Pediatr Clin N Am 53 (2006) 429 447

Prenatal Hydronephrosis
Sergio Fefer, MDa, Pamela Ellsworth, MDb,T
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Division of Urology, University of Massachusetts Memorial Hospital, 55 Lake Avenue North, Worcester, MA 01655, USA b Brown University and University Urological Associates, Providence, RI 02905, USA

A 22-year-old pregnant woman presented to the obstetrics and gynecology clinic for her first obstetric examination accompanied by her husband and her 5-year-old daughter. A prenatal ultrasound for dates was obtained, and it revealed an 8-week embryo well implanted in the uterus. She returned for a repeat ultrasound at 20 weeks. The fetus appeared appropriate size for gestational age. The placenta was well developed, as was the cranium, and the thoracicabdominal ratio was normal. The right kidney appeared normal, but the left kidney was abnormal. The left renal pelvis was dilated and measured 20 mm. The amniotic fluid volume was normal. A follow-up ultrasound obtained 6 weeks later demonstrated that the left renal pelvis was 25 mm and the sex of the fetus was male. The parents were referred to a pediatric urologist for prenatal consultation. The pediatric urologist discussed the recommended postnatal evaluation with the parents, which decreased their anxiety. The use of prenatal ultrasound has increased significantly over the past 20 years. In 1980, prenatal ultrasound was performed in 33% of pregnancies, whereas prenatal ultrasound was performed in 78% of pregnancies in 1987. The incidence of a significant structural abnormality detected by prenatal ultrasound is 1% [1]. The rate of prenatal abnormalities detected by screening ultrasound varies with the timing of fetal imaging, however. Detection rates increase when ultrasound is performed at midtrimester compared with earlier scanning [24]. Abnormalities of the genitourinary tract rank second in frequency of structural abnormalities on screening ultrasound. Approximately 50% of the structural abnormalities involve the central nervous system, 20% involve the genitourinary tract, 15% involve the gastrointestinal tract, and 8% involve the cardiopulmonary

T Corresponding author. E-mail address: Pamelaellsworth@aol.com (P. Ellsworth). 0031-3955/06/$ see front matter D 2006 Elsevier Inc. All rights reserved. doi:10.1016/j.pcl.2006.02.012 pediatric.theclinics.com

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system [5]. Hydronephrosis is the most common genitourinary tract anomaly detected on prenatal ultrasound studies. Not all cases of prenatally detected hydronephrosis are clinically significant. Several studies have assessed the threshold for diagnosing fetal hydronephrosis associated with persistent renal anomalies [6,7]. In most of these studies, persistent postnatal renal abnormalities are noted when the anteroposterior diameter (APD) of the fetal renal pelvis measures N6 mm at b20 weeks, N8 mm at 20 to 30 weeks, and N10 mm at N30 weeks gestation. To further characterize the dilatation of the collecting system and correlate fetal hydronephrosis with postnatal clinical relevance, a grading scale was developed for fetuses older than 20 weeks gestation Grade I: pelvic APD is 1 cm and the calyces are normal Grade II: pelvic APD is 1 to 1.5 cm but the calyces remain normal Grade III: pelvic APD is N1.5 cm and there is slight caliectasis Grade IV: pelvic APD N1.5 cm with moderate caliectasis Grade V: APD N1.5 cm with severe caliectasis and thinning of the renal cortex (b2 mm thick) (Figs. 14) [8,9]. The grade of hydronephrosis has been demonstrated to correlate with the potential for resolution of the hydronephrosis. Grade I hydronephrosis resolves in approximately 50% of patients, whereas grades II, III, and IV hydronephrosis resolve in 36%, 16%, and 3% of cases, respectively [10].

Fig. 1. Grade 1 hydronephrosis: renal pelvis is dilated without dilatation of the renal calices. (From Shimada K, Kakizaki H, Kubota M, et al. Standard methodology for diagnosing dilatation of the renal pelvis and ureter discovered in the fetus, neonate or infant. Int J Urol 2004;11(3):130; with permission.)

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Fig. 2. Grade 2 hydronephrosis: dilation of the renal pelvis. Some of the calices are dilated. (From Shimada K, Kakizaki H, Kubota M, et al. Standard methodology for diagnosing dilatation of the renal pelvis and ureter discovered in the fetus, neonate or infant. Int J Urol 2004;11(3):131; with permission.)

Although hydronephrosis may be a component of various congenital syndromes, isolated unilateral dilatation of the collecting system is the most frequent abnormality seen on prenatal ultrasound. Prenatal hydronephrosis is associated with various conditions, which vary in severity and prognosis and range from urethral atresia with complete urinary obstruction and fetal demise to transient physiologic dilatation of the collecting system that resolves spontaneously without sequelae. In addition to an assessment of the kidneys, the

Fig. 3. Grade 3 hydronephrosis: dilatation of the renal pelvis. All of calices are dilated. (From Shimada K, Kakizaki H, Kubota M, et al. Standard methodology for diagnosing dilatation of the renal pelvis and ureter discovered in the fetus, neonate or infant. Int J Urol 2004;11(3):131; with permission.)

