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182
11 Recto-bladder Neck Fistula
17. Vick LR, Gosche JR, Boulanger SC (2007) Primary
laparoscopic repair of high imperforate anus in neonatal males. J Pediatr Surg 42:1877–1881
18. Georgeson K (2007) Laparoscopic-assisted anorectal
pull-through. Semin Pediatr Surg 16:266–269
19. Liem NT, Hien PD (2007) Laparoscopic and transanal
approach for rectal atresia: a novel alternative.
J Pediatr Surg 42:E25–E27
20. Lopez M, Kalfa N, Allal H (2007) Anorectal malformation (ARM) with bladder fi stula: advantages of a
laparoscopic approach. Eur J Pediatr Surg. doi:
5/s-2007-965507
21. Srimurthy KR, Ramesh S, Shankar G (2008)
Technical modifi cations of laparoscopically assisted
anorectal pull-through for anorectal malformations.
J Laparoendosc Adv Surg Tech 18:340–343
22. Ichijo C, Kaneyama K, Hayashi Y (2008) Midterm
postoperative clinicoradiologic analysis of surgery for
high/intermediate-type imperforate anus: prospective
comparative study between laparoscopy-assisted and
posterior sagittal anorectoplasty. J Pediatr Surg
43:158–163
23. Grapin-Dagorno C, Fayad F (2008) Surgical treatment of high-type imperforate anus: role of laparoscopy. Bull Acad Natl Med 192:913–918
24. Ramanujam TM, Yik YI, Shitasanan N (2008)
Laparoscopically assisted anorectoplasty for high
anorectal malformations—a modifi ed technique.
Presented at the 15th Annual Meeting of the
International Pediatric Colorectal Club. Salamanca,
Spain, June 2008
10.105
25. El-Debeiky MS, Safan HA, Shafei IA (2009) Long- term
functional evaluation of fecal continence after laparoscopic-assisted pull-through for high anorectal malformations. J Laparoendosc Adv Surg Tech 19:S51–S54
26. Hay SA (2009) Transperineal rectovesical fi stula
ligation in laparoscopic assisted abdominoperineal
pull- through for high anorectal malformations.
J Laparoendosc Adv Surg Tech 19:S77–S79
27. Rollins MD, Downey EC, Meyers RL (2009) Division
of the fi stula in laparoscopic-assisted repair of anorectal malformations—are clips or ties necessary?
J Pediatr Surg 44:298–301
28. Podevin G, Petit T, Mure PY (2009) Minimally
invasive surgery for anorectal malformations in boys.
A multicentric study. J Laparoendosc Adv Surg Tech
19:S233–S235
29. Yamataka A, Kato Y, Lee KD (2009) Endoscopyassisted laparoscopic excision of rectourethral fi stula
in a male with imperforate anus. J Laparoendosc Adv
Surg Tech 19:S241–S243
30. Bischoff A, Levitt MA, Dickie B (2009) Technical
challenges of the laparoscopic approach for patients
with anorectal malformation and rectobladderneck
fi stula. J Laparoendosc Adv Surg Tech 19:264–291
31. Al-Hozaim O, Al-Maary J, AlQahtani A (2010)
Laparoscopic-assisted anorectal pull-through for anorectal malformations: a systematic review and the
need for standardization of outcome reporting.
J Pediatr Surg 45:1500–1504
Imperforate Anus Without Fistula in Males and Females
1 2
12.1 Introduction
Imperforate anus without fi stula is a very unique
kind of defect (Fig. 12.1 ). Most likely, it has a
very different embryologic origin, considering its
uniqueness, type of associated defects, and intrinsic anatomy. In the spectrum of anorectal malformations, one can see that the frequency of
associated defects is very much related with the
height of the location of the fi stula. Thus, a perineal fi stula type of malformation usually is rarely
associated to other defects, whereas a recto-bladder neck fi stula seems to have the highest incidence of associations with other malformations
mainly in the urinary tract. From that point of
view, an imperforate anus with no fi stula represents an exception because it is extremely unusual
for these patients to have other associated defects
such as tethered cord and abnormal sacrum.
