The European Anorectal Malformation Network (ARM-Net) patient registry: 10-year review of clinical and surgical characteristics.
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ABSTRACT:
SUBMITTER: Hageman IC
PROVIDER: S-EPMC10870250 | biostudies-literature | 2024 Jan
REPOSITORIES: biostudies-literature
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The European Anorectal Malformation Network (ARM-Net) patient registry: 10-year review of clinical and surgical characteristics.
Hageman Isabel C IC Midrio Paola P van der Steeg Hendrik J J HJJ Jenetzky Ekkehart E Iacobelli Barbara D BD Morandi Anna A Sloots Cornelius E J CEJ Schmiedeke Eberhard E Broens Paul M A PMA Fascetti Leon Francesco F Çavuşoğlu Yusuf H YH Gorter Ramon R RR Trajanovska Misel M King Sebastian K SK Aminoff Dalia D Schwarzer Nicole N Haanen Michel M de Blaauw Ivo I van Rooij Iris A L M IALM
The British journal of surgery 20240101 2
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Project description:AimThere is a knowledge gap regarding which patients with anorectal malformations (ARMs) are at highest risk of anorectal prolapse (AP), and which risk factors predispose to AP in ARM. The aims of the study were to define the frequency of AP after ARM reconstruction, and explore risk factors.MethodData from the ARM-Net registry inserted between 2007 and 2023 were used. Inclusion criteria were the reconstruction performed, no stoma at 1-year follow-up and all data available at 1-year follow-up. The statistics used were univariable and multivariable logistic regression models.ResultsAfter exclusions the incidence of AP was 163 in 1117 patients (14.6%) in data inserted by 31 centres from 12 countries. The AP incidence was unevenly distributed between the centres (interquartile range 6.3%-21.7%). AP was more frequent in boys than girls (20.9% vs. 8.1%; P < 0.001). In both sexes the incidence of AP was higher in complex ARM subtypes (P < 0.001). AP was most frequent after laparotomy- and laparoscopic-assisted reconstructions (50.0% and 37.5%, respectively). Spinal and sacral anomalies constituted risk factors for AP in univariable analyses, while tethered cord did not. Adjusted risk factors for AP were severity of ARM subtype (40% in long-channel cloaca and bladder neck fistula, OR 3.1, 95% CI 1.0-10.2), laparotomy-assisted posterior sagittal anorectoplasty (50%, OR 3.7, 95% CI 1.6-8.4) and larger neo-anus at 1-year follow-up (Hegar 13.6 vs. 13.1; OR 1.2, 95% CI 1.1-1.4). Constipation was not a risk factor for AP.ConclusionAnorectal prolapse is a frequent postoperative sequela. Adjusted analyses indicate that severity of ARM, abdominal open access during reconstruction and larger size of anus are risk factors.
| S-EPMC11788518 | biostudies-literature
Project description:Müllerian duct anomalies are rare in the general population, occurring in less than 3% of women, but much more prevalent in female patients with anorectal malformation, occurring in up to 30% of these patients. Unicornuate uterus with a rudimentary non-communicating horn is a congenital anomaly of Mullerian development which can be seen in isolation or in conjunction with other anomalies, with several case reports described in patients with VACTERL association. These anomalies may be asymptomatic until the patient develops dysmenorrhea or devastating obstetrical complications. We describe the successful surgical management of an obstructive Müllerian anomaly in a post-pubertal female patient with anorectal malformation.
| S-EPMC8372188 | biostudies-literature
Project description:Despite surgical correction, children with anorectal malformations may experience long-term bowel dysfunction, including fecal incontinence and/or disorders of evacuation. Anorectal manometry is the most widely used test of anorectal function. Although considerable attention has been devoted to its application in the anorectal malformation cohort, there have been few attempts to consolidate the findings obtained. This systematic review aimed to (1) synthesize and evaluate the existing data regarding anorectal manometry results in children following anorectal malformation repair, and (2) evaluate the manometry protocols utilized, including equipment, assessment approach, and interpretation. We reviewed four databases (Embase, MEDLINE, the Cochrane Library, and PubMed) for relevant articles published between 1 January 1985 and 10 March 2022. Studies reporting post-operative anorectal manometry in children (<18 years) following anorectal malformation repair were evaluated for eligibility. Sixty-three studies were eligible for inclusion. Of the combined total cohort of 2155 patients, anorectal manometry results were reported for 1755 children following repair of anorectal malformations. Reduced resting pressure was consistently identified in children with anorectal malformations, particularly in those with more complex malformation types and/or fecal incontinence. Significant variability was identified in relation to manometry equipment, protocols, and interpretation. Few studies provided adequate cohort medical characteristics to facilitate interpretation of anorectal manometry findings within the context of the broader continence mechanism. This review highlights a widespread lack of standardization in the anorectal manometry procedure used to assess anorectal function in children following anorectal malformation repair. Consequently, interpretation and comparison of findings, both within and between institutions, is exceedingly challenging, if not impossible. Standardized manometry protocols, accompanied by a consistent approach to analysis, including definitions of normality and abnormality, are essential to enhance the comparability and clinical relevance of results.
