the mesenchymal progenitors of the contractile smooth muscle cells (SMCs), while WNTs, SHH, and BMP4 induce their differentiation. How the activity of the underlying signalling pathways is controlled in time, space, and quantity to activate coordinately the SMC programme is poorly understood. Here, we provide evidence that the Zn-finger transcription factor GATA2 is involved in this crosstalk. In mice, Gata2 is expressed in the undifferentiated ureteric mesenchyme under control of RA signalling. Conditional deletion of Gata2 by a Tbx18cre driver results in hydroureter formation at birth, associated with a loss of differentiated SMCs. Analysis at earlier stages and in explant cultures revealed that SMC differentiation is not abrogated but delayed and that dilated ureters can partially regain peristaltic activity when relieved of urine pressure. Molecular analysis identified increased RA signalling as one factor contributing to the delay in SMC differentiation, possibly caused by reduced direct transcriptional activation of Cyp26a1, which encodes an RA-degrading enzyme. Our study identified GATA2 as a feedback inhibitor of RA signalling important for precise onset of ureteric SMC differentiation, and suggests that in a subset of cases of human congenital ureter dilatations, temporary relief of urine pressure may ameliorate the differentiation status of the SMC coat. © 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

Bladder agenesis, ectopic ureters and a multicystic dysplastic horseshoe kidney in one twin newborn with normal amniotic fluid index in utero.

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.

Boari flap reconstruction in a male infant with solitary kidney and associated megaureter.

A 1-year-old male infant presented with fever and abdominal lump for 3 months with increased leucocyte count (15 300/mm3) and serum creatinine (0.83 mg%). Abdominal ultrasound and renal scan demonstrated solitary left kidney with dilated tortuous left ureter. Voiding cystourethrogram was unequivocal. Left percutaneous nephrostomy was placed after poor response to perurethral catheterisation. His serum creatinine dropped to 0.58 mg/dL. Subsequent percutaneous nephrostogram and CT nephrostogram showed dilated left pelvicalyceal system, dilated, tortuous left ureter. A diagnosis of obstructed megaureter was made and ureteric plication and reimplantation planned. Intraoperatively, there were primitive ureteral valves until proximal one-third of the ureter. The distance between the upper ureter and bladder was ∼6 cm. This defect was bridged by Boari flap. The postoperative period was uneventful and now after 6 months of follow-up, he is doing fine.

Laparoscopic Intravesical Detrusorrhaphy With Ureteral Plication for Megaureter: A Novel Technique.

INTRODUCTION: To demonstrate the novel technique of laparoscopic intravesical detrusorrhaphy with ureteral plication for megaureter. TECHNICAL CONSIDERATIONS: From December 2012 to June 2014, 11 patients with primary unilateral megaureter underwent laparoscopic intravesical detrusorrhaphy with ureteral plication. By a transvesicoscopic approach using a 3-port technique, the ureter was mobilized and plicated with a 6F Foley catheter. After dissection of the bladder mucosa in a semilunar shape, the detrusor was incised vertically and repaired underneath the mobilized ureter to create a submucosal tunnel. The ureter was covered with bladder mucosa, and the ureteral orifice was anastomosed to its orthotopic neoureteric orifice. The mean age of the included patients at the time of surgery was 9.18 years (range, 12 months-24 years). All surgeries were successfully completed without open conversion, with a mean operation time of 214 minutes including preoperative evaluation. All patients tolerated the procedure without any complications. Within the mean follow-up duration of 12.6 months (range, 5-24 months), no complications related to the surgery were reported, and no vesicoureteral reflux was detected in the operated side on postoperative voiding cystourethrography. The distal ureteral diameter was significantly reduced (from 21.1 to 11.6 mm), and the grade of hydronephrosis was decreased on postoperative ultrasonography without deterioration of renal function. CONCLUSION: In this study, laparoscopic intravesical detrusorrhaphy with ureteral plication demonstrated successful short-term outcomes in management of megaureter in children and adults. Our technique can be an effective and feasible surgical option in the treatment of megaureter.

Transcaval Ureter: Multidetector Computed Tomography Demonstration.