The most important feature of this particular
defect is the fact that it represents approximately
4 % of all anorectal malformations, and half of
the cases are Down syndrome [ 1 ]. In addition,
from all patients with Down syndrome suffering
from anorectal malformations, over 95 % of them
have this specifi c type of defect. This fact represents a fascinating and unique opportunity to
speculate and investigate anorectal malformations from the genetic point of view. It is also
interesting to know that, as mentioned before,
half of the anorectal malformations without fi stula suffer from Down syndrome and the other
half seem to have a higher incidence of associa-
tion with other neurologic defects such as absent
corpus callosum.
Approximately 1.1–2.2 % of patients with
Down syndrome have an anorectal malformation
2 – 4 ], and the overwhelming majority has no
[
fi stula.
The association of Down syndrome and anorectal malformation without fi stula may occur in
identical twins [ 5 ].
The literature, prior to 1980, reported a high
incidence of this defect [ 6 ]. Stephens reviewed
his own series and those of eight authors and
found that approximately 10 % of all cases of
ARM suffered from this condition. We believe
Fig. 12.1 Diagram showing the intrinsic anatomy of
imperforate anus without a fi stula
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_12, © Springer International Publishing Switzerland 2015
183
184
ab
12 Imperforate Anus Without Fistula in Males and Females
Fig. 12.2 Distal colostogram done in a patient without a
fi stula. ( a ) The lack of hydrostatic pressure gives the false
impression that the rectum ends at the pubococcygeal line.
that those series included many misdiagnosed
cases, in whom a fi stula was not demonstrated
due to a lack of adequate imaging technology.
Even at present time, we see cases that come to
our center with a diagnosis of anorectal malformation “without fi stula,” based on a distal colostogram done with an inadequate technique (lack
of hydrostatic pressure). When we repeat the distal colostogram, applying enough hydrostatic
pressure, a fi stula is demonstrated (Fig.
12.2 ).
12.2 Anatomic Characteristics
Figure 12.1 shows a diagram of an imperforate
anus with no fi stula. Interestingly, the blind end
of the rectum is located at the same level of the
bulbar urethra in the overwhelming majority of
cases. In the past, the literature referred to imper-
( b ) Increased hydrostatic pressure allows us to see the real
location of the blind end of the bowel
forate anus with no fi stula, dividing the cases into
“high” and “low” location of the rectum [
6 ].
In our series of 83 cases, 76 of them had the
rectum located at the level of the bulbar urethra.
Five cases had the blind rectum located at the
level of the prostatic urethra, and we have only
seen two blind rectums, fl oating into the
peritoneum [
7 ]. That is an extremely unusual
defect, and we believe it belongs to another category. This is a debatable issue [ 8 ].
Some authors suggest that the absence of fi stula may contribute for these patients to suffer
from colon perforation earlier than those with fi stula [ 9 ]. The diagram in Fig. 12.1 shows that the
rectum and the posterior urethra share a long
common wall. This means that the repair of this
malformation is not necessarily easier than the
one of a rectourethral fi stula since the surgeon
has to work in that common wall and separate the
12. 3 Main Repai r
185
Fig. 12.4 Rectal lumen exposed in a patient with no
fi stula
There is no way to injure the urinary tract in
these patients because the rectum bulges right
through the wound. Two 5-0 silk stitches are
placed taking the posterior rectal wall, and the
Fig. 12.3 The white fascia. Deeper to the levator mecha-
nism, a white fascia is seen which covers the posterior
rectal wall. Arrow shows the white fascia
wall is opened with the needle-tip cautery. We
continue opening the rectum distally to reach
its blind end, to confi rm that there is no fi stula.
As we open the posterior rectal wall, 5-0 silk
rectum from the urinary tract preserving intact
the last one.
stitches are placed taking the edges of the rectum to expose the inside lumen of the rectum.
