J Indian Assoc Pediatr Surg. 2012 Jul-Sep; 17(3): 120–123.

doi: 10.4103/0971-9261.98131
PMCID: PMC3409900

Anorectal malformations: Definitive

management during and beyond
adolescence
Minakshi Sham, Dasmit Singh, and Dileep Phadke
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Abstract
Aim:

To evaluate our results of definitive repair of anorectal malformations in patients with

delayed presentation, during and beyond adolescence.

Material and Methods:

It is a retrospective analysis of all adolescent patients presenting for the first time for
definitive repairs and innate patients - colostomy performed during the neonatal period,
but who had lost to follow-up. It includes 15 patients (2 male and 13 female) aged from
13 to 32 years. Three well-decompressed female patients were managed by primary
anterior sagittal anorectoplasty (ASARP). Twelve patients underwent staged procedures.
Five patients (two male and three female) underwent posterior sagittal anorectoplasty
(PSARP). The oldest male patient underwent abdominal-PSARP.

Results:

All of them attained socially acceptable fecal continence at follow-up of 1–4½ years.
They are satisfied with the functional and cosmetic outcome of repair of their anomalies.

Conclusions:

Prospects of fecal continence are good when definitive repair of anorectal malformations
is done by an expert, even in the adolescent age group and beyond.

KEY WORDS: Anorectal malformations, anovestibular fistula, delayed presentation,

pouch colon
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INTRODUCTION
Presentation of anorectal malformations in the adolescence is not uncommon in resource-
poor environment.[1] Staged management is often recommended for treatment of
anorectal malformations, especially in adolescents.[1–4] We present our experience of
definitive repair (primary and staged) of anorectal malformations with delayed
presentation.

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MATERIALS AND METHODS

We have operated 15 patients with anorectal malformations between 13 to 32 years of age
from January 2006 to December 2010. There were 2 male and 13 female patients. It is a
retrospective, nonrandomized study of case records of above defined group and analysis
of our results in the study population.

Plain radiographs of the spine, ultrasonography of the abdomen, and micturating cysto-
urethrogram (MCU) were done in all. Fistulogram using water-soluble contrast medium
was done in all female patients. Ten female patients, who had constipation at the time of
presentation and grossly dilated rectosigmoid on the fistulogram, underwent completely
divided sigmoid colostomy as an initial step. The different procedures performed for
definitive correction included posterior sagittal anorectoplasty (PSARP), anterior sagittal
anorectoplasty (ASARP), or abdominal-assisted PSARP/ASARP. The operative time
required for different definitive procedures was about 2–5 h.

Neoanal dilatations were started from postoperative day 10 to 14 and continued for about
6 months, postcolostomy closure. Colostomy closure was done 6–12 weeks following the
definitive procedure.

Repair in male patients

Both male patients underwent repair by PSARP approach. The 32-year-old male required
abdominal mobilization, in addition to PSARP. The second patient also underwent
PSARP. However, mobilization of the pouch was quite difficult and time consuming
because of high placement of the rectum and convexity of the adolescent sacrum, making
it further difficult to access the anorectal pouch.

Repair in female patients

Majority of female patients underwent definitive repair by ASARP.[5,6] Primary ASARP

was done in three well-decompressed female patients. All other patients underwent staged
procedures. Three female patients underwent repair by the PSARP approach, because of
the surgeon's preference. Two female patients (one operated outside) required redo
definitive procedures. One female patient repaired by PSARP, had retracted and
anteriorly positioned anal opening. ASARP was done as redo operation in her with good
results.[7]

The 16-year-old adolescent girl had type IV pouch colon[8] (case 9) measuring 25 × 15 ×
15 cm with a very wide colovestibular fistula. She underwent pouch excision and
abdomino-ASARP in stages. The female child who had completed all three stages outside
and had presented to us with recurrent vestibular and large rectovaginal fistulae was
managed by initial redo colostomy. Division of fistula with vaginoplasty and Y-V
anoplasty were done for correction of her anomaly.

All patients were electively taught Kegel exercises starting 1 month after the definitive
repair. Colostomy closure was done between 6 and 12 weeks following the definitive
repair. Size of anal opening, anal tone, and bowel habits were assessed during follow-up
visits. The results (postoperative fecal continence) were judged by Kelly's continence
score,[9] 6 months, 1 year, and 4 years postoperatively. The follow-up period ranged
from 1 to 4½ years [Table 1].

Table 1
Case details
Difficulties encountered and technical modifications utilized

 Patient positioning on table was a major problem especially during PSARP. Prone
jack-Knife position is the standard position described for PSARP. Our patients
were placed prone with adequate padding underneath the groins; but with few
modifications. After induction of anesthesia on a trolley, and Foley catheterization
of the bladder, the patient was log rolled and placed prone onto the operation table
like a trauma patient. The repair was completed by standing on the right side of
the operation table, rather than standing at the foot end as has been described in
the standard text.
 Many authors have described sagittal splitting of the coccyx to gain access to high
rectal pouch.[2,4,10] We, in addition, resorted to removal of the coccyx
(coccygectomy) in three of our patients who underwent PSARP.
 The convexity of the adolescent sacrum and high placement of the rectum made
the dissection difficult. Gentle downwards and sideways traction was applied to
the anorectal pouch to make the neurovascular bundles taut and bring them under
view. Patiently done dissection using mixter forceps and use of bipolar diathermy
helped.
 Division of thick neurovascular bundles was required to mobilize adequate length
of the rectum. Bleeding was more as compared to definitive procedures in
younger children. This was tackled by using bipolar diathermy and adequately
coagulating the neurovascular bundles before cutting them.
 It was difficult to accommodate the dilated rectum within the sphincter muscle
complex. Tapering of the rectum over #14 Hegar dilator was done in 10 patients.
 The operative time ranged from 2 to 5 h as compared to 1¼ to 2 hours for similar
procedures performed by us in younger children.
 Management of ARM with pouch colon raises special problems. Proximal
diversion in the form of window colostomy, proximal ileostomy, end colostomy
after division of fistula, or end colostomy after coloplasty has been described as
the first step.[9,11] None of the above, best-suited our case. Here, the pouch was
grossly dilated and fecally loaded. Postoperatively, it would have been impossible
to clean the pouch through the vestibular fistula, had it been left behind. Also
complete excision of the pouch was impractical. Hence, we did subtotal excision
of the pouch alongwith descending end colostomy. Abdomino-ASARP was
performed for the definitive repair in this girl, rather than abdomino-PSARP, since
we believed approaching both the abdomen and the perineum were easy in the
supine- lithotomy position.

