Bilateral Ureteropelvic Junction Research Articles

V01-10 BILATERAL TESTICULAR VEIN SYNDROME MASQUERADING AS BILATERAL URETEROPELVIC JUNCTION OBSTRUCTION- LESSONS LEARNT

You have accessJournal of UrologyCME1 Apr 2023V01-10 BILATERAL TESTICULAR VEIN SYNDROME MASQUERADING AS BILATERAL URETEROPELVIC JUNCTION OBSTRUCTION- LESSONS LEARNT Ravimohan mavuduru, Shantanu Tyagi, Girdhar Bora, and Shrawan Singh Ravimohan mavuduru Ravimohan mavuduru More articles by this author , Shantanu TyagiShantanu Tyagi More articles by this author , Girdhar BoraGirdhar Bora More articles by this author , and Shrawan SinghShrawan Singh More articles by this author View All Author Informationhttps://doi.org/10.1097/JU.0000000000003221.10AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Vascular compression of ureter by aberrant lower polar vessels, retrocaval compression and ovarian vein has been described in the past. Still rarer is the ureteric obstruction with the crossing of testicular vein. We present a case of Bilateral testicular vein syndrome masquerading as Ureteropelvic junction obstruction (UPJO), its intra-operative diagnosis and management. METHODS: Index patient is a 18-year-old male, presented with history of bilateral flank pain since 7 months, colicky in nature, requiring intravenous analgesics. There was no significantly contributing past history. He was evaluated elsewhere with abdominal ultrasonography which was suggestive of bilateral gross hydronephrosis. Non-contrast computed tomography (NCCT), done during an acute episode revealed bilateral gross hydronephrosis with probable site of obstruction at UPJ. Diuretic renography in the form of DTPA Scan consolidated the findings of NCCT revealing bilateral compromised cortical function and obstruction to outflow at UPJ. With the given history and evaluation, patient was diagnosed to have Bilateral UPJO and planned for bilateral robot-assisted pyeloplasty. Intraoperatively, on the left side, at pyelotomy the ureter seemed dilated across UPJ raising questions over pre-operative diagnosis. Consequently distal patency check was undertaken to confirm the site of obstruction. After gentle distal ureteric dissection, extrinsic compression at the site of crossing left testicular vein was found to be the site of actual ureteric obstruction. To relieve this compression, the ureter was super positioned over a posteriorized gonadal vein with a subsequently successful distal patency check. Taking lessons from the left side, we started with distal ureteric dissection and surprisingly found extrinsic compression with right testicular vein on the right side as well. Site of compression was visualised as fibrosed ureteric segment which was excised and ureteroureterostomy was done super positioned over a posteriorized right gonadal vein. RESULTS: The patient had an uneventful post-operative course and bilateral double-J stents were removed at 3 weeks. Follow-up renogram at 3 months documented the resolving bilateral hydronephrosis and unobstructed drainage. CONCLUSIONS: Lessons learned from the given case are- Contrast-enhanced CT imaging could have suggested ureteric compression with gonadal vein pre-operatively, Distal patency check is vital when there is dilatation across PUJ, When in doubt, distal ureteric dissection is a better approach and preservation of ureteric vascularity is of paramount importance. Source of Funding: None © 2023 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 209Issue Supplement 4April 2023Page: e82 Advertisement Copyright & Permissions© 2023 by American Urological Association Education and Research, Inc.MetricsAuthor Information Ravimohan mavuduru More articles by this author Shantanu Tyagi More articles by this author Girdhar Bora More articles by this author Shrawan Singh More articles by this author Expand All Advertisement PDF downloadLoading ...