Figure
12.4 shows the lumen of the rectum in
a patient with imperforate anus with no fi stula.
12.3 Main Repair
The surgeon must remember at this point that the
anterior rectal wall is intimately attached to the
The posterior sagittal approach represents the
ideal way to repair this malformation [
1 , 7 , 10 ].
A Foley catheter is inserted in the bladder. The
patient is turned into the prone position, with the
pelvis elevated. The skin of the perineum and
both buttocks are washed, prepped, and draped
in the usual manner. A midline incision is done
with the needle-tip cautery running from the
middle portion of the sacrum down to the base of
the scrotum. The incision divides the skin, subcutaneous tissue, parasagittal fi bers, and muscle
complex. After the parasagittal fi bers have been
divided, we go through the ischiorectal fossa to
fi nd the levator mechanism. When the levator
muscle is divided, a white fascia appears very
clearly (Fig. 12.3 ). This white fascia must be
divided and separated from the real bowel wall.
posterior urethra and will require a meticulous
dissection in order to separate the rectum without injuring the urinary tract. For that, multiple
5-0 silk stitches are placed taking the edge of the
rectum in its lowest portion, and the dissection is
done using uniform traction. Applying uniform
traction with multiple silk stitches, a circumferential dissection is performed dividing bands
and vessels that held the rectum up in the pelvis (Fig.
12.5 ). Since the rectum is located sig-
nifi cantly low, once it is completely separated,
usually it requires a rather minor dissection to
gain enough length to achieve a low-tension anoplasty. In this particular malformation, it is not
uncommon to fi nd a very dilated rectum since the
patient had no fi stula. Therefore, the chances for
this patient to require tapering of the rectum are
186
a b
Fig. 12.5 Circumferential dissection to gain length. ( a ) Pulling down. ( b ) Pulling up
ab
12 Imperforate Anus Without Fistula in Males and Females
Fig. 12.6 Rectal tapering. ( a ) Open rectum after resection of its posterior aspect. ( b ) Reconstructed, tapered rectum
higher than in other cases of anorectal malformations. This is particularly true in those patients
who have spent a long time, from the time of
colostomy until the time of the main repair. If
the rectum is too bulky, it must be tapered, and
for that, we resect the posterior aspect of the
rectal wall and closed the rectum with two layers of interrupted long-term absorbable sutures
(Fig. 12.6 ). The rectum is then placed in front
of the levator mechanism, which is closed with
5-0 interrupted long-term absorbable sutures.
The posterior edges of the muscle complex on
each side of the midline are reapproximated in
the midline and sutured with interrupted 5-0
long-term absorbable sutures, taking a bite of the
posterior rectal wall to anchor the rectum in a
good position. The anoplasty is performed in the
center of the sphincter with 16 circumferential
stitches of 6-0 Vicryl sutures. The ischiorectal
fossa is obliterated with the same suture material as well as the subcutaneous tissue, and the
skin is closed with a subcuticular monofi lament
absorbable suture. These patients require minimal medication for pain. They can eat and drink
the same day as surgery. The Foley catheter can
be removed the next day. Dilatations are started
2 weeks after surgery according to our protocol.
Montalvo et al. designed an ingenious technique called “posterior fl ap anorectoplasty”
[ 11 – 13 ] to repair anorectal malformations
References
187
through a posterior sagittal approach, but without
separating the rectum from the urinary tract. The
bulging posterior wall of the rectum is used to
create a fl ap that is tubularized and switched
down to create the anus. We have no experience
with the procedure; however, we believe that it
can be used in patients who had no fi stula and
rectum dilated enough to allow this maneuver.
We do not believe that this approach should be
used in patients with fi stula, due to the risk of a
recurrent fi stula.
12.4 Function and Results
In terms of bowel control, the results are, as
expected, different in patients with Down syndrome and patients without Down syndrome. In
our series, patients without Down syndrome have
an 85 % chance of having voluntary bowel movements by the age of 3. This means that this malformation has an excellent functional prognosis.
