Abstract
Background
The European consortium on anorectal malformations (ARM-NET) was established to improve
the health care of patients and to identify genetic and environmental risk factors.
The aim of the present study was to present the first results on clinical data of
a large European cohort of ARM patients based on our registry.
Methods
In 2010, the registry was established including patient characteristics and data on
diagnosis, surgical therapy, and outcome regarding complications. Patients born between
2007 and 2012 were retrospectively added. A descriptive analysis of this cohort was
performed.
Results
Two hundred and three ARM patients were included. Syndromes or chromosomal abnormalities
were present in 9%. Perineal fistulas were seen most in boys (42%) and girls (29%).
Rare forms of ARM were found in 4% of the male and in 14% of the female patients.
Forty-five percent of the patients had additional urogenital abnormalities. However,
32% of the patients were never screened for bladder abnormalities. Eight percent were
never screened for renal malformations. In the majority of patients (79%), a PSARP
was performed for the definitive reconstruction.
Conclusion
This collaborative effort provides a representative basis to estimate incidence of
ARM types, to discuss differences and similarities in treatment, and health consequences
throughout Europe.
Key words
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References
- Maternal and paternal risk factors for anorectal malformations: a Dutch case–control study.Birth Defects Res A Clin Mol Teratol. 2010; 88: 152-158
- Prevalence estimation of anorectal malformations using German diagnosis related groups system.Pediatr Surg Int. 2007; 23: 1161-1165
- Chromosomal anomalies in the etiology of anorectal malformations: a review.Am J Med Genet A. 2011; 155A: 2692-2704
- Anorectal malformations.Orphanet J Rare Dis. 2007; 2: 33
- Analysis of 1,992 patients with anorectal malformations over the past two decades in Japan.J Pediatr Surg. 1999; 34: 435-441
- Anomalies associated with anorectal malformations according to the Krickenbeck anatomic classification.J Pediatr Surg. 2012; 47: 2273-2278
- Outcomes from the correction of anorectal malformations.Curr Opin Pediatr. 2005; 17: 394-401
- Research perspectives in the etiology of congenital anorectal malformations using data of the International Consortium on Anorectal Malformations: evidence for risk factors across different populations.Pediatr Surg Int. 2010; 26: 1093-1099
- Preliminary report on the International Conference for the Development of Standards for the Treatment of Anorectal Malformations.J Pediatr Surg. 2005; 40: 1521-1526
- VACTERL/VATER Association.Orphanet J Rare Dis. 2011; 6: 56
http://www.eucerd.eu/upload/file/EUCERDRecommendationCE.pdf.
- The tethered spinal cord in patients with anorectal malformations.J Pediatr Surg. 1997; 32: 462-468
- Sacral development in anorectal malformations and in normal population.Pediatr Radiol. 2001; 31: 858-862
- Advances in pediatric colorectal surgical techniques.Semin Pediatr Surg. 2010; 19: 86-95
- Long-term results of anterior sagittal anorectoplasty for the treatment of vestibular fistula.J Pediatr Surg. 2009; 44: 1913-1919
- Unexpected results of a nationwide, treatment-independent assessment of fecal incontinence in patients with anorectal anomalies.Pediatr Surg Int. 2012; 28: 825-830
Article info
Publication history
Accepted:
July 25,
2013
Received in revised form:
July 24,
2013
Received:
March 24,
2013
Identification
Copyright
© 2013 Elsevier Inc. Published by Elsevier Inc. All rights reserved.