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Urologic Problems in Anorectal Malformations
2 3
23.1 Introduction
Urogenital problems in patients with anorectal
malformation represent a very important source
of morbidity; in fact, it is more likely for a child
with an anorectal malformation to die from a urologic problem, rather than from gastrointestinal
problems or any other associated defect. Pediatric
surgeons must keep in mind that the patient with
an anorectal malformation represents a potential
life- threatening problem originated in the urogenital tract, mainly kidney damage and eventually kidney failure.
The association of urogenital defects with
anorectal malformations has been recognized for
a long time. The incidence has been estimated to
vary from 25 to 85 % [ 1 – 17 ]. The majority of
reports inform of an incidence around 30–50 %.
We believe that the difference found between different authors is a consequence of the thoroughness of the search. A high index of suspicion, in
an institution with high technologic support,
mostly likely will fi nd more urologic problems.
Table
23.1 shows the frequency of associ-
ated urologic defects for each type of anorectal
malformation. The anorectal defects are listed
according to the degree of complexity, being the
simplest the perineal fi stulas and in the opposite
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_23 .
extreme the cloacal exstrophy and other complex
malformations. It can be very easily seen that the
frequency of association increases directly proportional to the height of the defect and the complexity of the malformation. This information is
very valuable to increase the index of suspicion
of the clinician and allow an early detection of
the urologic problems, particularly those that
may produce renal damage and eventually kidney failure.
It is a relatively common experience to see a
patient that was born with an anorectal malformation that was repaired early in life, and the
parents were not alerted about the fact that the
patient had a urologic abnormality, and years
later, the parents have the very unpleasant experience of learning that the child needs a kidney
transplant. In some cases, the patient is born with
the kidneys already severely damaged, and the
role of the physician should be directed to avoid
worsening of that problem. On the other hand,
some patients are born with normal kidneys, but
the lack of care of a problem such as vesicoureteral refl ux and a poor functional bladder eventually may damage the kidneys, and the patient
may require a kidney transplant or even die. This
last situation is mostly preventable and should
not occur. All this means that it is very important to suspect, diagnose, and treat adequately
all those urologic problems associated with anorectal malformations. In addition, patients with
anorectal malformations should be followed on a
long-term basis, monitoring their kidney function
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_23, © Springer International Publishing Switzerland 2015
371
372
Table 23.1 Frequency of association of urologic defects for each type of anorectal malformation
Females
Absent kidney (%) Vesicoureteral refl ux (%) Hydronephrosis (%)
Cloaca CC >3 cm 26 40 45
Cloacal exstrophy 26 21 9
Cloaca CC <3 cm 17 21 22
Vestibular 10 13 6
Perineal 4 5 5
Males
Absent kidney
(%)
Bladder neck
fi stula
Prostatic 16 6 6 6 7
Bulbar 11 13 9 10 5
No fi stula 7 7 4 3 10
Perineal 2 3 6 1 2
31 29 24 15 18
Vesicoureteral
refl ux (%) Hydronephrosis (%) Hypospadias (%) UDT (%)
23 Urologic Problems in Anorectal Malformations
and the anatomy of the urinary tract, because we
know that many of them have anatomic or functional problems with a tendency to deteriorate.
23.2 Neonatal Approach
If a pediatric surgeon has no experience in the
diagnosis and management of urologic problems,
he should work together with a pediatric urologist in the initial evaluation of a patient with anorectal malformation.
All newborn babies with anorectal malformations should have a kidney ultrasound, within the
fi rst 24 h of life and before any surgical intervention. Female babies, particularly those with cloaca (single perineal orifi ce), must have, in
addition, a pelvic ultrasound to rule out the presence of a hydrocolpos.
We believe that a male baby with a normal
kidney ultrasound, that is passing urine satisfactorily, has a normal sacrum, and no evidence
of tethered cord can be operated on without any
further urologic tests. On the other hand, if the
baby has hydronephrosis, he will require further
urologic studies, including a voiding cystourethrogram. We do not believe that the voiding
cystourethrogram should be done routinely, in
all male babies with anorectal malformations,
because in our experience, it is not a good study
to diagnose the location of the recto-urinary fi stula. In addition, the urethral catheterization in
babies with rectourethral fi stulas sometimes is
diffi cult, because the patients frequently have
kinks or narrowing of the urethra, particularly at
the junction between the rectum and the urethra.