The sequence of intervention determines the risk of early postoperative acute kidney injury in infants with bilateral ureteropelvic junction obstruction

The management of bilateral ureteropelvic junction obstruction (UPJO) is greatly debated. We aim to identify the risk of early postoperative acute kidney injury (AKI) in relation to the sequence of intervention in children with bilateral UPJO managed in a sequential manner. A single center database was retrospectively reviewed for children ≤2 years who underwent bilateral pyeloplasty. According to the differential renal function on the preoperative renograms, patients were categorized into group A: pyeloplasty on the poorer functioning kidney first and group B: pyeloplasty on the better functioning side first. Serum creatinine and eGFR, using the modified Schwartz formula, were evaluated at four time points (I): before the first intervention (II): within 48h of the first intervention (III): before the second intervention and (IV): within 48h of the second intervention. Preoperative and postoperative values were compared. The incidence of early postoperative AKI in both groups was defined according to the Acute Kidney Injury Network (AKIN) criteria. The study comprised 46 children treated by staged pyeloplasty, 28 of them underwent pyeloplasty on the poorer functioning side first. Baseline serum creatinine and eGFR were not significantly different between both groups. Patients who underwent pyeloplasty on the poorer functioning side first, had a significant decline of eGFR after the first intervention (p=0.006). Conversely, no significant eGFR changes were observed after the first or second interventions in the other group (figure). Overall, 64.3% and 33.3% of patients developed some degrees of AKI when intervention was started on the poorer and better functioning renal units, respectively (p=0.04). Bilaterality is seen in approximately ¼ of patients with UPJO. Oftentimes, both renal units are asymmetrically affected with little data to guide surgeons on the optimal sequence of intervention. Following pyeloplasty, 52.2% of the evaluated children with bilateral UPJO had early postoperative AKI, mostly of low stage. Our data suggest that intervening first on the better functioning side allows for better recovery of the renal functional reserve and lowers the risk of postoperative AKI. In children with bilateral UPJO, starting intervention on the poorer functioning kidney is associated with increased risk of postoperative AKI. Long-term prospective studies are needed to confirm our findings.

Acute bilateral ureteropelvic junction obstruction as a rare cause of hypertensive crisis: a case report

BackgroundBilateral ureteropelvic junction obstruction is a common cause of secondary hypertension in the pediatric population, often due to congenital malformation. On the other hand, it is less frequently encountered in the adult population and is usually due to an acquired condition, most commonly by a bilaterally obstructing nephrolithiasis causing hydronephrosis and subsequent hypertension. The aim of this study was to investigate and highlight the underlying mechanisms by which acute bilateral ureteropelvic junction obstruction causes hypertensive crisis and why early detection and prompt treatment are necessary to mitigate the effects of elevated blood pressure on target organs.Case presentationA 41-year-old African American man with hypertensive cardiomyopathy presented with anuria. He was found to have elevated blood pressure with evidence of target organ damage on laboratory examination, demonstrated by sudden elevation of his serum creatinine level. He was initially treated with oral and intravenous antihypertensives, with minimal improvement. The work-up was unremarkable apart from the imaging finding of acute bilateral ureteropelvic junction obstruction from obstructing nephrolithiasis causing hydronephrosis. Bilateral ureteral stents were placed for decompression, with resolution of the hypertensive crisis and improvement of renal function.ConclusionThis case highlights the importance of prompt diagnosis and treatment of underlying acute bilateral ureteropelvic junction obstruction to mitigate the deleterious effects of sudden blood pressure elevation on target organs.

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.

Postnatal management of bilateral Grade 3-4 ureteropelvic junction obstruction.

