Pediatrics and Neonatology (2012) 53, 252e256

Available online at www.sciencedirect.com

journal homepage: http://www.pediatr-neonatol.com

ORIGINAL ARTICLE

Constipation is a Major Complication after Posterior

Sagittal Anorectoplasty for Anorectal Malformations
in Children
Ching-Fang Huang a, Hung-Chang Lee a,b,*, Chun-Yan Yeung a,
Wai-Tao Chan a, Chuen-Bin Jiang a, Jin-Cherng Sheu b,c, Nien-Lu Wang b,c,
Jr-Rung Lin d

a
Department of Pediatrics, Mackay Memorial Hospital, Taipei City, Taiwan
b
Department of Pediatrics, Taipei Medical University, Taipei City, Taiwan
c
Pediatric Surgery, Mackay Memorial Hospital, Taipei City, Taiwan
d
Clinical Informatics and Medical Statistics Research Center, Chang Gung University, Taiwan

Received Aug 16, 2011; received in revised form Nov 4, 2011; accepted Nov 9, 2011

Key Words Background: To assess whether constipation or fecal incontinence is a major late complication
anorectal after posterior sagittal anorectoplasty in patients with anorectal malformation (ARM).
malformation; Methods: We retrospectively enrolled 188 children, 85 low-type ARM (L-ARM) and 103 high-
constipation; type ARM (H-ARM), who had complete medical records of bowel habits and medication histo-
posterior sagittal ries after posterior sagittal anorectoplasty for anorectal malformation in Mackay Memorial
anorectoplasty Hospital. Stool characteristics as well as physical and medication history were evaluated.
procedure; The symptom severity (SS) scoring system was used to assess changes in bowel habits.
stool incontinence Results: During a mean follow-up period of 4.3 years, constipation was found to be the most
common late complication in both groups of patients (64.5% in the L-ARM group and 78.6%
in the H-ARM group). Compared to constipation, stool incontinence was much less frequent,
with 4.7% in L-ARM and 3.9% in H-ARM. There was no significant difference in mean SS scores
between the two groups.
Conclusion: Constipation was the most common late sequela in children after correction of
ARM in our study.
Copyright ª 2012, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights
reserved.

* Corresponding author. Department of Pediatrics, Mackay Memorial Hospital, Number 92, Section 2, Chungshan North Road, Taipei City
104, Taiwan.
E-mail address: ped2435@ms2.mmh.org.tw (H.-C. Lee).

1875-9572/$36 Copyright ª 2012, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights reserved.
http://dx.doi.org/10.1016/j.pedneo.2012.06.007
Constipation after posterior sagittal anorectoplasty 253