If the baby has tethered cord and/or a very
abnormal sacrum (sacral ratio less than 0.4), he is
at risk of suffering from deterioration of the bladder function, which eventually may affect the
kidneys and therefore is considered a high-risk
patient from the urologic point of view, even if
the initial kidney ultrasound is normal.
The female baby with a cloaca represents a
particularly diffi cult challenge (see Chap.
16 ).
Thirty percent of patients born with a cloaca have
a hydrocolpos that may produce an extrinsic
compression of the ureterovesical junction,
which results in megaureter and hydronephrosis.
As we mentioned in the Chap. 16 , before consid-
ering drainage of the urinary tract with a nephrostomy or ureterostomy, the surgeon must drain the
hydrocolpos and reevaluate the megaureter and
hydronephrosis. After the hydrocolpos has been
drained, the megaureter will improve or disappear, as well as the hydronephrosis. If the hydronephrosis or the megaureter persists, the baby
must be subjected to further evaluation to consider the possibility of making a ureterostomy,
nephrostomy, or vesicostomy. We have seen
23.4 Most Common Urologic Abnormalities in Male Patients with Anorectal Malformations
373
many babies with cloacas, hydronephrosis, and
megaureter, subjected to unnecessary ureterostomies, nephrostomies, and/or vesicostomies, in a
futile attempt to improve the hydronephrosis,
without draining the hydrocolpos (see Chap. 16 ).
There is a small group of patients with a cloaca
that are born with a very narrow (almost atresia) common channel which interferes with the emptying of
the bladder. After draining the hydrocolpos, an ultrasound may show that the bladder is still full and does
not empty well. An attempt to catheterize the bladder
may be extremely diffi cult; in those cases it is justifi ed to do a cystostomy or a vesicostomy.
Another possible indication for a vesicostomy
is the case of a baby with demonstrated massive
vesicoureteral refl ux, megaureter, hydronephrosis, and important risk factors for poor bladder
function, such as poor sacrum and tethered cord.
The newborn baby should not be taken to the
operating room until the surgeon has ruled out
and treated important associated urologic problems. After this, the baby is taken to the operating
room to perform either a colostomy or a primary
repair of the anorectal malformation.
23.3 The Importance
of the Colostomy Type
from the Urologic Point
of View
The location and type of colostomy may have
important urologic implications. It is important
for the neonatologist as well as the urologist to
remember this (see Chap.
The colostomy that we recommend is one created at the end of the descending colon and proximal to the sigmoid loop. The purpose of that
specifi c location is dual, fi rst, to avoid prolapse of
the proximal stoma, since the descending colon is
fi xed, and, second, to leave enough distal bowel
(loop of the sigmoid) to allow a successful pullthrough. In addition, we specifi cally recommend to
make the distal stoma (mucous fi stula) very narrow
in order to avoid prolapse, but still, we believe that
is important to keep it open to be able to irrigate the
distal bowel and to perform radiologic studies,
most specifi cally, a high-pressure distal colosto-
5 ).
gram. We strongly recommend separating both
stomas in order to be able to put a colostomy bag,
including only the proximal stoma and not the distal one. When both stomas are too close together,
the mother is forced to use a single bag to cover
both stomas, which may produce fecal contamination of the distal bowel and consequently of the
urinary tract. Loop colostomies, therefore, from
our point of view, are formally contraindicated in
anorectal malformations, because they represent a
source of fecal contamination of the urinary tract.
During the creation of the colostomy, we specifi cally recommend the surgeons to wash the
bowel distal to the mucous fi stula until it is completely collapsed and cleaned of meconium. If a
baby receives a colostomy and after the procedure
is not doing well and suffers from frequent urinary
tract infections and from episodes of acidosis, it is
extremely important for the physician to look at the
type of colostomy that the baby has, looking for an
explanation for that behavior. A loop colostomy
producing frequent urinary tract infections would
require an operation to separate the stomas. If the
surgeon did not wash the distal bowel, sometimes
the accumulation of meconium, plus mucus passing through the fi stula, produces urinary tract infections. Finally, when the colostomy was opened in
the transverse colon (which we consider contraindicated), the meconium may pass into the urinary
tract, but also urine passes from the urinary tract
into the colon where it is absorbed, producing
hyperchloremic acidosis that may interfere with the
growth and development of the newborn baby.
