REPATR OF ANORECTAL
MALFORMATIONS
"ONE STAGE OR THREE STAGES"
Mostafa Mostafa Rezk MD (1). Montaser Mohamed El-Kotby
MD (2). Mohamed Abdel-Monem El-Sayed M.B., B.Ch. - M.Sc
(1).
Abstract
Background:
The aim of this study is to assess the feasibility, safety, and
the outcome of one-stage repair of high and intermediate anorectal
malformation in a neonate by posterior sagittal aneorectoplasty
(PSARP) compared to the three-stage procedure.
Methods:
The study comprised fifty patients Classified into two groups, with
25 patients in each group.
Group A patients were managed by one-stage procedure
(posterior sagittal anorectoplasty) in early neonatal life.
Group B pateints were managed by traditional three-stages
procedure (colostomy- posterior sagittal anorectoplasty- colostomy
closure).
Follow up for continence was from parents history and
examination. MRI and/or CT for sphincter position and function
were done in selected cases.
Postoperative grading of continence was done according to the
following criteria: Grade A: Clean, Grade B: Soiling and Grade C:
Fecal matter.
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Results:
Complications were encountered in 4 (16%) patients in group A,
and 6 (24%) patients in group B.
(Group A: 13 52%) patients were classified as good, 11 (44%) as
moderate, and one (4%) - (male with rectobulbar fistula) - as poor.
Group B: 10 (40%) patients were classified as good, 13 (52%) as
moderate, and 2 (8%) - (female with rectovaginal fistula and male
with rectovesical fistula) – as poor.
Conclusion:
We found it is safe and feasible to do one-stage procedure
for anorectal malformations, with higher or at least similar
outcome.
Keywords: early PSARP, one stage, early neonate,
(1) Benha faculty of medicine, Surgery department.
(2) Cairo faculty of medicine , Pediatric surgery department.
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Introduction
Anorectal malformations are one of the most common
congenital defects. The usual reported incidence is between 1 per
1500 and 1 per 5000 live births. (1)
Despite the advances made in its management over the
last few decades, affected children continue to be a challenge to
the
pediatric
surgeons,
worldwide.
The
ultimate
goal
of
reconstruction is to create a functional and anatomically aesthetic
neoanus. (2)
The gold standard treatment for intermediate and high
anorectal malformations for the last two decades has been
neonatal colostomy followed by definitive surgery; posterior sagittal
anorectaoplasty (PSARP) or abdomino- perineal pull through and
then colostomy closure. (3)
Obviously, the three- stage procedure burdens the patients
and
their
parents
physiologically,
psychologically,
and
economically. Therefore, researchers, such as Goon H. (5) and
Moor T. (6), have tried to use 1-stage PSARP procedure to treat
high-type and intermediate-type anorectal anomalies in the
neonate with encouraging results. (7)
The techniques to achieve perineal gastrointestinal tract
continuity in this approach, based on the concept of improved fecal
continence after early perineal training for patients with high
imperforate anus has been proposed by others with limited followup. The theoretical basis for early restoration of gastrointestinal
127
continuity stems from the belief that the neuronal framework for
normal bladder and bowel function exists at the time of birth. (8)
Because neonates are not continent of urine or feces,
there is learning of "training" period in which long-lasting, activitydriven neuronal changes take place during neuronal circuitry
development. Theoretically, by delaying the repair of anorectal
anomalies, critical time may be lost in which neuronal networks
and synapses would have formed resulting in normal or nearnormal function. (8)
The advantages of bypassing the colostomy stage are
many. First, colostomy complications are eliminated completely.