1. Introduction Table 1 Symptom severity scoring system for constipation

and fecal incontinence in children.
Imperforate anus, also known as anorectal malformation
Item Score
(ARM), includes a wide spectrum of development defects,
ranging from distal atresia of the anal canal to high rectal Soiling
atresia. It is often associated with VACTER syndrome, None 0
Hirschsprung’s disease, and Down syndrome.1e4 Occasionally 1
Posterior sagittal anorectoplasty (PSARP) was initially Only if bowel loaded 5
proposed by Peña and de Vries in 1982 for surgical treat- Continuous day only 8
ment of ARM.5,6 Although low-type ARM (L-ARM) can be Continuous day and night 10
corrected with limited PSARP or anoplasty, correction of Delay in defecation
high-type ARM (H-ARM) requires a three-stage operation, Daily stool 0
including a temporary colostomy, PSARP, and closure of the Every 2e3 d 1
colostomy. While studies have demonstrated that con- Every 3e5 d 2
stipation is common following surgery for L-ARM and that Every 5e10 d 5
fecal incontinence is a common post-operative complica- > 10 d 8
tion following surgery for H-ARM,7e12 we have found, clin- Never 10
ically, that constipation is a common post-operative Pain and difficulty with defecation
problem in both types of anorectal malformation. None 0
The aim of this retrospective study was to assess the Occasionally 1
bowel habits of children after PSARP for anorectal Often 2
malformation. With most stools 4
With every stool 5
Laxative and enema usage
2. Methods None 0
Softeners only 1
We retrospectively reviewed the medical and surgical Softeners and daily stimulants 2
records of 214 children < 18 years of age, who underwent Extra weekend Movicol or picosulfate 4
posterior sagittal anorectoplasty for anorectal malforma- Extra high-dose Movicol or Klean-Prep 8
tion in Mackay Memorial Hospital during the period Laxatives and regular enema or suppositories 10
1988e2008. Among them, 10 (4.7%) died before 1 year of Child’s general health affected by the bowel problem
age and 16 patients were lost to follow-up or had incom- Well 0
plete bowel habit data. Therefore, a total of 188 children Occasionally ill 2
were enrolled in our analysis. The mean follow-up period Often ill 3
was 4.3 years (range Z 2e10 years). Ill most days 4
Patients with L-ARM were corrected directly with limited Never well 5
PSARP or anoplasty. Those with H-ARM were treated with Behavior related to the bowel problem
a three-stage operation, including a temporary colostomy, Cooperative 0
PSARP, and closure of the colostomy. Needs reminding to use the lavatory or potty 2
The followed-up patients were stratified into three Refuses to use the lavatory or potty 3
groups: (1) chronic constipation; (2) stool incontinence; and Also refuses medicines 4
(3) normal bowel movement group, according to stool Also generally difficult behavior 5
consistency, stool frequency, and frequency of encopresis Overall improvement of the symptoms since last seen
(stool soiling). Chronic constipation was defined according Nearly completely O.K. 0
to the Rome III criteria as the presence of lumpy to hard Much better 1
stool in > 25 % of bowel movements and < 3 defecations Some improvement 4
per week for more than 3 months’ duration. Stool soiling Still as difficult 8
was defined as a soft or semi-liquid stool unexpectedly Getting worse 12
leaking around an impacted stool. An abdominal X-ray or Amount of stool detected on abdominal examination
physical exam can find impacted stool in colon or rectum. None palpable 0
Those patients with stool soiling were classified as consti- Little palpable 1
pated in our study. Incontinence was defined as loose or Suprapubic only 2
watery stool passage without defecation sensation or effort To umbilicus 3
(involuntary), occurring > three times per week for more Beyond umbilicus 5
than 3 months’ duration, without fever, episodes of acute Reaching ribs 8
infectious disease, acute gastroenteritis, or other endo- Total 0e65
crine diseases. Changes in bowel habits were evaluated
within the 1st year after PSARP and again between the 2nd
year and the last follow-up recorded in the medical charts. 2.1. Statistical analysis
Severity of constipation in patients in the constipation
group was evaluated using the symptom severity (SS) score All measured values are presented as means  SD. Student t
questionnaire as reported by Keshtgar et al (Table 1).14 test was used to measure differences between the two
254
Table 2 Constipation severity in different genders.
SS score Male Female p
L-ARM (mean) 7.29  3.928 9.36  6.636 > 0.05
H-ARM (mean) 8.07  5.450 8.62  6.145 > 0.05
H-ARM Z high-type anorectal malformation; L-ARM Z low-type
anorectal malformation.
