Antenatally diagnosed hydronephrosis: current postnatal management

Michael T. Davenport Paul A. Merguerian Martin Koyle Pediatr Surg Int (2013) 29:207214

INTRODUCTION

 Since the late 1970s, prenatal screening with ultrasound has become a routine component of care for pregnant women worldwide. Studies have found that approximately 1 % of ultrasounds detect fetal anomalies. Of these detected anomalies, genitourinary abnormalities are amongst the most common, accounting for 20 % of identified anomalies

 Antenatal hydronephrosis (ANH), defined as dilation of the fetal renal collecting system affects between 1 and 5 % of pregnancies

 The differential diagnosis of antenatal hydronephrosis is quite broad ranging from ureteropelvic junction obstruction, vesicoureteral reflux, and posterior urethral valves Left untreated these pathologies may result in postnatal morbidity including nephrolithiasis, urinary tract infection, renal scarring and ultimately, renal loss, and chronic kidney disease

 ANH represents a spectrum, with most cases being a trivial and inconsequential finding on maternal fetal ultrasound. the vast majority of ANH is transient in nature and resolves spontaneously without intervention or complication, and hence is a benign, yet worrisome peculiarity

 Current practice regarding the evaluation and treatment of children with ANH remains in flux within the pediatric urology community and is far from uniform and often is based on dogma, training, and personal or institutional bias. Although algorithms have been devised to investigate the infant with ANH, none are perfect for each and every patient that is referred for evaluation

 This manuscript reviews the primary literature and consensus statements pertaining to ANH and sets forth our own recommendations regarding management of infants with this finding. The vast majority of this work is based on upper tract pathology relating to ureteropelvic junction obstruction as the subject is far too immense to similarly review all causes of ANH

NATURAL HISTORY OF ANTENATAL HYDRONEPHROSIS

PRENATAL DETECTION OF ANH

 Prenatal ultrasound screening is most commonly performed at 1820 weeks gestation, which also coincides with the point at which renal architecture becomes visibly distinct. The most commonly utilized parameter for determining the presence and severity of ANH on prenatal screening is the anteriorposterior diameter (APD) of the renal pelvis.

 as of yet no predetermined APD value which discriminates pathological from benign ANH Establishing such a threshold is difficult because of variation in APD associated with a number of factors including gestational age and maternal hydration status.

 Multiple studies have examined the APD measured on prenatal ultrasound necessary to predict postnatal pathology

Ismaili et al. prospectively followed 213 infants

APD cut off of 10 mm in the third trimester detected only 23 % of renal anomalies. APD cut off of 7 mm in the same patient cohort detected 68 % of abnormalities suggesting that a lower APD cut off provides greater sensitivity in detecting pathology

Coplen et al retrospectively evaluated a cohort of 257 neonates

APD cut off of 15 mm detected renal pathology in approximately 80 % of fetuses with a sensitivity of 73 % and a specificity of 82 %.

CLASSIFICATION OF ANTENATAL HYDRONEPHROSIS

 A number of grading systems have been utilized to classify ANH, but they are all complicated by subjectivity and inter-provider variability In order to overcome this subjectivity, more objective parameters have been implemented, namely APD

Lee et al
meta-analysis of 17 studies and a total of 1,308 subjects with antenatal hydronephrosis and were able to stratify ANH based on the size of the APD on prenatal ultrasound. Their analysis also found a difference in APD threshold based on gestational age.

 Mild disease was categorized by an APD <7 mm in the second trimester and <9 mm during the third trimester. Similarly, severe ANH was defined as an APD <10 in second trimester ultrasounds and C15 mm in the third trimester

 Animal models have shown that urinary obstruction not only results in renal dysplasia and kidney failure, but due to decreased amniotic fluid, normal pulmonary development is impeded Currently, intervention, such as open fetal surgery, vesicocentesis or renal pelvis aspiration, is reserved for fetuses with solitary kidney and severe hydronephrosis and oligohydramnios or in fetuses with posterior urethral valves and oligohydramnios. Intervention is only recommended in the second and third trimesters and carries significant morbidity and mortality limiting its utility

 While APD measurement provides an objective means of predicting pathology, most in the field would agree that other features are also important in determining severity of this finding. Therefore, features such as calyceal dilation and parenchymal thinning should be considered in grading the severity of ANH.

 The system grades hydronephrosis on a fivepoint scale with grade 0 representing normal renal ultrasound grade 1 demonstrates the onset of hydronephrosis grade 4 hydronephrosis with dilation of the pelvis and major calyces in addition to thinning of the parenchyma

the Society for Fetal Urology (SFU) took these factors into account

LONG-TERM OUTCOMES

 Perhaps the best data about long-term outcomes in patients with antenatal hydronephrosis come from the anecdotal accounts of Dhillon [11] and the experience at the Great Ormond Street Hospital in London and as further described by Thomas

 cohort of 76 children all having function < 40 % observed for a minimum of 16 years.

