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.

Intrinsic ureteric obstruction secondary to endometriosis: a rare clinical entity causing obstructive uropathy and renal failure.

Ureteral endometriosis is rare and can be a silent clinical entity, which can potentially lead to serious complications such as obstructive uropathy, sepsis and renal failure. A high clinical suspicion is required especially in childbearing age groups due to non-specific presentation such as renal colic, recurrent urinary tract infection (UTI), renal failure or asymptomatic hydronephrosis.A woman in her 40s presented with febrile UTI and flank pain. She reportedly suffered from recurrent UTIs in the past. Initial workup revealed an infected, obstructed left renal collecting system with gross hydronephrosis and hydroureter to the distal ureter on a significant gynaecological background of severe endometriosis requiring hysterectomy in the past.CT showed chronic obstructive changes and soft tissue nodules within the renal pelvis with no radio-opaque stones. She underwent emergent ureteric stent insertion. Functional imaging demonstrated only 1% contribution of the left kidney with a preserved estimated glomerular filtration rate of 65 mL/min/1.73 m2Endoscopic evaluation of ureters found extensive soft tissue lesions throughout the dilated left collecting system with biopsy-confirmed endometriosis. Subsequently, she underwent laparoscopic nephroureterectomy due to extensive ureteric involvement and chronically obstructed non-functioning kidney. Histopathology demonstrated completely obstructing ureteral endometriosis.Ureteric obstruction secondary to endometriosis can be due to extrinsic or intrinsic disease. In addition to initial assessment with CT urogram MRI may be helpful to evaluate soft tissue thickening. Endoscopic assessment with ureteroscopy and biopsy is required for tissue diagnosis. Surgery is often the treatment of choice, ranging from ureteroureterostomy, ureteroneocystostomy or nephroureterectomy in severe cases.Ureteral endometriosis is a rare clinical entity, clinicians should remain vigilant about common presentations of this rare entity, early diagnosis and prompt treatment is crucial to prevent progression to renal failure.

Routine Ureteral Stenting in Kidney Transplant Reduces Postoperative Hydronephrosis and Percutaneous Ureteral Interventions: A Single-Center Experience.

OBJECTIVES: The effect of routine ureteral stenting on postoperative hydronephrosis and percutaneous ureteral intervention in kidney transplant remains unknown. This study aimed to evaluate the effects of routine ureteral stenting on hydronephrosis and percutaneous ureteral intervention and the cost benefit of ureteral stenting in kidney transplant. MATERIALS AND METHODS: We retrospectively analyzed patients who underwent kidney transplant at a tertiary institution between 2005 and 2021. We adopted a ureteral stentingprotocol in2017, anda comparisonwas performed with previous patients without stents. RESULTS: In total, 539 patients underwent kidney transplant(271 with stents [51.3%], 268 without stents [49.7%]). Hydronephrosis was detected in 16 cases (5.9%) and 30 cases (11.2%) of groups with and without stents,respectively (P = .041). Among patients with hydronephrosis, the number of patients who underwent percutaneous ureteral intervention was significantly lower in the stent group than in the nostent group (1 [6.25%] vs 10 [33.33%]; P= .014).Twenty patients (3.71%) experienced major urologic complications (19 [7.1%] in the no-stent group, and 1 [0.4%] in the stent group; P = .001). No significant differences between the groups were shown in the incidence of urinary tract infections within 3 months of transplant (24 [8.9%] vs 22 [8.2%]; P = .846). No differences were shown between the groups in ureterovesical anastomosis time (24.4 vs 24.03 min; P = .699) or 1-year graft survival (97% vs 97.8%; P = .803). The healthcare cost was significantly lower in the stent group than in the no-stent group by $1702.05 ($15000.89 vs $16702.95; P < .001). CONCLUSIONS: Routine ureteral stenting in kidney transplant significantly decreased the incidence of postoperative hydronephrosis and percutaneous ureteral intervention. Stenting did notlead to increased urinary tract infections and was cost-effective.

Epidemiology of Infantile Ureteropelvic Junction Obstruction in the US.