The incidence of constipation, however, seems to
be higher than in other types of defects, since we
know now that a megarectum relates directly
with the chances of constipation. Surprisingly,
60 % of the patients with Down syndrome have
voluntary bowel movements by the age of three.
Of course, we must remember that Down syndrome babies have different degrees of developmental delay, and therefore, we very much
depend on that, to try to predict the chances of
bowel control.
Patients with Down syndrome have a wellknown tendency to be constipated. Patients with
anorectal malformations also have a tendency to
be constipated, provided their original rectum is
not resected and patients with anorectal malformation with no fi stula have a higher incidence of
constipation. Therefore, we must expect these
patients to suffer from severe constipation. After
the colostomy closure, the surgeon should be
very aggressive and proactive in giving the right
amount of laxatives to avoid constipation. When
not treated adequately, this problem may produce
chronic fecal impaction and overfl ow pseudoincontinence (see Chap.
We have been surprised to see patients born
with Down syndrome and anorectal malformation
25 ).
without fi stula coming to our center with a colostomy that is meant to remain on permanent basis.
Their doctors advised the parents to keep the
colostomy for life, because the patients with
Down syndrome are all incontinent. Our experience, as presented here, is quite different.
References
1. Torres R, Levitt MA, Tovilla JM, Rodriguez G, Peña
A (1998) Anorectal malformations and Down’s syndrome. J Pediatr Surg 33(2):194–197
2. Torfs CP, Bateson TF, Curry CJ (1992) Anorectal and
esophageal anomalies with Down syndrome. Am J
Med Genet 44(6):847; author reply 848–850
3. Zlotogora J, Abu-Dalu K, Lernau O, Sagi M, Voss R,
Cohen T (1989) Anorectal malformations and Down
syndrome. Am J Med Genet 34(3):330–331
4. Heinen F, Bailez M, Solana J (1994) Imperforate anus
and Down syndrome. [El ano imperforado en el
Sindrome de Down]. Rev Cir Infant 4(2):72–76
5. de Buys Roessingh AS, Mueller C, Wiesenauer C,
Bensoussan AL, Beaunoyer M (2009) Anorectal malformation and Down’s syndrome in monozygotic
twins. J Pediatr Surg 44(2):e13–e16. doi:
jpedsurg.2008.10.116
6. Stephens FD, Smith ED (1971) Chapter 7: Incidence,
frequency of types etiology. In: Ano-rectal malformations in children. Year Book Medical Publishers, Inc,
Chicago, pp 160–171
7. Bischoff A, Frischer J, Dickie BH, Peña A (2014)
Anorectal malformation without fi stula: a defect with
unique characteristics. Pediatr Surg Int 30(8):763–
766. doi:
8. Black CT, Sherman JO (1989) The association of low
imperforate anus and Down’s syndrome. J Pediatr
Surg 24(1):92–94
9. Chan KW, Lee KH, Tsui SY, Wong YS, Pang KY,
Mou JW, Tam YH (2014) Bowel perforation in newborn with anorectal malformation and no fi stula at
presentation. J Pediatr Surg 49(3):390–394. doi:
10. Fanjul M, Molina E, Cerdá J, Parente A, Laín A,
Cañizo A, Carrera N (2009) Characteristics of the
anorectal atresia without fi stula. Based on 12 cases.
Cir Pediatr 22(1):45–48
11. Montalvo-Marín A, Victoria-Morales G (1993)
Anorectoplasty with a distal tubular fl ap via a posterior sagittal approach. Bol Med Hosp Infant Mex
50(7):499–502. Spanish
12. Zea-Salazar LE, Cabrera-Johnson M (1994)
Anorrectolpastia Sagital posterior y colgajo rectal
[Posterior sagittal anorectoplasty and rectal fl ap]. Rev
Cir Infant 3:112–114
13. Montalvo-Marin A, del Campo NM, Alvarez-Solís
RM, Diaz-Lira MA (1998) Continence in patients
who undergo posterior rectal fl ap anorectoplasty. J
Pediatr Surg 33(12):1760–1764
10.1007/s00383-014-3527-5
10.1016/j.