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Fig. 4. Grade 4 hydronephrosis: calices are protruded in shape and extend into the parenchyma. (From Shimada K, Kakizaki H, Kubota M, et al. Standard methodology for diagnosing dilatation of the renal pelvis and ureter discovered in the fetus, neonate or infant. Int J Urol 2004;11(3):131; with permission.)

prenatal ultrasound should evaluate the ureters, bladder, amniotic fluid volume, and the fetal sex. When hydronephrosis is identified in one kidney it is important to determine whether there is a contralateral kidney and whether it appears normal. If the contralateral kidney is normal in appearance, fetal survival on the basis of the genitourinary abnormality is typically not compromised. On the contrary, bilateral hydronephrosis is more concerning, particularly in a male infant, and careful evaluation of the remainder of the genitourinary tract and amniotic fluid volume is indicated. A thickened bladder wall, distended bladder, and posterior urethral dilation in the setting of severe unilateral hydronephrosis or bilateral hydronephrosis are more indicative of posterior urethral valves. A cystic mass within the bladder associated with unilateral or bilateral hydronephrosis is consistent with a ureterocele. Several ultrasonographic findings are also helpful in the evaluation of unilateral fetal hydronephrosis. The demonstration of collecting system dilatation that is limited to the renal pelvis and calyces suggests ureteropelvic junction obstruction (UPJO). Although normal ureters are usually not visualized in the fetus, dilated ureters become easily recognizable and might indicate the presence of ureterovesicular junction obstruction, high-grade vesicoureteral reflux (VUR), mega-ureter, ureterocele, or ectopic ureter (Table 1). The major concern when facing a fetus with bilateral hydronephrosis is the presence of oligohydramnios as a result of significant urinary tract obstruction, which could adversely affect pulmonary development. The timing of obstruction is important, because early significant obstruction may result in renal dysplasia and, consequently, loss of renal function. The quantification of the amount of amniotic fluid is an overall sign of fetal health and indirectly points toward the severity of urinary tract obstruction. Fetal urine formation begins between the tenth and twelfth week of gestation [11]. As a fetus begins to urinate and swallow at approximately 11 weeks gestation, these physiologic functions become primary determinants of amniotic fluid volume and

prenatal hydronephrosis Table 1 Causes of antenatal hydronephrosis and ultrasound characteristics Ipsilateral ureter UPJO Vesicoureteral reflux Ureterocele Ectopic ureter Posterior urethral valves Multicystic kidney Primary obstructive or nonref luxing nonobstructing mega-ureter Urethral atresia Retrocaval ureter Prune belly syndrome Normal Dilated or normal Dilated Often dilated Often dilated (bilateral) Normal Dilated Dilated (bilateral) Dilated proximal and normal distal Dilated Bladder

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Normal Dilated or normal Dilated or normal (cystic massureterocele) Normal Thick wall, increased postvoid residual Normal Normal Thick wall, not emptying (oligohydramnios) Normal Dilated

composition [12,13]. The amniotic fluid volume reflects renal function and patency of the genitourinary tract. Oligohydramnios may have a significant impact on fetal survival. Pulmonary hypoplasia is the most common cause for mortality in neonates with obstructive uropathy. The most predictive factor in assessing the risk of pulmonary hypoplasia is the presence of mid-gestational oligohydramnios. Nakayami and colleagues [14] noted that in neonates with posterior urethral valves there is a 45% mortality rate, mostly because of pulmonary insufficiency. Early prenatal detection of urinary tract malformations associated with oligohydramnios affords the opportunity for prenatal intervention in select cases. Currently, fetal treatment programs recommend treatment only for fetuses at risk for neonatal death [15]. Before intervention for oligohydramnios it is important to assess the likelihood of salvageable renal function and document a normal karyotype. Currently, renal ultrasound and urine electrolytes are used to determine the likelihood of salvageable renal function. Urine electrolyte values associated with a good outcome include a urine sodium b100 mmol/L, a chloride b90 mmol/L and osm b210 mOsm/L [16]. The sensitivity of the urine electrolytes may be enhanced by sequential aspiration and analysis. Other new markers of renal function include beta-2 microglobulin, alpha-microglobulin, and retinal binding protein [16]. The vesicoamniotic shunt is the primary treatment modality for severe oligohydramnios associated with bladder outlet obstruction. It is a small, hollow catheter in which one end is placed into the bladder and the other into the amniotic cavity, which allows fetal urine to drain into the amniotic space. Initial results with vesicoamniotic shunts were disappointing; there was a 4.6% procedure-related mortality rate and overall survival rate of only 41% [17]. Refinements in technique and the use of antibiotics have improved the morbidity associated with the procedure and increased postnatal survival rates to 67% [17]. Intrauterine follow-up of fetuses with unilateral hydronephrosis is controversial. Although a dynamic, longitudinal evaluation of the urinary tract with serial

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ultrasound is important in cases of bilateral hydronephrosis, the role of third trimester ultrasound in cases of unilateral hydronephrosis is less clear. In the setting of unilateral hydronephrosis, if the hydronephrosis increases in subsequent ultrasound studies, in utero intervention or early delivery still would not be indicated.