OBJECTIVE: To describe sex- and diagnosis-specific comorbidities, outcomes, and secular trends associated with ureteropelvic junction obstruction (UPJO) in a large, real-world population diagnosed with hydronephrosis in infancy. MATERIALS AND METHODS: We identified all infants ≤1 year old with ≥1 claim in the Optum Clinformatics 2007-2020 nationwide population database and used univariable and multivariable Cox regression analyses to estimate associations of demographic and clinical characteristics of infants with a UPJO diagnosis with surgical status. RESULTS: Of 22,349 infants with hydronephrosis (1.1% of infants; males-1.4%, females-0.7%), 1722 (7.7%; 7.9%-males, 7.2%-females) had UPJO. Follow-up was ≥1 year in 1198 (70%) and ≥3 years in 555 (32%) cases, and UPJO repair was performed in 542 children (31.5%; 32.3%-males, 29.5%-females); 77.7% within 1 year and 97.3% within 3 years. UPJO repair was associated with prior urinary tract infection (UTI) (hazard ratio (HR) 1.41, 95% confidence interval (CI) 1.12-1.76) and South (HR 1.42, 95% CI 1.14-1.78) or Midwest (HR 1.60, 95% CI 1.26-2.04) geographic region but did not change over time. CONCLUSION: This population-based study provides a real-world view of postnatally diagnosed hydronephrosis, focusing on UPJO, for which 522 cases (∼1/3) had ≥3 years continuous coverage. UPJO-associated comorbidities were more common in females, and the frequencies of UPJO-associated surgery and comorbidities were higher than in other studies. Other than UTI, no other associated kidney or urinary tract diagnoses were associated with UPJO repair. We identified unique sex- and diagnosis-specific differences in associated comorbidities and interventions in children diagnosed with UPJO in the first year of life.

Giant right hydronephrosis with underlying double malignancy: a case report.

Giant hydronephrosis is mostly caused by ureteropelvic junction obstruction (UPJO). Giant hydronephrosis with concurrent malignancy is less common clinically and is easily misdiagnosed. We report a 77-year-old male who presented with sudden onset progressive abdominal distension in the last month. Abdominal computed tomography showed a right severe hydronephrotic kidney with loss of parenchymal thinning. Cystoscopy showed a 1x1cm papillary lesion protruding from the right ureteric orifice. He underwent a right radical nephroureterectomy with bladder cuff excision with lymph node dissection. Histopathology showed low-grade urothelial carcinoma of the ureter and incidental pleomorphic rhabdomyosarcoma in the right kidney. The patient refused chemotherapy and died 6 months later due to lung metastasis. Incidental pathologic finding of renal rhabdomyosarcoma in adults with giant hydronephrosis and urothelial carcinoma is a rare occurrence with diverse clinical presentations, prognoses, and outcomes.

Ureteropelvic Junction Obstruction/Hydronephrosis.

Congenital hydronephrosis can be classified and managed based on the Urinary Tract Dilation consensus scoring system. Ureteropelvic junction obstruction is one of the most common causes of hydronephrosis in the pediatric population. Although most cases can be managed conservatively with follow-up and serial imaging, some patients need surgical repair because of renal function deterioration, infections, or symptoms. Additional research to create predictive algorithms or develop noninvasive biomarkers for renal deterioration is necessary to better identify surgical candidates. The robotic-assisted approach for pyeloplasty is becoming increasingly widespread and associated with shorter hospital stay, high success rates, and low complication rates.

Rapport de cas Suspected congenital unilateral ureteral atresia and hydronephrosis in a 6-year-old dog.

A 6-year-old neutered male giant schnauzer dog was presented to an emergency clinic with stranguria and pollakiuria. On physical examination, the abdomen was generally and non-painfully distended. Diagnostic imaging revealed several large, anechoic, fluid-filled, space-occupying lesions from the cranial to caudal abdomen placing extramural pressure on the bladder and urethra and apparently causing the clinical signs. Unilateral ureteral atresia with secondary ipsilateral hydronephrosis and hydroureter were diagnosed on post-mortem examination. Due to a lack of history of abdominal surgery or trauma and the absence of scarring or stenosis of the ureter, the condition was suspected to be congenital. Key clinical message: Although rare, hydronephrosis and hydroureter secondary to a congenital ureteral defect should be considered when a dog is presented with abdominal distension and several peritoneal and retroperitoneal space-occupying lesions on diagnostic imaging.

