antenatally diagnosed hydronephrosis

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    Antenatally diagnosed hydronephrosis:

    current postnatal management

    Michael T. Davenport Paul A. Merguerian

    Martin Koyle

    Pediatr Surg Int (2013) 29:207214

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    INTRODUCTION

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    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

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    Antenatal hydronephrosis (ANH), defined as

    dilation of the fetal renal collecting system

    affects between 1 and 5 % of pregnancies

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    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 andultimately, renal loss, and chronic kidney disease

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    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

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    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 perfectfor each and every patient that is referred for

    evaluation

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    This manuscript reviews the primary literatureand consensus statements pertaining to ANHand sets forth our own recommendations

    regarding management of infants with thisfinding.

    The vast majority of this work is based onupper tract pathology relating to ureteropelvicjunction obstruction as the subject is far tooimmense to similarly review all causes of ANH

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    NATURAL HISTORY OF ANTENATALHYDRONEPHROSIS

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    PRENATAL DETECTION OF ANH

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    Prenatal ultrasound screening is mostcommonly performed at 1820 weeksgestation, which also coincides with the point

    at which renal architecture becomes visiblydistinct.

    The most commonly utilized parameter fordetermining the presence and severity of ANHon prenatal screening is the anteriorposteriordiameter (APD) of the renal pelvis.

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    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.

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    Multiple studies have examined the APD

    measured on prenatal ultrasound necessary to

    predict postnatal pathology

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    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

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    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 %.

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    CLASSIFICATION OF ANTENATALHYDRONEPHROSIS

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    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

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    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.

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    Mild disease was categorized by an APD

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    Animal models have shown that urinary obstructionnot only results in renal dysplasia and kidney failure,but due to decreased amniotic fluid, normal pulmonarydevelopment is impeded

    Currently, intervention, such as open fetal surgery,vesicocentesis or renal pelvis aspiration, is reserved for

    fetuses with solitary kidney and severe hydronephrosisand oligohydramnios or in fetuses with posteriorurethral valves and oligohydramnios. Intervention is

    only recommended in the second and third trimestersand carries significant morbidity and mortality limitingits utility

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    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 ofthis finding. Therefore,

    features such as calyceal dilation and

    parenchymal thinning should be considered ingrading the severity of ANH.

    th S i t f F t l U l (SFU)

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    the Society for Fetal Urology (SFU)

    took these

    factors into account The system grades hydronephrosis on a five-point 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

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    LONG-TERM OUTCOMES

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    Perhaps the best data about long-term

    outcomes in patients with antenatal

    hydronephrosis come from the anecdotal

    accounts of Dhillon [11] and the experience atthe Great Ormond Street Hospital in London

    and as further described by Thomas

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    cohort of 76 children

    all having function < 40 %

    observed for a minimum of 16 years.

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    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

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    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 improvedhydronephrosis

    Children with differential function of

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    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 orimproved renal function.

    Delayed pyeloplasty was performed in 45 of the182 neonates for decline in differential functionor UTI at a mean age of 18 months

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    OBSERVATION VERSUS EARLYINTERVENTION

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    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 surgicalintervention

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    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

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    ANTIBIOTIC PROPHYLAXIS

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    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 remainscontroversial

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    Retrospective cohort analysis

    Walsh et al:Infants with hydronephrosis 12times more likely to be hospitalized forpyelonephritis 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 neonateswith SFU grade 4 hydronephrosis, even whencontrolling for reflux

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    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

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    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

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    Canadian Urological Association (CUA) recognizedthe ambiguity surrounding the issue and onlyconferred a grade D recommendation for caseswithout reflux

    SFU recommended the use of prophylacticantibiotics in all cases of hydronephrosis exceptfor the most mild.

    recommends antibiotic use for children withadditional risk factors for UTI such as hydroureterand reflux

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    CIRCUMCISION

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    Even more ambiguous and controversial thanthe the use of prophylactic antibiotics

    Most recently, the American Academy of

    Pediatrics increased their support of theprocedure given the evidence of preventingurinary tract infection, HIV transmission, andpenile cancer

    but does not recommend routine circumcisionfor all newborns

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    Several studies have shown that circumcisioncan 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 theirmulticenter study of children withvesicoureteral reflux

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    HDN without hydroureter/ PUV : less prolific.

    Roth et al: none of the circumcised children

    grades 3 and 4 hydronephrosis developed UTI

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    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

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    RENAL ULTRASOUND

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    Important means of evaluating infants with

    ANH both in both initial and follow-up phases

    Allows for the differentiation of low- and high-

    risk disease based on SFU grading criteria of

    02 and 34, respectively

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    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 whichpatients will require surgical intervention and

    guide further treatment

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    Disadvantages

    a high degree of interoperator variability inskill and interpretation which can decreasepredictive value

    Hydration status of the infant can also affectthe ability of ultrasound to predict pathology.

