Incidence, diagnostics, therapeutic management and outcomes of paediatric intestinal pseudo-obstruction in the Netherlands: A 20-year retrospective cohort study.

OBJECTIVES: To describe incidence, clinical course, diagnostic and therapeutic management and long-term follow-up of paediatric intestinal pseudo-obstruction (PIPO) in the Netherlands between 2000 and 2020. METHODS: Multicenter, national, retrospective, observational study including patients aged <18 years diagnosed with PIPO and treated between 2000 and 2020 in Dutch academic medical centres. Outcomes included demographics, incidence, symptoms, diagnostic- and treatment methods used during follow-up, number of hospital admissions and mortality. RESULTS: Between 2000 and 2020, 43 children (median age 120 months, range 13 - 301, 54% female) were diagnosed with PIPO in the Netherlands. Mean incidence was 0.008/100,000/years (range 0/100 000-0.029/100 000). Twenty-six patients developed PIPO in the neonatal period. Initial symptoms were vomiting (n = 21/35, 60%) and abdominal distension (n = 14/35, 40%). Diagnostic strategies included imaging, manometry, histopathology, metabolic- and genetic screening, endoscopy and exploratory surgery. Treatment was divided in nutritional support, pharmacotherapy, colonic irrigation and surgical interventions, of which nutrition and surgery were the cornerstones for care. During the observed study period, the median number of hospital admissions was 22.5 (range 1-176) with a median of 157.5 days (range 3-840) during 20-year follow-up. Two patients (6%) died: one from sepsis and one due to a severe underlying neurological disease. Heterogeneity in diagnostic- en treatment methods existed between patients. CONCLUSIONS: PIPO is a rare, long-lasting complex disease requiring a high number of diagnostic and therapeutic interventions and hospital admissions. However, mortality rate is relatively low. Based on our results, we recommend centralization and standardization of care for this complex rare disease.

Congenital Heart Defects in Patients with Anorectal Malformations: A Retrospective Cohort Study of 281 Patients.

In patients born with anorectal malformations (ARM), additional congenital heart defects (CHD) can occur. We aimed to provide an overview on disease and treatment details of CHD identified in patients born with ARM, from a unique large cohort of a very rare disease. We performed a retrospective single-center cohort study between January 2000 and July 2023. All consecutive patients with ARM were included. Outcomes were the number of patients with CHD, and screening percentage and percentage of patients diagnosed with CHD over 3 time periods (2000-2006, 2007-2014, 2015-2023). We used uni- and multi-variable logistic regression analyses to search for associations between CHD present and baseline characteristics. In total, 281 patients were included. Some 241 (85.8%) underwent echocardiography, of whom 80 (33.2%) had CHD. Screening percentage with echocardiography increased (74.1% vs. 85.7% vs. 95.9%, p < 0.001) and percentage of patients diagnosed with CHD remained similar over time (30.2% vs. 34.5% vs. 34.0%, p = 0.836). Atrial and ventricular septal defects (n = 36, n = 29), and persistent left superior vena cava (n = 17) were most identified. The presence of VACTERL-association or a genetic syndrome was independently associated with the presence of CHD. CHD were present in 33% of patients with ARM that underwent echocardiography. Over time, the number of CHD identified through screening remained similar. Patients with the presence of VACTERL-association or a genetic syndrome had a higher risk of having CHD. Therefore, acknowledging the potential presence of CHD in patients with ARM remains important.

Pediatric thoracoscopic lung resections: a comprehensive analysis of congenital lesion cases.

BACKGROUND: Congenital lung lesions in pediatric patients may be managed conservatively or by video assisted thoracoscopic surgery (VATS). This study aimed to determine the complications after VATS for congenital lung lesions in children. METHODS: All children undergoing a lung resection between January 2009 and June 2022 were retrospectively identified. Children undergoing a primary open lobectomy or a resection other than a congenital lung lesion were excluded. Both early (<30 days) and late postoperative pulmonary complications were determined. The primary endpoint was postoperative complications within 30 days. RESULTS: In total, 56 patients were included, with a median age of 13 months (IQR 9-37). A VATS lobectomy were performed in 46 patients (82%), an extralobar sequestration in 8 patients (14%), an wedge resection in 1 patient and a segment resection in 1 patient. During the COVID pandemic, fewer resections were performed with an increase in symptomatic patients. A conversion to open occurred in 6 patients (11%), of which a preoperative lung infection was associated with an increased risk thereof(p = 0.004). The median follow-up was 22 months (IQR 7-57) and all patients were alive. A postoperative complication (Clavien Dindo ≥3) occurred in 9 patients and complications without the need of intervention in 6 patients. During follow-up a pneumonia occurred in 11 patients. CONCLUSION: There seems to be a shift towards delayed surgery with an increase in symptomatic congenital lung malformations, which might lead to an increase in postoperative complications.

