Congenital ureteropelvic junction obstruction Author Laurence S Baskin, MD, FAAP Section Editor Duncan Wilcox, MD Deputy Editor

Melanie S Kim, MD All topics are updated as new evidence becomes available and our peer review process is complete. Literature review current through: Sep 2013. | This topic last updated: Sep 12, 2013. INTRODUCTION Ureteropelvic junction (UPJ) obstruction is a partial or total blockage of the flow of urine that occurs where the ureter enters the kidney. The etiology of UPJ obstruction includes both congenital and acquired conditions. UPJ obstruction is the most common pathologic cause of antenatally detected hydronephrosis. The epidemiology, pathophysiology, clinical features, and management of congenital UPJ obstruction will be reviewed here. EPIDEMIOLOGY The reported incidence of ureteropelvic junction (UPJ) obstruction is 1 in 500 live births screened by routine antenatal ultrasound; however, not all cases require surgical intervention [1,2]. UPJ obstruction is the most common anatomical cause of antenatal hydronephrosis. In one retrospective study, functionally significant UPJ obstruction was noted in 1 in 1500 fetuses screened by antenatal ultrasound [ 3]. Boys are affected with UPJ obstruction more commonly than are girls [3,4]. Lesions are found more frequently on the left than on the right side. The reported rate of bilateral involvement ranges from 10 to 40 percent [1,4]. PATHOPHYSIOLOGY Congenital ureteropelvic junction (UPJ) obstruction is caused by anatomic lesions or functional disturbances that restrict urinary flow across the UPJ, resulting in hydronephrosis (figure 1 and picture 1) [1]. Most cases are thought to be due to partial obstruction, because complete obstruction results in rapid destruction of the kidney. In some cases, partial obstruction may also lead to progressive deterioration of renal function. However, in many infants, an equilibrium state may develop in which renal function remains stable. Development of the equilibrium state resulting in stable renal function depends on:

Urinary rate and output Anatomy and degree of UPJ obstruction Compliance of the renal pelvis In the fetus and infant, the pelvis, because of its increased compliance, can stretch and thereby accommodate large volumes of urine, which results in pelvic dilatation (hydronephrosis). The large capacity volume of a stretched pelvis decreases the risk of high intrapelvic pressure. As a result, kidneys with a large intrarenal pelvis are less vulnerable to damage from obstruction than those with a small intrarenal pelvis, especially during diuresis. ETIOLOGY Congenital ureteropelvic junction (UPJ) obstruction is usually caused by intrinsic stenosis of the proximal ureter, and less commonly by extrinsic compression of the UPJ. Intrinsic narrowing In most cases of UPJ obstruction, the upper segment of the ureter is narrowed or kinked, resulting in obstruction of urinary flow. Although the underlying mechanism is not proven, it is thought that there

