It's Not Cyclic Vomiting Syndrome Until Dietl's is Ruled Out: A Case for Point of Care Renal Ultrasound.

BACKGROUND: Abdominal pain and vomiting are frequent complaints for pediatric patients presenting to the Emergency Department (ED). When a diagnosis such as chronic abdominal pain, cyclic vomiting, or abdominal migraine has previously been made, it can lead to diagnostic momentum and confirmation bias on behalf of the treating physician. Dietl's crisis is a commonly missed diagnosis in the pediatric population that presents with intermittent episodes of pain and vomiting. It can be readily diagnosed at the bedside by the emergency physician (EP) through the employment of point of care ultrasound (POCUS). CASE SERIES: We present two cases of pediatric patients with episodic abdominal pain and vomiting who were previously diagnosed with cyclic vomiting syndrome. In both cases, pediatric gastroenterology evaluations had occurred with negative diagnostic testing having been performed. Both patients also presented to their primary pediatrician and the ED multiple times with each encounter resulting in treatment of symptoms and discharge. Each patient eventually presented to the ED when an EP was present who performed a renal POCUS. In each patient, the POCUS revealed severe unilateral hydronephrosis. Subsequent workup confirmed the diagnosis of Dietl's crisis as the etiology of symptoms. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: In pediatric patients, EPs should be wary of the diagnosis of cyclic vomiting syndrome until ureteral obstruction has been ruled out. A bedside POCUS can rapidly establish this diagnosis and potentially preclude recurrent health care visits, unnecessary diagnostic testing, and permanent loss of renal function.

Incidental discovery of unilateral hydronephrosis unveiling psoas major desmoid-type fibromatosis in a 24-year-old male: A case report with a 5-year follow-up.

RATIONALE: Desmoid-type fibromatosis (DTF), also known as aggressive fibromatosis, is a rare neoplasm originating from the fascial or musculoaponeurotic tissues. While benign and characterized by slow growth, it exhibits local aggressiveness and lacks specific clinical characteristics. However, in a considerable percentage of patients, it could be asymptomatic and discovered by accident during routine clinical examinations. Only a few cases of DTF arising from the psoas major muscle have been reported in the medical literature. PATIENT CONCERNS: A 24-year-old male, asymptomatic and without significant personal or family medical history, was diagnosed with grade 2 hydronephrosis by abdominal ultrasonography during a routine physical examination. This diagnosis was made 15 days after undergoing uncomplicated open-heart surgery to repair an atrial septal defect. DIAGNOSIS: Intravenous pyelogram revealed hydronephrosis with dilation of the pelvicalyceal system. Ureteroscopy ruled out any intrinsic lesions of the ureter. Contrast-enhanced computed tomography identified a 3.5 × 2 × 5.2 cm mass in the retroperitoneum, closely associated with the psoas muscle and enveloping the ureter adjacent to the iliac artery. Postoperative pathological analysis confirmed a definitive diagnosis of sporadic DTF. INTERVENTIONS: The patient underwent exploratory abdominal surgery, during which the tumor was resected without any intraoperative complications. RESULTS: After close monitoring over a 5-year follow-up period, which included periodic physical examinations, magnetic resonance imaging, and ultrasonography, no local recurrence was detected. LESSONS: Achieving an accurate preoperative diagnosis presents a challenge in cases involving retroperitoneal tumors originating from the psoas major muscle and encasing the ureter. However, the insertion of a double J stent is deemed a crucial step in the surgical process, facilitating the dissection and isolation of the ureter from the tumor while preserving kidney function.

[A young woman with hydronephrosis and ureteritis cystica]. / Een jonge vrouw met hydronefrose.

A 38-year-old woman with urosepsis and persistent unilateral hydronephrosis after antibiotic treatment. Antegrade pyelogram shows urine flow obstruction to the bladder. The whole ureter shows multiple small smooth-walled round lucent filling defects projecting into the lumen. The diagnosis ureteritis cystica was made.

The conundrum of high-grade hydronephrosis with non-obstructive drainage on diuretic renography.