Outcome after vaginal delivery of women with a previous medical history of surgically corrected anorectal malformations: a systematic review.

OBJECTIVE: Discussion remains on how to advise women with a past medical history of surgically corrected anorectal malformations (ARMs) regarding vaginal delivery. The aim of this review is to evaluate and review the reported obstetrical complications and outcomes after vaginal delivery for these women. DATA SOURCES: A systematic search was performed from inception up to 25 July 2022 in PubMed, Embase.com and Clarivate Analytics/Web of Science Core Collection, with backward citation tracking. STUDY ELIGIBILITY CRITERIA/APPRAISAL: All articles reported on the outcomes of interest in women with a past medical history of surgically corrected anorectal malformation and had a vaginal delivery were included with the exception of editorial comments or invitational commentaries. Screening, data extraction and risk of bias assessment was done by two authors independently with a third and fourth reviewer in case of disagreement. Tool for Quality assessment depended on the type of article. As low quality evidence was expected no meta-analysis was performed. RESULTS: Only five of the 2377 articles screened were eligible for inclusion with a total of 13 attempted vaginal deliveries in eight women. In three patients complications were reported: failed vaginal delivery requiring urgent cesarean section in two patients, and vaginal tearing in one patient. CONCLUSION: High quality evidence regarding outcomes and complications after vaginal delivery in women with a history of surgically corrected anorectal malformation is lacking. Therefore, based upon this systematic review no formal recommendation can be formulated regarding its safety. Future studies are essential to address this problem. TRIAL REGISTRATION: CRD42020201390. Date: 28-07-2020s.

Potential benefits of routine cystoscopy and vaginoscopy prior to reconstructive surgery in patients with an anorectal malformation.

PURPOSE: First, to assess the number of patients with anorectal malformations (ARM) in whom additional urological and/or gynecological anomalies were identified through routine screening with cysto- or vaginoscopy prior to reconstructive surgery. Second, to assess potential procedure-related complications. METHODS: Retrospective mono-center cohort study, including all ARM patients born between January 2019 and December 2022. Routine screening consisted of cystoscopy for male patients, with the addition of vaginoscopy for female patients. Chi-square was used to compare the screening percentages over time. RESULTS: In total, 38 patients were included, of whom 27 (71.1%) underwent cystoscopy ± vaginoscopy, without the occurrence of complications. Nine of 13 females (69.2%) underwent cysto- and vaginoscopy and 18 of 25 males (72.0%) underwent a cystoscopy. The percentage of patients that underwent these procedures improved over the 2 time periods (50.0% in 2019-2020 vs 90.0% in 2021-2022, p = 0.011). In 15 of 27 patients (55.6%) that underwent cystoscopy ± vaginoscopy, additional anomalies were found that were not identified through physical examination or US-kidney. CONCLUSIONS: In 56% of the patients that underwent cysto- ± vaginoscopy, additional anomalies were identified that were not with imaging studies or physical examination. This study emphasizes the potential benefit of routine cysto- and vaginoscopy in the diagnostic work-up of children with ARM. LEVEL OF EVIDENCE: III.

Spinal cord anomalies in children with anorectal malformations: a retrospective cohort study.

PURPOSE: First, to assess the number of spinal cord anomalies (SCA), specifically tethered spinal cord (TSC) in patients with anorectal malformations (ARM), identified with spinal cord imaging (i.e. spinal cord US and/or MRI). Second, to report outcomes after TSC treatment. METHODS: A retrospective mono-center study was performed. All ARM patients born between January 2000 and December 2021 were included. Screening for SCA consisted of spinal cord US and/or MRI. Radiology reports were scored on presence of SCA. Data were presented with descriptive statistics. RESULTS: In total, 254 patients were eligible for inclusion, of whom 234 (92.1%) underwent spinal cord imaging. In total, 52 (22.2%) patients had a SCA, diagnosed with US (n = 20, 38.5%), MRI (n = 10, 19.2%), or both US and MRI (n = 22, 42.3%), of whom 12 (23.5%) with simple, 27 (52.7%) intermediate, and 12 (23.5%) complex ARM types. TSC was identified in 19 patients (8.1%), of whom 4 (21.1%) underwent uncomplicated neurosurgical intervention. CONCLUSIONS: SCA were present in 22% of ARM patients both in simple, as well as more complex ARM types. TSC was present in 19 patients with SCA, of whom 4 underwent uncomplicated neurosurgical intervention. Therefore, screening for SCA seems to be important for all ARM patients, regardless of ARM type. LEVEL OF EVIDENCE: Level III.

