OBJECTIVE: Retroperitoneal fibrosis is a rare disease characterized by chronic inflammation and fibrosis in the retroperitoneal space that may wrap around the ureter and cause an obstruction. Here we present the cases of three patients diagnosed and treated between April and August 2022.
METHODS: Here we present three cases of retroperitoneal fibrosis. Imaging revealed hydronephrosis and/or ureteral dilation, whereas laboratory findings such as erythrocyte sedimentation rate, C-reactive protein, and immunoglobulin G4 were abnormal. Serum creatinine and blood urea nitrogen levels suggested renal injury. Additionally, two patients had unclear diagnoses from other hospitals. Later, all patients underwent ureteral stenting and laparoscopic ureterolysis at our hospital and simultaneous diagnosis by biopsy. The two patients with high immunoglobulin G4 levels exhibited extremely severe fibrosis. After discharge, each received maintenance prednisone to prevent recurrence as well as methotrexate, followed by follow-up every 3 months. Fortunately, serum creatinine, blood urea nitrogen, and intravenous urography findings remained normal with an average follow-up of 8.5 months.
CONCLUSIONS: Here we discovered interesting findings in the diagnosis and treatment of retroperitoneal fibrosis, including the stable effect of medical plus surgical treatment, an unclear ureteral obstruction etiology, and the relationship between immunoglobulin G4 level and fibrosis hardness. However, the mechanisms behind these new findings require further study.

BACKGROUND: Children with higher grades of hydronephrosis often undergo mercaptoacetyltriglycine nuclear renography scans (MAG3) to assess differential renal function (DRF) and drainage. Although MAG3 helps identify the potential need for pyeloplasty, its use incurs increased costs, radiation exposure, and stress for children and families. Several studies demonstrate pyramidal thickness (PT) ≤ 3 mm as a reliable predictive risk factor for pyeloplasty in children with a history of prenatal hydronephrosis. Our hypothesis was that renal sonographic measurements including PT and parenchymal thickness (ParT) correlate with DRF in children with high-grade unilateral hydronephrosis and may be used to better select the need and frequency of MAG3 scans in children at increased risk for diminished relative renal function. The objective of this project was to determine the correlation between sonographic renal measurements and DRF in patients with unilateral hydronephrosis, we assessed: 1) the correlation between PT, ParT, and the ratio of PT/ParT in hydronephrotic kidneys to DRF, 2) the correlation between the ratio of hydronephrotic PT/contralateral non-hydronephrotic PT and DRF, 3) the correlation between the ratio of hydronephrotic ParT/contralateral non-hydronephrotic ParT and DRF, and 4) the correlation between the ratio of (hydronephrotic PT/ParT)/(contralateral non-hydronephrotic PT/ParT) and DRF.
METHODS: We retrospectively reviewed 71 children with grades 3 or 4 unilateral hydronephrosis. Most patients presented with a history of prenatally detected hydronephrosis at median age (IQR) of 112 days (43-274). Measurements of PT and ParT were completed on 98 renal ultrasounds and DRF was collected from corresponding MAG3 scans. Threshold values were identified visually through scatterplots. Spearman\'s correlation coefficient and Fisher\'s p-values were calculated.
CONCLUSIONS: Ratios of PT and ParT in hydronephrotic kidneys to contralateral non-hydronephrotic kidneys were positively correlated with DRF. Ratios of hydronephrotic PT/non-hydronephrotic PT > 0.8 and hydronephrotic ParT/non-hydronephrotic ParT >0.7 occurred more frequently in patients with a DRF >40% (p = 0.11 and p = 0.001, respectively). A PT > 3 mm and ParT >5 mm occurred significantly more frequently in patients with a DRF >40% (p = 0.008 and p = 0.006, respectively).
CONCLUSIONS: Renal sonographic measurements including threshold values of PT > 3 mm, ParT > 5 mm, ratio of hydronephrotic PT/contralateral non-hydronephrotic PT (>0.8), and ratio of hydronephrotic ParT/contralateral non-hydronephrotic ParT (>0.7) are good predictors of DRF >40% in unilateral high-grade hydronephrosis. These identified threshold values have potential utility in determining the need for nuclear renal scans in children with high-grade hydronephrosis.

