ORIGINAL ARTICLE

Functional Outcome of Anorectal Malformations and Associated

Anomalies in Era of Krickenbeck Classification
Saqib Hamid Qazi1, Ahmad Vaqas Faruque1, Muhammad Arif Mateen Khan1 and Umama Saleem2

ABSTRACT
Objective: To describe the management and functional outcome of anorectal malformations and associated anomalies
according to Krickenbeck classification.
Study Design: Case series.
Place and Duration of Study: The Aga Khan University Hospital, Karachi, from January 2002 to December 2012.
Methodology: Anorectal anomalies were classified according to Krickenbeck classification. Data was collected and
proforma used regarding the primary disease associated anomalies, its management and functional outcome, according
to Krickenbeck classification. Cases included were: all those children with imperforate anus managed during the study
period. Qualitative variables like gender and functional outcome were reported as frequencies and percentages.
Quantitative variables like age were reported as medians with interquartile ranges.
Results: There were 84 children in study group. Most common associated anomaly was cardiac (38%), followed by
urological anomaly (33%). All children were treated by Posterior Sagittal Anorectoplasty (PSARP). Fistula was present in
64 out of 84 (76%) cases. The most common fistula was rectourethral (33%), followed by recto vestibular (31%). According
to Krickenbeck classification, continence was achieved in 62% children; however 27% children were constipated, followed
by 12% children having fecal soiling.
Conclusion: Functional outcome of anorectal malformation depends upon severity of disease. A thorough evaluation of
all infants with ARM should be done with particular focus on cardiovascular (38%) and genitourinary abnormalities (33%).

Key Words: Anorectal malformation. Functional outcome. Krickenbeck classification.

INTRODUCTION cation in 1970 and Wingspread classification in 1984.

Anorectal malformations (ARMs) are common Pena's classified ARMs on the bases of presence and
anomalies in neonates.1 The catastrophe happens as a position of fistula. He also shared his experience with a
result of antenatal dysmorphogenesis of cloaca and new surgical technique, i.e. Posterior Sagittal Anorecto-
urorectum.2 Currently survival rate of ARMs is much plasty (PSARP).
improved because of advancement in surgical All these classifications categorized the ARMs but there
techniques and availability of improved neonatal were variations in terms of follow-up of these children as
perioperative intensive care facilities. Mostly, children it was difficult to compare the functional outcome of
are diagnosed postnatally, and have associated other these children. Krickenbeck group in 2005 published
anomalies; mostly, urological, cardiac, and musculo- their findings and incorporated criteria from Wingspread
skeletal systems.3 Majority of these babies have and Pena's works. They gave the concept of
associated recto urinary fistula. Recto urethral and recto categorization of ARMs in to three categories, i.e.
vestibular fistulae are the most common presentations diagnostic category, surgical procedure category, and
respectively in males and females.3 functional outcome category. Since then it is this
Ammussat, the father of proctoplasty, in 1835 attempted Krickenbeck classification that has been used to
the first classification of ARMs. His work was followed by measure functional outcome of patients of anorectal
Ladd and Gross in 1934, producing the first standard malformation.
classification of this anomaly. Further development led To the best of authors' knowledge, there is no national
to an international classification on the basis of study available regarding the classification of this
puborectalis muscle, followed by International classifi- anomaly according to this classification. This study was
conducted with the objective of describing the
Department of Surgery1 / Medical Student2, The Aga Khan management and functional outcome of anorectal
University Hospital, Karachi. malformations and associated anomalies, according to
Correspondence: Dr. Muhammad Arif Mateen Khan, Head, Krickenbeck classification.
Section of Pediatric Surgery, The Aga Khan University Hospital,
Karachi. METHODOLOGY
E-mail: [email protected] It was retrospective case series. Prior approval was
Received: October 27, 2014; Accepted: December 14, 2015. taken from the Medical Record Audit Committee at The

204 Journal of the College of Physicians and Surgeons Pakistan 2016, Vol. 26 (3): 204-207
Functional outcome of anorectal malformations and associated anomalies