Postnatal assessment Physical examination of newborns with unilateral antenatal hydronephrosis is usually normal. A palpable, transilluminating abdominal mass occasionally may be present and is associated with cases of severe UPJO or multicystic kidney disease. A distended bladder might be identified as a palpable mass in the suprapubic area in infants and can lead to the diagnosis of bladder outlet obstruction by posterior urethral valves in boys or obstructing ectopic ureterocele in girls. The observation of spontaneous voiding with normal urinary stream within the first 24 hours of life does not rule out an obstructive process. On the contrary, failure to void within the first 48 hours of life favors the diagnosis of obstructive uropathy, such as posterior urethral valves or urethral atresia. Urethral atresia is usually not compatible with life because of the associated severe oligohydramnios present in utero. Postnatal serum creatinine levels are rarely obtained with unilateral hydronephrosis and a normal contralateral kidney but are indicated when there are abnormalities of both kidneys. Interpretation of the serum creatinine obtained during the first day or two of life is limited because the value reflects the mothers creatinine. If the serum creatinine is elevated at the time of initial evaluation, serial creatinine levels should be obtained until the levels normalize or plateau. Newborns delivered at term should have serum creatinine levels at approximately 0.4 mg/dL at the end of their first week of life. A serum creatinine of N0.8 at 1 year of life is associated with an increased risk of renal insufficiency [18,19].

Postnatal management Newborns with antenatal diagnosis of hydronephrosis should be started on prophylactic antibiotics after birth until follow-up radiographic studies are obtained. Traditionally, amoxicillin is used as the prophylactic antibiotic of choice in newborns. At 8 weeks of life, when an infants liver is mature, trimethoprim-sulfamethoxazole or nitrofurantoin may be used if continued prophylaxis is warranted. All infants with prenatally detected hydronephrosis should undergo a postnatal renal/bladder ultrasound study (Fig. 5). Concern has been raised regarding the timing of the postnatal renal/bladder ultrasound. It was initially believed that in the absence of suspicion of potential life-threatening anomalies (eg, posterior urethral valves), a postnatal renal ultrasound should not be performed until after

Prenatal hydronephrosis

Girls

Boys

Prophylaxis Postnatal US VCUG

Prophylaxis Postnatal US VCUG

VUR

Negative VCUG VUR

Negative VCUG

Continue prophylaxis

Unilateral or Bilateral Hydro

If high grade, consider DMSA to assess for dysplasia

prenatal hydronephrosis

VCUG and US at 1 yr Consider circumcision

Mild to moderate

Severe

Mild to moderate

Moderate to severe VCUG and US at 1 yr Lasix Mag3 Renal scan at 1 month Repeat US

Repeat renal US

Lasix Mag3 renal scan

Repeat renal US

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Fig. 5. Postnatal management scheme for prenatal hydronephrosis.

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48 hours of life. The rationale for this decision was the premise that an initial low glomerular filtration rate coupled with relative dehydration caused by poor feeding could lead to oliguria during the first 48 hours of life and underestimate the degree of renal dilatation in a partially obstructed system. Docimo and Silver [20] reviewed the records of 101 neonates with prenatally detected hydronephrosis who underwent sonography within 48 hours of birth. Thirty-three had a normal postnatal study (either no or mild hydronephrosis) and documented follow-up studies. None of the children had a significant obstructive renal lesion within the first year of life, and 1 had an obstructive pattern on diuretic renography at 18 months of age after previous studies were unremarkable. Wiener and OHara [21] performed a prospective study that compared prenatal ultrasound findings at 48 hours of birth to those at 7 to 10 days of life. The authors noted that the grade of hydronephrosis changed between the initial sonogram in the first 48 hours of life and the second sonogram at 7 to 10 days in most evaluable renal units. In those renal units with significant uropathy, however, there was no significant difference in the grade of hydronephrosis between the two ultrasounds. Although it is ideal to obtain the postnatal renal ultrasound at approximately 7 days of life, in individuals in whom compliance or other factors may prevent this, a renal ultrasound obtained at 48 hours of life is acceptable. Postnatal persistence of prenatally diagnosed hydronephrosis requires further evaluation. The Society for Fetal Urology developed a grading scale to assess the severity of postnatal urinary tract dilatation (Table 2). The degree of dilatation of the collecting system and thickness of renal parenchyma are the cornerstones of this grading system. There is a strong correlation between the grade of hydronephrosis and the likelihood of surgical intervention being required. Similar to the prenatal scenario, ultrasound examination of the remainder of the genitourinary tract, including ureters, bladder, and urethra, is important in establishing the final diagnosis. The presence of persistent unilateral hydronephrosis on postnatal ultrasound requires further evaluation to determine the cause (Fig. 5.) In these cases, prophylactic antibiotic are continued until further radiologic evaluation is completed. A voiding cystourethrogram is obtained to evaluate for ipsilateral VUR and, in boys, posterior urethral valves. Further evaluation with a furosemide 99mTc mercaptoacetyltri-glycine (lasix Mag3) renal scan is determined by the grade of hydronephrosis and the presence/absence of VUR. Infants with postnatal mild to moderate hydronephrosis should undergo a repeat ultrasound in lieu of a diuretic
Table 2 Society for Fetal Urology grading system of congenital hydronephrosis Grade 0 1 2 3 4 Central renal complex Intact Slight splitting of the pelvis Evident splitting of pelvis and calices Wide splitting of pelvis and calices Further splitting of pelvis and calices Renal parenchyma Normal Normal Normal Normal Reduced

