Journal of Neonatal Surgery 2013;2(1):3

ORIGINAL ARTICLE
Single-Stage Surgical Correction of Anorectal Malformation Associated
with Rectourinary Fistula in Male Neonates
Ernesto Leva1, Francesco Macchini1,* Rossella Arnoldi1, Antonio Di Cesare1, Valerio Gentilino1,
Monica Fumagalli2, Fabio Mosca2, Akbar Bhuiyan3, Maurizio Torricelli1, Tahmina Banu3
1

Dept. of Pediatric Surgery, FONDAZIONE IRCCS CA GRANDA Ospedale Maggiore Policlinico, Milan Italy

Neonatal ICU, FONDAZIONE IRCCS CA GRANDA Ospedale Maggiore Policlinico, Milan Italy

Dept. of Pediatric Surgery, CHITTAGONG MEDICAL COLLEGE HOSPITAL, Chittagong Bangladesh

ABSTRACT
Introduction: The treatment of children affected by ano-rectal malformations (ARM) is characterized by some unsolved problems. The three-stage surgical correction has been known
to be most effective in preventing complications, but recently new approaches have been
proposed. We describe our experience with the newer approaches.
Methods: Twenty three male newborns, affected by ARM and recto-urinary fistula, were
treated in 2 different centers in 8 years. Nineteen neonates (birth weight 2.4 - 3.5 kg) received a primary posterior sagittal anorectoplasty (PSARP) at the Department of Pediatric
Surgery of the Chittagong Medical College Hospital (group 1). Four term neonates (birth
weight 2.9 - 3.4 kg) received a primary pull-through with combined abdomino-perineal approach at the Pediatric Surgery Department of Fondazione C Granda of Milan (group 2).
Results: Among patients of Group 1, 11 patients had a recto-bulbar fistula and 8 a rectoprostatic fistula. Among the Group 2, 2 had a recto-bulbar fistula and 2 a recto-prostatic fistula. The site of fistula was decided at the time of surgery. In Group 1, 5 post-surgical complications were recorded (26%); 1 child died of sepsis, 3 had dehiscence and 1 stenosis,
which resolved with dilatation. In Group 2, the only post-operative complication of small
rectal prolapse resolved spontaneously after a few months on follow-up. Group 2 patients
were followed-up in a dedicated multidisciplinary colorectal center.
Conclusions: Primary repair of ARMs with recto-urinary fistula is a feasible, safe and effective technique in the neonatal period. A combined abdominal and perineal approach seems
to guarantee better results. A dedicated team is mandatory, both for the surgical correction
and for a long-term follow-up.
Key words: Anorectal malformation, Primary repair, Recto-urinary fistula

INTRODUCTION
The management of children affected by anorectal malformations (ARM) is still affected by
some unsolved problems. Pena advocates 3
staged management- colostomy at birth, posterior sagittal ano-recto-plasty (PSARP) and closure of colostomy for males neonates affected
*

Corresponding Author

by ARM associated with recto-urinary fistula [13]. This surgical approach is considered the
most effective in preventing incontinence,
thanks to its meticulous respect of the perineal
structures [4]. In the last 2 decades, with the
advent of laparoscopy, new approaches to
ARMs were proposed, at first by Willital and
then by Georgeson [5]. The laparoscopy was
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Single-Stage Surgical Correction of Anorectal Malformation Associated with Rectourinary Fistula in Male
Neonates

very useful in identifying the rectal wall, isolating and closing the fistula, thus facilitating
the anorectoplasty. The results of two different
techniques of primary repair performed in two
Departments of Pediatric Surgery are here analysed: in the first group a posterior sagittal approach was chosen, while in the second one a
combined abdominal and perineal correction
was preferred.
MATERIALS AND METHODS
From January 2002 to December 2009, 23
male newborns affected with ARM associated
with recto-urinary fistula were treated in 2 different centers within first 48 hours of lives.
The diagnosis of ARM with recto-urinary fistula
was easily established within first 24 hours of
birth. An invertogram was obtained 24 hours
after birth in all the patients to determine the
distance between the rectal pouch and the proposed anal site. Neonates without evidence of
meconium in urine after 24 hours from birth
and neonates with a delayed presentation after
48 hours from birth were excluded by the
study. No other exclusion criteria, such as associated cardiac diseases, were considered.
Group 1 consisted of 19 neonates (gestational
age 35 - 41 weeks; birth weight 2.4 - 3.5 kg)
who underwent a primary PSARP at the Department of Pediatric Surgery of the Chittagong
Medical College Hospital. Among them, 37%
were born by caesarean section. Four patients
had cardiac malformations (atrioseptal defect
(n=2); ventriculoseptal defect (n=1); pulmonary
stenosis (n=1)), while 9 had genito-urinary malformations (vesicoureteral reflux (n=4); hypospadias (n=3); multicystic kidney (n=1) and 1
ectopic kidney (n=1).
Group 2 consisted of 4 term neonates (birth
weight 2.9 - 3.4 kg) who received a primary
pull-through with combined abdomino-perineal
approach at the Pediatric Surgery Department
of Fondazione C Granda of Milan, Italy. All of
them had a spontaneous delivery and a regular
post-natal course. There were no associated
cardiac and renal malformations in this subgroup. The study of lumbo-sacral anomalies