is an embryologic disruption of the proximal ureter that alters circular musculature development and/or collagen fibers, and composition between and around the muscular cells [5]. Other causes of intrinsic UPJ obstruction, albeit rare, include valvular mucosal folds, persistent fetal ureteral convolutions, and ureteral polyps. Extrinsic compression In about 10 percent of pediatric UPJ obstruction, an aberrant or accessory renal artery or arterial branch may cross the lower pole of the kidney, resulting in compression of the UPJ and blockage of urinary flow (image 1). CLINICAL PRESENTATION Fetal and neonatal In the developed world, where maternal ultrasound screening is commonly performed, most cases of ureteropelvic junction (UPJ) obstruction are diagnosed during the postnatal evaluation of antenatal hydronephrosis. The Society of Fetal Urology developed criteria for the diagnosis and grading of antenatal hydronephrosis based upon the degree of pelvic dilatation, number of calyces seen, and the presence and severity of parenchymal atrophy (image 2 and image 3). (See "Overview of antenatal hydronephrosis".) In the absence of antenatal screening, newborns may present with a palpable abdominal mass caused by an enlarged obstructed kidney. Other presentations include urinary tract infection, hematuria, or failure to thrive. Renal failure is an unusual presentation, and occurs in infants with a single obstructed kidney or with bilateral involvement. Older children Clinical manifestations in older children include intermittent flank pain or abdominal pain. The pain may worsen during brisk diuresis (for example, after consumption of caffeine or alcohol). These symptoms may be accompanied by nausea and vomiting, leading to an evaluation of the gastrointestinal tract [6]. Children may also present with renal injury after experiencing minor trauma [7], hematuria, renal calculi [8], or hypertension [6,9]. In these cases, an incidental finding of hydronephrosis on ultrasound may be the first clue of the possibility of UPJ obstruction as the underlying diagnosis (image 2). Other anomalies UPJ obstruction may be associated with other genitourinary anomalies, such as a horseshoe kidney [10], or be a component of or associated with a syndrome such as CHARGE syndrome [ 11]. DIAGNOSIS The diagnosis of ureteropelvic junction (UPJ) obstruction is generally suspected when imaging studies, usually ultrasonography, demonstrate hydronephrosis. The diagnosis is confirmed by diuretic renography. Ultrasonography Most cases of UPJ obstruction present as a result of detecting hydronephrosis by prenatal ultrasonographic screening (image 2). Although UPJ obstruction is the most common anatomical etiology of hydronephrosis, further imaging evaluation is required to differentiate UPJ obstruction from other frequent causes of antenatal hydronephrosis, such as transient and functional hydronephrosis, ureterovesical junction obstruction, and vesicoureteral reflux (VUR). In older children with abdominal pain and suspected UPJ obstruction, an ultrasound examination should be performed during the acute painful episode (Diettel's crisis) to demonstrate hydronephrosis (image 1). The sonogram often is normal when the pain subsides.

Diuretic renography Diuretic renography (renal scan and the administration of a diuretic) is used to diagnose urinary tract obstruction. It measures the drainage time from the renal pelvis (referred to as washout) and assesses total and each individual kidney's renal function. The washout measurement correlates with the degree of obstruction. In general, a half-life greater than 20 minutes to clear the isotope from the kidney is considered indicative of obstruction, although other urologists use split function, comparing the function of the two kidneys, as a means to identify patients for surgery. A more complete description of the diuretic renography is found separately. (See "Postnatal management of antenatal hydronephrosis", section on 'Diuretic renography'.) CT scan Computed tomography (CT) is an alternative to ultrasonography in the symptomatic child (image 4); however, it is not the preferred modality due to its radiation exposure. In UPJ obstruction, the CT scan typically shows hydronephrosis without a dilated ureter. Voiding cystourethrogram A voiding cystourethrogram (VCUG) is performed in patients with hydronephrosis to confirm the presence or absence of VUR of both the affected and contralateral kidneys. Ten percent of patients with UPJ obstruction have contralateral low-grade vesicoureteral reflux. In addition, VCUG assesses the patency of the urethra in males to detect posterior urethral valves. Identification of VUR is important because children with concurrent VUR and UPJ obstruction may be at higher risk for severe infection. (See "Presentation, diagnosis, and clinical course of vesicoureteral reflux", section on 'Diagnosis' and "Clinical presentation and diagnosis of posterior urethral valves", section on 'Diagnosis'.) DIFFERENTIAL DIAGNOSIS The differential diagnosis of ureteropelvic junction (UPJ) obstruction includes other causes of hydronephrosis. Imaging studies (eg, diuretic renography, serial ultrasounds, and voiding cystourethrogram [VCUG]) differentiate UPJ obstruction from the following conditions:

Vesicoureteral reflux (VUR) Transient hydronephrosis Functional hydronephrosis Other urological anomalies including posterior urethral valves, congenital megaureter, ureterocele, and multicystic dysplastic kidney EVALUATION Newborn The goal of evaluating a newborn with antenatal hydronephrosis is to identify all infants with significant renal or urinary tract abnormalities including ureteropelvic junction (UPJ) obstruction, while limiting unnecessary radiographic studies in infants with clinically insignificant findings or normal infants. (See "Postnatal management of antenatal hydronephrosis", section on 'Approach'.) Imaging studies are performed in a step-wise fashion. Decisions for further imaging depend upon the result of each study as follows:

Postnatal ultrasound Evaluation begins with a postnatal ultrasound to confirm the presence of persistent hydronephrosis. The timing of performing imaging studies evaluation is dependent upon whether there is bilateral involvement and the severity of antenatal hydronephrosis (algorithm 1 and algorithm 2). Infants with

bilateral involvement or with a severe hydronephrotic solitary kidney require evaluation in the first few days of life because of the increased likelihood of significant disease and need for early intervention. In general, however, examination should be avoided in the first two days after birth, because hydronephrosis may not be detected because of extracellular fluid shifts that will underestimate the degree of hydronephrosis. Therefore, in unilateral disease regardless of severity, ultrasonography should be performed at least after 48 hours of life. We use the Society of Fetal Urology system to grade the severity of hydronephrosis from grade 0 (no hydronephrosis) to grade IV (severe hydronephrosis) (image 3).

Voiding cystourethrogram (VCUG) If there is persistent, significant hydronephrosis, a VCUG is performed to detect vesicoureteral reflux, and in males, posterior urethral valves. (See "Postnatal management of antenatal hydronephrosis", section on 'Approach'.) If the VCUG is negative (no reflux) and the infant has persistent postnatal severe hydronephrosis (renal pelvis diameter 15 mm), a diuretic renography is performed to detect possible obstruction. At this stage of evaluation, referral to a center with expertise in pediatric urologic care is warranted because the renal scan is an invasive procedure, requiring placement of an intravenous line and bladder catheter. In general, diuretic renography can be performed after six weeks of life because immediate surgical intervention is rarely required, and the diuretic renography is more difficult to interpret in the newborn period because of immature renal function. If the VCUG is negative (no reflux) and the infant has persistent postnatal hydronephrosis that is not severe (renal pelvis diameter <15 mm), a follow-up ultrasound is performed at three months of age to monitor the degree of hydronephrosis. If dilatation progresses to the threshold of renal pelvis diameter 15 mm, a diuretic renography should be obtained at that time to determine the relative function of the affected kidney. Older child Ultrasonography should be performed in older patients with symptoms that are suggestive of UPJ obstruction. The examination should be conducted when the child is symptomatic, as the obstruction may be intermittent. The diagnosis of intermittent UPJ obstruction is confirmed if hydronephrosis is present when the child is symptomatic, and resolves when the child is well. A diuretic renal scan will document baseline renal function and may also provoke symptoms during the diuretic phase of the study, which confirms the diagnosis. Surgery is indicated to prevent further episodes of symptoms (eg, pain). (See 'Symptomatic patients' below.) MANAGEMENT There are no randomized trials that provide evidence for the optimal management of congenital ureteropelvic junction (UPJ) obstruction. The following discussion is based upon observational studies, and the experience and opinions of experts in the field. Prenatal Unilateral UPJ obstruction does not require prenatal intervention or early delivery, unless prenatal decompression of an enormously dilated renal pelvis is necessary to prevent dystocia or pulmonary compression [12,13]. There is no evidence that prenatal intervention in infants with either a single obstructed kidney or bilateral involvement improves renal outcome. Antenatal surgery should only be considered in the rarest of cases and only in select centers with expertise after a complete evaluation is performed. The criteria and setting for antenatal surgery is discussed in detail separately. (See "Overview of antenatal hydronephrosis", section on 'Management'.) Symptomatic patients Children who are symptomatic usually require operative intervention. If radiographic evaluation reveals hydronephrosis during pain, which resolves when symptoms subside, surgical intervention is warranted. In these patients, pyeloplasty is performed and consists of resecting the atretic or stenotic segment, and reattaching the normal ureter to the renal pelvis, thereby relieving the obstruction. If the obstruction is due to an aberrant renal blood vessel, the UPJ is repositioned anatomically above the blood vessel preventing further obstruction. (See 'Surgical options' below.)