When a pediatric urologist is called to see a baby
with this kind of problem, he should look at the
type of colostomy that the baby has.
23.4 Most Common Urologic
Abnormalities in Male
Patients with Anorectal
Malformations
23.4.1 Absent Kidney
The most common congenital urologic defect
associated to anorectal malformations is an
absent, dysplastic, multicystic, or nonfunctional
374
23 Urologic Problems in Anorectal Malformations
a
b
c
Fig. 23.1 Absent kidney (multicystic, dysplastic). ( a ) Ultrasound showing an image of a multicystic kidney. ( b ) Renal
scan showing an absent kidney. ( c ) MRI showing a horseshoe kidney
kidney. In our series, 106 out of our 909 male
patients and 165 out of 1,123 females were born
with this problem.
Figure 23.1 shows the images of a patient with
an absent kidney. Figure 23.1a shows the ultrasound
and Fig. 23.1b shows a renal scan. This is by far the
most common type of anatomic abnormality seen in
anorectal malformations. The kidney may be totally
absent or rather represented by a nonfunctional
multicystic and/or dysplastic kidney remnant.
Figure 23.2a shows the images of an ultrasound in a patient with hydronephrosis, and
Fig. 23.2b shows a case with hydronephrosis
and megaureter in a patient with anorectal malformation. This problem was present in 198 out
of 1,123 female patients and 70 out of 909 male
patients. The presence of a megaureter in patients
with anorectal malformations, most of the time,
represents the existence of severe vesicoureteral
refl ux, since the cases of ureterovesical obstruction are very unusual. Of course, we must remember that cloaca patients may have an extrinsic
compression of the ureterovesical junction that is
released when a dilated vagina is drained.
Figure 23.3 shows the images of a voiding cystourethrogram of a baby suffering from
vesicoureteral refl ux (Fig. 23.3a unilateral and
Fig. 23.3b bilateral). In our series, this problem
was present in 207 out of 1,123 females and 133
out of 909 males. The incidence of refl ux in cases
of anorectal malformations has been estimated to
vary from 30 to 60 % [ 18 – 21 ]. In our series it
occurred between 14 and 18 % of the cases.
The presence of severe megaureter and vesicoureteral refl ux in a newborn baby is an ominous sign. That baby will require special care to
protect the kidneys, particularly in cases with real
or potential neurogenic bladder, such as patients
with severe sacral deformity and/or tethered
cord. The best way to protect the kidneys in those
babies is with a temporary vesicostomy, since
trying to perform a ureteral reimplantation, with
ureteral tapering, in an infant with a well-known
poor functional bladder most likely will not succeed and may put the marginal renal function of
the patient at risk.
There is one particular group of male
patients born with a recto-bladder neck fi stula.
23.4 Most Common Urologic Abnormalities in Male Patients with Anorectal Malformations
a
b
375
Fig. 23.2 Hydronephrosis (ultrasound image). ( a ) Bilateral hydronephrosis. ( b ) Hydronephrosis and megaureter
a
b
Fig. 23.3 Vesicoureteral refl ux (VCUG). ( a ) Unilateral. ( b ) Bilateral
376
23 Urologic Problems in Anorectal Malformations
Fig. 23.4 VCUG in a patient with a single kidney with
hydronephrosis and refl u x
Twenty- fi ve patients of a group of 111 patients
born with a bladder neck fi stula had an absent
kidney, megaureter, and hydronephrosis in the
opposite site (Fig. 23.4 ). These patients are at
high risk to end up with a kidney transplant.
The fact that a baby is born with hydronephrosis means that those kidneys had been
suffering in utero and at birth already have a
signifi cant degree of damage. We have seen
patients with hydronephrosis at birth and with
a normal creatinine; they maintain the normal
level of serum creatinine during the fi rst years
of life, but when they have a growth spurt,
sometimes the creatinine suddenly elevates,
indicating that most likely the patient was born
with a limited kidney function, which became
obvious when the patient grows signifi cantly.
Those cases with bladder neck fi stula, single
kidney, megaureter, and hydronephrosis should
be considered high-risk patients.