and intra-spinal defects was postponed; X-ray

of sacrum and spinal US or MRI was done before age of 2 months. For Group 2, continence
was evaluated on the basis of the Krickenbeck
consensus statement [6].
In Group 1, PSARP and submucosal dissection
were performed according to Penas technique.
With a posterior sagittal approach, the muscle
complex was open, the rectum was reached and
the urinary fistula was ligated, and then an
anorectoplasty was performed.
In Group 2, through a pfannenstiel incision,
the abdomen was explored. The dilated descending colon was opened, decompressed from
the meconium and subsequently closed. Rectal
dissection begins at the peritoneal reflection
and this was facilitated by an antero-superior
traction applied to the empty bowel remaining
close to the bowel wall. The dissection and ligation of the fistula resulted easy in the present
population of newborns. Legs were elevated.
Penas muscle stimulator was used to determine the correct anal site. Vertical incision with
a complete visualisation of the muscle complex
was performed, a Step Verres needle was
passed through the muscle complex and dilatations of the new anal canal to Hegar number 11
were done. After this, the rectum was pulled
through and the anoplasty was performed.
RESULTS
In Group 1 (n=19), 11 patients had a rectobulbar fistula and 8 a recto-prostatic fistula. In
Group 2 (n=4), 2 had a recto-bulbar fistula and
2 a recto-prostatic fistula.
The site of fistula was decided at the time of
surgery. A good dissection of the rectal pouch,
isolation and ligation of the fistula and a correct anorectoplasty was achieved in all patients.
Average duration of the operation was 2 hours
and 10 minutes (range: 1h50min - 3h30min) in
Group 1 and 2 hours and 35 minutes (range:
2h 20min - 3h 10min) in Group 2.
No intra-operative and anaesthesia complications were recorded in either centers. Feeding
Journal of Neonatal Surgery Vol. 2(1); 2013

Single-Stage Surgical Correction of Anorectal Malformation Associated with Rectourinary Fistula in Male
Neonates

was started on day 5 in Group 1 and on day 12

in Group 2. All neonates passed meconium after an average of 48 hours post-surgery.
In Group 1, 5 post-surgical complications were
recorded (26%); 1 child died after 3 days from
surgery for sepsis, 3 presented with dehiscence
within 48 hours from surgery (one severe,
which required colostomy and two successfully
solved with conservative treatment), and 1 developed stenosis which required prolonged dilatation. Two patients were lost to follow-up.
Sixteen patients were toilet trained and reached
regular bowel movements and no fecal incontinence in a mid-term follow-up of 3 years. Five
out of these patients had vertebral dysraphism
(such as anomalies in number and form of vertebrae), but none had neurospinal defects (such
as tethered cord, or intradural lipoma). None of
these boys had any urinary complaints; no
voiding cystourethrograms (VCUG) were done.
In Group 2, only 1 surgical complication was
recorded, consisting in a small rectal prolapse,
which spontaneously resolved on follow-up after a few months. None of the patients presented strictures or dehiscence in the short
term follow-up and the quality of life was considered good for all the children as related by
parents. All these patients achieved regular
bowel movements. All these patients achieved
regular bowel movements and no soiling in a
mid-term follow-up of 3 years was recorded,
while 2 of them needed daily laxative for a stasis of grade 2 according to Krickenbeck consensus statement. Patients of this group were followed-up in a Multidisciplinary Colorectal Center (mean time of follow-up of 3 years), with
regular ambulatory evaluations by a dedicated
team of specialist, formed by neurosurgeons,
urologists, gynaecologists, psychologists and
pediatric surgeons.
None of them presented associated spinal defects. VCUG were normal in all boys.
DISCUSSION
ARM comprise a spectrum of diseases ranging
from minor defects, characterized by a good
functional prognosis, to more complex ones,
Journal of Neonatal Surgery Vol. 2(1); 2013

