PAEDIATRIC SURGERY
Hydronephrosis in children:
pelviureteric junction
dysfunction
Harriet J Corbett
Liam McCarthy
Abstract
Hydronephrosis is commonly caused by dysfunction of the pelviureteric
junction (PUJ). Other causes, which are usually associated with hydrour-
eter as well hydronephrosis, include vesicoureteric reflux, vesicoureteric
obstruction and bladder pathology. Common causes of PUJ dysfunction
include intrinsic stenosis and aberrant lower pole vessels. Surgery, usually
an AndersoneHynes type pyeloplasty, is indicated in patients with pain,
infection or haematuria. Surgery may be performed open or laparoscopi-
cally. Asymptomatic patients, often detected during antenatal screening,
require a baseline MAG3 scan and serial monitoring with ultrasound scan-
ning. Surgery is indicated in this cohort if there is increasing hydronephro-
sis with an anterior posterior diameter (APD) greater than 3 cm, function
below 40% or a drop in function of more than 10% on serial MAG3 scans.
Keywords Antenatal hydronephrosis; antero-posterior diameter;
crossing vessels; Dietls crisis; hydronephrosis; pelviureteric junction
obstruction
Introduction
The junction of the renal pelvis with the ureter is known as the
pelviureteric junction (PUJ). Suspected dysfunction of this part of
the urinary tract is responsible for a considerable number of
referrals to urologists, both paediatric and adult. PUJ dysfunction
(PUJD) is often called obstruction but there is rarely a true
obstruction at this point. A more accurate description of PUJD is
that of abnormal flow of urine from the pelvis to the ureter which
results in gradual and progressive damage to the kidney. Typi-
cally detected by ultrasound scanning (USS), there is hydro-
nephrosis, i.e. dilatation of the renal pelvis, often in association
with dilatation of the calyces but there is rarely ureteric dilatation
in isolated PUJD.
Incidence/demographics
Hydronephrosis of one or both kidneys is detected frequently on
antenatal scans; in around 50% of cases this is thought to be
Harriet J Corbett MD FRCS(Paed) is a Consultant in Paediatric Urology at
Alder Hey Childrens Hospital, Liverpool, UK. Conflicts of interest: none
declared.
Liam McCarthy PhD FRCS(Paed) is a Consultant Paediatric Urologist at
Birmingham Childrens Hospital, UK. Conflicts of interest: none
declared.
SURGERY 31:3
due to PUJD. The incidence of PUJD (based on the rate of
operative correction) is approximately 1:750e1:1000. Hydro-
nephrosis is more common on the left than right and is bilateral
in 10e20% of cases. There is an increased chance of other
urinary tract anomalies such as vesicoureteric reflux in children
with PUJD.
Aetiology
In most cases there is an intrinsic segment of stenosis at the PUJ
which effectively results in dysfunction of the peristaltic wave
that moves urine from the pelvis to the ureter. Histopathology of
this tissue usually reveals a thin wall with reduced muscle and
some fibrosis. In older children, aberrant or additional vessels to
the lower pole of the kidney that pass anterior to the PUJ may
cause an extrinsic obstruction. Vessels passing behind the PUJ at
this point are a normal variant. These crossing vessels, as they
are known, cause around 33% of PUJD in older children. Inter-
estingly they are rarely the cause of hydronephrosis in children
less than 4e5 years of age.
Other less common causes of PUJD include anatomical
ureteric folds or kinks, external fibrous bands, retrocaval posi-
tioning of the ureter resulting in compression between the infe-
rior vena cava and spine and fibroepithelial polyps. PUJD can
occur in anatomical variants such as horseshoe kidneys and
duplex systems (classically in the lower pole). It may also be
found as a phenomenon secondary to vesicoureteric reflux,
particularly high-grade reflux. Finally, renal calculi can impact at
the PUJ and cause obstruction, although non-obstructing calculi
may co-exist with PUJD, caused by urinary stasis due to impaired
urine flow through the PUJ.
Natural history
The natural history of antenatally detected PUJD is important, as
the incidence of this finding is high. In 27% there is resolution of
hydronephrosis, 56% stay stable whilst 17% of cases are found
to progress, that is, the kidney becomes more hydronephrotic or
there is functional deterioration.
Presentation
PUJD may present in a variety of ways. With the increasing
accuracy of antenatal USS, a large number of asymptomatic cases
are detected prior to birth although a small cohort will be
detected incidentally during childhood USS for other reasons.
The commonest symptomatic presentation is with loin pain,
which may be acute or a more insidious aching pain. Acute pain
may be associated with loin swelling. Both pain and swelling
may resolve after passing a large volume of urine some hours
later; such episodes are known as Dietls crisis. Other symp-
tomatic presentations include urinary tract infection, pyoneph-
rosis, haematuria and raised blood pressure. In addition, PUJD is
often discovered following trauma as hydronephrotic kidneys are
more vulnerable to injury; symptoms may be precipitated by
trauma in a previously well patient.
Antenatal detection and initial postnatal management
Hydronephrosis is one of the commonest abnormalities detected
on antenatal screening. The differential diagnosis includes
135 2013 Published by Elsevier Ltd.
 PAEDIATRIC SURGERY