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renal scan. In the setting of postnatal moderate to severe hydronephrosis, a lasix Mag3 renal scan is obtained typically at 1 month of age when the kidneys are more mature. Two parameters are evaluated on the lasix Mag3 renal scan: (1) the split renal function and (2) the half time (tO), the time it takes for half of the radionuclide to leave the renal collecting system. Normal differential renal function is believed to be 45 to 50/55 to 50. If the renal function of the hydronephrotic kidney is b40%, compromised function is present [22,23]. A normal half-time is less than 10 minutes, and a half-time that indicates some element of obstruction is more than 20 minutes. The area between 10 and 20 minutes is an indeterminate region and warrants continued observation. There are limitations in the assessment of half-time, including the state of hydration, the renal function, the volume and contractility of the renal pelvis, patient position, bladder filling, and timing and dose of diuretic administration. A combination of split renal function and half-time is often used to determine if a significant obstruction is present. Careful review of the postnatal ultrasound and the lasix Mag3 renal scan is important to assess the location of the obstruction. The absence of an ipsilateral dilated ureter confirms a UPJO, whereas the presence of ipsilateral ureteral dilatation suggests a mega-ureter, obstructive or nonobstructive. The subsequent evaluation of an infant with prenatally detected hydronephrosis whose postnatal ultrasound is normal is controversial. The primary area of controversy is the role of postnatal VCUG in this setting. Recent publications showed that routine use of VCUG to evaluate all newborns with prenatal diagnosis of hydronephrosis resulted in detection of VUR in only 12% to 21% of the cases [24,25]. Although VCUG is commonly performed, it is an invasive test that exposes infants to ionizing radiation and carries the risk of new onset of urinary tract infection (UTI) [2628]. In the setting of a normal postnatal ultrasound, the pros and cons of a VCUG should be discussed with the family. Several attempts have been made to identify infants with the highest chance of having VUR by carefully evaluating the prenatal ultrasound, with the goal of reducing unnecessary postnatal invasive testing. Herndon and colleagues [29] noted that the prenatal ultrasound characteristics that support the presence of VUR include bilateral mild to moderate renal pelvic dilation, dilatation of the collecting system that increases with voiding during gestation, visualization of a ureter, and family history of reflux.

Causes of prenatal hydronephrosis Ureteropelvic junction obstruction UPJO accounts for 44% to 65% of the cases of prenatal hydronephrosis [10]. UPJO occurs in 1 in 2000 live births, boys are more commonly affected, and 90% of cases are unilateral [30]. The most common cause of UPJO in the pediatric

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population is an adynamic ureteral segment at the junction between the ureter and the renal pelvis, the ureteropelvic junction. This nonfunctional ureteral segment creates a resistance that compromises urine passage from the renal pelvis into the proximal ureter. Extrinsic compression of the proximal ureter by the presence of accessory lower pole renal vessels may cause UPJO in children, but it is more commonly seen in adults [31]. Rarely, hydronephrosis may be the result of intrinsic anomalies, such as ureteral valves or polyps or extrinsic bands. Historically, a child with a UPJO presented with a palpable abdominal mass or UTI during the first and second year of life or gastrointestinal complaints in older children. In utero identification is currently the most common presentation of UPJO. Concerns have been raised that in utero identification and early postnatal confirmation will lead to an increased number of surgical procedures being performed in children with asymptomatic urinary tract dilatation. A hospital-based study by Brown and colleagues [32] supported this concern. Wiener and colleagues [33] examined the annual rate of pyeloplasty in the population before the advent of maternal ultrasound and compared it to the rate after the introduction of maternal ultrasound, however, and concluded that maternal ultrasound did not lead to an overdiagnosis of UPJO but rather the detection of the disease at an earlier age. This finding suggests that prenatally and

Fig. 6. Postnatal Lasix Mag 3 renal scan demonstrates decreased renal function. Percent renal function at 2 minutes is 33.8% left and 66.2% right.

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postnatally detected hydronephrosis represents a continuous spectrum of the same disease entity. A corollary of early detection is the question of timing of intervention. Is it better to intervene earlier or observe and intervene when the obstruction is noted to compromise renal function? With the advent of diuretic renography the initial emphasis was placed on the drainage time (tO) and drainage curve. In the 1980s, Ransley and colleagues [22] proposed that the emphasis should be placed on the renal function instead of the drainage curve and half-time drainage (Figs. 6 and 7). The critical value of renal function suggestive of obstruction is controversial. Recommendations were made by several authors that hydronephrosis with ipsilateral differential renal function more than 30% to 40% should be treated conservatively with periodic renal ultrasound studies and diuretic renography [22,23]. Several authors have demonstrated that in most children with UPJO conservative management is safe. Only 10% to 25% of children followed conservatively ultimately require surgical intervention, and most children who require surgical intervention do so within the first 2 years of life [22,34]. In children in whom the hydronephrosis does not resolve during follow-up, it takes approximately 30 months for maximal ultrasound improvement in the hydronephrosis [34].

Fig. 7. Postnatal Lasix Mag 3 renal scan in same patient demonstrates abnormal drainage curve for left kidney.