often associated with other anomalies with poor

functional prognosis [1-3]. The management of
these children is still affected by some unsolved
problems.
The most important advancement in the treatment of ARMs came from Pena in the early
1980s, with the introduction of a new operative
approach (PSARP), effective for the entire spectrum of ARMs [7]. The well-known technique
consists in dissecting the external anal sphincter complex and the levator muscle, by staying
carefully in the midline and thus avoiding lesions of the surrounding structures. In particular, this technique needs a 3 stage-approach for
the treatment of recto-urinary fistula in male,
consisting in a colostomy at birth, the PSARP
repair within 2 months and a subsequent closure of colostomy [1, 3]. Over the years, the
crucial role of the muscle complex in achieving
continence and preserving the sensory and as
motor functions became evident.
In the early 1990s, a minimal invasive technique for correction of ARM was proposed for
children with recto-urinary fistula by Willital,
later popularized by Georgeson [5].
In past years, a few papers describing singlestage procedure at birth have been published
[8-10]; these were received with some criticism
by the international experts of the disease. The
proposal consisted in performing a single surgical procedure, unifying the anatomical criteria
of Pena and the innovations brought by minimal invasive surgery [5].
Here, the experiences of 2 different centers on
the primary repair of ARMs with recto-urinary
fistula are reported.
At the Department of Pediatric Surgery of the
Chittagong Medical College Hospital, the decision to treat males affected by ARM with rectourinary fistula by a primary repair was taken in
consideration of the limited facilities of the area
and of the difficulties of a staged surgical program in children living in suburban villages.
Post-surgical complications were especially related to the lack of availability of antibiotics,
parenteral nutrition and laboratory tests. On

Single-Stage Surgical Correction of Anorectal Malformation Associated with Rectourinary Fistula in Male
Neonates

the contrary, in a mid-term follow-up, all children presented satisfactory results.

Following the experience of the team from
Bangladesh, a single-stage surgical approach
was applied to 4 male children affected by ARM
with recto-urinary fistula in the Department of
Pediatric Surgery of Policlinico of Milan (Group
2). Post-surgical complications were few and
self-limited.
Based on the present experience, few facts have
to be taken in consideration.
Appropriate selection criteria have to be decided for the newborns that would be subjected
to primary repair. The main objective evidence
is the presence of meconium in the urine, as
sign of recto-urinary fistula. The fistula limits
the risk to search the rectum somewhere, allowing surgeons to follow the anatomical structures, to isolate the rectum and the fistula and
to reconstruct the anatomy of the low pelvic
floor and perineum with the same principles
and dogmas of posterior sagittal approach. If
there is no evidence of meconium in the urine
or in the perineum after 24 hours from birth in
male newborns, staged management is advocated. The invertogram was realised to be a futile investigation as the surgical approach did
not change on the fact that how high the rectal
pouch ended.

It is clear that a primary repair represent an

attractive choice for the treatment of ARMs. In
fact, it reduces the number of operations from
3 to 1, with the obvious advantages in terms of
surgical and anaesthesia risks; it avoids colostomy, with lower surgical complications and
familiar discomfort in terms of management,
psychological and economic burdens [8]; it allows the passage of stools since the beginning,
with a theoretical early establishment of the
brain-defecation reflexes [9, 10]. Furthermore,
a precocious closure of the fistula can avoid
continued urinary tract colonization through it
[10].
Review of the literature did not reveal any experience of laparoscopy in the single-stage repair
of ARMs during the neonatal period. We found
that laparotomy was effective in isolating distally the fistula in these newborns. Furthermore, it may represent a valid approach in case
of dilated rectal pouch, allowing an enterotomy
and the emptying of the distal bowel (never
needed in the present population).
If further studies will demonstrate that the results of the primary repair are similar to that of
the traditional approach in terms of functional
outcome, the primary repair will represent a
valid option for surgeons trained and dedicated
to the surgery of ARM.
CONCLUSION

The satisfactory results, in terms of post-surgical complications, were obtained taking extreme care to respect some fundamental principles: to dissect the perineum close to the rectum in order to avoid injuries to the pelvic autonomic nerve of bladder and penis; to isolate
carefully the recto-urinary fistula, taking attention not to damage the urethra; to dilate
progressively the perineum to avoid strictures;
to perform the anoplasty with the same principles of PSARP, included the use of the Pena
stimulator to determine the anal site [7].

Primary repair of ARMs with recto-urinary fistula is a feasible, safe and effective technique in
the neonatal period. A combined abdominal
and perineal approach seems to guarantee better results. Further studies are needed to evaluate if the incidence of surgical and functional
complications is similar to the traditional
staged management. The role of a dedicated
team is mandatory, both for the surgical correction and for a long-term follow-up.
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Address for correspondence

Francesco Macchini,
Department of Pediatric Surgery - Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, via Commenda 10 20122 Milan - Italy
E mail: [email protected]
Leva et al, 2013

Submitted on: 04-09-2012

Accepted on:

27-11-2012

Published on: 01-01-2013

Conflict of interest: None
Source of Support: Nil

How to cite:
Leva E, Macchini F, Arnoldi R, Di Cesare A, Gentilino V, Fumagalli M, et al. Single-stage surgical correction of anorectal
malformation associated with rectourinary fistula in male neonates. J Neonat Surg. 2013; 2: 3.

Journal of Neonatal Surgery Vol. 2(1); 2013