Transcaval ureter is a rare congenital anomaly characterized by an inferior cava vein duplication producing a vascular ring around the right ureter, usually determining hydroureteronephrosis. The knowledge of this vascular anomaly on imaging examinations permits to avoid erroneous diagnosis of retroperitoneal masses or adenopathy and preoperatively advise the surgeon of potential sources of complications. We describe a case of transcaval ureter studied with multidetector computed tomography. To our knowledge, this is the first case in which computed tomography multiplanar and volume-rendering reconstructions show this rare anomaly.

Diagnostic work-up and laparoscopic correction of an ectopic ureter.

INTRODUCTION: A duplex renal collecting system is a common congenital anomaly in children. Continuous dribbling (especially if after the toilet-training period) should raise suspicion of the presence of an ectopic ureter, which is most often associated with ureteral duplication. This video will demonstrate the complete diagnostic work-up necessary in these cases. CASE REPORT: A 10-year-old girl presented with continuous dribbling. Ultrasonography and computerized tomography depicted a duplex system on the left side, with the upper pole ureter ectopically inserting into the vaginal cavity and good upper pole renal parenchyma. A careful urethrocystoscopy showed a topic right ureteral orifice and a topic lower pole left ureteral orifice. Retrograde pyelography was performed and displayed normal left lower pole anatomy. A vaginography was performed, which showed reflux to the ectopic ureter. Vaginoscopy clearly identified the ectopic ureteral orifice. A guide wire was introduced through this meatus and retrograde contrast injection confirmed the diagnosis of an ectopic ureter. RESULTS: At laparoscopy, a larger upper pole ureter and a normal lower pole ureter on the left side were identified. A termino-lateral ureteroureteral anastomosis was performed. After the procedure, the child reported immediate resolution of urinary dribbling. CONCLUSION: In order to optimize its surgical correction, efforts should be made to appropriate localization of the ectopic ureter.

[Laparoscopic and open heminephrectomy in children: results comparison].

UNLABELLED: Objectives. To compare the results of laparoscopic and open heminephrectomy for kidneys duplication in children. Materials and methods. 42 patients underwent laparoscopic heminephrectomy and 20 patients underwent open heminephrectomy for non-functioning kidney moiety. Transperitoneal access with 3-4 trocars was used in the first group. Retrospective analysis of demographic, intra- and postoperative parameters, developed complications was performed. RESULTS: The mean operative duration was 127 minutes (90-205) and 120 minutes (100-219) in laparoscopic and open surgery groups, respectively. There were not intraoperative complications and need for conversion in laparoscopic group. Two children required additional surgery for perirenal cyst and ureteric stump inflammation. Hemotransfusion after open surgery was performed in 2 cases. Complications included pleural sine laceration and complete function loss of remaining moiety. The mean hospital stay was 7.2 days (5-12) and 11.5 days (8-17) in laparoscopic and open surgery groups, respectively. CONCLUSIONS: Laparoscopic transperitoneal heminephrectomy can be successfully used in children. Duration of laparoscopic and open interventions is comparable. But hospital stay is less in case of laparoscopic interventions.

A bladder stone within a congenital paraureteral diverticulum: a case report.

Paraureteral diverticula are rare in pediatric population. We here present a bladder stone in the congenital paraureteral diverticulum presenting with vesicoureteral reflux. To the best of our knowledge, stone formation in paraureteral diverticulum has not been reported previously. A 5-year-old boy was admitted with the complaint of dysuria. Abdominal ultrasonography revealed a bladder stone in a diverticulum arising from right posterolateral wall and a small right kidney. Voiding cystourethrogram confirmed large bladder diverticulum with vesicoureteral reflux. Cystoscopy revealed a giant stone in the paraureteral diverticulum. The stone was removed with open diverticulectomy and right Cohen ureteroneocystostomy was performed.

Laparoscopic ureteral ligation (clipping): a novel, simple procedure for pediatric urinary incontinence due to ectopic ureters associated with non-functioning upper pole renal moieties.

