35 research outputs found
Rectal atresia and rectal stenosis:the ARM-Net Consortium experience
Purpose: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry. Methods: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed. Results: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0Â years (IQR 2.3â9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema. Conclusion: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging.</p
Inter- and Intraobserver Variation in the Assessment of Preoperative Colostograms in Male Anorectal Malformations: An ARM-Net Consortium Survey
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: 4â1,106 days).
Surgeons were asked to classify the fistula in bulbar, prostatic, bladder-neck, no
fistula, and âunclear anatomyâ 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 surgeonsâ scores (interobserver variation)
was also calculated using Krippendorffâs 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 âpoorâ and âgoodâ 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
(Krippendorffâs 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
Bowel function and associated risk factors at preschool and early childhood age in children with anorectal malformation type rectovestibular fistula:An ARM-Net consortium study
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. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome. Methods: A multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4â7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups âpoorâ †11, and âfairâ 11 < BFS < 17) and good outcome (BFS â„ 17) were formed. Univariable analyses were performed to detect risk factors for outcome. Results: The study included 111 RVF-patients. Median BFS was 16 (range 6â20). The âbelow normalâ group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management. Conclusions: Although median BFS at 4â7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management. Level of Evidence: Level III
COMPARE LAAO: Rationale and design of the randomized controlled trial "COMPARing Effectiveness and safety of Left Atrial Appendage Occlusion to standard of care for atrial fibrillation patients at high stroke risk and ineligible to use oral anticoagulation therapy"
Contains fulltext :
252137.pdf (Publisherâs version ) (Open Access)BACKGROUND: Left atrial appendage occlusion (LAAO) provides an alternative to oral anticoagulation (OAC) for stroke prevention in patients with atrial fibrillation (AF). In patients with a long-term or permanent contraindication for OAC randomized controlled trial (RCT) data is lacking. STUDY OBJECTIVES: To assess the efficacy and safety of LAAO in AF patients who are ineligible to use OAC. The co-primary efficacy endpoint is (1) time to first occurrence of stroke (ischemic, hemorrhagic, or undetermined) and (2) time to first occurrence of the composite of stroke, transient ischemic attack (TIA), and systemic embolism (SE). The primary safety endpoint is the 30-day rate of peri-procedural complications. STUDY DESIGN: This is a multicenter, investigator-initiated, open-label, blinded endpoint (PROBE), superiority-driven RCT. Patients with AF, a CHAâDSâ-VASc score â„2 for men and â„3 for women and a long-term or permanent contraindication for OAC will be randomized in a 2:1 fashion to the device- or control arm. Patients in the device arm will undergo percutaneous LAAO and will receive post-procedural dual antiplatelet therapy (DAPT) per protocol, while those in the control arm will continue their current treatment consisting of no antithrombotic therapy or (D)APT as deemed appropriate by the primary responsible physician. In this endpoint-driven trial design, assuming a 50% lower stroke risk of LAAO compared to conservative treatment, 609 patients will be followed for a minimum of 1 and a maximum of 5 years. Cost-effectiveness and budget impact analyses will be performed to allow decision-making on reimbursement of LAAO for the target population in the Netherlands. SUMMARY: The COMPARE LAAO trial will investigate the clinical superiority in preventing thromboembolic events and cost-effectiveness of LAAO in AF patients with a high thromboembolic risk and a contraindication for OAC use. NCT TRIAL NUMBER: NCT04676880
Origin of the Local Bubble
We present a new unbiased search for OB associations in the Solar neighbourhood
which have hosted the progenitor stars of the core collapse supernovae responsible for the
Local Bubble in the interstellar gas. For this purpose we have analyzed a volume complete
set (with a diameter of 400 pc) of B stars drawn from the Hipparcos catalogue and the Arivel
data base, from which candidate members were selected by a kinematical criterion. After
careful dereddening the star colours we have constructed a colour-magnitude diagram and
confirmed that the Upper Scorpius, Upper Centaurus Lupus, and Lower Centaurus Crux
subgroups of the Sco OB2 association are the youngest nearby OB associations. We dated
their ages with theoretical isochrones in the range of 20â30 Myr, in agreement with previous
work. We have traced backwards in time the paths of the stars and found that they entered
the volume of the present bubble at 10 to 15 Myr ago. We argue that the Local Bubble began
to form then and estimate that 14 to 20 supernovae have exploded since. The implied energy
input into the ambient medium can be shown to be sufficient to excavate a bubble of the
presently observed size
The Physics of Star Cluster Formation and Evolution
© 2020 Springer-Verlag. The final publication is available at Springer via https://doi.org/10.1007/s11214-020-00689-4.Star clusters form in dense, hierarchically collapsing gas clouds. Bulk kinetic energy is transformed to turbulence with stars forming from cores fed by filaments. In the most compact regions, stellar feedback is least effective in removing the gas and stars may form very efficiently. These are also the regions where, in high-mass clusters, ejecta from some kind of high-mass stars are effectively captured during the formation phase of some of the low mass stars and effectively channeled into the latter to form multiple populations. Star formation epochs in star clusters are generally set by gas flows that determine the abundance of gas in the cluster. We argue that there is likely only one star formation epoch after which clusters remain essentially clear of gas by cluster winds. Collisional dynamics is important in this phase leading to core collapse, expansion and eventual dispersion of every cluster. We review recent developments in the field with a focus on theoretical work.Peer reviewe
"This bicycle gives me a headache", a congenital anomaly
Contains fulltext :
128545.pdf (publisher's version ) (Open Access)BACKROUND: The combination of a presacral mass, a sacral bone deformity, and an anorectal malformation are also known as the Currarino triad or Currarino syndrome. The syndrome is associated with a very high rate of severe and intractable constipation and urinary incontinence. However, it can also result in less common complaints and symptoms. Although the syndrome is known since 1981 and the involved genes are clarified to a great extent, the diagnosis may be delayed or missed if unrecognized. CASE PRESENTATION: A 24-year old female presented with periodical headaches. She was born with an imperforate anus, absent rectum and colon, double bladder, and sacral defect. Soon after birth she underwent several surgical procedures for anorectal and bladder reconstructions. The patient now came to her pediatric urologist for urinary incontinence and mentioned severe headaches on the side, particularly when riding a bike. Finally, she solved her headache problem by stopping to ride her bicycle.On physical examination no abnormalities were found except the ileostomy that was present ever since soon after birth and her urinary incontinence. Blood tests showed no abnormalities. Additional MRI showed a large and previously not known anterior meningocele at the level of the sacrum. Surgical treatment consisted of closure of the dura by posterior approach. CONCLUSION: In this case report we describe the late discovery with an atypical presentation of an anterior meningocele in a young adult with urinary incontinence, a sacral defect, an anorectal malformation and headaches during bicycle riding. After surgical treatment of our patient the meningocele regressed. Three months after successful surgery she had no complaints and was able to ride a bike again