| S-EPMC10094903 | biostudies-literature
Project description:Aim: Male patients with anorectal malformations (ARM) are classified according to presence and level of the recto-urinary fistula. This is traditionally established by a preoperative high-pressure distal colostogram that may be variably interpreted by different surgeons. The aim of this study was to evaluate the inter- and intraobserver variation in the assessment by pediatric surgeons of preoperative colostograms with respect to the level of the recto-urinary fistula. Materials and Methods: Sixteen pediatric surgeons from 14 European centers belonging to the ARM-Net Consortium twice scored 130 images of distal colostograms taken in sagittal projection at a median age of 66 days of life (range: 4-1,106 days). Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no fistula, and "unclear anatomy" example. Their assessments were compared with the intraoperative findings (kappa) for two scoring rounds with an interval of 6 months (intraobserver variation). Agreement among the surgeons' scores (interobserver variation) was also calculated using Krippendorff's alpha. A kappa over 0.75 is considered excellent, between 0.40 and 0.75 fair to good, and below 0.40 poor. Surgeons were asked to score the images in "poor" and "good" quality and to provide their years of experience in ARM treatment. Results: Agreement between the image-based rating of surgeons and the intraoperative findings ranges from 0.06 to 0.45 (mean 0.31). Interobserver variation is higher (Krippendorff's alpha between 0.40 and 0.45). Years of experience in ARM treatment does not seem to influence the scoring. The mean intraobserver variation between the two rounds is 0.64. Overall, the quality of the images is considered poor. Images categorized as having a good quality result in a statistically significant higher kappa (mean: 0.36 and 0.37 in the first and second round, respectively) than in the group of bad-quality images (mean: 0.25 and 0.23, respectively). Conclusions: There is poor agreement among experienced pediatric colorectal surgeons on preoperative colostograms. Techniques and analyses of images need to be improved in order to generate a homogeneous series of patients and make comparison of outcomes reliable.
| S-EPMC7531276 | biostudies-literature
Project description:Interventions: Group 1: Survey of surgical treatment of benign and malignant endocrine and neuroendocrine tumors as well as associated complications.
Primary outcome(s): Frequency of disease
Study Design: Allocation: ; Masking: ; Control: ; Assignment: ; Study design purpose: health services research
| 2431489 | ecrin-mdr-crc
Project description:Anorectal malformations (ARMs) consist of a range of anomalies that are often associated with other anomalies The purpose of the study is to assess the incidence of associated congenital anomalies that are seen in patients with ARMs. An observational prospective study was conducted on 162 cases with ARM from February 2019 to January 2020, and data were collected on patient demographics, type of ARM, and associated anomalies using a prestructured questionnaire and analysis done using SPSS (IBM), version 23, software. Relevant statistical analysis was done, and the results are presented in tables and charts. Of 162 cases studied, 70 of them were males and 92 were females with a male-to-female ratio of 0.76:1. The majority of male patients (45%) had rectourethral fistulas, whereas 63% of the females had rectovestibular fistula. While 76 (47%) patients presented with isolated ARM, 86 (53%) had ≥1 associated congenital malformations. Forty-eight (30%) patients presented with a single associated anomaly, whereas 20 (12%) patients had≥3 associated anomalies. The commonest associated anomalies were urologic 26.5% followed by genital (22.8%), cardiac 20.4%, and musculoskeletal 16.6%, and 12.3% of them had vertebral; anorectal; cardiac; tracheoesophageal fistula; renal; limb association. More than half of the children have other associated abnormalities. We found urogenital anomalies to be the most common associated congenital defects. A lower incidence of cardiac and spinal cord anomalies was noted suggesting a need for active workup to be in line with the latest standards of care.
| S-EPMC11419488 | biostudies-literature
Project description:BACKGROUND:Fifty to sixty percent of patients with anorectal malformation (ARM) have at least one associated anomaly (AA). We determined the incidence of AA with the subtypes of ARM classified in accordance with the Krickenbeck classification and analyzed differences in the incidence rates of major and minor AAs according to organ system. METHODS:From January 1999 to May 2017, we retrospectively analyzed congenital anomalies in patients who underwent an anoplasty for ARM at our institution. The AAs were divided into nine organ systems. To analyze the difference in the incidence of AAs, we calculated odds ratios (ORs) using cases of perineal fistula as the base group. RESULTS:Of the 460 patients, 256 (55.7%) were male, 299 (65%) had at least one anomaly, and 274 (59.6%) had major AAs. According to organ system, AAs were most common in the genitourinary (28%), cardiovascular (25%), and spinal/vertebral systems (22.6%). Major AA was most common in the cardiovascular (23%) and spinal/vertebral and genitourinary systems (19.3%). According to ARM subtype, AAs were common in the order of cloaca (93.9%), rectovaginal fistula (85.7%), and recto-bladder neck fistula (85%). For the incidence of AAs, cloaca (OR, 15.7) and recto-bladder neck fistula (OR, 5.74) showed significantly higher ORs. In the analysis of major AAs, the cloaca (OR, 19.77) showed the highest OR, followed by no fistula (OR, 4.78) and recto-bladder neck fistula (OR, 3.83). CONCLUSION:A considerable number of patients with ARM had AAs. Our data are useful for predicting AAs in patients with ARM.
