Publications by authors named "Ivo De Blaauw"

Article Synopsis
  • - Anorectal malformations (ARM) are rare congenital defects occurring in about 3.32 per 10,000 pregnancies, involving a range of issues from mild anal canal displacement to severe fusion of the anorectum, vagina, and urethra.
  • - The developmental basis of ARM stems from improper septation of the hindgut during early pregnancy, with most cases being non-syndromic and lacking clear genetic causes.
  • - Surgical correction, primarily through posterior sagittal anorectoplasty since 1982, is common, but there is a need for further improvements in surgery outcomes, prenatal detection, and understanding the psychosocial impacts on patients.
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Unexplainable gastrointestinal complaints occasionally occur after Roux-en-Y Gastric Bypass (RYGB) surgery. We therefor investigated the impact of microbiota composition and metabolites on gastrointestinal complaints after RYGB. In the BARICO study (Bariatric surgery Rijnstate and Radboudumc neuroimaging and Cognition in Obesity), microbiota and metabolites were measured before surgery, and 6, and 24 months after surgery.

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Article Synopsis
  • - Anorectal malformations (ARMs) are uncommon congenital issues affecting the anus and rectum, which require complicated management; European experts are working on unified guidelines for better care.
  • - The guidelines were created based on the Dutch Quality Standard for ARMs and involved a literature review and evaluation by a panel of experts from several European countries, leading to adaptations and new recommendations.
  • - Emphasizing the need for specialized care throughout a patient's life, the guidelines outline essential diagnostic and follow-up processes for children with ARMs to ensure comprehensive management.
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Introduction:  Being born with an anorectal malformation (ARM) can have profound and lifelong implications for patients and parents. Organization of care and communication between health care providers is an overlooked area of patient care. The European Reference Network eUROGEN for rare and complex urogenital conditions assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM.

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Article Synopsis
  • * A group of 15 experts from Europe created guidelines to help doctors and hospitals properly treat patients with ARMs based on new research and existing standards.
  • * The guidelines cover treatments, after-surgery care, toilet training, and managing issues like incontinence, emphasizing the need for a well-coordinated healthcare team.
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Introduction:  Anorectal malformations (ARMs) are complex congenital anomalies of the anorectal region, oftentimes also affecting the genitourinary system. Although successful surgical correction can often be achieved in the neonatal period, many children will experience functional problems in the long term. The European Reference Network for rare and complex urogenital conditions (eUROGEN) assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM.

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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).

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The term "recurrent constellations of embryonic malformations" (RCEM) is used to describe a number of multiple malformation associations that affect three or more body structures. The causes of these disorders are currently unknown, and no diagnostic marker has been identified. Consequently, providing a definitive diagnosis in suspected individuals is challenging.

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Obesity is a multifactorial disease associated with low-grade inflammation. The gut is thought to be involved in obesity-related inflammation, as it is continuously exposed to antigens from food, microbiota and metabolites. However, the exact underlying mechanisms are still unknown.

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Article Synopsis
  • * Participants were divided into three groups: clinical exposure only, continuous training, and continuous training with self-assessment, with advancements in suturing skills measured through pre- and post-tests.
  • * Results showed all groups improved overall, but the self-assessment group initially performed better, although it did not maintain that advantage after training, suggesting that reflective practices may enhance early surgical skill development.
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Background: The aim of this study is to evaluate whether the parameters 'time' and 'distance', measured by SurgTrac, correlate with the assessment of the same skills by blinded experts.

Methods: Basic open suturing tasks were executed by medical students. SurgTrac software measured objective parameters by tracking fingers.

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Congenital vascular malformations (CVMs) are the result of an aberrant development during embryogenesis. Although these lesions are present at birth, they are not always visible yet. Once symptomatic, patients suffer from pain, bleeding, ulcers, infections or lymphatic leakage, depending on the subtype of vessels involved.