Zinner Syndrome (ZS) is a rare congenital genitourinary abnormality defined by seminal vesicle cysts, ejaculatory duct obstruction, and unilateral renal dysplasia or agenesis. Patients can be asymptomatic, while others experience pain, urinary or ejaculatory symptoms and infertility. A patient that presented with painless gross hematuria was found to have a large pelvic cystic structure, an absent left kidney, multiple fluid collections in the region of the left seminal vesicle and right hydronephrosis. Hydronephrosis is atypical in ZS. This patient eventually developed right flank and pelvic pain treated with robotic-assisted laparoscopic excision of the pelvic cystic structure and extravesical ureteral reimplantation.

Calyceal rupture, defined as the extravasation of urine from the renal calyces into the perinephric or paranephric spaces, typically results from increased intrapelvic pressure due to urinary tract obstruction. This condition can lead to the formation of a perinephric urinoma and severe complications, such as infection, abscess formation, and impaired renal function. Timely diagnosis and management are crucial to prevent these adverse outcomes. Calyceal rupture often results from urolithiasis, with other causes including strictures, tumors, and congenital abnormalities. The rupture occurs when intrapelvic pressure exceeds the tensile strength of the calyceal walls, leading to urine leakage and potential inflammation or sepsis. Calyceal ruptures are quite rare, with their exact incidence not well-documented due to the infrequency of the condition and potential underreporting. Although relatively uncommon, the condition is more prevalent in individuals with recurrent nephrolithiasis and other predisposing factors. Timely recognition and intervention, guided by imaging studies such as non-contrast CT scans, are essential. Conservative management with medical therapy is effective in many cases, but surgical intervention may be necessary for larger stones or complications. This report presents the case of a 36-year-old female with calyceal rupture secondary to nephrolithiasis, presenting with severe flank pain. Upon initial presentation, the patient underwent a thorough workup, including imaging studies, appropriate medical management, and continuous monitoring. She was stabilized, her pain was effectively managed, and she was discharged with a scheduled outpatient follow-up. This case highlights the importance of early diagnosis, comprehensive management, and vigilant monitoring in preventing complications and promoting favorable outcomes.

BACKGROUND: When indicated, ureteroceles and ectopic ureters in duplicated collecting systems can be managed via upper or lower urinary tract surgical approaches, or a combination of both. Open ureteroureterostomy (UU) has been described to address these conditions in the absence of lower pole (LP) vesicoureteral reflux (VUR). We report outcomes from multiple centers worldwide with mid-term follow-up.
METHODS: Our study consists of a retrospective review of records and imaging of children who underwent open distal UU via inguinal incision for duplicated collecting system without lower pole VUR in institutions from North America, South America, and the Caribbean. Descriptive statistics and univariate analysis were utilized.
RESULTS: The records of 127 patients who underwent open distal UU for double collecting system between 2009 and 2022 were reviewed. Of those, 65% were female (n = 82), with a mean age at operation of 18 months (range 3-180). Main presentation at surgery included prenatal hydronephrosis (64%, n = 81), followed by febrile urinary tract infections (28%, n = 36), and urinary incontinence or other (8%, n = 10). The patients with antenatal or incidental diagnosis of hydronephrosis were classified as preoperative Society for Fetal Urology (SFU) grade 3 (n = 64) and 4 (n = 54) (96%). Those who were classified with SFU grade 0-2 (4%) had symptomatic clinical presentations including febrile urinary tract infections (UTIs) (n = 3), urinary incontinence (n = 3), and a 12-year-old patient with recurrent abdominal pain (n = 1). Mean operative time from skin incision to skin closure was 86 min (range 45-240). Mean hospital stay was estimated at 1.1 days (range 0.5-4). In our cohort of 127 patients, 3 (2%) developed Clavien-Dindo grade I (2 with ureteral stent displacement and 1 with a urine leak/managed conservatively), 6 (5%) with grade II (3 febrile and 3 non-febrile UTIs managed with oral antibiotics), and 2 (2%) with grade IIIb complications (urine leaks requiring surgical management), which were appropriately treated. None presented grade IV or V complications. A double J stent was used in 56 patients (44%), and a Penrose drain was left in 10 (8%). A total of 125 children (98%, n = 125/127) showed sonographic improvement of hydronephrosis, or resolution of symptoms with stable ultrasound findings. Successful outcome was similar for both ureterocele and ectopic ureter subgroups: 49/49 patients with ureterocele showed 100% improvement, 76/78 children with ectopic ureter showed improvement in 97% of the cases. On univariate analysis, outcomes were similar regardless of the use of ureteral stents (p = 0.11). Mean follow-up was 28.2 months (range 12-85).
CONCLUSIONS: Open distal UU is a good alternative for the definitive surgical management of ectopic ureters and ureteroceles without LP VUR. This multicentric and mid-term follow-up study demonstrates that open distal UU offers high success rates, low morbidity, short operative times and hospital stays, and satisfactory aesthetic outcomes. Additionally, the surgery is performed extra-peritoneally and may not require the use of stents or drains, depending on surgeon preference. Further investigations are being conducted to determine the role of UU in the settings of double collecting systems associated with ipsilateral LP VUR.

