Abstract
Purpose
“Low” anorectal malformations (ARMs) are considered minor anomalies of the distal
rectum and anal–canal development. Nonetheless, the prognosis of affected patients
is far from excellent, as some degree of constipation is a frequent complaint in the
long-term follow-up. Constipation in “low” ARM has been reported in 42%–70% of cases.
Vestibular fistulas seem to have the highest rate of constipation (not less than 61.4%).
The aim of this study was to evaluate all the histological wall abnormalities of ARM
with recto-perineal and recto-vestibular fistulas in order to identify features that
could explain the bowel dysfunctions. Moreover, the resection of distal perineal and
vestibular fistulas (last 3 cm) allowed evaluating functional results in “low” ARM series with extensive fistula
resection.
Methods
One hundred four specimens were collected from 52 patients (32 recto-perineal and
20 recto-vestibular fistulas) during the posterior sagittal anorectoplasty (PSARP).
The distal 3 cm of aberrant anorectal canals (fistulas) was systematically resected and divided
longitudinally. One portion was fixed for immuno-histochemical stainings (PGP 9.5,
S-100, NSE), H&E, and tricromic stainings. The frozen sections of the second portion
were incubated for enzyme histochemical stainings (AChE, etc.). The follow-up of 42
of 52 ARM was postoperatively evaluated at 3–8 years of age, and the assessment of the outcome after PSARP repair was in line with
Krickenbeck's 2005 meeting parameters.
Results
Muscle coat was abnormal in all cases (100%), showing aspect and absence of organization
into the circular and longitudinal layers. The connective tissue was found to be irregular
and abnormally represented in 100% of cases. Abnormal vascularization was detected
in 5 cases (9.6%). All vestibular (100%) and 71.8% of perineal fistulas showed different
degrees of enteric nervous system (ENS) anomalies. In the series of 42 patients followed
up at least after 3 years of age, 40 cases (95.2%) showed postoperative good continence without use of
laxatives (according to Krickenbeck's 2005 criteria).
Conclusion
Every wall component of the distal rectum can be affected by different structural
abnormalities in “low” ARMs. Pediatric surgeons should take into consideration the
implications of these structural abnormalities during radical treatment. The resection
of a significant portion of the distal fistula seems to permit better functional results.
Key words
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Article info
Publication history
Accepted:
March 8,
2013
Received:
February 23,
2013
Identification
Copyright
© 2013 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