This is even more important in developing countries where
colostomy is socially unacceptable, non-availability of colostomy
bags, most of the parents are illiterates and cannot manage
colostomies (which these unfortunate patients have for 6 to 8
months), and there are no stomal nurses. (9)
The aim of this study is to assess the feasibility, safety, and
the outcome of one-stage repair of high and intermediate anorectal
malformation in a neonate by posterior sagittal aneorectoplasty
(PSARP) compared to the three-stage procedure
Patients and methods
This is a randomized controlled comparative study, has been
conducted during the period between 2008- 2014 in Pediatric
Surgical Department, Abu-El Reesh Children Hospital, Cairo
University. The study included fifty patients with high and
intermediate ano-rectal malformation. Informed consent was taken
128
from all of subjects after discussing with them about the aim of the
study. The patients were classified into two groups:
Group A: twenty five patients are operated upon using one-stage
procedure of posterior sagittal anorectoplasty during early neonatal
period (once presented to hospital).
Group B: twenty five patients are operated upon using classic
three-stage
procedure
(colostomy-
posterior
sagittal
anorectoplasty- closure of colostomy) and used as a control group.
Ethical consideration:
A clear written informed consent was taken from all of subjects
after discussing with them about the aim of the study.
Group A: The included patients were 14 males and 11 females;
presented during the first few hours of life. Patients were
investigated
by
invertogram
(or
cross-table
x-ray)
and
ultrasonography (after at least 12 hours of birth). The anomaly
types were 13 with high anomaly (8 males and 5 females), and 12
with intermediate level (6 males and 6 females).
All were operated by posterior sagittal anorectoplasty in one
stage of formal repair at early neonatal life. The use of
magnification (surgical loupes) during operative procedure and
electric stimulator facilitated to identify sphincter components and
proper splitting of the muscle complex.
Groupe B: The included patients were 13 males and 12 females;
presented with their parents seeking the definite surgical
129
correction. They were investigated and the anomaly types were12
with high level anomaly (7 males and 5 females), and 13 with
intermediate level (6 males and 7 females).
Classic 3 stages of (colostomy, posterior sagittal anorectoplasty,
closure of colostomy) then done:
Left ileac defunctioning colostomy was done to avoid the
development of intestinal obstruction and divert meconium away
from the urinary tract.
Distal loopogram was done as a preoperative procedure before
posterior sagittal anorectoplasty; to assess distal level of large
bowl and reveal any fistula.
Posterior sagittal anorectoplasty was done from the age of sex
months onward.
Closure of colostomy was done after the correct size of the anus
is reached (after dilatation schedule)
Postoperative follow up:
The patient was observed in the operating room until fully
recovered, then, placed on his side to avoid aspiration and
minimize pressure on the suture line in the recovery room.
For patients that has done colostomy follow up included local
care of the colostomy wound.
Parenteral administration of broad spectrum antibiotics started 2
hours postoperatively and continues for 3 to 5 days. Then oral
administration may be continued for longer period if needed.
130
Oral feeding started 24 hours of the postoperative period.
Local care of the wound was done by irrigation of saline and
antiseptics every 8 hours, also done following any soiling with fecal
matter. Wound care continued for 2 or 3 days after removal of
sutures.
Urethral catheter was removed on the 3rd postoperative day in
patients without fistula, and on the 5th post operatine day in
patients with fistula.
Removal of sutures started at the 8th to 12th postoperative day
according to the degree of wound healing.
Anal calibration and gentle dilatation started 2 or 3 weeks
postoperatively, using a corresponding dilator to the anal size and
xylocaine ointment to minimize pain. All patients were subjected to
dilatation schedule.
Follow up for continence was from parents history (for pateints <
1 year old), and examination for older patients. MRI and/or CT for
sphincter position and function were done in selected cases.
Postoperative grading of continence was done according to
the following criteria: Grade A: Clean, Grade B: Soiling and Grade
C: Fecal matter.
Patients had been selected with the following criteria:
-Inclusion criteria:
1) Patients with high and intermediate ano-rectal malformation
2) With or without fistula.
3) Full term.
131
4) Birth weight > 3 kg.
5) Distance of rectum, assessed by x-ray and ultrasound, not
more than 2-3 cm.
6) Had no abdominal distension at birth (and during 1st 24 hours)
in group A.
7) Not
associated
with
other
congenital
anomalies
(i.e.:
VACTREL).
8) Had no other congenital or acquired diseases e.g. enzymatic
defeciencies, hormonal defeciencies, serious infections, etc.)