Aga Khan University Hospital, Karachi. Medical records recto-urethral fistula was present in 21/64 (33%),
of all the patients were reviewed who had needed followed by recto-vestibular fistula in 20/64 (31%,
surgical intervention and presented over a period of 10 Table I).
years from January 1, 2002 to December 31, 2012 at the Three-stage procedures were performed in 64/84 (76%).
Aga Khan University Hospital, Karachi, Pakistan. The All children with fistula were managed initially by making
inclusion criterion was all children with anorectal divided descending colostomy followed by PSARP, while
malformations who were born and presented to 5 children (06%) were offered single-stage limited
Emergency / Pediatric Surgery clinics at the study centre PSARP. All of them were newborn and were offered
and needed surgical intervention during the study
single-stage procedure (Table II). There was one child
period. Children who were shifted out of the Hospital and
who had a perineal repair and 8 children were managed
those with inadequate follow-up information were
by cutback anoplasty (Table III). All children with PSARP
excluded from the study.
had colostomy closure later on. There were 18 children
There was a standard protocol of managing all children who developed complications later (Table IV). The most
presented with anorectal malformation with fistula as common complication was anal stenosis (8/84, 10%).
multistage surgery; first divided sigmoid colostomy Four children later presented with recurrent fistulae,
followed by standard Posterior Sagittal Anorectoplasty which were managed by Redo PSARP.
(PSARP), and colostomy closure usually 6 to 8 months The functional outcome of the patients, who were more
after the PSARP. Regular fortnightly follow-up initially than 3 years, was assessed according to the
and then monthly clinic visits were required depending Krickenbeck classification and found that 32/52 (62%)
on the outcome of all these children in the surgical were continent. Most of them had low anorectal
outpatient clinic for monitoring of regular anal dilatation anomalies (17/32, 53%). However, there were 14/52
and postoperative wound care. Throughout the (27%) children who had constipation followed by
postoperative course, there was a close communication different grading of soiling (6/52, 12%), reported by the
with parents all the time regarding the need of regular parents (Figure 1).
anal dilatation and toilet training.
Table I: Frequency of different types of fistulae associated with anorectal
Cases were identified via Hospital Information anomalies.
Management System (HIMS) by using International Presence of fistula - 64
classification of Disease (ICD-9-CM) codes. ARMs were Rectourethral 21/64 (33%)
classified and functional outcomes were assessed Rectovesicular 11/64 (17%)
according to Krickenbeck classification using detailed Rectovestibular 20/64 (31%)
questionnaire completed at each child visit whenever Rectoperineal 06/64 (9%)
possible or by contacting the parents via telephone. Rectovaginal 06/64 (9%)

Collected data were double entered in Epi-Data (version

3.2) by two different data entry persons. SPSS Table II: Types of surgical interventions.
(Statistical Software for Social Sciences) version 17 was Surgical intervention - 84
used for statistical analysis. Qualitative variables like Divided descending colostomy 64 (76%)
gender and functional outcome were reported as Total PSARP 69 (82%)
frequencies and percentages. Quantitative variables like Limited PSARP (single stage) 05 (6%)

age were reported as medians with interquartile ranges. Anoplasty 08 (10%)

Perineal repair 01 (1%)
All the possible efforts were made to maintain the
confidentiality of patients. No identifiable information
Table III: Frequency of other associated anomalies with anorectal
was collected. Data was stored under lock and key in the malformations.
custody of the principal investigator. Other anomalies - 13

RESULTS
Ectopic anus 05/84 (6%)
Anal atresia 03/84 (4%)

There were 84 children including 57 (68%) males and 27 Pouch colon 02/84 (2%)

(32%) females. The median age at presentation was the Cloacal malformation 03/84 (4%)

day of birth, as 56% of children presented immediately

after birth. Associated anomalies were present in 45 Table IV: Surgical complications.
(53%) children. Cardiac anomalies (17/45, 38%) were Surgical complication - 18
the most common co-existed anomalies with anorectal Anal stenosis 08 (10%)
malformations, followed by urological anomalies in Stoma prolapsed 05 (6%)
15/45 (33%) children. Fistula was present in 64/84 Recurrence of fistula 04 (5%)

(76%) children. Out of those who had associated fistula, Stoma stenosis 01 (1%)

Journal of the College of Physicians and Surgeons Pakistan 2016, Vol. 26 (3): 204-207 205
Saqib Hamid Qazi, Ahmad Vaqas Faruque, Muhammad Arif Mateen Khan and Umama Saleem