Introduction:Bilateral hydronephrosis on prenatal ultrasound can be managed expectantly or with surgical intervention. The treatment strategies and outcomes are not clearly defined.Methods:We conducted a retrospectively audit of outcomes of management of prenatally detected severe bilateral ureteropelvic junction obstruction (UPJO) in our institution.Patients with bilateral Grade 3–4 hydronephrosis were included. Those with complications like rupture, underwent bilateral intervention within 4 weeks; in the remaining, unilateral pyeloplasty was performed at 4–12 weeks. The contralateral renal unit was re-evaluated at a later date for further improvement or deterioration. All the patients were followed up with ultrasonography and renogram at 3 months, 6 months, and 1-year post operatively. The case records were analyzed for the resolution of antero-posterior diameter (APD) or the improvement in single-kidney glomerular filtration rate (s-GFR) in the operated units.Results:Over 15 years, 28 patients (56 renal units) had bilateral UPJO (male-to-female ratio = 13:1). Twelve units underwent neonatal intervention to tackle the complications (6 bilateral pyeloplasty), 17 units underwent early pyeloplasty, and 15 underwent late pyeloplasty. Twelve of the twenty-two (54%) contralateral units, which were stented/observed, resolved spontaneously. Receiver operating characteristics analysis revealed that those with initial APD <25 mm and initial s-GFR >35 ml/m were more likely to improve during the observation. Ten of the forty-four operated units (22%) failed to show an improvement. Units with initial s-GFR <10 ml/m had poor chance of postoperative functional recovery.Conclusions:In neonates with bilateral UPJO, the worse affected kidney is operated first, as it still has the potential to recover. The contralateral milder UPJO unit is known to recover spontaneously following unilateral pyeloplasty. In those with bilateral Grade 4 UPJO and mass, bilateral pyeloplasty is feasible. Alternatively, unilateral pyeloplasty + contralateral cystoscopic retrograde stenting may prevent rupture or functional deterioration in the opposite kidney.

Bilateral Ureteral Obstruction Related to Pelvic Rhabdomyosarcoma Presenting with Acute Kidney Injury: A Case Report

Bilateral renal obstruction is a rare critical condition, requiring a prompt diagnostic approach and treatment to restore the renal function. The most commonly observed obstructive uropathy in children is congenital malformation, such as posterior urethral valves and bilateral ureteropelvic junction obstruction. Malignant pelvic masses obstructing the ureter are widely reported in adults but are rarely observed in children. The treatment of ureteral obstruction related to pelvic malignancy is a therapeutic challenge with a median survival duration of 3–7 months in adults; however, pediatric patients with pelvic malignancy leading to ureteral obstruction had better outcomes, with a reported 5-year mortality rate of 20%, than the adult patients. Here, we report a rare case of bilateral ureteral obstruction associated with pelvic rhabdomyosarcoma presenting with acute kidney injury treated by ureteral diversion with double J stent, and concommittent emergency hemodialysis, leading to restoration of good renal function. We suggest that bilateral ureteral obstruction should be released as soon as possible using surgical or interventional approach to minimize the obstruction period, and subsequential chemotherapy may contribute to improvement of survival and recovery of renal function. Key words: Urinary bladder neck obstruction, Acute kidney injury, Child, Pelvic Neoplasms, Prognosis

BILATERAL URETEROPELVIC JUNCTION OBSTRUCTION: FACTORS GUIDING MANAGEMENT

Background: The management of prenatally detected hydronephrosis due to bilateral ureteropelvic junction obstruction (UPJO) remains controversial. No definite recommendations exist regarding management, and many authors recommend unilateral or bilateral pyeloplasty. Objective: This study was conducted to evaluate the clinical outcome of patients with bilateral UPJO treated at a tertiary care hospital. Materials and Methods: This descriptive study was performed in the Department of Pediatric Surgery, Lucknow. All patients with prenatally diagnosed bilateral hydronephrosis and postnatal diagnosis of bilateral UPJO, who underwent initial unilateral pyeloplasty, were prospectively evaluated and were included in the study. The outcome of contralateral hydronephrosis was assessed as resolution or surgery. Results: A total of 16 patients underwent initial unilateral pyeloplasty and met the inclusion criteria (mean age: 2.6 months). Of all 16 patients, 12 (75%) had resolution and 4 (25%) patients required additional contralateral pyeloplasty during the follow-up. The mean follow-up period was 18 months (range 9–36 months) and the mean anteroposterior pelvic diameter and pelvis-to-cortex (P/C) were significantly different among the groups (p<0.01). Conclusion: Our findings suggest that in children with bilateral UPJO type of hydronephrosis, improvement or resolution of the contralateral renal unit can occur following initial unilateral pyeloplasty for the worse renal unit. In patients in whom both the renalunits have similar split renal function and grades of hydronephrosis, pelvic AP diameter, cortical thickness, and P/C ratio can be used to determine the likelihood of contralateral delayed pyeloplasty.