vesicoureteric reflux, posterior urethral valves, vesicoureteric
junction obstruction (VUJO) and non-refluxing, non-obstructed
megaureter. Additional antenatal investigations may be recom-
mended as hydronephrosis is a soft marker for other disorders
including trisomies, particularly trisomy 21.
All infants will require a postnatal USS, the timing of which is
guided by the antenatal scan findings. Babies with bilateral
hydronephrosis, dilated ureters and/or bladder pathology
warrant urgent postnatal investigation as they may have bladder
outlet obstruction caused by posterior urethral valves. At the
other end of the spectrum are babies with very mild unilateral
hydronephrosis, in whom the scan may be performed as an
outpatient at several weeks of age. All other infants should have
a scan after 48 hours of age as relative dehydration and low urine
output mean scans prior to this time may appear normal and,
thus, falsely reassuring. Most will be followed up by a paediatri-
cian or a paediatric urologist. Infants with an anteroposterior
diameter (APD) of the renal pelvis greater than 10 mm are given
antibiotic prophylaxis (normally trimethoprim 2 mg/kg orally
at night).
Investigations
Ultrasound scans (USS) e the degree of hydronephrosis is
assessed with USS by measuring the transverse APD of the renal
pelvis. The maximal point of dilation is unreliable as it may be
extra-renal and therefore depends upon hydration. The APD
must therefore be measured correctly at the renal hilum
(Figure 1). The upper limit of the normal APD is 8e10 mm, but
the degree of calyceal dilatation must also be considered. In
addition, the USS should assess the thickness of renal cortex and
the rest of the urinary tract. A dilated ureter on the same side
The kidney in the longitudinal [a] and transverse
[b] planes
Kidney
Pelvis
Anterior
Posterior
Ureter
a. Longitudinal view of b. Transverse antero-
the kidney. The dashed posterior view of the
line indicates the kidney and pelvis
transverse plane showing where the
antero-posterior diameter
should be measured
(arrow)
Figure 1 Figure (b) illustrates the correct position to measure the antero-
posterior diameter (APD) during ultrasound scanning.
SURGERY 31:3
does not rule out a PUJ problem. Figure 2 shows the USS images
of three patients with differing degrees of hydronephrosis.
Isotope renography e MAG3 (99mTechnetium-labelled mer-
captoacetyl triglycerine) is given intravenously. MAG3 is taken
up by the renal tubules and excreted by a first pass effect. The
activity is detected by a gamma camera and processed to give
split renal function and excretion (drainage) curves. Often furo-
semide is given prior to the peak of activity. Analysis of the
MAG3 study depends upon split renal function and excretion
curves. Figure 3 shows MAG3 images in a patient with obstruc-
tion due to PUJD before and after surgery.
Intravenous urogram (IVU) e the IVU (also known as an
intravenous pyelogram) may be used in some cases. It is good for
showing the anatomy, and if used at the time of pain will help to
make the diagnosis (Figure 4). The radiation dose is high,
however, so the study is used very sparingly in children.
Micturating cystourethrogram (MCUG) e this may be indi-
cated where vesicoureteric reflux is thought to co-exist or be the
cause of hydronephrosis, or bladder outlet obstruction is
suspected.
Retrograde pyelograms are performed in theatre by catheter-
izing the ureter and are useful for clarifying the level of
obstruction and confirming normal anatomy of the VUJ prior to
pyeloplasty. The investigation is utilized in some centres but due
to the accuracy of modern USS and isotope renography their use
is diminishing.
Management of symptomatic patients
Patients with symptoms due to PUJD require surgery. If the
function of the kidney is good then a pyeloplasty or pyelopexy
will usually be performed. If there is poor function in the kidney,
i.e. less than 10e15% on MAG3, then a nephrectomy may be
recommended. Drainage procedures, such as ureteric stenting or
nephrostomy, have not been shown to increase function in the
affected kidney. Temporary drainage by nephrostomy is recom-
mended in children presenting with pyonephrosis, however,
whilst the infection is treated.
Surgery
The AndersoneHynes dismembered pyeloplasty is a tried and
tested procedure (Figure 5). It aims to fashion an unobstructed
funnel for good urine drainage with the ureter. Where there are
crossing vessels to the lower pole of the kidney, the pyeloplasty
is performed anterior to the vessels. More recently some
surgeons perform a pyelopexy (pexy of the renal pelvis) or a pexy
of the vessels to separate the vessels and the compressed
segment of PUJ, rather than a dismembered anastomosis. Pye-
loplasty or pexy procedures may be done via an open incision
or laparoscopically. The laparoscopic technique, performed via
a retroperitoneal or transperitoneal approach, may be quite
challenging in small children where the working space is limited.
Success rates for laparoscopic surgery are reported to be equiv-
alent to open surgery but the procedure takes much longer and
a recent metaanalysis found a higher rate of secondary proce-
dures. However requirements for analgesia and length of stay are
lower after laparoscopic surgery.
One of the current controversies of pyeloplasty surrounds
stenting of the anastomosis. The anastomosis may be left
136 2013 Published by Elsevier Ltd.
 PAEDIATRIC SURGERY