All patients were subjected to history taking (from parents),
examined thoroughly, and investigated; to assess general
condition and type and level of the anomaly.
Statistical methodology:
The data were recorded on an “Investigation report form”. These
data were tabulated, coded then analyzed using the computer
program SPSS (Statistical package for social science) version 16 to
obtain.
Descriptive data:
Descriptive statistics were calculated for the data in the form of:
1.
Mean.
2.
Standard deviation (±SD).
3.
Number and percent
Analytical statistics:
In the statistical comparison between the different groups, the
significance of difference was tested using one of the following tests:132
1- Student's t-test:-Used to compare between mean of two
groups of numerical (parametric) data.
2- For continuous non- parametric data, Mann-Whitney U- test
was used for inter-group analysis,
3- Pearson correlation coefficient (r) test was used correlating
different parameters.
4- Inter-group comparison of categorical data was performed by
using chi square test (X2)
P value >0.05 was considered statistically non-significant. P
value <0.05 was considered statistically significant (S). And a P
value <0.0001 was considered highly significant (HS) in all
analyses.
Results
The study comprised fifty patients with high and intermediate
ano-rectal malformation. Classified according to their management
into 2 groups: Group A and group B (25 patients in each group).
Group A: considered as the study group; managed by one-stage
procedure (posterior sagittal anorectoplasty) in early neonatal life.
Group B: considered as a control group; managed by three-stage
procedure (colostomy- posterior sagittal anorectoplasty- colostomy
closure).
Sex distribution of both groups regarding fistula and its types is
shown in table (13).
Group A: 11 (44%) males and 8 (32%) females were with fistula.
Also, 3 (12%) males and 3 (12%) females were without.
133
Group B: 9 (36%) males and 10 (40%) females were with fistula.
Also, 4 (16%) males and 2 (8%) females were without.
Table (13): Shows the two study groups (cases and controls)
as regards sex distribution and type of fistula
Group A
Group B
Sex distribution
Sex distribution
Male
female
Male
female
Rectovesical
4 (16%)
0 (0%)
4(16%)
0 (0%)
Rectoprostatic
4 (16%)
0 (0%)
3(12%)
0 (0%)
Rectobulbar
3 (12%)
0(0%)
2(8%)
0 (0%)
Rectovaginal
0 (0%)
4 (16%)
0 (0%)
5 (20%)
Rectovestibular
0 (0%)
4 (16%)
0 (0%)
5 (20%)
Anorectal
3(12%)
3 (12%)
4(16%)
2 (8%)
11 (44%)
8 (32%)
9 (36%)
10 (40%)
Type of fistula
pvalue
malformation
without fistula
Total no. of
cases with
fistula
134
>0.05
Figure (49): Shows the two study groups (cases and controls) as regards sex distribution and type of
fistula
60
50
40
30
20
20
16
16
16
20
16
12
16
16
12
12 12
8
10
0
0
0
0
0
8
0
0
0
0
0
0
Rectovesical
Rectoprostatic
Group A Male
Rectobulbar
Group A Female
Rectovaginal
Group B Male
133
Rectovestibular
Group B female
Anorectal malformation
without fistula
Table (14): Showing types of complications in the two study
groups (cases and controls); group A and B patients
Type of
No. of Group A No. of Group B
complication
cases
cases
Mild infection
1 (4%)
3 (12%)
Severe infection
1 (4%)
1 (4%)
Mucosal prolapse
1 (4%)
1 (4%)
Annular stricture of
1 (4%)
1 (4%)
pvalue
the neoanus
Total no of cases
4 (16%)
6 (24%)
>0.05
Sever infection = purulent discharge with gapping of more than 2 suture sites
Figure (50): Showing types of complications in the two study
groups (cases and controls); group A and B patients
10
9
8
7
6
5
4
3
3
2
1
1
1
1
1
1
1
1
0
mild infection
Severe infection
Mucosal prolapse
Group A
134
Group B
Annular stricture
Complications were encountered in 4 (16%) patients in group A,
and 6 (24%) patients in group B.
