Anorectal malformations (ARM) encompass a spectrum of rare congenital defects of the rectum and anus, requiring specialized reconstructive surgery. To improve epidemiological and clinical research in rare diseases such as ARM, collaborative efforts and patient registries are key. This retrospective study pools clinical data over a 30-year period from two ARM patient registries (The Royal Children's Hospital (RCH) in Melbourne, Australia, and the ARM-Network Consortium in Europe).
View Article and Find Full Text PDFIn the past, an anteriorly located anus was often misdiagnosed and treated as an anorectal malformation (ARM) with a perineal fistula (PF). The paper aims to define the criteria for a normal anus, an anterior anus (AA) as an anatomic variant, and milder types of ARM such as congenital anal stenosis (CAS) and PF. An extensive literature search was performed by a working group of the ARM-Net Consortium concerning the subject "Normal Anus, AA, and mild ARM".
View Article and Find Full Text PDFJ Pediatr Surg
September 2022
Background: Outcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging.
View Article and Find Full Text PDFObjective: To review the Anorectal Malformation Network experience with perineal groove (PG) focusing on its clinical characteristics and management.
Study Design: Data on patients with PG managed at 10 participating Anorectal Malformation Network centers in 1999-2019 were collected retrospectively by questionnaire.
Results: The cohort included 66 patients (65 females) of median age 1.
Purpose: Since pediatric surgeons aim to follow their patients with anorectal malformations (ARM) into adulthood the aim of this study was to investigate how pediatric surgeons deal with sexual issues related to ARM.
Methods: In 2018, a questionnaire was developed by the working group "Follow-up and sexuality" of the ARM-Net consortium and sent to all consortium-linked pediatric surgeons from 31 European pediatric surgical centers. Obtained data were statistically analyzed.
Introduction: Intractable full-thickness rectal prolapse (IRP) unresponsive to conservative treatment remains a major problem after anorectoplasty for high or intermediate anorectal malformation (ARM). Surgical management must aim for a permanent fixation of the rectum to the presacral fascia. While in children with IRP following ARM repair the optimal procedure has not been established yet, laparoscopic posterior mesh-rectopexy (Well's procedure) has demonstrated efficacy in adults.
View Article and Find Full Text PDFPurpose: In 1989, Sauer introduced a technique to treat patients with total colonic aganglionosis (TCA) where he preserved the ileo-cecal valve and the right hemicolon to retain water and electrolyte absorption. This report examines the long-term outcome of patients who underwent this technique.
Methods: All TCA patients treated between 1981 and 2005 according to Sauer were invited in 2013 to participate in a questionnaire survey to assess their long-term outcome focusing on bowel function and quality of life (QoL), using standardized scores.
Background: Since the introduction of the ALARA ("as low as reasonably achievable") concept, ultrasound (US) has been progressively advocated for paediatric diagnostic imaging. This study aimed to analyse the role and accuracy of US in paediatric renal trauma.
Methods: From 1999 to 2009, the tertiary-care-hospital database was retrospectively evaluated for renal trauma with regards to aetiology, type of injury, diagnostics, management and outcome.