BACKGROUND: Patients with high grade hydronephrosis (HN) and non-obstructive drainage on mercaptoacetyltriglycine (MAG-3) diuretic renography (renal scans) can pose a dilemma for clinicians. Some patients may progress and require pyeloplasty; however, more clarity is needed on outcomes among these patients. OBJECTIVE: Our primary objective was to predict which patients with high-grade HN and non-obstructive renal scan, (defined as T ½ time <20 min) would experience resolution of HN. Our secondary objective was to determine predictors for surgical intervention. STUDY DESIGN: Patients with prenatally detected HN were prospectively enrolled from 7 centers from 2007 to 2022. Included patients had a renal scan with T ½<20 min and Society for Fetal Urology (SFU) grade 3 or 4 at last ultrasound (RBUS) prior to renal scan. Primary outcome was resolution of HN defined as SFU grade 1 and anterior posterior diameter of the renal pelvis (APD) < 10 mm on follow-up RBUS. Secondary outcome was pyeloplasty, comparing patients undergoing pyeloplasty with patients followed with serial imaging without resolution. Multivariable logistic regression was used for analysis. RESULTS: Of the total 2228 patients, 1311 had isolated HN, 338 patients had a renal scan and 129 met inclusion criteria. Median age at renal scan was 3.1 months, 77% were male and median follow-up was 35 months (IQR 20-49). We found that 22% (29/129) resolved, 42% of patients had pyeloplasty (54/129) and 36% had persistent HN that required follow-up (46/129). Univariate predictors of resolution were age≥3 months at time of renal scan (p = 0.05), T ½ time≤5 min (p = 0.09), SFU grade 3 (p = 0.0009), and APD<20 mm (p = 0.005). Upon multivariable analysis, SFU grade 3 (OR = 4.14, 95% CI: 1.30-13.4, p = 0.02) and APD<20 mm (OR = 6.62, 95% CI: 1.41-31.0, p = 0.02) were significant predictors of resolution. In the analysis of decision for pyeloplasty, SFU grade 4 (OR = 2.40, 95% CI: 1.01-5.71, p = 0.04) and T ½ time on subsequent renal scan of ≥20 min (OR = 5.14, 95% CI: 1.54-17.1, p = 0.008) were the significant predictors. CONCLUSIONS: Patients with high grade HN and reassuring renal scan can pose a significant challenge to clinical management. Our results help identify a specific candidate for observation with little risk for progression: the patient with SFU grade 3, APD under 20 mm, T ½ of 5 min or less who was 3 months or older at the time of renal scan. However, many patients may progress to surgery or do not fully resolve and require continued follow-up.

Utility of F-15 diuretic MAG3 renography in assessment of paediatric hydronephrosis.

INTRODUCTION: Diuretic renography is crucial in evaluation of paediatric hydronephrosis. Furosemide is conventionally given 15-20 min after radiolabelled tracer (F+15/F+20 protocol), however this is equivocal in around 15% of patients. Giving furosemide 15 min prior to tracer (F-15 MAG3 protocol) has been suggested as an additional tool in the investigation of patients with suspected upper urinary tract obstruction. However, the role of this method in assessment and management of paediatric hydronephrosis is not widely reported. OBJECTIVE: To investigate utility of F-15 renograms in children with hydronephrosis being assessed for Pelvi-Ureteric Junction Obstruction (PUJO). STUDY DESIGN: Retrospective review of patients <16 years old undergoing F-15 MAG3 renogram between 2018 and 2021 in our tertiary paediatric surgical centre. Data collected included patient demographics, mode of presentation, investigations, management and outcomes. RESULTS: Eighteen patients were included. Median age at F-15 renogram was 7.3 years. Eleven patients presented with antenatal hydronephrosis, 5 with symptoms in childhood and 2 with incidental hydronephrosis on trauma imaging. Fourteen patients were symptomatic. Ten had a prior non-obstructed F+20 renogram but persisting symptoms suggestive of PUJO. Seven had previous equivocal F+20 renograms. One symptomatic patient directly underwent an F-15 renogram. A conclusive result was obtained in 16/18 (89%); 11 patients had obstructed curves and 5 non-obstructed. Two asymptomatic patients' scans were inconclusive. All symptomatic patients had conclusive scans. Of 11 patients with an obstructed F-15, 9 have undergone pyeloplasty to date. All have had post-operative resolution in symptoms and static or improved post-operative ultrasound. One patient with an inconclusive scan underwent pyeloplasty due to persisting hydronephrosis and parent preference. Three patients with non-obstructed F-15 renograms have been discharged. One symptomatic patient with a non-obstructive F-15 had a ureteric stent inserted due to persistent flank pain; 1 continues under surveillance. DISCUSSION: It is known that conventional F+20 MAG3 renograms can give equivocal results. Published experience suggests that F-15 renograms are conclusive in the majority of patients. Routine primary use is, however, discouraged as they can 'over diagnose' obstruction and limit the study of tracer transit under physiological flow rates. This study indicates that the F-15 renogram is a useful adjunct in the assessment of patients with symptoms suggestive of PUJO who have previously had an equivocal or a non-obstructed F+20 renogram. CONCLUSION: F-15 renogram was conclusive in 89% of patients. We recommend using F-15 renograms to aid surgical decision-making in children with equivocal F+20 renograms, especially in the presence of symptoms.