Group A: one had mild infection (female with rectovaginal).
Another had severe infection (male with rectobalbar fistula) and
managed by antibiotics, antiseptics and secondary sutures. One
developed mucosal prolapse (female with rectovestibular fistula)
managed by trimming.
The last developed annular stricture of the neoanus (male with
rectovesical fistula) managed by local excision of the stenosed ring
and resuturing of the excised edges.
Group B: three had mild infection (two males with rectoprostatic
fistula, and one female with rectovestibularl fistula). One had
severe infection (female with rectovaginal fistula). Another
developed mucosal prolapse (male with rectovesical fistula). The
last one developed annular stricture of the neoanus (female with
rectovestibular fistula). The same management as group A was
done for all.
The operative time for group A ranged from 1 hour to 2.5 hours,
and from 1 hour and 15 minutes to 2 hours and 45 minutes for
group B.
All patients of both groups were subjected to anal dilation schedule
as mentioned before.
Colostomy closure in group B patients was done as soon as the
neoanus became soft, supple, pliable and of optimal size for age.
135
Table (15): Showing day of discharge from hospital in both
groups
Day of
No. of patients
No. of patients
discharge
in group A
in group B
0-3
6 (24%)
0 (0%)
4-6
8 (32%)
0 (0%)
7-9
9 (36%)
20 (80%)
10-12
2 (8%)
5 (20%)
p-value
<0.001
Figure (51): Showing day of discharge from hospital in both
groups
90
80
80
70
60
50
30
36
32
40
24
20
20
8
10
0
0
0
0--3
4--6
7--9
Group A
10--12
Group B
Group A patients discharged earlier: 6 (24%) patients at the 3rd
postoperative day, 8 (32%) patients after removal of urinary
catheter (males with rectovesical or rectourethral fistulae) at the 5 th
136
postoperative day. The remaining patients were discharged at 7th
day onwards.
As for group B patients; they were discharged starting from the 7 th
postoperative day onwards.
The main hospital stay for group A was 6.7 days, while was 8.6
days in group B.
Table (16): Showing postoperative grading of continence
in
both groups
grade
No. of patients
No. of patients
in group A
in group B
A (Good)
13 (52%)
10 (40%)
B (Fair)
11 (44%)
13 (52%)
C (Poor)
1 (4%)
p-value
>0.05
2 (8%)
Grade A: Clean (age 6-12 months). Grade B: Soiling. Grade C: Fecal matter.
Postoperative grading for continence was done in the follow up
period (6 to 12 months). According to criteria in table (16), data
filled were taken from history from parents in patients < 1 year, and
examination for older patients.
Discussion
Despite many researches and improved understanding of
embryology and pathophysiology of ARM, the problem of obtaining
better functional results still remains unsolved. (37)
The surgical approach to repairing these defects changed
dramatically in 1980 with the introduction of the posterior sagittal
approach, which allowed surgeons to view the anatomy of these
defects clearly, to repair them under direct vision, and to learn
137
about the complex anatomic arrangement of the junction of rectum
and genitourinary tract. (26)
The main concerns for the surgeon in correcting these
anomalies are bowel control, urinary control, and sexual function.
With early diagnosis, management of associated anomalies and
efficient meticulous surgical repair. (26)
The surgical approach to repairing these defects changed
dramatically in 1980 with the introduction of the posterior sagittal
approach, which allowed surgeons to view the anatomy of these
defects clearly, to repair them under direct vision, and to learn
about the complex anatomic arrangement of the junction of rectum
and genitourinary tract. (26)
The main concerns for the surgeon in correcting these
anomalies are bowel control, urinary control, and sexual function.
With early diagnosis, management of associated anomalies and
efficient meticulous surgical repair. (26)
Along with the improvement in operating techniques in
neonatal surgery, a single-stage PSARP procedure has been
developed for the treatment of imperforate anus. (43)
The concept of a single-stage procedure for the treatment of
congenital intermediate-type imperforate anus is not yet widely
accepted since such a procedure may result in lifelong
sequelae.(43)
Mortality rate was zero in the study, but, may be due to
exclusion of cases associated with other congenital disorders.