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In children without a significant obstruction, the optimal timing for subsequent studies is not well established. The timing of studies often varies with the degree of dilatation on ultrasound and the split renal function and half-time on renal scan. Dhillon [35] recommends earlier and more frequent isotope scans in neonates with a severe degree of dilatation (APD N20 mm) or calyceal involvement or infants with more than 40% renal function at 3 months of age whose dilatation persists on subsequent renal ultrasounds. The duration and follow-up for children with persistent hydronephrosis remains unclear and varies with the degree of dilatation. Surgical intervention Currently, the presence of symptoms, declining renal function on renal scan, and increasing hydronephrosis on ultrasound are clear indications for surgical correction. A decrease in renal function of 10% or more on subsequent renal scan is believed to indicate high-grade obstruction and warrants surgical intervention [22,23]. A study performed by the Society of Fetal Urology in infants and children with high-grade obstructive hydronephrosis concluded that children b6 months of age with high-grade obstructive unilateral hydronephrosis with good renal function were better served by pyeloplasty than observation [36]. Surgical correction of UPJO involves excision of the adynamic portion, tailoring of the redundant renal pelvis, and reapproximation of the ureter in a dependent fashion to the renal pelvis. This procedure is traditionally performed through a retroperitoneal approach, either by an incision on the back (dorsal lumbotomy) or, more commonly, a transverse incision on the lateral aspect of the abdomen. More recently, pediatric urologists with advanced laparoscopic skills have performed the procedure laparoscopically [37].

Vesicoureteral reflux VUR is often believed to be a benign condition in the absence of UTIs. The big bang theory of renal scarring suggests that first febrile UTI often may be a cause of renal scarring. Uncircumcised infant boys have a higher risk of UTIs in the first year of life than girls. Infant boys with prenatally detected VUR (hydronephrosis on prenatal ultrasound) tend to have higher grades of VUR, which may be associated with renal dysplasia [38]. In infants with prenatally detected hydronephrosis, postnatal management with prophylaxis and subsequent VCUG allows for earlier detection of infants at greater risk for UTIs and possible scarring. Unfortunately, postnatal renal ultrasound has little value in the diagnosis of VUR. Postnatal ultrasound results are frequently normal in most cases of VUR identified by prenatal ultrasound. Some authors have argued that VUR detected in the context of a normal postnatal ultrasound is a self-limiting condition that could be left alone. Others

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have demonstrated no correlation between the degree of prenatal or postnatal grade of hydronephrosis and the grade of VUR. Of the children with prenatal hydronephrosis in one study, there was no postnatal hydronephrosis in 27% of the grade 25 refluxing units [24]. One should consider performing a postnatal VCUG to evaluate for reflux even in infants with a normal postnatal ultrasound, particularly infants with prenatal ultrasound findings that suggested VUR [29]. Children with prenatally detected VUR are managed similarly to children with UTI-related reflux. They are placed on antibiotic prophylaxis and followed with sequential renal ultrasound and VCUG to assess renal growth and resolution of VUR. The indications for surgical correction of prenatally detected VUR are the same as those for UTI-related VUR, including breakthrough UTIs, persistence of reflux, and poor compliance. Posterior urethral valves Posterior urethral valves represent a congenital valvular obstruction caused by a mucosal membrane in the posterior urethra that results in different grades of bladder outlet obstruction and proximal urinary tract dilatation. The incidence of posterior urethral valves is generally accepted to be between 1 in 5000 and 1 in 8000 live male births [39]. Posterior urethral valves represents 3% to 9% of all prenatally diagnosed cases of hydronephrosis [40,41]. Prenatal ultrasound findings that suggest posterior urethral valves include enlarged bladder, thickened bladder wall, posterior urethral dilation, unilateral or bilateral hydronephrosis, increased renal echogenicity, and oligohydramnios (Fig. 8) [42]. Such ultrasound anomalies suggestbut are not definitive forposterior urethral valves. El Ghoneimi and colleagues [43] noted that only 53% of male fetuses with megacystis and bilateral hydronephrosis had posterior urethral valves. The detection of posterior urethral valves after 24 weeks gestation is associated with a better prognosis than cases detected before 24 weeks [42]. Second trimester findings that portend a poor postnatal outcome include moderate or severe upper tract dilation, renal pelvic APD N10 mm, and increased echogenicity or cystic changes in the renal parenchyma. In male infants with oligohydramnios and salvageable renal function, early detection and management via placement of a vesicoamniotic shunt may enhance survival by improving pulmonary function. Currently limited data are available to demonstrate that prenatal detection and intervention with vesicoamniotic shunt in high-risk neonates improve renal function. Early identification and intervention decrease the complications of postnatal septicemia and uremia, however. Male newborns with severe bilateral hydronephrosis and oligohydramnios require emergent postnatal medical management for correction of pulmonary dysfunction and electrolyte disturbances. A catheter should be placed immediately and urine output should be monitored. Once stabilized, a newborn should undergo a VCUG to confirm the presence of posterior urethral valves. If valves are detected, transurethral surgical ablation of the posterior urethral valves is performed. Follow-up imaging is required to ensure that the obstruction is

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Fig. 8. Prenatal ultrasound appearance of severe posterior urethral valves in a male fetus. The bladder is moderately distended and there is severe dilatation of the collecting system of both kidneys with normal appearing renal parenchyma. (From Peters CA. Perinatal urology. In: Walsh OC, Retik AB, Vaughn ED, et al, editors. Campbells urology. 8th edition. Philadelphia: WB Saunders; 2002. p. 1787.)

relieved and to evaluate bladder emptying and upper tract dilatation. Further evaluation and management of infants with posterior urethral valves are dictated by their renal function, bladder function, and the presence/absence of other anomalies, such as high-grade reflux or a nonfunctioning kidney. A nadir serum creatinine of N0.8 mg/dL at 1 year of age indicates a long-term risk of renal insufficiency [18,19].

