Eur J Pediatr Surg 2025; 35(02): 112-119
DOI: 10.1055/s-0044-1791257
Original Article

European Reference Network eUROGEN Guidelines on the Management of Anorectal Malformations, Part II: Treatment

Ophelia Aubert 
1   Department of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany
,
Willemijn F. E. Irvine
2   Department of Evidence Based Medicine and Methodology, Qualicura Healthcare Support Agency, Breda, The Netherlands
,
Dalia Aminoff
3   AIMAR-Associazione Italiana Malformazioni AnoRettali, Rome, Italy
,
Ivo de Blaauw 
4   Department of Pediatric Surgery, Radboudumc Amalia Children's Hospital, Nijmegen, The Netherlands
,
Salvatore Cascio
5   Department of Pediatric Surgery, School of Medicine, University College Dublin and Children's Health Ireland, Dublin, Ireland
,
Célia Cretolle
6   Department of Pediatric Surgery, Urology, and Transplantation, Hôpital Necker Enfants Malades, APHP, Université de Paris Cité - National Rare Disease Center MAREP, Paris, France
,
Barbara Daniela Iacobelli
7   Department of the Fetus-Newborn-Infant, Ospedale Bambin Gesù - Medical and Surgical, Rome, Italy
,
Konstantinos Mantzios
2   Department of Evidence Based Medicine and Methodology, Qualicura Healthcare Support Agency, Breda, The Netherlands
,
Paola Midrio
8   Pediatric Surgery Unit, Cà Foncello Hospital, Treviso, Veneto, Italy
,
Marc Miserez
9   Department of Abdominal Surgery, UZ Leuven, KU Leuven, Leuven, Flanders, Belgium
,
Sabine Sarnacki
6   Department of Pediatric Surgery, Urology, and Transplantation, Hôpital Necker Enfants Malades, APHP, Université de Paris Cité - National Rare Disease Center MAREP, Paris, France
,
Eberhard Schmiedeke 
10   Department of Pediatric Surgery and Urology, Centre for Child and Youth Health, Klinikum Bremen-Mitte, Bremen, Germany
,
Nicole Schwarzer
11   Selfhelp Organization for Parents and Patients Born with Anorectal Malformations—SoMA e.V., Munich, Germany
,
Cornelius Sloots
12   Department of Pediatric Surgery, Erasmus Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands
,
Pernilla Stenström 
13   Department of Pediatrics, Clinical Sciences Lund University, Skane University Hospital Lund, Lund, Sweden
,
Martin Lacher   *
14   Department of Pediatric Surgery, University Hospital Leipzig, Leipzig, Germany
,
Jan-Hendrik Gosemann   *
14   Department of Pediatric Surgery, University Hospital Leipzig, Leipzig, Germany
› Author Affiliations
Funding This study was supported by the European Union.
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Abstract

Introduction Anorectal malformations (ARMs) are rare birth defects affecting the anorectum and oftentimes the genitourinary region. The management of ARM patients is complex and requires highly specialized surgical and medical care. The European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM applicable on a European level.

Methods The Dutch Quality Standard for ARM served as the basis for the development of guidelines. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from seven European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering the current evidence, expert opinion, and the European context.

Results Surgical and medical treatment of ARM, postoperative instructions, toilet training, and management of fecal and urinary incontinence were addressed. Seven new studies were identified. The panel adapted 23 recommendations, adopted 3, and developed 8 de novo. The overall level of newly found evidence was considered low.

Conclusion Treatment of ARM patients requires a multidisciplinary team and expertise about anatomical and surgical aspects of the disease, as well as long-term follow-up. This guideline offers recommendations for surgical and medical treatment of ARM and associated complications, according to the best available evidence and applicable on a European level.

Keywords

ARM - guidelines - quality standard - evidence-based - consensus

Collaboration

This work was performed in collaboration with experts involved in ERN ERNICA, EUPSA, and Qualicura.


* These authors contributed equally.


Supplementary Material



Publication History

Received: 14 August 2024

Accepted: 27 August 2024

Article published online:
19 September 2024

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