groups. A p value < 0.05 was considered to indicate
statistical significance.
3. Results
The overall male to female ratio was 1.32 (107:81). The
mean post-operational SS scores are shown in Table 2.
There were no significant difference between men and
women.Eighty-five children with L-ARM underwent limited
PSARP or anoplasty at a mean age of 2.6 months. The other
103 children with H-ARM needed to receive three-stage
procedures. The mean age of the operations was as
follows: colostomy at 2.4 days of age, PSARP at 5.0 months
of age, and closure of colostomy at 8.8 months of age.
Within the 1st year after limited PSARP in the L-ARM
patients, 54.1% were found to have constipation, which was
only slightly higher than that of normal bowel movement
(38.8%) and incontinence (7.1%). However, after a mean
follow-up period of 4.3 years, the prevalence of constipation
increased to 64.5%. In children with H-ARM, constipation was
the most common complication during both time periods
(72.8% and 78.6%, respectively), (Figures 1 and 2).
There were no significant differences in mean SS scores
between the two groups during the follow-up period (L-
ARM Z 7.67  5.67 and H-ARM Z 8.25  5.65, p > 0.05)
(Table 3). Incontinence decreased in both groups with time.
In the H-ARM group, 38.8% children had significant
constipation-related complications (such as anal fissure,
mucosal prolapse, and fistula formation) compared with
15.3 % of children in the L-ARM group (Table 4).
4. Discussion
Anorectal malformations occur in 1 out of 4000 to 5000
newborns.13,15 Posterior sagittal anorectoplasty, first
described by Peña and de Vries in 1982,5,6 has gradually
become the treatment of choice for correcting high-level
anorectal malformations.
Figure 1 Patients with low-type anorectal malformations (L)
and high-type anorectal malformations (H) were stratified into
a constipation group, a stool incontinence group, or a normal
bowel movement group.
C.-F. Huang et al
Figure 2 The proportions of each group with (A) L-type ano-
rectal malformations and (B) H-type anorectal malformations.
In the literature, constipation has been reported after
surgery for L-ARM, while stool incontinence was a more
common complication of H-ARM after correction.16e18
However, some studies reported opposite results.19,20 In
our study, using a local population base, we believe con-
stipation was a common late complication of PSARP,
although it was more common in H-ARM (64.5% in the L-ARM
group and 78.6% in the H-ARM) (Table 5). The prevalence of
voluntary and normal bowel function among patients in our
study (23.4%) was markedly higher than those reported in
other long-term follow-up studies (range Z 0e15%).9,10,21
Kiely and Pena predicted that continence might be ex-
pected within weeks of stoma closure in most patients, but
could not be improved as time passed.16,22 In our study,
constipation was a more persistent problem.Whether
PSARP is the main cause of constipation for ARM is still
controversial. In contrast to the previous surgery, PSARP
was used to identify an upstream large intestine section
that has a ganglionic response, followed by resection and
tailoring to an appropriate size. Then, it was taken down
and placed into the newly-built artificial anus. This
approach resulted in a wider upper portion and a narrower
lower portion of intestine. This caused fecal obstruction
and hence, resulted in constipation. Furthermore, Holseh-
neider and other scholars have recently pointed out that
rectal end innervations disturbance caused during surgery,
might produce a high incidence of constipation.23 In 2007,
Senel et al also made a hypothesis that constipation
possibly relates to the recto-anal inhibitory reflex.24 PSARP
preserved the internal circular muscles and hence reduced
Table 3 Constipation severity assessment under severity
scoring system.
First year within PSARP Long-term follow-up
L-ARM 6.57  4.251 8.45  5.666
H-ARM 7.67  5.666 8.25  5.649
H-ARM Z high-type anorectal malformation; L-ARM Z low-type
anorectal malformation; PSARP Z posterior sagittal ano-
rectoplasty procedure.
Constipation after posterior sagittal anorectoplasty
Table 4 Complications associated with constipation.
Complication after H-ARM L-ARM
constipation (n) (n)
Anal fissure 5 2
Fistula 1 0
Infection 4 2
Mucosa prolapse 16 6
Anal stenosis 14 3 anorectoplasty procedure; L-FU year Z those children with
Total 40 13
H-ARM Z high-type anorectal malformation; L-ARM Z low-type
anorectal malformation.
the chance of incontinence. Abnormal colonic motility was
also found in patients with ARM.25 Overall, it is reasonable
to believe that constipation is a more common problem
after PSARP in both types of H-ARM.In some reports, stool
