13 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
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
Perineal Groove : An Anorectal Malformation Network, Consortium Study
Objective: 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 1.4 months at diagnosis. The leading referral diagnosis was anal fissure (n = 20 [30.3%]): 23 patients (34.8%) had anorectal malformations. Expectant management was practiced in 47 patients (71.2%). Eight (17%) were eventually operated for local complications. The median time to surgery was 14 months (range, 3.0-48.6 months), and the median age at surgery was 18.3 months (range, 4.8-58.0 months). In the 35 patients available for follow-up of the remaining 39 managed expectantly, 23 (65.7%) showed complete or near-complete self-epithelization by a mean age 15.3 months (range, 1-72 months) and 4 (11.4%) showed partial self-epithelization by a mean age 21 months (range, 3-48 months). Eight patients showed no resolution (5 were followed for â€3 months). Nineteen patients (28.7%) were primarily treated with surgery. In total, 27 patients were operated. Dehiscence occurred in 3 of 27 operated patients (11.1%). Conclusions: PG seems to be an underestimated anomaly, frequently associated with anorectal malformations. Most cases heal spontaneously; therefore, expectant management is recommended. When associated with anorectal malformations requiring reconstruction, PG should be excised in conjunction with the anorectoplasty
The European Anorectal Malformation Network (ARM-Net) patient registry:10-year review of clinical and surgical characteristics
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
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