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RESULTS
No deaths occurred in the postoperative period. Vaginal tear occurred in one, while partial
tear of most distal part of fistula occurred in two children. One patient with primary
ASARP developed superficial wound infection and dehiscence. Retraction of the anus
was noted in one female patient who had undergone repair by PSARP approach. She also
had anteposed anus. This was corrected by an ASARP with reconstruction of the perineal
body. Neoanal narrowing occurred in one female patient (case 3), who did not follow the
regime of neoanal dilatations. This required correction by Y-V anoplasty. The female
child who had completed all three stages outside and had presented to us with recurrent
vestibular and large rectovaginal fistula (case 5) was managed by initial redo colostomy.
Division of fistula with vaginoplasty and Y-V anoplasty were done for correction of her
anomaly. The cosmetic result was very good, despite a redo surgery.

Among patients staged with colostomy, troublesome peri-anal excoriations following

colostomy closure was a significant problem in two patients. It required diet modulations,
regular applications of egg albumin, zinc oxide, etc., and good peri-anal care, i.e.,
keeping the area clean and dry. This settled over a period of 15 days or so. All our
patients are satisfied with the functional and cosmetic outcome of repair of their
anomalies at a follow-up of 1 to 4½ years. Our continence results are 75%–80%, i.e.,
comparable to other large series treating adolescent patients.[12]

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DISCUSSION
Adolescent anorectal malformations comprised 15%–20% ARM patients operated at our
institution. Female preponderance was noted probably because of poverty, lack of access
to advanced healthcare in rural areas of Maharashtra, or neglect of the female children in
our society. Also the need for emergency colostomy in neonatal period in male children
with high and intermediate lesions may have had an impact towards early definitive
repairs in them.

It is desirable to complete the procedure of anorectoplasty before the age of 6 months.

[13] Hashmi et al. have also described primary ASARP in well-decompressed female
children with delayed presentation.[14] Sinha et al. have performed primary ASARP in
three adolescent female patients with low ARM with good results.[12] They have also
tried to analyze the causes of delayed presentation of ARM, which include wrong advice
regarding the correct age of treatment, inadequate management of ARM elsewhere,
delayed diagnosis, lack of money, and lack of social support.[12] Poverty, presence of
associated cardiac anomalies [Table 1], inadequate knowledge about the management of
ARM, and lack of access to advanced healthcare in rural areas of Maharashtra (India)
were the factors noted by us in the present study. Delayed presentation has been defined
as male patients presenting after 7 days of birth and female patients with presentation
beyond 6 months of age in study by Sinha et al.,[12] whereas in our case all patients were
above 13 years - all female patients coming to our centre for the first time and
colostomised males presenting for definitive repair for the first time.

Though, most of the intermediate and high malformations in both males and females are
amenable to correction by PSARP approach, in few cases of very high lesions, adequate
mobilization of the distal pouch to obtain a tension free skin-mucosa anastomosis and a
normally located neoanus may still be difficult. Abdominal mobilization of the high
rectum/vagina is often combined with PSARP in these cases.[2–4] In our study too (case
4), since the patient had recto-bladder neck fistula, mobilization of the rectal pouch by
PSARP approach alone was difficult. Also the problem was compounded by the patient's
age, ascent of colostomy with age, and the curvature of the sacrum, making it further
difficult. Hence, we resorted to abdomino-PSARP.

From this study, we wish to emphasize the fact that no patient requiring definitive
correction of his/her anorectal malformations should be denied the procedure (in the
absence of gross sacral anomalies) on the basis of age alone. In fact, there are ample
reports in literature of definitive procedures either primary or secondary procedure, done
even in adult patients with ultimate achievement of socially acceptable continence. Gil-
Vernet et al. have described good results with Rehbein abdominoperineal pull-through
plus a PSARP for high anorectal malformations in the age group of 3-20 years.[15]
Similarly good level of continence was achieved by Clifford et al.[16] by employing
PSARP as a secondary procedure with failed primary repairs. Our continence results of
75%–80% are comparable to other series treating adolescent patients.[2] Creating
awareness among the public and health care professionals about the problem of anorectal
malformations, the need for early operative correction in them and making pediatric
surgical health care accessible at peripheral health care centers should be our ultimate
goal.[17]

Our's being a nonrandomized study with a small sample size, the results may be skewed.
Similarly, our follow-up is short, ranging from 1 to 4½ years. Our patients are under
regular follow-up and we will evaluate our long-term results. Nevertheless, we think, our
results are representative and the sample size is adequate enough to encourage more and
more pediatric surgeons to undertake definitive repairs even in older patients with ARM
and restore their right to normal defecation, which they very much deserve. Also, to the
best of our knowledge, this is one of the largest series from India treating adolescents and
adult patients with ARM.

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Footnotes
Source of Support: Nil

Conflict of Interest: None declared.

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