Atrésie urétérale unilatérale congénitale suspectée et hydronéphrose chez un chien de 6 ans. Un chien schnauzer géant mâle castré âgé de 6 ans a été présenté à une clinique d'urgence avec une strangurie et une pollakiurie. À l'examen physique, l'abdomen était distendu de manière générale et non-douloureuse. L'imagerie diagnostique a révélé plusieurs grandes lésions anéchoïques, remplies de liquide et occupant de l'espace de l'abdomen crânien à caudal, exerçant une pression extra-murale sur la vessie et l'urètre et causant apparemment les signes cliniques. Une atrésie urétérale unilatérale avec hydronéphrose ipsilatérale secondaire et hydro-uretère ont été diagnostiqués lors de l'autopsie. En raison d'un manque d'antécédents de chirurgie abdominale ou de traumatisme et de l'absence de cicatrices ou de sténose de l'uretère, la condition a été suspectée d'être congénitale.Message clinique clé :Bien que rares, l'hydronéphrose et l'hydro-uretère secondaires à une anomalie urétérale congénitale doivent être envisagées lorsqu'un chien présente une distension abdominale et plusieurs lésions péritonéales et rétropéritonéales occupant de l'espace à l'imagerie diagnostique.(Traduit par Dr Serge Messier).

Giant Hydronephrosis: A Rare Case Report and Literature Review.

Giant hydronephrosis (GH), characterized by the presence of more than 1 L of fluid in the renal collecting system, is a rare urological condition, particularly in adults. Obstruction of the pyeloureteral junction is the most common cause of GH. We report the case of a 51-year-old man who presented with dyspnea, edema of the lower limbs, and major abdominal distension. The patient was diagnosed with obstruction of the pyeloureteral junction, causing a left giant hydronephrotic kidney. After renal drainage of 27 L of urine, a laparoscopic nephrectomy was performed. GH usually manifests as asymptomatic abdominal distension or vague symptoms. However, very few published reports describe cases of GH initially presenting with respiratory and vascular manifestations.

Renal tamponade in a patient with hydronephrosis-related Page kidney.

A 48-year-old woman presented with hyperreninemic hypertension and renal dysfunction and was diagnosed with hydronephrosis-related Page kidney. The pathophysiology was "renal tamponade", in which the kidney was compressed by the renal pelvis and Gerota's fascia, resulting in intrarenal microvascular ischemia. Ureteral stent placement promptly improved the hyperreninemic hypertension and renal dysfunction, and additional perirenal fluid drainage gradually improved these conditions. These observations indicated the following three points. First, renal compression-induced renin-angiotensin-aldosterone system upregulation plays an important role in the pathogenesis of Page kidney. Second, physicians should consider perirenal fluid drainage as a therapeutic option in addition to ureteral stenting in patients with hydronephrosis-related Page kidney. Third, bilateral perirenal subcapsular hematomas in this case could be caused by hydronephrosis. Hydronephrosis-induced intrarenal pressure elevation possibly caused chronic perirenal subcapsular hemorrhage at the vulnerable sites of the renal cortex and peeling of the renal capsule from the cortex, resulting in the bilateral massive subcapsular hematomas and Page kidney.

Can pyeloplasty restore normal renal function in patients with severe unilateral ureteropelvic junction obstruction and DRF < 35.

PURPOSE: To assess the outcome of successful pyeloplasty in infants with Ureteropelvic Junction Obstruction (UPJO) and a differential renal function of (DRF) < 35% whether they can attain normal postoperative renal function or not. METHODS: All children who were diagnosed with antenatal hydronephrosis due to UPJO were presented to our institutions and were prospectively followed up. Pyeloplasty was performed based on predefined indications such as: initial DRF ≤40%, progression of hydronephrosis, and febrile urinary tract infection (UTI). A total of 173 children, who had successful surgical intervention due to impaired DFR, were divided according to their pre-intervention DRF value as follows: DRF <35% (group I) and DRF 35-40% (group II). The renal morphology and function changes were recorded and used for comparison between both groups. RESULTS: Group I was comprised of 79 patients, and group II included 94 patients. Pyeloplasty achieved significant improvement in the anatomical and functional indices in both groups (p-value <0.001). The degree of improvement in Anteroposterior diameter (APD) and cortical thickness was comparable in both groups (P-value, 0.64 and 0.44 respectively). While the improvement in the DRF was significantly higher in group I (16.06 ± 6.6) than in group II (6.25 ± 2.66) (P-value <0.001). Despite that, a significantly higher percentage of infants in group II (61.7%) achieved normal final DRF compared with only (10.1%) in group I (Figure). CONCLUSION: Even in severely impaired renal function (<35%), successful pyeloplasty can recover a significant part of lost renal function. However, most of these patients do not achieve normal postoperative renal function.

Incidental Diagnosis of Renal Pelvic Tumor in Patients Who Underwent Percutaneous Nephrolithotomy: A Report of 6 Cases and Review of the Literature.