| S-EPMC7152527 | biostudies-literature
Project description:BackgroundPrimary treatment of an anorectal malformation (ARM) is surgical restoration of the anatomy. These children can experience many problems later in life; therefore, a long-term follow-up by an experienced team is needed. The aim of the ARM and OUtcome Review (ARMOUR-study) is to identify the lifetime outcomes that are important from a medical and patients' perspective and develop a core outcome set (COS) that can be implemented in a care pathway to support individual ARM management decisions.MethodsFirst, a systematic review will identify clinical and patient-reported outcomes described in studies conducted in patients with an ARM. Second, qualitative interviews with patients of different age categories and their caregivers will be held to ensure that the COS will include outcomes that are relevant from the patient's perspective. Finally, the outcomes will be taken forward to a Delphi consensus exercise. Using multiple web-based Delphi rounds, key stakeholders (medical experts, clinical researchers and patients) will prioritise outcomes. During a face-to-face consensus meeting, the final COS will be determined. These outcomes can be evaluated in a life-long care pathway for patients with ARM.DiscussionThe development of a COS for ARMs aims to reduce heterogeneity in outcome reporting between (clinical) studies, enhancing the availability of comparable data, which will facilitate evidence-based patient care. Assessment of the outcomes in the COS during individual care pathways for ARM can support shared decisions regarding management. The ARMOUR-project has ethical approval and is registered with the Core Outcome Measures in Effectiveness Trials (COMET) initiative.Level of evidenceTreatment study level II.
| S-EPMC10016268 | biostudies-literature
Project description:Introductionand importance: Foetal hydrocolpos and anorectal malformation are difficult to diagnose prenatally due to abundance of differential diagnoses. This case report presents the challenges of diagnosing such disorders.Case presentationA G3P2A0 woman came at 32 weeks of pregnancy with a referral for foetal ovarian cyst. Ultrasound revealed a singleton breech pregnancy, estimated foetal weight 3528 g. A septate abdominal cyst measuring 11.31 × 7.17 cm and polyhydramnios were present. Elective caesarean section delivered a female baby weighing 2820 g and measuring 43 cm. Neonatal examination revealed a right lateral suprapubic mass and a rectovestibular fistula. A sinoscopy revealed a suspected hydrocolpos. An abdominal hydrocolpos drainage was performed; a patent urachus and normal bilateral adnexa were present.Clinical discussionHydrocolpos is a rare congenital disorder due to distal obstruction of various etiologies. It may be mistaken with other pathologies, including fetal ovarian cysts. A genitourinary congenital abnormality may occur in conjunction with other abnormalities, including gastrointestinal tract anomalies. The presence of imperforate anus and/or fistula should alert the clinician of a possible association with VACTERL syndrome.ConclusionHydrocolpos is a rare congenital genitourinary disorder with various differential diagnoses. Simultaneous presence of other abnormalities is likely, with possible association to other syndromes.
| S-EPMC9793165 | biostudies-literature
Project description:BackgroundAnorectal malformations comprise a broad spectrum of disease. We developed a percutaneous anorectoplasty (PARP) technique as a minimal-invasive option for repair of amenable types of lesions.MethodsPatients who underwent PARP at five institutions from 2008 through 2021 were retrospectively analyzed. Demographic information, details of the operative procedure, and perioperative complications and outcomes were collected.ResultsA total of 10 patients underwent the PARP procedure during the study interval. Patients either had low perineal malformations or no appreciable fistula. Most procedures were guided by ultrasound, fluoroscopy, or endoscopy. Median age at PARP was 3 days (range 1 to 311) days; eight patients were male. Only one intraoperative complication occurred, prompting conversion to posterior sagittal anorectoplasty. Functional outcomes in most children were highly satisfactory in terms of continence and functionality.ConclusionsThe PARP technique is an excellent minimal-invasive alternative for boys born with perineal fistulae, as well as patients of both sexes without fistulae. The optimal type of guidance (ultrasound, fluoroscopy, or endoscopy) depends on the anatomy of the lesion and the presence of a colostomy at the time of repair.
| S-EPMC9140123 | biostudies-literature