Ureterocele A ureterocele is a cystic dilatation of the distal portion of the ureter. Although most ureteroceles present in girls (female:male ratio of 4:1) and in association with ureteral duplication (80%), multiple anatomic variants and clinical presentations have been reported [44,45]. More frequently the ureterocele is associated with obstruction of the upper pole moiety of a duplicated collecting system, and the postnatal ultrasound demonstrates unilateral upper pole hydroureteronephrosis. The upper pole moiety is often abnormal in appearance with little, if any, parenchyma. With large ureteroceles, bladder outlet obstruction may be present and bilateral hydronephrosis may be seen on ultrasound. Historically, the diagnosis of a ureterocele was made during the evaluation of a UTI in infancy and early childhood. More increasingly, ureteroceles are

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being detected on prenatal ultrasound evaluation. Ureteroceles have been detected on prenatal ultrasounds as early as 17 weeks gestation. Kitagawa and colleagues [41] were able to identify 62.5% of ureteroceles by 20 weeks gestation and the remainder at 21 to 23 weeks gestation. Although prenatal detection of ureteroceles does not routinely alter the course of a pregnancy, in rare cases in utero intervention may be indicated for ureteroceles associated with bladder outlet obstruction and oligohydramnios [4648]. When a ureterocele is identified on prenatal ultrasound, serial ultrasound studies are obtained to follow the amniotic fluid volume, bladder volume, degree of hydronephrosis, and echogenicity of the kidneys. Current indication for in utero intervention includes progressive bladder outlet obstruction with increasing megacystis and oligohydramnios. In utero interventions are designed to decompress the ureterocele and restore bladder emptying and amniotic fluid volume [4648]. The impact of the prenatal diagnosis of ureterocele on the ultimate renal function is controversial. A few series support better upper pole renal function with prenatal diagnosis, yet others indicate that there is no difference in function of the obstructed upper pole [4951]. Although controversy remains as to the impact of prenatal ultrasound on the function of the affected upper pole moiety, it is clear that prenatal detection of ureteroceles does impact the morbidity associated with ureterocele [4951]. Prenatal diagnosis has allowed for early institution of prophylactic antibiotics and has led to a decreased incidence of UTIs from 70% to 80% historically to 3% to 15% currently [5153]. Upadhyay and colleagues [53] demonstrated that prenatal diagnosis of ureterocele is associated with a decreased rate of secondary procedures independent of the type of ureterocele. In infants who underwent partial nephrectomy there was a 16% reoperation rate in the prenatally detected group compared with a 38% reoperation rate in the postnatally detected group.

Mega-ureter Mega-ureter is a term applied to the presence of ureteral diameter of more than 1 cm. Primary mega-ureter occurs three to four times more often in boys and is two to three times more common on the left side [54]. Currently, because of the widespread use of maternal screening ultrasound, most cases of mega-ureter are diagnosed prenatally. Postnatal ultrasound, VCUG, and renal scan are helpful to confirm the cause of the mega-ureter. Mega-ureter may be classified as primary or secondary and as refluxing, obstructive (defined by lasix Mag 3 renal scan), refluxing obstructive, or nonrefluxing nonobstructive mega-ureter [55]. In the absence of documented obstruction, mega-ureters are followed conservatively with periodic radiologic evaluations and antibiotic prophylaxis until the dilatation is deemed stable or resolved. Surgical management involves excision of the obstructive distal segment, tapering of the distal ureter (if needed), and ureteral reimplantation.

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Ectopic ureter Ectopic ureters are associated with ipsilateral hydronephrosis and ureteral dilation on prenatal and postnatal ultrasound. A dilated ureter is often seen posterior to the bladder on pelvic ultrasound. As with ureteroceles, ectopic ureters are often associated with the upper pole of a duplex kidney (80%) and poor upper pole function [56]. Ectopic ureters may have various abnormal insertions, the site varying with the childs sex. In girls, the ectopic ureter may insert distally to the bladder neck or into the vagina, which leads to incontinence [57]. In boys, the most common site of insertion is the posterior urethra. Prenatal detection leads to early identification and management decreasing the morbidity associated with ectopic ureters. Surgical intervention is indicated for ectopic ureters. The procedure varies with the renal function and may involve excision of a nonfunctioning upper pole or ureteral reimplantation or ureteroureterostomy.

Multicystic dysplastic kidney A multicystic dysplastic kidney may be confused with a severe UPJO. Careful review of the renal ultrasound and assessment of renal function differentiate between the two. On ultrasound, the multicystic dysplastic kidney demonstrates a collecting of renal cysts of varying size with no larger central or medial cyst (Fig. 9). Renal functional studies demonstrate b10% function of the multicystic dysplastic kidney. They are more common on the left side, and contralateral anomalies include UPJO (3%12%) and VUR (18%43%) [58]. A VCUG is

Fig. 9. Multicystic dysplastic kidney with multiple, variable-sized cysts without a central dominant cyst. (From Peters CA. Perinatal urology. In: Walsh OC, Retik AB, Vaughn ED, et al, editors. Campbells urology. 8th edition. Philadelphia: WB Saunders; 2002. p. 1787.)

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needed to rule out contralateral VUR. Management of multicystic dysplastic kidneys is conservative, with periodic ultrasound performed because they tend to involute. Observation is indicated until they are no longer visible because of rare reported cases of Wilms tumor arising in multicystic dysplastic kidneys. Indications for removal include increasing size, respiratory compromise, or suspicion of Wilms tumor.