Bilateral ureteropelvic junction obstruction with ureteric stone in a female Nigerian infant: the necessity for renal and urinary tract ultrasound in acute kidney injury running head: bilateral ureteropelvic junction obstruction

Bilateral ureteropelvic junction obstruction with ureteric stone in a female Nigerian infant: the necessity for renal and urinary tract ultrasound in acute kidney injury running head: bilateral ureteropelvic junction obstruction

Devastating Renal Injury After a Low Energy Trauma: Was the Force Sufficient?

Introduction: Renal traumas are quite commonly encountered in emergency departments. Low-energy traumas are often considered non-significant, but the presence of a pre-existing renal lesion predisposes the kidneys to more severe damage in the setting of even minor traumas, which may be accompanied by devastating results, should they remain unattended. Case Presentation: Here we report an incidental diagnosis of bilateral ureteropelvic junction obstruction in a patient who presented with unexpected shattered kidney following a low-energy flank injury. Conclusions: Patients with a pre-existing renal lesion are vulnerable to severe traumatic renal injuries, even after minor trauma. The severity of renal injuries may be much more than expected, making the clinical and imaging findings complex and confusing in these patients.

Bilateral ureteropelvic junction disruption following blunt abdominal trauma: a case report

Bilateral ureteropelvic junction disruption following blunt abdominal trauma: a case report

Predictors of Renal Functional Improvement After Pyeloplasty in Ureteropelvic Junction Obstruction: Clinical Value of Visually Assessed Renal Tissue Tracer Transit in 99mTc-mercaptoacetyltriglycine Renography

To determine the clinical value of visually assessed renal tissue transit time (TTT) in 99mTc-mercaptoacetyltriglycine (99mTc-MAG3) renography for patients undergoing pyeloplasty. Medical records of 164 patients who underwent dismembered pyeloplasty were retrospectively reviewed. Baseline and postoperative renal ultrasonography and 99mTc-MAG3 renography were performed. Two urologists blinded to clinical data evaluated the renography and classified TTT as timely or delayed based on visualization of the tracer in the kidney pelvis between 2 and 10 minutes. Renal functional change after pyeloplasty was compared between patients in the timely and delayed groups. A total of 126 patients (median age, 9 months) were evaluated after excluding patients with bilateral ureteropelvic junction obstruction, a single functioning kidney, duplicated ureter, or <3 months of follow-up. There were no differences between 89 patients with timely TTT and 37 patients with delayed TTT in mean preoperative hydronephrosis grade (3.7 vs 3.8) and pelvic diameter (3.1 cm vs 3.4 cm). Although the pre- and postoperative mean values of differential renal function (DRF) were significantly higher in the timely group than in the delayed group (47.2% vs 38.3% and 47.9% vs 44.6%), DRF change was greater in the delayed group (6.3% vs 0.6%). In multivariate analysis, delayed TTT was the only significant predictor of >5% improvement in renal function after pyeloplasty. Delayed TTT in 99mTc-MAG3 renography was a significant predictor of renal functional improvement after pyeloplasty in ureteropelvic junction obstruction. Because substantial improvement of renal function is anticipated, we recommend immediate pyeloplasty in patients with delayed TTT and decreased DRF.

Fibroproliferative response to urothelial failure obliterates the ureter lumen in a mouse model of prenatal congenital obstructive nephropathy.

Congenital obstructive nephropathy (CON) is the most prevalent cause of pediatric chronic kidney disease and end-stage renal disease. The ureteropelvic junction (UPJ) region, where the renal pelvis transitions to the ureter, is the most commonly obstructed site in CON. The underlying causes of congenital UPJ obstructions remain poorly understood, especially when they occur in utero, in part due to the lack of genetic animal models. We previously showed that conditional inactivation of Sec10, a central subunit of the exocyst complex, in the epithelial cells of the ureter and renal collecting system resulted in late gestational bilateral UPJ obstructions with neonatal anuria and death. In this study, we show that without Sec10, the urothelial progenitor cells that line the ureter fail to differentiate into superficial cells, which are responsible for producing uroplakin plaques on the luminal surface. These Sec10-knockout urothelial cells undergo cell death by E17.5 and the urothelial barrier becomes leaky to luminal fluid. Also at E17.5, we measured increased expression of TGFβ1 and genes associated with myofibroblast activation, with evidence of stromal remodeling. Our findings support the model that a defective urothelial barrier allows urine to induce a fibrotic wound healing mechanism, which may contribute to human prenatal UPJ obstructions.