A follow-up ultrasound should be obtained approximately 4 to 12 weeks after surgery. Over time, nearly all patients (up to 95 percent) have radiographic improvement and resolution of their symptoms. Pyelonephritis Patients with UPJ obstruction who present with acute pyelonephritis are treated with antibiotics. Surgical repair is performed when the infection has resolved. If pyelonephritis does not respond to antibiotics, a temporary percutaneous pyelostomy tube should be placed to relieve the obstruction. Kidney stones Kidney stones sometimes develop in the obstructed renal pelvis. Treatment involves open pyelolithotomy and pyeloplasty. Asymptomatic patients The ultimate goal of management is to preserve renal function and avoid unnecessary surgery. Managing asymptomatic patients is challenging, because the natural course of UPJ obstruction is variable. It appears that many patients will have stable renal function and improvement in the degree of hydronephrosis during long periods of observation. However, others will have deterioration of their renal function, and appear to benefit from surgical correction. Because there is no reliable method to predict whether the function of a kidney with UPJ obstruction will remain unaffected or deteriorate with time, there is controversy on the role of watchful observation with surgical intervention only if there is a deterioration in the patient's clinical status (ie, decrease in renal function and/or development of symptoms). This approach may prevent unnecessary surgery in some asymptomatic patients. These points were illustrated in a large retrospective case series of 116 patients with grade III to IV hydronephrosis and obstructive diuretic renograms [14]. Of the 116 patients, 83 presented with antenatal hydronephrosis. Management was divided into three categories: observation, immediate pyeloplasty, or pyeloplasty after observation. The choice of therapy was dependent upon ultrasound determination of the grade of hydronephrosis and degree of parenchymal thinning, and renal function on nuclear renal scan. The following findings were noted:

Immediate pyeloplasty performed in 32 children (28 percent) resulted in a pre- and postoperative change of average renal function (measured in 27 patients) from 30 to 39 percent and median grade of hydronephrosis from 4 to 3. Observational management performed in 37 patients (32 percent) resulted in a change of median hydronephrosis from grade 4 to 2.25 in 29 patients during an 18-month follow-up. In 15 patients, despite worsening hydronephrosis, repeat renal scans demonstrated stable renal function. Delayed pyeloplasty occurred in 47 patients (41 percent). Before and after pyeloplasty, relative renal function changed from 36 to 42 percent, and median grade of hydronephrosis from 4 to 3. Surgical complications occurred in four patients (3 percent), including uncomplicated bowel injury, urine extravasation, persistent hematuria, and incisional abscess. These were all treated conservatively. Of the 33 symptomatic patients who presented postnatally, 29 underwent pyeloplasty. Of the 83 patients with antenatal presentation, 50 were surgically corrected. The authors concluded no one diagnostic study could determine when surgical intervention was needed. Their experience suggested that initial renal function based upon renal scan and serial ultrasonographic monitoring were the findings most commonly used in determining whether or not to perform pyeloplasty. In many patients, conservative management of observation alone had no adverse effect, and in some cases, demonstrated improvement.