References
[1] Grisoni ER, Gauderer MWL, Wolfson RN, et al. Antenatal ultrasonography: the experience in a high risk perinatal center. J Pediatr Surg 1986;21:358 61. [2] DOttavio G, Mandruzzato G, Meir YJ, et al. Comparisons of first and second trimester screening for fetal anomalies. Ann N Y Acad Sci 1998;847:200 9. [3] Whitlow BJ. The value of sonography in early pregnancy for the detection of fetal abnormalities in an unselected population. Br J Obstet Gynaecol 1999;106:929 36. [4] Taipale P, Ammala M, Salonen R, et al. Two-stage ultrasonography in screening for fetal anomalies at 1314 and 1822 weeks of gestation. Acta Obstet Gynecol Scand 2004;83:1141 6. [5] Elder JS. Antenatal hydronephrosis: fetal and neonatal management. Pediatr Clin North Am 1997;44(5):1299 321. [6] Mandell J, Peters C. Perinatal urology. In: Walsh PC, Retik AB, Stamey TA, et al, editors. Campbells urology. 7th edition. Philadelphia7 WB Saunders; 1998. p. 247 8. [7] Siemens DR, Prouse KA, MacNeily AE, et al. Antenatal hydronephrosis: thresholds of renal pelvic diameter to predict insignificant postnatal pelvicaliectasis. Tech Urol 1998;4(4):198 201. [8] Grignon A, Filion R, Filiatrault D, et al. Urinary tract dilatation in utero: classification and clinical application. Radiology 1986;160(3):645 51. [9] Fernbach SK, Maizels M, Conway JJ. Ultrasound grading of hydronephrosis: introduction to the system used by the Society for Fetal Urology. Pediatr Radiol 1993;23:478 80. [10] Lim DJ, Park JY, Kim JH, et al. Clinical characteristics and outcome of hydronephrosis detected by prenatal ultrasonography. J Korean Med Sci 2003;18(6):859 62. [11] Chevalier R, Howards S. Renal function in the fetus, neonate, and child. In: Walsh PC, Retik AB, Vaughan ED, et al, editors. Campbells urology. 8th edition. Philadelphia7 WB Saunders; 2002. p. 1766. [12] Rabinowitz R, Peters MT, Vyas S, et al. Measurement of fetal urine production in normal pregnancy by real-time ultrasonography. Am J Obstet Gynecol 1989;161:1264 6. [13] Curran M, Nijland MJ, Mann SE, et al. Human amniotic fluid mathematical model: determination and effect of intramembranous sodium flux. Am J Obstet Gynecol 1998;178(3):484 90. [14] Nakayama DK, Harrison MR, deLorimier AA. Prognosis of posterior urethral valves presenting at birth. J Pediatr Surg 1986;21:43 5. [15] Manning FA, Harrison MR, Rodeck C. Catheter shunts for fetal hydronephrosis and hydrocephalus: report of the International Fetal Surgery Registry. N Engl J Med 1986;315:336 40. [16] Glick PL, Harrison MR, Golbus MS, et al. Management of the fetus with congenital hydronephrosis. II. Prognostic criteria and selection for treatment. J Pediatr Surg 1985;20(4):376 87. [17] Freedman AL, Johnson MP, Gonzalez R. Fetal therapy for obstructive uropathy: past, present. . ..future? Pediatr Nephrol 2000;14(2):167 76. [18] Walker RD, Padron M. The management of posterior urethral valves by initial vesicostomy and delayed valve ablation. J Urol 1990;144(5):1212 4. [19] Reinberg Y, Gonzalez R, Fryd D, et al. The outcome of renal transplantation in children with posterior urethral valves. J Urol 1988;140(6):1491 3. [20] Docimo SG, Silver RI. Renal ultrasonography in newborns with prenatally detected hydronephrosis: why wait? J Urol 1997;157(4):1387 9.