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.

Application of STREAM-URO and APPRAISE-AI reporting standards for artificial intelligence studies in pediatric urology: A case example with pediatric hydronephrosis.

INTRODUCTION: Artificial intelligence (AI) and machine learning (ML) in pediatric urology is gaining increased popularity and credibility. However, the literature lacks standardization in reporting and there are areas for methodological improvement, which incurs difficulty in comparison between studies and may ultimately hurt clinical implementation of these models. The "STandardized REporting of Applications of Machine learning in UROlogy" (STREAM-URO) framework provides methodological instructions to improve transparent reporting in urology and APPRAISE-AI in a critical appraisal tool which provides quantitative measures for the quality of AI studies. The adoption of these will allow urologists and developers to ensure consistency in reporting, improve comparison, develop better models, and hopefully inspire clinical translation. METHODS: In this article, we have applied STREAM-URO framework and APPRAISE-AI tool to the pediatric hydronephrosis literature. By doing this, we aim to describe best practices on ML reporting in urology with STREAM-URO and provide readers with a critical appraisal tool for ML quality with APPRAISE-AI. By applying these to the pediatric hydronephrosis literature, we provide some tutorial for other readers to employ these in developing and appraising ML models. We also present itemized recommendations for adequate reporting, and critically appraise the quality of ML in pediatric hydronephrosis insofar. We provide examples of strong reporting and highlight areas for improvement. RESULTS: There were 8 ML models applied to pediatric hydronephrosis. The 26-item STREAM-URO framework is provided in Appendix A and 24-item APPRAISE-AI tool is provided in Appendix B. Across the 8 studies, the median compliance with STREAM-URO was 67 % and overall study quality was moderate. The highest scoring APPRAISE-AI domains in pediatric hydronephrosis were clinical relevance and reporting quality, while the worst were methodological conduct, robustness of results, and reproducibility. CONCLUSIONS: If properly conducted and reported, ML has the potential to impact the care we provide to patients in pediatric urology. While AI is exciting, the paucity of strong evidence limits our ability to translate models to practice. The first step toward this goal is adequate reporting and ensuring high quality models, and STREAM-URO and APPRAISE-AI can facilitate better reporting and critical appraisal, respectively.

Long-term follow-up of congenital hydronephrosis in a single-center study.

OBJECTIVES: Many congenital hydronephroses spontaneously resolve. This study evaluated a long-term follow-up of more than 4 years of patients with congenital hydronephrosis at a single center. METHODS: In total, 215 patients (286 kidneys) with congenital hydronephrosis were included. Hydronephrosis outcomes (resolution, improvement, and persistence) and time-to-outcome were evaluated. RESULTS: Fourteen patients underwent early surgical intervention until the age of 2 years. A total of 189 congenital hydronephrosis cases (66%) showed resolution at a median of 16 months (interquartile range: 7-21 months) and 169 (80%) of 210 kidneys with grade I to II hydronephrosis showed resolution at a median of 14 months (interquartile range: 6-23 months). Of 76 kidneys with grade III to IV hydronephrosis, 24 (32%) showed resolution at a median of 29 months (interquartile range: 24-41 months), and 56 (74%) showed improvement to grade II or less at a median of 12 months (interquartile range: 5-23 months). Of the 76 kidneys with grade III to IV hydronephrosis, five required delayed pyeloplasty at a median of 66 months (interquartile range: 42-89 months). One patient was asymptomatic, with a marked worsening of hydronephrosis and decreased renal function 6 years after the resolution of hydronephrosis. CONCLUSIONS: None of the patients with grade I to II hydronephrosis required surgical treatment, and a shorter follow-up may be sufficient. Grade III to IV severe hydronephrosis should be considered for a longer and more careful follow-up, given the possibility of asymptomatic exacerbation of hydronephrosis.

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).