soiling was also classified as constipation. Rintala and Lin-
dahl mentioned, in 2001, that “Constipation is a major
complication in patients who have undergone PSARP for
high ARM. Overflow incontinence is the main cause of fecal
soiling in these patients ”.26 It was inferred that with the
advancement in the medical treatments, severe con-
stipation has been reduced substantially and so too has the
overflow incontinence (Table 6). Thus, this might be the
reason for constipation being the most common sequela in
children after correction of ARM in our study.
It was presumed that the reasons for persistent stool
incontinence post-operatively include overflow continence
resulting from severe constipation, secondary to a short
colon and congenital anomalies, and included significant
sacral agenesis or intraspinal anomalies.27 However, in our
current study, the ratio of sacral anomaly was as low as
3.7%, which might explain our more satisfying prognosis.
A number of scoring systems are used to assess post-
operative bowel habits for patients with ARM,28 but most of
them only apply to the grading of continence. While most of
these methods ultimately categorize the subjective
outcome as “good”, “fair” or “poor”, the SS scoring system
used in this study was based on objective parameters and
integrated physical examination and medication cate-
gories. The parameters in Kelly’s scoring system of fecal
continence,29 including “continence, staining, sphincter”,
fail to indicate constipation. Rintala’s scoring system of
continence includes more details,30 but fails to grade the
severity of constipation and does not objectively control for
social problems. Moreover, most of the existing scoring
Table 5 Bowel habit assessment after PSARP during
childhood.
ARM type Incontinence Constipation
Peña17 H 57% 43.1%
Langemeijer et al18 H 56% 5%
This study H 3.9% 78.6%
L 4.7% 64.5%
ARM Z anorectal malformation; HZ high; L Z low; PSARP Z
post-sagittal anorectoplasty procedure.
255
Table 6 Relationship with stool soiling and constipation.
L-1st year L-FU year H-1st year H-FU year
Constipation (n) 46 55 75 81
Stool soiling (n) 13 6 20 6
Stool soiling (%) 28.3% 10.9% 26.7% 7.4%
L-1st year Z those children with low-type anorectal malforma-
tions were evaluated within the 1st year after post-sagittal
low-type anorectal malformations were evaluated between the
2nd year and the last follow-up recorded in the medical charts
(mean Z 4.3 years); H-1st year Z those children with high-type
anorectal malformations were evaluated within the 1st year
after post-sagittal anorectoplasty procedure; H-FU year Z
those children with high-type anorectal malformations were
evaluated between the 2nd year and the last follow-up recorded
in the medical charts (mean Z 4.3 years).
systems are not suitable for young children, especially
neonates and infants.31e34 The retrospective nature of this
study precluded us from evaluating social and behavioral
problems in our patients. Therefore, we did not include
either category in the questionnaire. In our experience,
complications related to constipation can exacerbate the
severity of constipation. In this study, children with H-ARM
had a significantly higher rate of constipation-related
complications compared to the L-ARM group (38.8% vs.
15.3%, p < 0.05). However, there were no significant
differences in SS scores between the two groups during the
follow-up period. That result might focus our local clini-
cians’ attention on preventing constipation after posterior
sagittal anorectoplasty. At present, for patients with post-
operative constipation, early treatment is mainly anal
dilatation for at least 6 months.16,35 Other methods include
bowel training and biofeedback. If the above measures fail,
stool softeners or laxatives will be used. We believe that
the improvement of the overall clinical bowel continence
was due to successful treatment of constipation and fol-
lowed complications including stool impaction and overflow
incontinence.
5. Conclusion
In conclusion, constipation was a common late problem
after PSARP in both types of ARM, although it was more
frequent in H-ARM. There was a higher rate of constipation-
related complications in H-ARM, but the severity of con-
stipation via SS showed no significant difference between
both types.
References
1. Kim PC, Superina RA, Ein S. Colonic atresia combined with
Hirschsprung’s disease: a diagnostic and therapeutic chal-
lenge. J Pediatr Surg 1995;30:1216e7.
2. Flageole H, Fecteau A, Laberge JM, Guttman FM. Hirsch-
sprung’s disease, imperforate anus, and Down’s syndrome:
a case report. J Pediatr Surg 1996;31:759e60.
3. Raboei EH. Patients with anorectal malformation and Hirsch-
sprung’s disease. Eur J Pediatr Surg 2009;19:325e7.
256