BACKGROUND: Robot-assisted radical cystectomy (RARC) has gained momentum in the management of muscle invasive bladder cancer (MIBC). Predictors of RARC outcomes are not thoroughly studied. We aim to investigate the implications of preoperative hydronephrosis on oncological outcomes.
METHODS: This study analysed data from the Asian RARC consortium, a multicentre registry involving nine Asian centres. Cases were divided into two groups according to the presence or absence of pre-operative hydronephrosis. Background characteristics, operative details, perioperative outcomes, and oncological results were reviewed. Outcomes were (1) survival outcomes, including 10-year disease-free survival (DFS) and overall survival (OS), and (2) perioperative and pathological results. Multivariate regression analyses were performed on survival outcomes.
RESULTS: From 2007 to 2020, 536 non-metastatic MIBC patients receiving RARC were analysed. 429 had no hydronephrosis (80.0%), and 107 (20.0%) had hydronephrosis. Hydronephrosis was found to be predictive of inferior DFS (HR = 1.701, p = 0.003, 95% CI = 1.196-2.418) and OS (HR = 1.834, p = 0.008, 95% CI = 1.173-2.866). Subgroup analysis demonstrated differences in the T2-or-above subgroup (HR = 1.65; p = 0.004 in DFS and HR = 1.888; p = 0.008 in OS) and the T3-or-above subgroup (HR = 1.757; p = 0.017 in DFS and HR = 1.807; p = 0.034 in OS).
CONCLUSIONS: The presence of preoperative hydronephrosis among MIBC patients carries additional prognostic implications on top of tumour staging. Its importance in case selection needs to be highlighted.

A rare disorder called newborn hydronephrosis is mostly caused by the obstruction of the pyeloureteral junction. We describe a case study of a male neonate who underwent Anderson-Hynes pyeloplasty to effectively cure hydronephrosis in a single kidney that was further complicated by early renal failure. Considering kidney failure can result in progressive renal fibrosis, early management is essential. The gold standard is Anderson-Hynes pyeloplasty, which is usually advised for individuals who weigh over 10 kg. Percutaneous nephrostomy is frequently used in the early stages of care for newborns in order to reduce dilatation and restore renal elasticity. After surgery, the resolution of hydronephrosis may take up to 24 months. We note that pyeloureteral junction obstruction can appear as a single anomaly or a component of a multifactorial illness. This study aims to contribute to the discourse surrounding the optimal timing of Anderson-Hynes pyeloplasty in pediatric patients, providing insights into clinical management strategies and outcomes.

Ureteric duplication is a rare anomaly in the urinary tract, with an incidence of 0.5% to 3%. Bilateral double ureters are even rarer, occurring in 1 of 500 individuals. A 25-year-old man presented with right flank pain and nausea. Physical examination revealed tenderness at the right renal angle. Urinalysis demonstrated microscopic hematuria, and Ultrasound showed enlargement in the lower pole of the right kidney, while the upper pole appeared normal. Intravenous pyelography confirmed bilateral complete ureter duplication. However, after a week of observation, a 5 mm calcium oxalate stone was passed, and this event demonstrated the underlying cause of hydronephrosis in the lower pole of the right kidney. Bilateral complete ureter duplication is a rare anomaly in the urinary tract. To our knowledge, the presence of bilateral complete ureter duplication with a single stone in the right limb of the right double ureter is a unique case that has not been reported in the existing literature.

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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).
METHODS: 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.