Similar results were seen in a Belgium case series of 81 patients with antenatally diagnosed unilateral UPJ obstruction with an anteroposterior renal pelvic diameter 15 mm. In this cohort, early pyeloplasty was performed in eight patients (10 percent) based on evidence of differential renal function (DRF), and in one case parental choice, and late pyeloplasty in 23 patients (28 percent) who were initially managed conservatively because of evidence of deteriorating or persistently low DRF, increasing hydronephrosis, poor renal emptying, urinary tract infection, and one case each of nephrolithiasis and parental choice. Postoperative assessment in 28 of the 31 patients showed improved, stable, or poorer DRF in 7, 19 and 2 patients, respectively. Conservative management was performed in the remaining 50 patients (62 percent). The following sections discuss the two management approaches of observation and surgical repair. Observation For experts in the field who consider observation an option, the main criterion for observation in cases with significant hydronephrosis on sonogram is demonstration of greater than 40 percent of split renal function of the affected kidney by diuretic renography, even if washout is delayed [15-17]. This threshold value indicates that the narrowing of the UPJ is not impairing renal function. In these patients, serial renal ultrasounds are obtained to monitor any change in the degree of hydronephrosis that indicate changes in functional obstruction. Renal ultrasound examinations are performed every four months until the child reaches one year of age, every six months for the next two years, and then annually [18]. If the renal sonogram shows increasing hydronephrosis, diuretic renography is repeated to confirm that renal function is stable. If renography shows deterioration of >10 percent on the affected side, or relative function less than 40 percent, surgery is recommended. These studies are performed more frequently (every three to four months) if relative renal function is borderline, and significant hydronephrosis is present. Of note, relative renal function may not be a reliable measure in patients with bilateral involvement. In patients with bilateral disease, glomerular filtration rate can be measured with serum creatinine and/or by renal scan. If renal function is significantly decreased, surgery is indicated to relieve obstruction. Although antibiotic prophylaxis has been recommended because urinary stasis is believed to be a risk factor for urinary tract infection (UTI), it does not appear that it is beneficial in children with UPJ obstruction.

In one study of 92 children with severe hydronephrosis secondary to UPJ obstruction who were not treated with antibiotic prophylaxis, UTI occurred in 4.3 percent of the patients after a mean follow-up time of 27 months [19]. The mean age at the time of UTI was 6.1 months. All four patients with UTI presented with fever, and one developed urosepsis. The authors concluded that antibiotic prophylaxis was not beneficial in patients with severe obstruction due to UPJ obstruction. In a Turkish prospective study, there were no UTIs or evidence of renal scarring based on dimercaptosuccinate renography in 84 infants with UPJ obstruction who were not treated with prophylactic antibiotics at the end of one year follow-up [20]. As a result, in our practice, antibiotic prophylaxis is given in patients with severe, grade IV hydronephrosis until the time of the voiding cystourethrogram (VCUG). If the VCUG does not show reflux, the antibiotics are discontinued. Surgery Many clinicians recommend surgery when significant hydronephrosis (Grade IV) is noted on the sonogram regardless of the relative renal function of the two kidneys. The argument for surgical intervention is that function may be preserved or improved with correction of the blockage [16].

Surgical options Surgical options include the following:

Open dismembered pyeloplasty The standard surgical procedure is open dismembered pyeloplasty [6]. Outcomes are excellent, with resolution of the obstruction in 90 to 95 percent of cases, including newborns [21,22]. Ultrasound examination is repeated approximately four to six weeks after surgery. If the hydronephrosis does not improve, a diuretic renogram should be performed to assess for persistent obstruction and/or a loss of renal function. Laparoscopic pyeloplasty Laparoscopic pyeloplasty in infants and children is performed in some centers, with excellent short-term results [23-28]. Robotic pyeloplasty also is becoming routinely used, especially in the older child [29,30]. However, in newborns, overall morbidity may be less with open pyeloplasty than with laparoscopic or robotic techniques, because the classic pyeloplasty incision is the same length as the incision needed for the combined laparoscopic ports, and postoperative morbidity is similar. In general, laparoscopic and robotic techniques are performed transperitoneal in contrast to retroperitoneal for open pyeloplasty. Laparoscopic and robotic techniques also require postoperative placement of internal stents, increasing the economic burden of pyeloplasty, that are subsequently removed under general anesthesia; these are not usually needed with open procedures [31]. Others Alternative surgical techniques, such as percutaneous and retrograde endopyelotomy, have been used in adults. However, experience is limited in children [32]. Our approach Based upon our clinical experience, observation is appropriate for asymptomatic patients with UPJ type hydronephrosis that is mild to moderate in severity (ie, Society of Fetal Urology [SFU] grade II to III) (image 3). We follow these patients with ultrasound examination every four (Grade III) to six (Grade II) months in the first year of life, then every 12 to 18 months thereafter. If there is an increase in the degree of hydronephrosis, diuretic renography is performed. If the affected kidney has less than 40 percent of split renal function, or there is a serial loss greater than 10 percent from a previous study, surgical intervention is recommended. Newborns who have persistent SFU grade IV hydronephrosis require closer observation. In these patients, we obtain a diuretic renogram at six to eight weeks of age. If the hydronephrotic kidney has decreased renal function (less than 40 percent of split renal function) compared with its normal contralateral mate, we recommend surgical intervention. More commonly, the hydronephrotic kidney will have equal function compared with the normal contralateral kidney. In this case, we repeat a sonogram in four to six weeks. If the sonogram shows improvement, we continue to follow with serial ultrasounds at three to four month intervals. If we see any worsening of the hydronephrosis, we repeat a diuretic renogram. These serial sonograms usually show continued SFU grade IV hydronephrosis without substantial change from previous studies. In this case, we offer the family either continued observation, which will require ongoing monitoring with sonograms and possible diuretic renography, or surgical intervention. Of note, in our practice, the washout curve or half-life calculation is not typically used to determine the need for surgery in any of these patients; however, others use a delay in excretion indicative of obstruction and renal function in considering surgical correction. The relative renal function typically does not change if the initial and follow-up renograms demonstrate stable renal function. Surgical intervention is performed for the following indications:

Symptoms including pain, infection, and renal stones.

Increasing grade of hydronephrosis, and decrease in function of the affected kidney so that it is less than the threshold of 40 percent of split renal function, or there is a serial loss greater than 10 percent. Parental and/or patient preference to avoid continued invasive testing, and allow definitive correction of the problem. Massive hydronephrosis with a renal pelvic diameter >50 mm. This approach appears to limit surgical correction to one-half of patients diagnosed with antenatal UPJ obstruction. This was illustrated in a long-term retrospective study of 343 children with UPJ obstruction detected by antenatal ultrasound that reported 52 percent of patients underwent surgical correction based upon renal deterioration of greater than 5 percent [33]. In this cohort of patients, 235 patients had initial relative renal function (RRF) >40 percent, 68 had RRF between 30 and 40 percent, and 40 had RRF <40 percent. Initial RRF <40 percent and initial grade III and IV hydronephrosis were independent predictors for surgery. Postoperative follow-up Renal ultrasonography is performed four to six weeks postoperatively. If the ultrasound shows improvement (ie, decrease in the severity of hydronephrosis), the patient can then be followed by repeat ultrasound at increasing time intervals (ie, initially one year and then every two to three years) [34]. If the followup ultrasound does not show improvement, diuretic renography is performed to detect persistent obstruction, which may require an additional surgical procedure. OUTCOME There are limited data on the long-term outcome of children with ureteropelvic junction (UPJ) obstruction, which is confined to those who underwent pyeloplasty. In one series of 49 postpubertal adolescents with antenatally diagnosed UPJ obstruction who underwent surgical repair between 1989 and 1992, relative renal function improved from 37 percent preoperative to 43 percent after puberty [35]. There was also a decrease in time for isotope washout. Two patients (4 percent) required additional surgery for increasing hydronephrosis. Another study of 401 children who underwent open pyeloplasty reported a similar rate of 5 percent of recurrent obstruction [36]. It remains unknown what proportion of patients with congenital UPJ obstruction who do not undergo surgical correction will become symptomatic as adultsand/or require surgical intervention. It also is not known whether there is a difference in long-term renal function between the observational and initial surgical approaches. It is clear that long-term follow up studies are needed to answer these questions so that optimal management decisions can be made. INFORMATION FOR PATIENTS UpToDate offers two types of patient education materials, The Basics and Beyond the Basics. The Basics patient education pieces are written in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon. Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on patient info and the keyword(s) of interest.)