Krickenbeck International Classification emerged in

2005, which is based on consensus recommendations
of world authorities.11 This classification system is
composed of 3 distinct elements: a diagnostic category,
a surgical procedure category, and a category
documenting functional outcome criteria. With the
inclusion of all defects including rarer ones and surgical
options, Krickenbeck classification aims to rationalize
functional outcome among different clinical and surgical
groups to allow more meaningful comparisons.12
Since the development of this system is fairly recent,
there is paucity of literature on large long-term outcome
Figure 1: Follow-up according to Krickenbeck classification (age > 3 years,
n = 52).
studies using this classifications especially in our region.
Long term functional outcome in children with ARM,
DISCUSSION primarily entails bowel function which is of vital
importance as fecal incontinence and/or constipation
Anorectal malformations comprise a wide spectrum of
remain major postoperative complications that impede
defects ranging from slight malpositioning of the anus
social and psychological development of these
with excellent functional outcomes to complex
patients.13,14 Continences, defined as the ability to
anomalies of the hindgut and urogenital organs that are
initiate voluntary bowel movement with no soiling,
difficult to manage.4 Commonly known as imperforate
regular bowel habits with no constipation, in turn defined
anus, it affects 1 in 4000 to 5000 live births worldwide
as the passage of infrequent or hard stools, and overall
with a slight male predominance.5 The anomaly may
quality of life, are the parameters looked at when
occur in isolation but is commonly associated with other
assessing functional prognosis in such patients.15 In this
anomalies with incidence ranging from 40 - 60% in
study, there were 32/52 (62%) children who were
different series. The commonest of these are in the
continent. Mother's education is part and parcel of the
urinary tract (35%), the vertebral system (18%), and in
better functional outcome as most of these children were
the developing heart (10%). A known association of
toilet trained mainly because of mother's compliance to
anomalies is known as the VACTERL group (vertebral,
toilet training drills.
anorectal, cardiac, tracheoesophageal, renal and limb).6
The Krickenbeck classification allows for international
Many classification systems for ARM have been devised
criteria for their treatment and development of a uniform
over the years; the first of which was in 1970s which
international scoring system for comparable follow-ups.
described low, high, intermediate and miscellaneous
One of the first such studies was conducted in 2008 by
lesions for both genders based on the position of the
Hassett et al. which evaluated the 10-year outcome of
terminal rectum to the levator ani. The same anatomic
children born with ARM and treated by posterior sagittal
relationship formed the basis of the widely used 1984
anorectoplasty.3 This group of authors advocated with
Wingspread classification where the categories
attempts to rationalize and demonstrate application of
aforementioned were subdivided for males and females
the Krickenbeck classification for both diagnosis and
separately.7,8 A more surgically oriented classification
functional outcome in terms of constipation, urinary
was then proposed in 1995 by Pena as a result of his
control and soiling.
experience with posterior sagittal anorectoplasty
(PSARP).4 This was based on the presence and position Here, the researchers evaluated the follow-up of these
of fistula and on the relationship of the terminal colon to children according to Krickenbeck classification and we
the levator sling muscles of the pelvic floor. The found that continence, followed by constipation and fecal
advantage of the classification of Pena was that the type soiling, was the most common functional outcome in this
of the fistula provided information not only about study. Commonly, constipation occurs as a consequence
localization of the blind pouch but also on the anticipated of chronic dilatation of the rectal pouch due to failure to
extent of mobilization of the atretic rectal segment evacuate stool adequately. Mostly, it is seen in low
necessary to perform sacro perineal or abdomino- fistulae.16-18 Soiling occurs because of defects in the
sacroperineal pull-through.9,10 This classification system sphincter mechanism or as a consequence of overflow
was also the first one which attempted to determine from chronic constipation.19 Similar results were seen in
prognosis for each group in terms of functional bowel this study as 9/18 (50%) children, who were constipated,
outcomes. had low anorectal anomalies.
With recognition of rarer anomalies not previously Cardiovascular anomalies (38%) followed by urological
included in any classification and development of anomalies (33%) were the most commonly associated
advanced surgical procedures other than PSARP, the anomalies in this study. Similar results were found in a

206 Journal of the College of Physicians and Surgeons Pakistan 2016, Vol. 26 (3): 204-207
Functional outcome of anorectal malformations and associated anomalies