Presentation, Management and Long-Term Outcome of Ureteropelvic Junction Obstruction in Duplex Kidneys

Ureteropelvic junction obstruction in association with a duplex collecting system is a rare but challenging upper urinary tract pathology. We report our 21-year experience with this anomaly in terms of presentation, diagnostic evaluation and management. We retrospectively identified all patients with ureteropelvic junction obstruction in a duplex collecting system between 1991 and 2012. We reviewed each case for presenting symptoms, anatomy and management. Median followup was 10.8 years (range 2 to 22). Ureteropelvic junction obstruction in duplex kidneys was diagnosed in 21 patients. Ten patients presented with clinical symptoms such as flank pain and urinary tract infection but 11 were asymptomatic. Six patients were diagnosed by prenatal ultrasound. The lower pole and the upper pole were affected in 22 and 3 renal units, respectively. Bilateral ureteropelvic junction obstruction was found in 4 cases. Duplication was complete in 5 patients, incomplete in 11 and undetermined in 5. Surgery was performed in 14 patients, including pyelopyelostomy or ureteropyelostomy in 7, dismembered pyeloplasty in 6 and heminephrectomy in 1. Reintervention was required in 1 case. Conservative treatment was adopted in 7 patients with clinically insignificant obstruction and unimpaired renal function. In all of these patients upper urinary tract dilatation gradually improved during 3 years. Ureteropelvic junction obstruction in a duplex kidney is a rare but challenging anomaly that requires careful evaluation. Treatment should be individualized according to clinical presentation (symptomatic/asymptomatic), anatomy (lower/upper pole), duplication type (complete/incomplete) and obstruction with time (severity/development) on dynamic renogram.

Stentless pyeloplasty for ureteropelvic junction obstruction in children

Introduction Stenting after pyeloplasty is an established practice and helps in ensuring a patent anastomosis until healing has completed. Stents, however, may cause complications such as infection and displacement and increase the cost of management; therefore, stentless pyeloplasty is now considered as feasible alternative. Patients and methods From August 2008 to October 2010, we retrospectively analyzed the results of stentless surgery in patients with ureteropelvic junction (UPJ) obstruction. In all, 42 patients with UPJ obstruction were managed. Nine patients who were treated conservatively, one who underwent nephrectomy, and one pyelostomy for pyonephrosis in solitary kidney were excluded. Age range at surgery was 14 days–12 years with a mean age of 12.7 months. Results There were 23 male patients and eight female patients with a male-to-female ratio of 3 : 1. Fourteen patients had left, 12 right, and five had bilateral UPJ obstruction. A total of 34 pyeloplasties were performed in 31 patients. Of the five patients with bilateral UPJ obstruction, three underwent bilateral pyeloplasty and remaining two underwent unilateral pyeloplasty with conservative management on the opposite sides. Two patients underwent laparoscopic pyeloplasties and 32 pyeloplasties by open technique. Double J stent was placed at initial surgery in three patients and 31 stentless pyeloplasties were performed. The mean operative time was 75 min. The mean perinephric drain removal time was 2 days. None of the patients had persistent urinary leak. The mean hospital stay was 3.2 days. Reduction in anteroposterior diameter was noticed in 91% cases on 12 weeks follow-up scan. Complications included persistent or increase in hydronephrosis in three (9%) patients. In all the three patients, cystoscopic stenting was attempted. In two patients, size 4 Fr double J stent was passed easily into the renal pelvis. One patient improved, whereas other still has a dilated pelvis with static anteroposterior diameter after removal of stent at 6 weeks; patient is kept on close surveillance on regular ultrasonography. Re-exploration was performed in one patient, which showed kinking at the anastomosis site. Pyeloplasty was revised and patient improved. Other complications included lumbar hernia in one patient, which improved at 6-month follow-up, and stitch granuloma in one patient, which improved after removal of residual stitch. Conclusion Stentless surgery for UPJ obstruction is a safe and feasible technique; it reduces the cost of surgery and avoids multiple procedures. Keywords : management, pyeloplasty, stentless, ureteropelvic junction obstruction