4. Fratino G, Granata C, Dodero P. An association between ano-
rectal malformations and Down’s syndrome. Pediatr Med Chir
1992;14:157e9.
5. Peña A, Devires PA. Posterior sagittal anorectoplasty; impor-
tant technical considerations and new applications. J Pediatr
Surg 1982;17:796e811.
6. de Vries PA, Peña A. Posterior sagittal anorectoplasty. J Pediatr
Surg 1982;17:638e43.
7. Paidas CN. Fecal incontinence in children with anorectal mal-
formations. Semin Pediatr Surg 1997;6:228e34.
8. Peña A, Hong A. Advances in the management of anorectal
malformations. Am J Surg 2000;180:370e6.
9. Doolin EJ, Black CT, Donaldson JS, Schwartz D,
Raffensperger JG. Rectal manometry, computed tomography,
and functional results of anal atresia surgery. J Pediatr Surg
1993;28:195e8.
10. Rintala R, Mildh L, Lindahl H. Fecal continence and quality of
life in adult patients with an operated high or intermediate
anorectal malformation. J Pediatr Surg 1994;29:777e80.
11. Hassink EA, Rieu PN, Severijnen RS, vd Staak FH, Festen C. Are
adults content or continent after repair for high anal atresia? A
long-term follow-up study in patients 18 years of age and older.
Ann Surg 1993;218:196e200.
12. Peña A, Guardino K, Tovilla JM, Levitt MA, Rodriquez G,
Torres R. Bowel management for fecal incontinence in patients
with anorectal malformations. J Pediatr Surg 1998;33:133e7.
13. Drossman DA, Corazziari E, Talley NJ, Thompson WG,
Whitehead WE. Rome II: a multinational consensus document
on functional gastrointestinal disorders. Gut 1999;45:1e81.
14. Keshtgar AS, Ward HC, Sanei A, Clayden GS. Botulinum toxin,
a new treatment modality for chronic idiopathic constipation
in children: long-term follow-up of a double-blind randomized
trial. J Pediatr Surg 2007;42:672e80.
15. Santulli TV. The treatment of imperforate anus and associated
fistulas. Surg Gynecol Obstet 1952;95:601e14.
16. Harjai MM, Bipin Puri, Vincent PJ, Nagpal BM. Fecal inconti-
nence after posterior sagittal anorectoplasty: follow-up of 2
years. MJAFI 2003;59:194e6.
17. Peña A. Anorectal malformations. Semin Pediatr Surg 1995;4:
35e47.
18. Langemeijer RA, Molenaar JC. Continence after posterior
sagittal anorectoplasty. J Pediatr Surg 1991;26:587e90.
19. Levitt MA, Kant A, Peña A. The morbidity of constipation in
patients with anorectal malformations. J Pediatr Surg 2010;
45:1228e33.
C.-F. Huang et al
20. Rintala R, Lindahl H, Marttinen E, Sariola H. Constipation is
a major functional complication after internal sphincter-saving
posterior sagittal anorectoplasty for high and intermediate
anorectal malformations. J Pediatr Surg 1993;28:1054e8.
21. Nixon HH, Puri P. The results of treatment of anorectal
anomalies: a thirteen to twenty year follow- up. J Ped Surg
1977;12:27e37.
22. Kiely EM, Pena A. Anorectal malformations. In: Paediatric Surgery.
5th ed. St Louis: Mosby-Year Book, Inc.; 1998. p. 1425e48.
23. Holschneider AM, Ure BM, Pfrommer W, Meier-Ruge W. Inner-
vation patterns of the rectal pouch and fistula in anorectal
malformations: a preliminary report. J Pediatr Surg 1996;31:
357e62.
24. Senel E, Demirbag S, Tiryaki T, et al. Postoperative anorectal
manometric evaluation of patients with anorectal malforma-
tion. Pediatr Int 2007;49:210e4.
25. Zuccarello B, Romeo C, Scalfari G, et al. Scintigraphic evalu-
ation of colonic motility in patients with anorectal malforma-
tions and constipation. J Pediatr Surg 2006;41:310e3.
26. Rintala RJ, Lindahl HG. Fecal continence in patients having
undergone posterior sagittal anorectoplasty procedure for
a high anorectal malformation improves at adolescence, as
constipation disappears. J Pediatr Surg 2001;36:1218e21.
27. Rintala R. Postoperative internal sphincter function in ano-
rectal malformations d a manometric study. Pediatr Surg Int
1990;5:127e30.
28. Bhatnagar V. Postoperative assessment in anorectal malfor-
mations. I JAPS 2005;10:80e5.
29. Mollitt DL, Malangoni MA, Ballantine TVN, Grosfeld JL. Colos-
tomy complications in children. Arch Surg 1980;115:455e60.
30. Rintala RJ, Lindahl H. Is normal bowel function possible after
repair of intermediate and high anorectal malformations?
J Pediatr Surg 1995;30:491e4.
31. Al-Salem AH, Grant C, Khawaja S. Colostomy complications in
infants and children. Int Surg 1992;77:164e6.
32. Uba AF, Chirdan LB. Colostomy complication in children. Ann
Afr Med 2002;9:9e12.
33. Sowande OA. Complications of colostomy in infants and chil-
dren. Niger J Surg 1999;6:19e22.
34. Chalapathi G, Chowdhary SK, Rao KL, et al. Risk factors in the
primary management of anorectal malformations in Northern
India. Pediatr Surg Int 2004;20:408e11.
35. Kifayat K. Anorectal malformations: functional outcome of
posterior sagittal anorectoplasty. JPMI 2008;22:304e8.