00
Minimally Invasive Approach to Anorectal Malformations
1 3
13.1 Introduction
The minimally invasive approach to the repair of
congenital malformation is here to stay. All of us,
pediatric surgeons, are concerned about infl icting
pain and/or being too invasive to our patients.
Every effort aimed to reduce the suffering of our
patients, as well as the length of stay in the hospital and subsequently the cost of the operations, is
welcomed. We embrace ideas that have, as the
end result, a less traumatic operation without
compromising the standard of care or provoking
more sequelae in our patients. That explains why
the minimally invasive approach to repair malformations had so much impact in pediatric surgery
and the surgical fi eld in general.
Since the introduction of this new technology,
there are now operations in which the laparoscopic or thoracoscopic approach represents the
gold standard. There are others in which these
new approaches have not been applied, and there
is another group in which the approach is controversial. Anorectal malformations have not been
an exception, and many pediatric surgeons have
used and continue trying to use more and more
the laparoscopic approach to the repair of anorectal malformations, many of them with the correct
indication, others in malformations that can be
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_13 .
repaired either way laparoscopically or posterior
sagittally, and others in cases in whom we believe
that laparoscopy is formally contraindicated.
Our basic contention and belief is that laparoscopy is primarily indicated to replace a laparotomy. In other words, a classic indication is a
procedure that is usually done opening the abdomen and now can be done equally well, through
the small orifi ces of the ports necessary for minimally invasive operation. In male patients with
anorectal malformations, we have to open the
abdomen 10 % of the times, specifi cally in those
cases that have a recto-bladder neck fi stula. We
consider that group a good potential indication
for laparoscopy. We use the word “potential”
because even in those particular types of defects,
laparoscopy has certain limitations, as will be
shown later.
Approximately 30 % of patients with cloacas
require a laparotomy. Yet, all of them require a
posterior approach in order to repair the urogenital component of the malformation. Conceivably,
a laparoscopic approach could be useful to separate the rectum from the bladder neck in those
unusual cases that have the rectum connected to
the bladder neck. Yet, those patients require
rather sophisticated and technically demanding
maneuvers to repair the urogenital component of
the malformation (see Chap.
We decided to review the literature related with
the laparoscopic approach of anorectal malformations. We found 52 papers published between 1998
and 2014. Forty-one of them [ 1 – 41 ] presented
16 ).
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_13, © Springer International Publishing Switzerland 2015
189
190
13 Minimally Invasive Approach to Anorectal Malformations
Tethered
in few
Tethered
cord
0
in 1
all
in 1
cases
all cases
Rectal
atresia Vaginal Vest Perineal Cloaca High Inter Low Sacrum
No
fi stula
Bladder
neck PR Bulbar
2 3
used
Year Authors Cases Comments N/A Urethral
18. 2009 El-Debeiky 15 15
19. 2009 Hay 12 12
20. 2009 Bischoff 6 6
21. 2009 Lopez 1 1 Normal
22. 2009 Yang 11 2 3 3 3
23. 2009 Uchida 24 2 15 3 2 2
24. 2009 Yamataka 1 1
25. 2009 Podevin 34 3 20 10 1
6. 2003 Lin 9 5 1 3
7. 2003 Iwanaka 12 1 2 5 1 2 1
8. 2003 Raghupathy 11 8 3
9. 2003 Tei 5 1 3 1
10. 2005 Kudou 13 7 2 1 2 1
11. 2005 Koga 1 1
12. 2005 Kubota 5 2 2 1
13. 2006 Lima 7 6 1 Abnormal
14. 2006 Hakgüder 4 1 3 Abnormal
15. 2007 Vick 6 3 3
16. 2007 Liem 2 2
1. 1998 Willital 2 2
2. 2000 Georgeson 10 7 1 1 1 1
3. 2001 Ettayebi 1 1
4. 2001 Yamataka 3 3
Table 13.1 List of authors and cases reported
5. 2002 Yamataka 6 3 3 Normal in
17. 2008 Ichijo 15 5 4 1 1 2 2 Normal in
26. 2009 Rollins 5 No clips or ties
13.1 Introduction
191
1
agenesis
both groups
had SR
<0.6
Tethered
in 5, No
0.4–0.7 in
T/C in 8,
Unknown
in 2
6, > 0.7 in
6
0.64 in
prostatic,
and 0.54 in
recto-
bladder
neck
18
specify type
or number.