Absence of Canonical Smad Signaling in Ureteral and Bladder Mesenchyme Causes Ureteropelvic Junction Obstruction

Obstruction of the ureteropelvic junction (UPJ) is a common congenital anomaly frequently associated with ureteral defects. To study the molecular mechanisms that modulate ureteral development, we inactivated Smad4, the common Smad critical for transcriptional responses to TGF-β and Bmp signaling, in the ureteral and bladder mesenchyme during embryogenesis. Loss of canonical Smad signaling in these tissues caused bilateral UPJ obstruction and severe hydronephrosis beginning at embryonic day 17.5. Despite a reduction in quantity of ureteral smooth muscle, differentiation proceeded without Smad4, producing a less severe phenotype than Bmp4 mutants; this finding suggests that at least some Bmp4 functions in ureteral smooth muscle may be Smad-independent. The absence of canonical Smad signaling in the ureteral mesenchyme, but not in the urothelium itself, led to urothelial disorganization, highlighting the importance of mesenchymal support for epithelial development. Transcript profiling revealed altered expression in known Bmp targets, smooth muscle-specific genes, and extracellular matrix-related genes in mutant ureters before the onset of hydronephrosis. Expression of the Bmp target Id2 was significantly lower in Smad4 mutants, consistent with the observation that Id2 mutants develop UPJ obstruction. In summary, Smad4 deficiency reduces the number and contractility of ureteral smooth muscle cells, leading to abnormal pyeloureteral peristalsis and functional obstruction. The subsequent bending and luminal constriction of the ureter at the UPJ marks the transition from a functional obstruction to a more intractable physical obstruction, suggesting that early intervention for this disease may prevent more irreversible damage to the urinary tract.

Bilateral ureteropelvic junction obstruction presenting with hypertension and cerebral vascular accident

Cerebrovascular accident and renal hypertension secondary to ureteropelvic junction obstruction (UPJO) are extremely rare. A 6-year-old girl presented with intracranial hemorrhage because of hypertension secondary to the bilateral UPJO. This was successfully treated with craniotomy and subsequent percutaneous nephrostomy placement and bilateral pyeloplasty. Brain computerized tomography revealed right-sided intracranial hemorrhage, and renal ultrasonography confirmed bilateral severe hydronephrosis. Craniotomy with evacuation of intracerebral hematoma and bilateral nephrostomy under ultrasound guidance were performed. One week later, she underwent bilateral pyeloplasty in 2 stages. The patient has been well with normalized renal function and is completely cured of her hypertension in long-term follow-up.

Laparoscopic Simultaneous Bilateral Dismembered Pediatric Pyeloplasty Applying Three Midline Ports

The objective of this study was to describe our experience with laparoscopic simultaneous bilateral dismembered pyeloplasty in two pediatric cases by using three midline ports. A 5-year-old boy and a 5-month-old male infant with bilateral ureteropelvic junction obstruction underwent bilateral laparoscopic transperitoneal dismembered pyeloplasty. A 10-mm trocar was placed through the umbilicus, and two 5-mm trocars were also placed in the midline superior and inferior to the umbilicus. On the left side, dismembered pyeloplasty was performed through a transmesocolic approach. Double-J ureteral stent was inserted through one of the trocars on both sides. The postoperative period was uneventful. Patients were discharged on postoperative days 4 and 5. Oral feeding was tolerated on the first postoperative day. In a 6-month follow-up period, excretory urography and renal scan showed resolution of obstruction. Laparoscopic dismembered pyeloplasty can be performed in a single session for pediatric patients with bilateral ureteropelvic junction obstruction. This can obviate the need for two sessions of anesthesia induction and intubation. However, more studies with large number of patients are needed to confirm this result.