 52% significant or complete resolution of hydronephrosis without recurrence 11 % stable hydronephrosis without complication or intervention. 37 % eventually underwent pyeloplasty for increased dilation, decreased differential function or onset of symptoms such as infection

Ulman et al
104 neonates with severe unilateral ANH 23 children eventually required surgery. Of the remaining , 69 % resolved within 2.5 yrs 31% had persistent but improved hydronephrosis Children with differential function of<40 %: 24 % had an improvement in function to a mean of 47 % by 18 months

Ismaili et al.
Retrospective data for a cohort of 234 neonates Over longer period of time, up to 13 years. 22 % required early pyeloplasty for reduced function. Remaining 182 children managed conservatively with observation and 137 found to have stable or improved renal function. Delayed pyeloplasty was performed in 45 of the 182 neonates for decline in differential function or UTI at a mean age of 18 months

OBSERVATION VERSUS EARLY INTERVENTION

 Majority children with antenatally diagnosed hydronephrosis will have spontaneously resolving dilation or remain asymptomatic with persistent dilation ~ 2533 % of cases will worsen over the course of observation with decreased renal function or infection and require surgical intervention

 Organizations such as the Society for Fetal Urology (SFU) and Canadian Urological Association (CUA) have put forth recommendations to guide practitioners, But the community remains divided on their interpretation of the available data

ANTIBIOTIC PROPHYLAXIS

 As of date, there have not been any prospective randomized trials evaluating the utility of prophylactic antibiotics in children with ANH. There are multiple conflicting retrospective studies, some showing an increased risk of UTI and others not, and the topic remains controversial

Retrospective cohort analysis

Walsh et al:Infants with hydronephrosis 12 times more likely to be hospitalized for pyelonephritis in the first year of life Coelho et al.: incidence of infection with mild, moderate, and severe hydronephrosis was 11, 18, and 36 %, respectively, at 36 month Lee et al.: rate of infection ~40 % in neonates with SFU grade 4 hydronephrosis, even when controlling for reflux

 An equal number of studies that demonstrate a low risk of urinary tract infections in children with ANH without vesicoureteral reflux. Estrada et al. :1,514 /2,076 with ANH grade 2 hydronephrosis screened for VUR Of the 828 patients who did not have reflux, only 11, or 1.3 % ultimately developed UTI

 Roth et al.: h/o UTI in only 4.3 % of 92 children with grade 3 or 4 hydronephrosis without reflux a slightly higher rate ofUTI, 8.3 %, in children with hydroureter

 Canadian Urological Association (CUA) recognized the ambiguity surrounding the issue and only conferred a grade D recommendation for cases without reflux SFU recommended the use of prophylactic antibiotics in all cases of hydronephrosis except for the most mild. recommends antibiotic use for children with additional risk factors for UTI such as hydroureter and reflux

CIRCUMCISION

 Even more ambiguous and controversial than the the use of prophylactic antibiotics Most recently, the American Academy of Pediatrics increased their support of the procedure given the evidence of preventing urinary tract infection, HIV transmission, and penile cancer but does not recommend routine circumcision for all newborns

 Several studies have shown that circumcision can prevent UTI with VUR and PUV. Mukherjee et al.:retrospective analysis of 78 uncircumcised pts with PUV 27 circumcised : 83 % reduction in the incidence of UTI Herndon et al. found similar results in their multicenter study of children with vesicoureteral reflux

 HDN without hydroureter/ PUV : less prolific. Roth et al: none of the circumcised children grades 3 and 4 hydronephrosis developed UTI

 all retrospective studies to date, no prospective randomized trials to verify the utility of circumcision. Unlikely in the future given the delicate and personal nature of the procedure decision to pursue circumcision must be individualized to each child and family

RENAL ULTRASOUND

 Important means of evaluating infants with ANH both in both initial and follow-up phases Allows for the differentiation of low- and highrisk disease based on SFU grading criteria of 02 and 34, respectively

Advantages
absence of radiation ease of use non-invasive readily accessible in most locations The available data: monitoring SFU criteria and measuring APD can help predict which patients will require surgical intervention and guide further treatment

Disadvantages
a high degree of interoperator variability in skill and interpretation which can decrease predictive value Hydration status of the infant can also affect the ability of ultrasound to predict pathology. At birth, infants are relatively dehydrated and therefore an ultrasound performed immediately after birth can underestimate the degree of hydronephrosis

 Vast majority of ANH is detected during the second trimester around 20 weeks gestation Abnormal findings then generally followed with repeat prenatal ultrasound during the third trimester and almost always with renal ultrasound during the postnatal period SFU: recommendation in all cases of ANH CUA: grade A recommendation

 SFU & CUA recommend postponing the initial postnatal renal ultrasound until at least 1 week after birth unless necessitated by symptoms such as febrile infection or rising creatinine Children with additional risk factors for renal damage including those with severe bilateral hydronephrosis and any grade of hydronephrosis in a solitary kidney: ultrasound be performed prior to discharge from the hospital at birth.