a b

c d

e f

Ultrasound images showing varying degrees of hydronephrosis. Images a, c and e are taken in the longitudinal plane and b, d and f in the
transverse plane. Images a and b show mild hydronephrosis. Images c and d show moderate hydronephrosis with some calyceal dilatation.
Images e and f show severe hydronephrosis with marked calyceal dilatation.

Figure 2

un-stented or an indwelling JJ ureteric stent may inserted pre- or
peri-operatively. Alternatively a nephrostomy-type stent (a
temporary externalized drain) is inserted during surgery. Stents,
indwelling or nephrostomy-type, are more likely to be used in
laparoscopic surgery. Studies comparing stented methods and
stentless surgery have not been able to show any advantage of
one method over another. The main disadvantage of indwelling
and nephrostomy-type stents are urinary tract infection, and for
indwelling JJ ureteric stents the requirement for a second
procedure to remove the stent.
Complications of pyeloplasty include urine leak, infection,
stenosis of the anastomosis and stone formation. The failure rate of
pyeloplasty is less than 2%. Follow-up after surgery includes an USS
and a functional study between 6 months and 1 year. The
postoperative imaging takes some time to improve and in some
children will never completely return to normal. It should be
remembered that the aim of surgery is to preserve function and
remove symptoms, and not just to improve the imaging appearance.
Management of the asymptomatic patient
This group is largely made up of patients with hydronephrosis
detected by antenatal USS, but there will be a small number
whose hydronephrosis was detected incidentally. The protocol
for following patients with hydronephrosis is well established.
The aim of management is to preserve function in the affected
kidney. Serial monitoring with USS is indicated until the hydro-
nephrosis has stabilized; a baseline MAG3 study is also required.