The management of vesicoureteral refl ux in
patients with anorectal malformations deserves a
special comment. The general principles of management of this disorder, in patients without anorectal malformations, should not be extrapolated
and applied for cases with anorectal malformation
for the following reasons:
A. Patients with anorectal malformations
frequently suffer from a spectrum of sacral
abnormalities that have a demonstrated effect
on bladder function.
B. At least 25 % of patients with ARM suffer
from tethered cord, which also has a signifi cant negative effect on bladder function.
C. The repair of an anorectal malformation,
when done inappropriately, produces different degrees of neurogenic bladder.
D. The repair of complex cloacas includes a sig-
nifi cant dissection between the vagina(s), the
bladder neck, the trigone, and the urethra. All
these have a very signifi cant effect on the
bladder function. In addition, the scar produced by that dissection may represent a serious challenge to perform a reimplantation.
It is becoming obvious that the bladder function
is a fundamental factor to take into consideration
when making a decision about a ureteral reimplantation. A ureteral reimplantation, done in a patient
with neurogenic bladder and incapacity to empty,
most likely will fail. The decision to reimplant a
ureter must not be taken without a previous urodynamic evaluation. This is particularly true in patients
with megaureter and hydronephrosis. The ureteral
reimplantation must be performed being sure that
the patient is able to empty the bladder spontaneously or with intermittent catheterization through
the urethra [
22 ] or through an appendicovesicos-
tomy tract (Mitrofanoff principle) [ 23 , 24 ].
Because of all these reasons, in general, we try
to avoid a reimplantation in a newborn with refl ux,
megaureter, and hydronephrosis. A vesicostomy is
a better option, under those circumstances; it protects the upper tracts and gives us time to reevaluate the case and make an appropriate decision.
23.4.2 Urethral Problems
Figure 23.5 shows an image of male urethrogram
of a patient born with an anorectal malformation
and a recto-urinary fi stula. The kink shown in the
study most likely corresponds to the location of the
fi stula. However, we believe that the VCUG is not
the ideal study to determine the location of the fi stula. Some patients suffer from a urethral stricture
23.5 Bifi d Scrotum
377
Fig. 23.5 Radiologic image of a kinked urethra. The nar-
rowing and/or the kink is located at the junction of the
rectum and the urethra (fi stula site)
or a kink of the urethra at the same location of the
junction of the rectum (fi stula) (Fig. 23.6 ). The
posterior sagittal approach represents an ideal way
to repair urethral strictures. This can be done at the
same time of the repair of the anorectal malformation. We have operated on our patients with this
kind of problem and found relatively simple the
urethral repair. The results have been good.
Figure 23.7 shows operative pictures of a repair of
a urethral stricture located at the same location of
Fig. 23.6 Distal colostogram showing a urethral stricture
at the junction of the rectum with the urethra
the fi stula. A urethroplasty was performed using
a fl ap of rectal tissue (Figs. 23.7a–d ).
It is relatively common to see cases that have
a very acute kink of the urethra at the same location where the rectum joins the urinary tract.
When the doctors, radiologists, or surgeons try to
pass a catheter, the catheter stops at that place or
rather goes into the rectum. To try to overcome
this obstacle, it is recommended to use a coude
type of catheter (curved tip) to get access to
the bladder. Sometimes, however, even with the
use of this catheter, it is not possible to catheterize the bladder. In such cases it is necessary to
from a urethral stricture, then there is no way to
pass the catheter into the bladder. Under those
circumstances we allow the catheter to go into
the rectum and approach the patient posterior
sagittally; when the rectum is opened, one can
see the catheter inside, and then under direct
vision, one can redirect the catheter into the bladder. If the surgeon fi nds that there is a urethral
narrowing, we have been using a little piece of
the local rectal tissue to create a small fl ap to
enlarge the area of the stricture of the urethra
with good results (Fig.
23.7 ).
pass the cystoscope using a wire in order to
catheterize the bladder. There is a maneuver that
proved to be useful to pass a Foley catheter under
23.5 Bifi d Scrotum
those circumstances. A lacrimal probe is inserted
in the hole of the catheter, and the lacrimal probe
is curved at its tip and can be externally directed
(see Chap.
9 ). If the patient, in addition, suffers
In our series of 909 cases of male patients with
anorectal malformation, we found 102 patients
suffering from a bifi d scrotum (Fig. 23.8 ). Most