446

fefer

&

ellsworth

[21] Wiener J, Ohara S. Optimal timing of initial postnatal ultrasonography in newborns with prenatal hydronephrosis. J Urol 2002;168(4, Suppl 2):1826 9. [22] Ransley PG, Dhillon HK, Gordon I, et al. The postnatal management of hydronephrosis diagnosed by prenatal ultrasound. J Urol 1990;144:584 7. [23] Koff SA. Postnatal management of antenatal hydronephrosis using an observational approach [editorial comment]. Urology 2000;55:609. [24] Farhat W, McLorie G, Deary D, et al. The natural history of neonatal reflux associated with antenatal hydronephrosis. J Urol 2000;164(3):1057 60. [25] Brophy M, Austin F, Yan Y, et al. Vesicoureteral reflux and clinical outcomes in infants with prenatally detected hydronephrosis. J Urol 2002;168:1716 9. [26] Ismaili K, Avni FE, Hall M, and the Brussels Free University Perinatal Nephrology (BFUPN) Study Group. Results of systematic voiding cystourethrography in infants with antenatally diagnosed renal pelvis dilation. J Pediatr 2002;141(1):21 4. [27] Yerkes E, Adams M, Pope JC, et al. Does every patient with prenatal hydronephrosis need voiding cystourethrography? J Urol 1999;162:1218 20. [28] Vates TS, Shull MJ, Underberg-Davis SJ, et al. Complications of voiding cystourethrography in the evaluation of infants with prenatally detected hydronephrosis. J Urol 1999;162(3, Suppl 2):1221 3. [29] Herndon CD, McKenna PH, Kolon T, et al. A multicenter outcomes analysis of patients with neonatal reflux presenting with prenatal hydronephrosis. J Urol 1999;162:1203 8. [30] Livera LN, Brookfield DS, Egginton JA, et al. Antenatal ultrasonography to detect fetal renal abnormalities: a prospective screening programme. BMJ 1989;298(6685):1421 3. [31] Lowe F, Marshall SF. Ureteropelvic junction obstruction in adults. Urology 1984;23:331 5. [32] Brown T, Mandell J, Lebowitz RL. Neonatal hydronephrosis in the sonographic era. AJR Am J Roentgenol 1987;148(5):959 63. [33] Wiener JS, Emmert GK, Mesrobian HG, et al. Are modern imaging techniques over diagnosing ureteropelvic junction obstruction? J Urol 1995;154(2, Suppl 2):659 61. [34] Ulman I, Jayanthi VR, Koff SA. The longterm followup of newborns with severe unilateral hydronephrosis initially treated nonoperatively. J Urol 2000;164(3, Suppl 2):1101 15. [35] Dhillon HK. Prenatally diagnosed hydronephrosis: the Great Ormond Street Experience. BJU 1998;81(Suppl 2):39 44. [36] Palmer L, Maizels M, Cartwright P, et al. Surgery versus observation for managing obstructive grade 3 to 4 unilateral hydronephrosis: a report from the Society for Fetal Urology. J Urol 1998; 159:222 8. [37] Bonnard A, Fouquet V, Carricaburu E, et al. Retroperitoneal laparoscopic versus open pyeloplasty in children. J Urol 2005;173(5):1710 3. [38] Paltiel HJ, Lebowitz RL. Neonatal hydronephrosis due to primary vesicoureteric reflux: trends in diagnosis and treatment. Radiology 1989;170(3, Suppl 1):787 9. [39] Kaplan GW, Scherz HC. Posterior urethra. In: Kelalis P, King L, Belman AB, et al, editors. Clinical pediatric urology. 3rd edition. Philadelphia7 WB Saunders; 1992. p. 835. [40] Gloor JM. Management of prenatally detected fetal hydronephrosis. Mayo Clin Proc 1995; 70(2):145 52. [41] Kitagawa H, Pringle KC, Stone P, et al. Postnatal followup of hydronephrosis detected by prenatal ultrasound: the natural history. Fetal Diagn Ther 1998;13(1):19 25. [42] Hutton KA, Thomas DF, Davies BW. Prenatally detected posterior urethral valves: qualitative assessment of second trimester scans and prediction of outcome. J Urol 1997;158(3, Suppl 2): 1022 5. [43] El Ghoneimi A, Desgrippes A, Luton D, et al. Outcome of posterior urethral valves: to what extent is it improved by prenatal diagnosis? J Urol 1999;162(3, Suppl 1):849 53. [44] Schlussel RN, Retik AB. Ectopic ureter, ureterocele, and other anomalies of the ureter. In: Walsh PC, Retik AB, Vaughan ED, et al, editors. Campbells urology. 8th edition. Philadelphia7 WB Saunders; 2002. p. 2022. [45] Coplen DR, Duckett JW. The modern approach to ureteroceles. J Urol 1995;153(1):166 71. [46] Soothill PW, Bartha JL, Tizard J. Ultrasound guided laser treatment for fetal bladder outlet obstruction resulting from ureterocele. Am J Obstet Gynecol 2003;188(4):1107 8.

prenatal hydronephrosis

447

[47] Quintero RA, Homsy Y, Bornick PW, et al. In-utero treatment of fetal bladder outlet obstruction by a ureterocele. Lancet 2001;357:1947 8. [48] Hansen WF, Cooper CS, Yankowitz J. Ureterocele causing anhydramnios successfully treated with percutaneous decompression. Obstet Gynecol 2002;99(5, Suppl 2):953 6. [49] Bolduc S, Upadhyay J, Sherman C, et al. Histology of the upper pole is unaffected by prenatal diagnosis in duplex system ureteroceles. J Urol 2002;168(3):1123 6. [50] Van Savage JG, Mesrobian HG. The impact of prenatal ultrasonography on the morbidity and outcome of patients with renal duplication anomalies. J Urol 1995;153(3):768 70. [51] Hulbert WC, Rabinowitz R. Prenatal diagnosis of duplex system hydronephrosis: effect on renal salvage. Urology 1998;52(Suppl 2):23. [52] Shekarriz B, Upadhyay J, Fleming P, et al. Long-term outcome based on the initial surgical approach to ureterocele. J Urol 1999;162(part 2):1072 6. [53] Upadhyay J, Bolduc S, Braja L, et al. Impact of prenatal diagnosis on the morbidity associated with ureterocele management. J Urol 2002;167(6):2560 5. [54] Williams DI, Hulme-Moir I. Primary obstructive mega-ureter. Br J Urol 1970;42(2):140 9. [55] Smith ED. Report of working party to establish an international nomenclature for the large ureter. In: Bergsman D, Duckett JW, editors. Birth defects. Original Articles Series 1977;13(5):38. [56] Schulman CC. The single ectopic ureter. Eur Urol 1976;2(2):64 9. [57] Ellerker AG. The extravesical ectopic ureter. Br J Surg 1958;45:344. [58] Atiyeh B, Husmann D, Baum M. Contralateral renal abnormalities in multicystic-dysplastic kidney. J Pediatr 1992;121(1):65 7.