    At birth, infants are relatively dehydrated andtherefore an ultrasound performedimmediately after birth can underestimate thedegree of hydronephrosis

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    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

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    SFU & CUA recommend postponing the initialpostnatal renal ultrasound until at least 1 weekafter birth

    unless necessitated by symptoms such as febrileinfection or rising creatinine

    Children with additional risk factors for renaldamage including those with severe bilateral

    hydronephrosis and any grade of hydronephrosisin a solitary kidney: ultrasound be performedprior to discharge from the hospital at birth.

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    The CUA recommends using ultrasound inconjunction with SFU grading criteria toclassify patients into observational groups and

    cases requiring additional evaluation. SFU grades 02 can be observed closely with

    annual imaging to detect worsening ofhydronephrosis, but more severe disease,

    grades 34, often necessitate a moreextensive workup

    Dhill d i h G

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    Dhillon and associates at the Great

    Ormond Street Hospital

    Measuring APD can help predict the need forsurgical 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 tocorrect obstruction.

    Additionally, they followed 36 kidneys with anAPD < 40 mm and found that all of themeventually required surgical correction

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    VOIDING CYSTOURETHROGRAM

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    Standard component in the evaluation of

    infants with ANH to detect vesicoureteral

    reflux and lower tract pathology such as

    posterior urethral valves, ureteroceles orbladder diverticula

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    SFU & CUA have recommended that all casesof ANH found on renal ultrasound to have SFUgrade 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, asthe modality is more invasive than ultrasound

    and these children have not been shown toprogress to significant pathology

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    The recommendations become equivocal,however, when dealing with ANH classified asSFU grade 3 as this group of kidneys has the mostconflicting data regarding progression ofpathology

    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

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    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 abnormalappearance of the bladder

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    SUMMARY ANDRECOMMENDATIONS

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    Antenatal hydronephrosis is the most commonlydiagnosed

    anomaly on prenatal ultrasound. Meta-analysis hasshown

    that these children have an increased risk of pathology

    postnatally when compared with children within thenormal

    population. However, the degree of this risk, like the

    severity of hydronephrosis, varies largely betweenchildren.

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    RECOMMENDATIONS

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    SFU grades 02

    Long-term data indicate that infants with low-

    grade antenatal hydronephrosis have

    resolution of dilation or remain stable without

    pathological complication in the majority ofcases

    Consequently, initial surgical intervention is

    not indicated or recommended by either theSFUorCUA

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    a protocol of expectant management

    As per the recommendations of both the SFU

    and CUA, all infants with antenatally

    diagnosed hydronephrosis should have a renalultrasound shortly after birth, but no sooner

    than 2 weeks of life to avoid the initial

    postnatal diuretic phase

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    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 daysafter birth in an otherwise healthy infant

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    counsel the family on performing a VCUG toevaluate for reflux.

    If VCUG is negative for reflux we would

    recommend discontinuing antibiotic prophylaxis.If no VCUG is performed, again a frank discussionwith the family is in order, and a decision madeafter providing the data available and respecting

    their sense of comfort. Circumcision also becomes a decision based on

    similar models

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    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 renalscan should be performed.

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    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 riskof UTI in this group of children

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    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 renalpathology.

    most often require surgical intervention to

    prevent said adverse events.

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    that renal ultrasound should be performed after 2weeks of

    life to reassess renal dilation. Furthermore, because ofthe

    increased risk of pathological outcomes in thesechildren,

    VCUG should be encouraged. If vesicoureteral reflux is

    found a DMSA scan may be offered in selected cases in

    order to evaluate differential function.

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    If the VCUG is

    negative for reflux a diuretic renogram should beperformed

    to elucidate etiology of hydronephrosis and plan for

    potential surgical management. Again, because of the

    conflicting data regarding antibiotic prophylaxis andcircumcision

    in children with ANH, we recommend that they be reserved for symptomatic cases

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    Hydroureter

    risk of developing urinary tract infection isgreater than in children with dilation limited tothe kidney

    It is our recommendation, and that of the SFU,that these children undergo imaging within thefirst 7 days, including at least a renal ultrasound.

    Similarly, these patients should also be placed on

    antibiotic prophylaxis until imaging studies arecomplete because of their increased risk ofinfection.

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    Bilateral hydronephrosis

    there is new evidence, which, while limited,suggests that children bilateral hydronephrosismay benefit from early evaluation and

    antibiotic prophylaxis. at increased risk of infection

    Increases with the grade of hydronephrosiswith bilateral severe hydronephrosis havinghigher incidence of infection than mildercases.

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    we recommend, along with the SFU and CUA, thatthese children be placed on antibiotic prophylaxiswhile awaiting studies to be completed.

    Bilateral hydronephrosis not only carries an increased

    risk of infection, it may also be a sign of more severeunderlying pathology such as posterior urethral valvesin boys.

    Therefore, it is recommended that these children be

    evaluated with ultrasound and potentially VCUG priorto being discharged from the hospital after birth