Only “high/
intermediate”
and no numbers
Totals 446 0 21 16 93 185 63 17 2 29 8 2 8 2 0 0
27. 2010 Raschbaum 3 MRI guided 3
28. 2010 Bailez 5 5 1 sacral
29. 2010 Kimura 13 1 10 1 1
30. 2010 Koga 5 3 2 Normal None
31. 2010 Lopez 1 1 No
32. 2011 Wong 18 Does not
33. 2011 Bailez 17 8 9 50 % in
34. 2011 De Vos 20 3 13 3 1
35. 2011 Tong 33 1 22 6 4
36. 2012 Miglani 3 2 1
37. 2012 England 24 3 7 9 3 2
38. 2013 Jung 25 6 16 3
39. 2013 Liem 19 15 4
40. 2013 Bischoff 15 13 2 < 0.4 in 2,
41. 2014 Ming 24 11 13 Average
192
Table 13.2 Summarized results of a literature review on minimally invasive approach to anorectal malformation
13 Minimally Invasive Approach to Anorectal Malformations
185 prostatic fistula
357
males
446
cases
45
females
44
gender not
specified
series of cases (Table 13.1 ). Ten papers [ 42 – 52 ]
were rather informative, written by experts but
without discussing specifi c cases.
The total number of cases reported in the literature according to our review is 446. These
included 357 males, 45 females, and 44 in whom
the gender was not specifi ed (Table 13.2 ).
The group of male cases included 185 recto-
prostatic fi stulas, 93 bladder neck, 63 bulbar, and
16 labeled as “urethral” without specifying the
location of the fi stulas.
The female group included 29 “vaginal fi stula,” 8 vestibular, and 8 cloacas. The group of
patients without gender specifi cation included 21
cases, 17 without a fi stula, 2 recto-perineal, 2
“rectal atresia,” and 2 cases labeled simply as
“high” (Table
13.2 ).
13.2 Males
Anorectal malformations in males are divided
into the following categories:
• Recto-bladder neck fi stula
• Rectourethral prostatic fi stula
93 Bladder neck
63 Bulbar
16 “Urethral”
29 “vaginal”
8 Vestibular
8 cloaca
17 without fistula
21 not specified
2 perineal
2 rectal atresia
2 “High”
• Rectourethral bulbar fi stula
• Anorectal malformation with no fi stula
• Perineal fi stula
We believe that mainly those patients with
recto-bladder neck fi stulas may benefi t from the
laparoscopic approach. In our own experience,
the laparoscopic approach provides an excellent
exposure of the peritoneal fl oor and the distal part
of the bowel as it approached the bladder neck
(Animation 13.1 ). The dissection of the rectum,
as well as the ligation of the fi stula, is an easy
maneuver. The main reason for this is that in anorectal malformations, the higher the malformation, the shorter the common wall existing
between the rectum and the urinary tract, and
therefore the rectum ends in the bladder neck in a
T fashion, which makes the ligation of the fi stula
an easy task, without any risk of damaging neighboring structures. To repair this malformation,
the next step after the ligation of the fi stula is the
mobilization of the rectum. We found that sometimes this is feasible, and the entire procedure
can be completed after making a small incision in
the perineum. Yet, sometimes the mobilization of
the rectum (as explained in Chap. 11 ) requires a