OBJECTIVE: A simplified approach for the surgical management of symptomatic ectopic ureters, associated with a non-functioning upper moiety, with laparoscopic ureteric clipping is presented in this research paper. MATERIALS AND METHODS: Prospectively collected data on nine consecutive girls with ectopic ureters associated with urinary incontinence who underwent laparoscopic clipping between February 2011 and December 2013. Surgical technique consisted of cystoscopy and insertion of ureteral catheter in the lower pole ureter to aid in identification and clipping of the ectopic ureter, which was achieved by standard trans-peritoneal laparoscopy. RESULTS: Median age was eight years (range 4-17 years). Diagnosis was based on clinical findings, which were supported by: ultrasound (US), nuclear scans and magnetic resonance urography in Cases 9, 8 and 5, respectively. Bilateral complete duplication was present in two patients; the combination of cystoscopy and laparoscopy allowed adequate identification of the ectopic ureter causing incontinence in both. All nine patients were immediately dry after surgery and remain asymptomatic after a maximum follow up of 27 months. Eight out of nine patients had developed some degree of asymptomatic upper pole hydronephrosis on follow-up US. CONCLUSION: Laparoscopic clipping holds promise as a simple alternative to other more-complex surgical procedures in the treatment of incontinence due to an ectopic ureter. Despite favorable and encouraging initial results, further follow up is warranted in order to determine the fate of expected associated upper-pole hydronephrosis.

An unusual presentation of bilateral Hutch diverticula.

Summary Hutch diverticula are rare congenital diverticula. The general consensus is that they occur secondary to a congenital failure of normal muscle development around the ureteral orifice where Waldeyer sheath anatomically covers the space between the intravesical ureter and muscular layer of the bladder. Our case highlights the radiological appearance of Hutch diverticula and the need for contrast-enhanced imaging to enable accurate evaluation and diagnosis. It illustrates how the presence of periureteric diverticula can contribute to ureteric obstruction and calculus formation; complicate the interpretation of renal tract imaging and the subsequent management of renal calculi.

Diagnosis and management of urinary ectopia.

Ectopic ureters are the most common cause of urinary incontinence in young dogs but should be considered as a differential in any incontinent dog for which the history is not known. Ectopic ureters can be diagnosed with excretory urography, fluoroscopic urethrography or ureterography, abdominal ultrasonography, cystoscopy, helical computed tomography, or a combination of these diagnostic procedures. Other congenital abnormalities can also occur in dogs with ectopic ureters, including renal agenesis or dysplasia, hydronephrosis, and/or hydroureter and vestibulovaginal anomalies; therefore, the entire urinary system must be evaluated with ultrasonography if cystoscopy is the only other diagnostic tool used before surgery. Novel surgical techniques and adjunctive medical management have improved the prognosis for dogs with urinary ectopia.

Detection of recurrent transmission of 17q12 microdeletion by array comparative genomic hybridization in a fetus with prenatally diagnosed hydronephrosis, hydroureter, and multicystic kidney, and variable clinical spectrum in the family.

OBJECTIVE: This study was aimed at detection of recurrent transmission of the 17q12 microdeletion in a fetus with congenital anomalies of the kidney and urinary tract. MATERIALS AND METHODS: A 35-year-old woman was referred to the hospital at 20 weeks' gestation because of hydronephrosis in the fetus. The mother was normal and healthy. Her second child was a girl who had bilateral dysplastic kidneys that required hemodialysis, and died at the age of 5 years. During this pregnancy, the woman underwent amniocentesis at 18 weeks' gestation because of advanced maternal age. Cytogenetic analysis revealed a karyotype of 46,XY. Prenatal ultrasound showed left hydronephrosis with a tortuous ureter, right hydronephrosis, and increased echogenicity of the kidneys. Fetal magnetic resonance imaging showed right dilated renal calyces, left hydronephrosis, hydroureter, and multicystic kidney. The pregnancy was subsequently terminated. Array comparative genomic hybridization (aCGH) and fluorescence in situ hybridization were applied for genetic analysis using umbilical cord, maternal blood, and cultured amniocytes. RESULTS: aCGH analysis on umbilical cord detected a 1.75-Mb deletion at 17q12 including haploinsufficiency of LHX1 and HNF1B. aCGH analysis on maternal blood detected a 1.54-Mb deletion at 17q12 including haploinsufficiency of LHX1 and HNF1B. Metaphase fluorescence in situ hybridization analysis on cultured amniocytes and maternal blood lymphocytes using 17q12-specific bacterial artificial chromosome probe showed 17q12 microdeletion in the fetus and the mother. CONCLUSION: Prenatal diagnosis of recurrent renal and urinary tract abnormalities in the fetus should include a differential diagnosis of familial 17q12 microdeletion.