Postoperative complications were encountered in 10 patients
in the study (4 in group A and 6 in group B).
Four patients (one in group A and three in group B) had mild
wound infection in the form of a small area of redness and serous
discharge at a suture site. That was treated by antibiotics and
antiseptics.
One patient in each group had severe infection in the form of
purulent discharge and gapping of 2 or 3 sutures.
They were treated by administration of antibiotics and
antiseptics followed by secondary sutures.
138
One patient in each group developed annular stricture of the
neoanus managed by local excision of the stenosed ring and
resuturing of the excised edges.
Also one patient in each group developed mucosal prolapse,
and were managed by trimming.
Higher results were found in a study by Osifo et al; that had
no mortality was recorded on six months to four years follow-up.
Apart from minor superficial perianal surgical site infection in one
baby which responded to antibiotics, no post-operative sepsis or
breakdown of repair was recorded. (44)
Our result were higher than the results in a study done by
Elbatarny et al; postoperative complications included; superficial
wound infection (18.4%), deep wound infection (7.8%); recurrent
rectovestibular fistula (4.1%), anal stenosis (11.4%), mucosal
prolapse(14.2%). (45)
There as a significant difference in hospital stay (p-value
<0.001) between both groups, as the main hospital stay for group
A was 6.7 days, while was 8.6 days in group B. With earlier
discharge (at 3rd postoperative day onwards) in group A. Oral
feeding allowed 24 hours postoperative.
In a study by Osifo et al; Oral intake was commenced and
tolerated on the second post-operative day, and the babies were
hospitalized for between eight and 10 (median 9) days after
surgery. (44)
Postoperative grading for continence was done in the follow
up period (6 to 12 months). Collected data were from history from
parents in patients < 1 year, and examination for older patients.
Postoperative grading for continence was done in the follow
up period (6 to 12 months). Collected data were from history from
parents in patients < 1 year, and examination for older patients.
Patients were classified into three categories: good,
moderate and poor.
139
Good patients had no or minimal soiling (clean) in between
motions, while moderate had significant soiling in between motions
with obstinate constipation despite regular washouts. Poor patients
had obvious fecal matter in between motions
Group A patients were classified as: 13 patients were good,
11 as moderate, and one - (male with rectobulbar fistula) - as poor.
Group B patients were classified as: 10 patients were good,
13 as moderate, and 2 - (female with rectovaginal fistula and male
with rectovesical fistula) – as poor.
The total results were 23 (46%) good patients, 24 (48%)
moderate and 3 (6%) poor.
Postoperative grading for continence was done in the follow
up period (6 to 12 months). Collected data were from history from
parents in patients < 1 year, and examination for older patients.
Patients were classified into three categories: good,
moderate and poor.
Good patients had no or minimal soiling (clean) in between
motions, while moderate had significant soiling in between motions
with obstinate constipation despite regular washouts. Poor patients
had obvious fecal matter in between motions
Group A patients were classified as: 13 patients were good,
11 as moderate, and one - (male with rectobulbar fistula) - as poor.
Group B patients were classified as: 10 patients were good,
13 as moderate, and 2 - (female with rectovaginal fistula and male
with rectovesical fistula) – as poor.
The total results were 23 (46%) good patients, 24 (48%)
moderate and 3 (6%) poor.
140
Conclusion
In this study we found it is safe and feasible to do one-stage
procedure for anorectal malformations, with higher or at least
similar outcome.
Early correction may attain better development of normal
stooling patterns at the appropriate time during the development of
somatosensory cortical reflexes and this will be reflected on the
outcome of the postoperative continence in these patients.
1) Patient positioning
141
2) Start of dissection in posterior sagittal anorectoplasty
(PSARP)
142
3) Identification of sphincter parts (direction of muscle fibers)
143
4) Closure after repair done
144
5) Post-operative follow up after scheduled dilatation
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