 The CUA recommends using ultrasound in conjunction with SFU grading criteria to classify patients into observational groups and cases requiring additional evaluation. SFU grades 02 can be observed closely with annual imaging to detect worsening of hydronephrosis, but more severe disease, grades 34, often necessitate a more extensive workup

Dhillon and associates at the Great Ormond Street Hospital

Measuring APD can help predict the need for surgical intervention Differential function > 40 % over a period of 613 years with conservative management. For kidneys with an APD between 30 and 40 mm, 21 out of 25 eventually required surgery to correct obstruction. Additionally, they followed 36 kidneys with an APD < 40 mm and found that all of them eventually required surgical correction

VOIDING CYSTOURETHROGRAM

 Standard component in the evaluation of infants with ANH to detect vesicoureteral reflux and lower tract pathology such as posterior urethral valves, ureteroceles or bladder diverticula

 SFU & CUA have recommended that all cases of ANH found on renal ultrasound to have SFU grade 4 dilation undergo VCUG to rule out reflux and other potential pathology Also recommend that VCUG be deferred for less severe cases of ANH, SFU grades 02, as the modality is more invasive than ultrasound and these children have not been shown to progress to significant pathology

 The recommendations become equivocal, however, when dealing with ANH classified as SFU grade 3 as this group of kidneys has the most conflicting data regarding progression of pathology recommended that a more individualized approach be taken with these patients and the decision to pursue VCUG be made on a case by case basis

 Factors which would influence the decision to recommend VCUG include any findings which suggest lower urinary tract disease like posterior urethral valves. These findings include bilateral hydronephrosis, dilated ureter, duplex kidney, abnormal renal echogenicity, and abnormal appearance of the bladder

SUMMARY AND RECOMMENDATIONS

 Antenatal hydronephrosis is the most commonly diagnosed anomaly on prenatal ultrasound. Meta-analysis has shown that these children have an increased risk of pathology postnatally when compared with children within the normal population. However, the degree of this risk, like the severity of hydronephrosis, varies largely between children.

RECOMMENDATIONS

SFU grades 02
Long-term data indicate that infants with lowgrade antenatal hydronephrosis have resolution of dilation or remain stable without pathological complication in the majority of cases Consequently, initial surgical intervention is not indicated or recommended by either the SFUorCUA

 a protocol of expectant management As per the recommendations of both the SFU and CUA, all infants with antenatally diagnosed hydronephrosis should have a renal ultrasound shortly after birth, but no sooner than 2 weeks of life to avoid the initial postnatal diuretic phase

SFU grade 3
antibiotic prophylaxis until the studies are completed, particularly if the family chooses to pursue a VCUG. Ultrasound should be performed at 714 days after birth in an otherwise healthy infant

 counsel the family on performing a VCUG to evaluate for reflux. If VCUG is negative for reflux we would recommend discontinuing antibiotic prophylaxis. If no VCUG is performed, again a frank discussion with the family is in order, and a decision made after providing the data available and respecting their sense of comfort. Circumcision also becomes a decision based on similar models

 We would recommend repeating the ultrasound at around 3 months of age and if the degree of hydronephrosis remains the same or worsens a Tc-MAG 3 diuretic renal scan should be performed.

SFU grade 4
should be placed on prophylactic antibiotics until the studies are completed. Particularly, if there is ureteral dilation, a VCUG should be encouraged. If the VCUG shows no reflux antibiotics may be discontinued, even realizing that the retrospective data suggests an increased risk of UTI in this group of children

 Children with grade 4 hydronephrosis have the most severe renal anomalies and, as shown in the above longterm follow-up data, the greatest risk for developing renal pathology. most often require surgical intervention to prevent said adverse events.

 that renal ultrasound should be performed after 2 weeks of life to reassess renal dilation. Furthermore, because of the increased risk of pathological outcomes in these children, VCUG should be encouraged. If vesicoureteral reflux is found a DMSA scan may be offered in selected cases in order to evaluate differential function.

 If the VCUG is negative for reflux a diuretic renogram should be performed to elucidate etiology of hydronephrosis and plan for potential surgical management. Again, because of the conflicting data regarding antibiotic prophylaxis and circumcision in children with ANH, we recommend that they be reserved for symptomatic cases

Hydroureter
risk of developing urinary tract infection is greater than in children with dilation limited to the kidney It is our recommendation, and that of the SFU, that these children undergo imaging within the first 7 days, including at least a renal ultrasound. Similarly, these patients should also be placed on antibiotic prophylaxis until imaging studies are complete because of their increased risk of infection.

Bilateral hydronephrosis
there is new evidence, which, while limited, suggests that children bilateral hydronephrosis may benefit from early evaluation and antibiotic prophylaxis. at increased risk of infection Increases with the grade of hydronephrosis with bilateral severe hydronephrosis having higher incidence of infection than milder cases.

 we recommend, along with the SFU and CUA, that these children be placed on antibiotic prophylaxis while awaiting studies to be completed. Bilateral hydronephrosis not only carries an increased risk of infection, it may also be a sign of more severe underlying pathology such as posterior urethral valves in boys. Therefore, it is recommended that these children be evaluated with ultrasound and potentially VCUG prior to being discharged from the hospital after birth