Acute renal failure due to bilateral pieloureteral stone impaction in a 10-month-old boy

Urolithiasis (UL) can present with its classic signs and symptoms, such as flank or abdominal pain and gross hematuria. However, atypical complaints can be more common in younger children. We report here a case of bilateral ureteropelvic junction (UPJ) stones in a 10-month-old boy who only showed nonspecific symptoms at the time of presentation. The initial blood test revealed renal failure (serum creatinine 3.4 mg/dl), hyperkalemia (6.4 mEq/l), hyperphosphoremia (9.4 mEq/l) and mild metabolic acidosis. Medical treatment for electrolyte disorders was started. The ultrasonography revealed impacted stones in both ureteropelvic junctions. A pigtail catheter was placed in each ureter. High urine flow was promptly achieved after the pigtail procedure, and the serum creatinine level dropped quickly from 4.5 to 0.32 mg/dl. Quantitative determination of urinary amino acids by ion exchange chromatography showed high cystine levels of 8.43 mmol/g creatinine. Outpatient follow-up was scheduled every 3 months to monitor patient compliance with potassium citrate. In the first 6 months, the patient underwent three febrile urinary tract infections (UTIs). Since both pigtail catheters were removed, he has been free of UTIs and stones. Our case emphasizes the need for considering UL in infants who complain with unclear signs, because UL can only show nonspecific symptoms in children younger than 1 year old. Since cystinuria can cause loss of renal function due to urinary system obstruction and UTI, an early diagnosis and a close follow-up are the key to achieving the best long-term outcome.

Bilateral Ureteral Leiomyoma With Bilateral Ureteropelvic Junction Obstruction

Leiomyomas are benign tumors characterized by overgrowth of visceral smooth muscle in the respiratory, gastrointestinal, and female reproductive tracts. They rarely develop in the urinary system and only 10 cases of unilateral ureteral leiomyoma (UL) have been reported since 1955. No cases of bilateral UL or ureteropelvic junction obstruction due to UL have ever been reported. We present a case of bilateral UL with bilateral ureteropelvic junction obstruction. To the best of our knowledge, this is the first such case report in the English literature.

Management of Severe Bilateral Ureteropelvic Junction Obstruction in Neonates with Prenatally Diagnosed Bilateral Hydronephrosis

PurposeThe management of prenatally detected bilateral ureteropelvic junction obstruction (UPJO) remains controversial. We attempted to develop a treatment plan for patients with severe bilateral UPJO.Materials and MethodsWe evaluated the records of 13 patients with prenatally diagnosed grade 3 or more bilateral hydronephrosis that led to the postnatal diagnosis of severe bilateral UPJO. Ultrasonography and 99mtechnetium mercaptoacetyltriglycine (99mTc-MAG3) renal scans were performed within 1 month. Four renal units had grade 3 and 22 had grade 4 hydronephrosis. All 13 patients were managed by unilateral pyeloplasty, and the patients' mean age was 3 months. At 1 month postoperatively, we decided whether delayed surgery in the opposite renal unit was necessary according to the findings of ultrasonography and 99mTc-MAG3 scans.ResultsOf 13 patients, 11 underwent initial pyeloplasty on renal units with more severe hydronephrosis or lower relative renal function (RRF) on 99mTc-MAG3 scans. The remaining 2 patients simultaneously underwent percutaneous nephrostomy on renal units with a lower RRF and initial pyeloplasty on renal units with a higher RRF. In 5 patients, contralateral hydronephrosis had spontaneously improved at 1 month postoperatively, and 8 patients underwent delayed contralateral pyeloplasty at 2 months postoperatively.ConclusionsIn children with severe bilateral UPJO, the non-operated renal units with grade 3 and some with grade 4 hydronephrosis improved spontaneously after unilateral pyeloplasty. Therefore, delayed pyeloplasty of the opposite side should be considered at 1 month following initial pyeloplasty.