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Background: Alcohol misuse among medical professionals poses a significant concern, and there is a lack of clarity in (inter)national guidelines regarding alcohol use during work. Moreover, there exists an insufficient body of research on the specific impact of alcohol on fine motor skills within the medical sector, specifically surgery. This study aims to investigate the impact of alcohol on fine motor skills in a minimally invasive surgical setting.

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Surface electroenterography is a potential non-invasive alternative to current diagnostics of colonic motility disorders. However, electrode positioning in electroenterography is often based on general anatomy and may lack generalizability. Furthermore, the repeatability of electroenterography measurements is unknown.

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Background: Serum alpha-fetoprotein (AFP) is often used as tumour marker for recurrent sacrococcygeal teratoma (SCT). We aimed to assess the normal dynamics of serum AFP levels after initial resection and diagnostic accuracy of serum AFP levels the follow-up for recurrence in SCT.

Methods: This retrospective study included 57 patients treated for SCT in the six pediatric surgical centers in the Netherlands from 1980 to 2018.

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Current diagnostics in Hirschsprung's disease are often challenging and invasive. This study aims to investigate whether surface electroenterography can non-invasively discern healthy subjects from subjects suffering from Hirschsprung's disease. Nine healthy subjects (seven children, two adults) and eleven subjects suffering from surgically untreated Hirschsprung's disease (nine children, two adults) underwent an electroenterography procedure.

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Background: Congenital diaphragmatic hernia (CDH) is a rare birth defect with substantial mortality. This study aims to generate a population-based overview of CDH care and outcomes in the Netherlands. Moreover, it assesses interhospital variations between the two Dutch CDH expert centres.

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Article Synopsis
  • * Associated syndromes include trisomy 21 and Mowat-Wilson syndrome, with genes like RET and EDNRB linked to the disease's occurrence.
  • * Diagnosis involves imaging and biopsy, and treatment usually requires surgical removal of the affected bowel, with various surgical techniques available and ongoing research aimed at improving diagnosis and patient outcomes.
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Objectives: Studies concerning cardiopulmonary outcomes of adults born with congenital diaphragmatic hernia (CDH) are sparse. Moreover, they don't include participants who have been treated with extracorporeal membrane oxygenation (ECMO) during the neonatal period. This study evaluated the cardiopulmonary morbidities in young adults born with CDH.

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Unlabelled: A cross-sectional study was performed to evaluate health-related quality of life (HRQOL) in children with congenital vascular malformations (CVM) and to investigate factors associated with an impaired HRQOL. Children (2-17 years) with CVMs who visited the HECOVAN expertise center between 2016-2018 were included. The PedsQL 4.

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Background: Transition of care (TOC; from childhood into adulthood) of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) ensures continuation of care for these patients. The aim of this international study was to assess the current status of TOC and adult care (AC) programs for patients with ARM and HD.

Methods: A survey was developed by members of EUPSA, ERN eUROGEN, and ERNICA, including patient representatives (ePAGs), comprising of four domains: general information, general questions about transition to adulthood, and disease-specific questions regarding TOC and AC programs.

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Patient registries serve to overcome the research limitations inherent in the study of rare diseases, where patient numbers are typically small. Despite the value of real-world data collected through registries, adequate design and maintenance are integral to data quality. We aimed to describe an overview of the challenges in design, quality management, and maintenance of rare disease registries.

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Background: Registries are important in rare disease research. The Anorectal Malformation Network (ARM-Net) registry is a well-established European patient registry collecting demographic, clinical, and functional outcome data. We assessed the quality of this registry through review of the structure, data elements, collected data, and user experience.

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Introduction:  Sufficient training is needed to acquire and retain the procedural skills needed for the surgical correction of congenital anatomical malformations. This study aims to assess the opinions of trainees and pediatric surgeons on the use of simulation-based continued at-home training, which can help to acquire these skills.

Methods:  This study consisted of two parts.

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