OBJECTIVES: To report the clinical characteristics of patients who were incidentally diagnosed as renal pelvic tumor during or after percutaneous nephrolithotomy (PNL). METHODS: The profiles of about 8,000 patients who underwent PNL in our center from 2011 to 2021 were retrospectively reviewed, 6 patients were diagnosed with renal pelvic tumor during or after PNL. Clinical data of these six patients were collected and summarized. RESULTS: Four males and two females with the average age of 59 years were researched. Chief complaints included loin pain, gross hematuria, and wound infection after PNL. Two patients had a definite long history of stones. Urinary tract infection occurred in 5 patients and all six patients had gross hematuria or microscopic hematuria. CT images revealed severe hydronephrosis in 4 patients and moderate hydronephrosis in 2 patients. Both staghorn and multiple stones were separately seen in 3 patients. Five patients were diagnosed with renal pelvic urothelial carcinoma via biopsy during PNL. One patient was diagnosed as renal pelvic tumor with extensive invasion by postoperative CT scan and received palliative treatment. Of two patients underwent radical surgery, one patient obtained long survival, one patient received endoscopic tumor resection and also obtained long survival. Two patients underwent renal artery embolization, one patient was still alive after 22 months, the other patient showed tumor growth in 3 weeks. CONCLUSIONS: The possibility of renal pelvic tumor should be considered in stone patients with several risk factors including elder age, long history of stones, complicated stones, hydronephrosis especially pyonephrosis, obvious hematuria etc.

Bedside Ultrasound in the Emergency Department Enables Rapid Diagnosis of PUJ Obstruction Syndrome.

Presentation A 27-year-old male presented to the Emergency Department with acute severe left flank pain following ingestion of 5 pints of beer. Approximately 20 bouts of similar episodes over the past year, in the setting of alcohol ingestion. Despite attending GP, no diagnosis reached yet. Diagnosis "Pelvo-ureteric junction (PUJ) obstruction Syndrome". Bedside ultrasound in the Emergency Department during the acute pain crisis: massive hydronephrosis left kidney. Finding confirmed on CT scan. Subsequent 99m-Tec renogram showed markedly decreased renal function on the left. Treatment Interval Pyeloplasty two months later. Conclusion Delayed recognition is the norm for PUJ obstruction syndrome, as CT/MRI/US studies often do not display hydronephrosis if the patient is asymptomatic. We could not find any reports in the literature of diagnosing PUJ obstruction syndrome using bedside ultrasound in the Emergency Department. We advise acquiring rapid bedside ultrasound imaging in suspected cases of PUJ obstruction syndrome, enabling earlier diagnosis.

Role of urinary Neutrophil Gelatinase-Associated Lipocalin (NGAL), Monocyte Chemoattractant Protein-1(MCP-1), and Interleukin-6(IL-6) as biomarkers in pediatric patients with hydronephrosis.

OBJECTIVES: The decision to surgically intervene in a hydronephrotic kidney in children is based on many debatable guidelines, some requiring repeated ultrasounds or renal scans. Urinary proteins have the potential to reflect renal disorders and hence can be the alternatives to such scans. Here, we aim to assess the role of urinary Neutrophil Gelatinase-Associated Lipocalin, Monocyte Chemoattractant Protein-1, and Interleukin-6 (IL-6) in such patients. METHODS: Seventeen children had obstructive hydronephrosis requiring pyeloplasty (UPJO), while seven were kept on conservative management in view of non-obstructive dilation (NOD). Urine samples were measured for the three urinary proteins at the time of presentation and following pyeloplasty using commercially available ELISA kits. RESULTS: The levels of all three urinary proteins were significantly higher in patients with UPJO children compared to the NOD group. Cut-off values to differentiate obstructive from non-obstructive hydronephrosis were obtained. A significant fall in the post-operative value of urinary IL-6 was also observed. CONCLUSION: This study highlights the potentiality of urinary proteins as biomarkers in identifying children with hydronephrosis and picking out the ones with obstructive hydronephrosis who will require pyeloplasty. The drop in levels after pyeloplasty can be employed to evaluate the effectiveness of pyeloplasty when sent serially.

Ureteropelvic junction obstructions: Is side a prognostic factor?