Basics topics (see "Patient information: Prenatal hydronephrosis (The Basics)")

SUMMARY AND RECOMMENDATIONS

Ureteropelvic junction (UPJ) obstruction is a partial or total blockage of the flow of urine that occurs where the ureter enters the kidney, and results in hydronephrosis (figure 1 and picture 1). (See 'Pathophysiology' above.) Although the etiology of UPJ obstruction includes both congenital and acquired conditions, congenital causes are more common. UPJ obstruction is the most common pathologic cause of antenatally detected hydronephrosis with a reported incidence of up to 1 in 500 fetuses screened by antenatal ultrasound. (See 'Epidemiology' above.) Congenital UPJ obstruction is usually caused by intrinsic stenosis of the proximal ureter, less commonly by extrinsic compression of the UPJ by an aberrant or accessory renal artery or arterial branch, and rarely from an intraluminal fold. (See 'Etiology' above.) In the developed world, most cases of UPJ obstruction are diagnosed during the postnatal evaluation of antenatal hydronephrosis. In the absence of antenatal screening, infants may present with an abdominal mass (enlarged obstructed kidney), urinary tract infection, hematuria, or failure to thrive. In older children, the most common clinical manifestations of UPJ obstruction are intermittent flank and abdominal pain. Other symptoms include renal injury after experiencing minor trauma, hematuria, renal calculi, or rarely hypertension. (See 'Clinical presentation' above.) The diagnosis of UPJ obstruction is generally suspected when imaging studies, usually ultrasonography, demonstrate hydronephrosis. The diagnosis is confirmed by diuretic renography. (See 'Diagnosis' above.) Imaging studies (eg, diuretic renography, serial ultrasounds, and voiding cystourethrogram [VCUG]) differentiate UPJ from other causes of hydronephrosis, including vesicoureteral reflux (VUR), transient and functional hydronephrosis, and other urological anomalies (eg, posterior urethral valves, congenital megaureter, ureterocele, and multicystic dysplastic kidney). (See 'Differential diagnosis' above.) The initial evaluation of a patient with suspected UPJ obstruction includes ultrasonography to demonstrate persistent hydronephrosis, VCUG to detect the presence or absence of VUR, and a diuretic renogram that demonstrates the delay in isotope clearance at the UPJ confirming partial or total blockage of urinary flow. (See 'Evaluation' above.) There are no randomized trials that provide evidence for the optimal management of congenital UPJ obstruction. The following management regimen is based upon observational studies, and the experience and opinions of the author. We suggest NOT to perform prenatal intervention or early delivery in fetuses with unilateral UPJ obstruction (Grade 2C). In extremely rare cases, decompression of an enormously dilated renal pelvis may be considered to prevent dystocia or fetal pulmonary compression. (See 'Prenatal' above.) In symptomatic patients, we recommend surgical intervention to relieve the obstruction and resolve symptoms (Grade 1B). (See 'Symptomatic patients' above.) In asymptomatic patients with unilateral UPJ obstruction and a split renal function greater than 40 percent of the affected kidney, we suggest observation and monitoring with serial ultrasounds and diuretic renography (Grade 2C). Indications for surgical intervention include increasing hydronephrosis and decrease in split renal function below 40 percent or a serial loss greater than 10 percent in subsequent studies, the development of symptoms, massive hydronephrosis with a renal pelvic diameter greater than 50 mm, and/or parental and patient preference. This approach limits surgical correction to about half of affected children. (See 'Asymptomatic patients' above.) Limited long-term data confined to children who underwent surgical correction demonstrate a correction of the obstruction defined by stable or improved renal function, decreased hydronephrosis, and lack of symptoms in

most patients. The rate of recurrent obstruction requiring additional intervention is about 5 percent. Long-term data on patients who were managed by observation alone are not currently available. (See 'Outcome' above.) Use of UpToDate is subject to the Subscription and License Agreement. REFERENCES
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