SURGERY 31:3 137 2013 Published by Elsevier Ltd.

 PAEDIATRIC SURGERY

An intravenous urogram in a patient with symptomatic left

pelviureteric junction dysfunction (PUJD). The top image is the
control film. The bottom film, taken approximately 20 minutes
following administration of intravenous contrast, shows bilateral
excretion which is normal on the right. On the left the pelvis is
dilated with calyceal clubbing due to PUJD.

Figure 4

surgery in relation to the degree of hydronephrosis (data from

Figure 3 Pre- and postoperative MAG3 images of a patient with obstruc- the Great Ormond Street Natural History series).
tion due to PUJD. Finally, management of the patient with bilateral hydro-
nephrosis may be difficult as there is not a normal kidney to
Any significant deterioration in hydronephrosis on USS will compare to. The same management principles should be fol-
usually prompt a repeat MAG3. Patients with less than 40% lowed where possible. In instances where there is a solitary
initial function or a drop in function of more than 10% in asso-
ciation with PUJD should be considered for surgery. PUJD with
over 40% function should be managed expectantly if the Diagram of a dismembered AndersonHynes type
hydronephrosis is small, but should be managed operatively if pyeloplasty
the APD is over 3 cm.
Infants in whom the hydronephrosis was detected antenatally
will often be followed up by a paediatric urologist. They should
have a MAG3 study and a repeat USS at approximately 3 months
of age, with serial USS after that time to monitor the hydro-
nephrosis. All infants except those with hydronephrosis less
than 10 mm should be given antibiotic prophylaxis and this is
usually continued for 1e2 years. It should be noted that there is
scant evidence for antibiotic prophylaxis but most surgeons a b c
prefer to use it. As with the group above, infants with an APD
over 3 cm or function of less than 40% on MAG3 are candidates
for pyeloplasty. Table 1 outlines the overall risk of requiring Figure 5

SURGERY 31:3 138 2013 Published by Elsevier Ltd.

 PAEDIATRIC SURGERY

 function below 40% or a drop in function of more than

Risk of requiring surgery for hydronephrosis 10% on repeat MAG3
 increasing hydronephrosis with APD >3 cm.
Degree of Transverse anteroposterior Chance of
Symptomatic patients usually undergo a pyeloplasty; pyelopexy
hydronephrosis diameter (mm) requiring
may be recommended in cases with crossing vessels, whilst
surgery (%)
those with very poor function are best managed by neph-
Mild <15 Very low
rectomy. A
Moderate 15e20 11
Severe 20e30 40
30e40 75 FURTHER READING
>40 100 Dhillon HK. Prenatally diagnosed hydronephrosis: the Great Ormond
Street experience. Br J Urol 1998; 81(suppl 2): 39e44.
Table 1 Gordon I, Dhillon HK, Gatanash H, Peters AM. Antenatal diagnosis of
pelvic hydronephrosis: assessment of renal function and drainage as
functioning kidney with PUJD, it is accepted practice to under-
a guide to management. J Nucl Med 1990; 32: 1649e54.
take a pyeloplasty when the APD is greater than 2 cm.
Lee DJ, Kim PH, Koh CJ. Current trends in pediatric minimally invasive
urologic surgery. Korean J Urol 2010; 51: 80e7.
Conclusion
Nguyen HT, Herndon CD, Cooper C, et al. The Society for Fetal Urology
Asymptomatic patients with hydronephrosis thought to be due to consensus statement on the evaluation and management of antenatal
PUJD require serial monitoring with USS and a baseline MAG3 hydronephrosis. J Pediatr Urol 2010; 6: 212e31.
scan. Surgery, usually an AndersoneHynes type pyeloplasty, is Thomas DFM, Duffy PG, Rickwood AMK, eds. Essentials of pediatric
indicated if there is: urology. 2nd edn. Informa Healthcare, 2008.

SURGERY 31:3 139 2013 Published by Elsevier Ltd.