Background: Ureteropelvic junction obstructions (UPJOs) occur more frequently on the left than on the right side. Among patients diagnosed during the neonatal period, those with left-side UPJO have a more severe course than those with right-side UPJO. Aim: This study examined clinical advances in the surgical management of right and left symptomatic UPJOs preoperatively and postoperatively, based on a retrospective analysis of cases. Patients and Methods: In this retrospective clinical trial, 650 patients were evaluated at the time of diagnosis and at surgery. Results: Left-side UPJO was diagnosed in 66.1% of patients (P = 0.017). The median age of the patients at surgery for left- and right-side UPJO was 1.5 and 4.2 years, respectively (P = 0.001). At the preoperative evaluation, the ratio of parenchymal thickness (RPT) on the UPJO side versus the contralateral side was 0.55 ± 0.3 and 0.7 ± 0.3 for patients with left-side and right-side UPJO, respectively (P = 0.029). RPT during the first postoperative year was 0.83 ± 0.2 for patients treated on the left side and 0.9 ± 0.3 for those treated on the right side (P = 0.25). The respective values at 3 years postoperatively were 0.8 ± 0.3 and 0.9 ± 0.2 (P = 0.09). The preoperative kidney function value in the left-side group was 42.5 ± 13.4, which declined to 39.52 ± 15.8 at the 3-year follow-up examination. In the right-side group, preoperative kidney function was 38.8 ± 16.1, which increased to 40.2 ± 13.2 at 3 years postoperatively. Both the decline and improvement were significant (both P = 0.006). Conclusions: Those with left-side UPJO had a more severe course than those with right-side UPJO.

Initial observational management of hydronephrosis in infants with reduced differential renal function and non-obstructive drainage parameters.

INTRODUCTION: Hydronephrosis secondary to ureteropelvic junction (UPJ) obstruction is a common finding in infants with prenatally-diagnosed hydronephrosis and often results in pyeloplasty due to obstructive drainage parameters and/or renal function compromise. However, little is known regarding the natural history of hydronephrosis with reduced differential renal function (DRF) but non-obstructive drainage. OBJECTIVE: We sought to explore our experience with initial observational management of these patients. STUDY DESIGN: A retrospective review of our institutional database of all diuretic MAG-3 renal scans obtained between 2000 and 2016 was performed. We included patients with antenatally-detected unilateral hydronephrosis ≥ SFU grade 2, first MAG-3 scan prior to 18months of age, DRF <40% and post-furosemide half-time (T1/2) <20 min. Exclusion criteria were: hydroureter, VUR, solitary kidney, duplication anomalies. Outcomes of interest were a progression of T1/2 ≥ 20 min and/or further decline in DRF >5%. RESULTS: Of 704 patients with unilateral hydronephrosis, 91 had DRF≤40%, of which 29 (18 boys, 11 girls) met our inclusion criteria and were followed for a mean of 2.8 years (1.4 months-6.6 years). Mean age at first sonogram was 2.3 months. 2 patients had SFU grade 2, 16 had grade 3, and 9 had grade 4 hydronephrosis, and 2 unknown grade. Median half-time on initial MAG-3 scan across all patients was 10 min (3-20 min). Initial MAG3 scan was performed at a median of 2.3 months of age (0.3-17 months). 22/29 patients had >1 MAG3 scan. Of the 7 remaining, 5 were lost to follow-up and 2 demonstrated improvement in hydronephrosis. Worsening drainage occurred in 10/22(45%), median final T1/2 was 45.5 min 8 of these underwent pyeloplasty and 2 were lost to follow up. 4/22 patients (18%) had progressive decline in DRF (mean 8.3%, range 6-10%). 3/4 maintained non-obstructive drainage patterns and stable/improved hydronephrosis, and 1 underwent pyeloplasty. 13/18 remaining patients had stable DRF and 5 had improvement in DRF. 7(39%) of these underwent surgery for worsening drainage (Summary Figure). Overall, 7/29(24%) patients had sufficient resolution of hydronephrosis to be discharged from our care, 8(28%) are under continued observation, 9(31%) underwent pyeloplasty, and 5(17%) were lost to follow-up. In the observational group [median follow-up 4.5 years (3.7-6.6 years)], all 8 demonstrated improved non-obstructive drainage (T1/2 <20 minutes) and/or improvement in hydronephrosis. 4/10(40%) with DRF <35% underwent pyeloplasty versus 5/19(26%) with DRF 35-40%(p=0.67). CONCLUSION: Initial observational management of unilateral hydronephrosis with reduced DRF and nonobstructive drainage is recommended as most kidneys maintain nonobstructive drainage and do not demonstrate further decline in DRF. Even when DRF decreases, the majority remain non-obstructive. Worsening drainage over time more often leads to the decision for pyeloplasty rather than change in DRF.