study from Singapore, in which they found similar 4. Pena A. Anorectal malformations. Semin Pediatr Surg 1995;
associated anomalies (28% and 19%, respectively).20 4:35-47.
5. Blesa Sanchez E. Anorectal malformations: anatomy,
To the best of authors' understanding, there is no
classification and diagnosis. Cir Pediatr 1988; 1:58-61.
national study published which stratifies the functional
outcome of this anomaly. This study is one of its own 6. Cho S, Moore SP, Fangman T. One hundred three consecutive
kind from Pakistan, addressing the need of uniform patients with anorectal malformations and their associated
anomalies. Arch Pediatr Adolesc Med 2001; 155:587-91.
application of this classification for measuring functional
outcome. The authors strongly suggest to make an 7. Kelly JH. Cine radiography in anorectal malformations.
anorectal malformation registry at national level so that J Pediatr Surg 1969; 4:538-46.
we will come across the multicenter functional outcome 8. V.D. Upadhyaya AG, Srivastava P, Hasan Z, Sharma S.
of this anomaly for better understanding and Evolution of management of anorectal malformation through
management. Like all retrospective studies, there are the ages. Internet J Surg 2008; 17. Available from: http://
the same limitations, as some time it was very difficult to ispub.com/IJS/17/1/3593
recall although all possible measures were taken to 9. Pena A. Advances in the management of fecal incontinence
double-confirm the findings by other observers. In secondary to anorectal malformations. Surg Annu 1990; 22:
addition to the parents’ understanding about the 143-67.
functional outcome, there were variations of responses 10. Pena A, Hong A. Advances in the management of anorectal
by mothers in terms of different grading of constipation. malformations. Am J Surg 2000; 180:370-6.
11. Holschneider A, Hutson J, Pena A, Beket E, Chatterjee S,
CONCLUSION Coran A, et al. Preliminary report on the international
conference for the development of standards for the treatment
Functional outcome of anorectal malformation is related
of anorectal malformations. J Pediatr Surg 2005; 40:1521-6.
to severity of disease. Children with low anorectal
malformations usually have a good functional outcome; 12. Rintala RJ. Congenital anorectal malformations: anything
however, soiling is likely to be a long-term complication. new? J Pediatr Gastroenterol Nutr 2009; 48:S79-82.
There should be uniform approach to know the 13. Davies MC, Creighton SM, Wilcox DT. Long-term outcomes of
functional outcome which should be part and parcel for anorectal malformations. Pediatr Surg Int 2004; 20:567-72.
preoperative counselling to parents. A thorough 14. Kaselas C, Philippopoulos A, Petropoulos A. Evaluation of
evaluation of all infants with ARM should be done with long-term functional outcomes after surgical treatment of
particular focus on cardiovascular and genitourinary anorectal malformations. Int J Colorectal Dis 2011; 26:351-6.
abnormalities. 15. Aminoff D, La Sala E, Zaccara A. Follow-up of anorectal
Acknowledgement: We would like to acknowledge the anomalies: the Italian parents' and patients' perspective.
J Pediatr Surg 2006; 41:837-41.
parents of all those children who participated in this
study, without which this study was not possible. Also we 16. Rintala RJ, Lindahl HG, Rasanen M. Do children with repaired
would like to acknowledge Dr. Noman Shahzad, resident low anorectal malformations have normal bowel function?
J Pediatr Surg 1997; 32:823-6.
in general surgery at Aga Khan University Hospital,
Karachi for his help in statistical analysis and revision of 17. Pakarinen MP, Koivusalo A, Lindahl H. Prospective controlled
manuscript. long-term follow-up for functional outcome after anoplasty in
boys with perineal fistula. J Pediatr Gastroenterol Nutr 2007;
REFERENCES 44:436-9.

1. Risto J. Rintala, Mikko P. Pakarinen. Imperforate anus: long- 18. Yeung CK, Kiely EM. Low anorectal anomalies: a critical
and short-term outcome. Semin Pediatr Surg 2008; 17:79-89. appraisal. Pediatr Surg Int 1991; 6:333-5.

2. Qi BQ, Williams A, Beasley S. Clarification of the process of 19. Cheu HW, Grosfeld JL. The atonic baggy rectum: a cause of
separation of the cloaca into rectum and urogenital sinus in the intractable obstipation after imperforate anus repair. J Pediatr
rat embryo. J Pediatric Surg 2000; 35:1810-6. Surg 1992; 27:1071-3.

3. Hassett S, Snell S, Hughes-Thomas A, Holmes K. 10-Year 20. Shireen A. Nah, Caroline CP Ong, Narasimhan K. Lakshmi, Te-
outcome of children born with anorectal malformation, treated Lu Yap, Anette S. Jacobsen, Yee Low. Anomalies associated
by posterior sagittal anorectoplasty, assessed according to the with anorectal malformations according to the Krickenbeck
Krickenbeck classification. J Pediatr Surg 2009; 44:399-403. anatomic classification. J Pediatr Surg 2012; 47: 2273-8.

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