Do Antibiotics Reduce the Incidence of Infections After Percutaneous Endoscopic Gastrostomy Placement in Children?

OBJECTIVE: Percutaneous endoscopic gastrostomy (PEG) provides a long-term solution for tube dependency. Pediatric guidelines recommend prophylactic antibiotic treatment (ABT) based on adult studies. AIM: To compare wound infection and other complications in children receiving a PEG with and without prophylactic ABT. METHODS: Retrospective study including children 0 to 18 years undergoing PEG placement. Patients with (2010-2013) and without (2000-2010) ABT were compared with respect to the occurrence of wound infection and other complications. RESULTS: In total, 297 patients were included (median age 2.9 years, 53% boys). Patients receiving ABT per PEG protocol (n = 78) had a similar wound infection rate (17.9% vs 21%, P = 0.625), significantly less fever (3.8% vs 14.6%, P = 0.013), leakage (0% vs 9.1%, P = 0.003) and shorter hospital admission (2 vs 4 days, P = 0.000), but more overgranulation (28.2% vs 8.7%, P = 0.000) compared with those without (n = 219). Patients receiving any ABT, per PEG protocol or clinical indication (n = 115), had similar occurrence of wound infection (19.1% vs 20.9%, P = 0.768), fever (7.8% vs 14.3%, P = 0.100) and leakage (3.5% vs 8.8%, P = 0.096), a significantly shorter hospital admission (3 vs 4 days, P = 0.000), but more overgranulation (21.7% vs 8.8%, P =0.003) compared with those without (n = 182). CONCLUSIONS: Prophylactic ABT does not seem to reduce the occurrence of wound infection but it might be beneficial with respect to fever, leakage and duration of hospital admission, but not overgranulation. A randomized controlled trial is needed to confirm our results.

Quality of life and colorectal function in Crohn's disease patients that underwent ileocecal resection during childhood.

Psychosocial and functional outcomes after intestinal resection in pediatric Crohn's disease (CD) are lacking. Therefore, we (I) assessed health-related quality of life (HRQOL), colorectal function, and satisfaction with surgery and (II) investigated their relationship with surgical outcomes, after ileocecal resection for CD. Crohn's patients that underwent ileocecal resection during childhood were included. HRQOL and colorectal function were assessed using SF-36 and COREFO, respectively, and compared with reference values. Satisfaction was scored on a 5-point Likert scale. In total, 80 patients (50% male, median age 23.0 years) were included. Physical HRQOL was impaired (SF-36 [mean]: CD, 47 vs. general, 54; p < 0.001), while mental HRQOL was similar to that in the general population. Overall colorectal function was impaired (COREFO [mean]: CD, 12.6 vs. normal, 7.2; p < 0.001). Worse colorectal function was associated with increasing clinical disease activity and longer interval since resection. Majority of patients was satisfied with surgery (81% satisfied/very satisfied, 11% neither satisfied nor dissatisfied, 8% dissatisfied/very dissatisfied). Decreased satisfaction with surgery was associated with increased clinical disease activity but not related to colorectal function.Conclusions: Physical HRQOL and colorectal function in CD patients who underwent ileocecal resection during childhood seem impaired and related to adverse surgical outcomes. This emphasizes the need for post-operative monitoring and prophylactic therapies. What is Known: • Up to 25% of pediatric-onset Crohn's disease (CD) patients undergo an intestinal resection within 5 years from diagnosis. • Many children and adults with CD experience disruption of their daily activities and health-related quality of life (HRQOL). What is New: • Physical HRQOL and colorectal function are impaired in patient with CD that underwent ileocecal resection during childhood. • Increasing clinical disease activity, a longer interval since surgery, severe complications related to surgery, and recurrent surgeries are all associated with worse colorectal function.

15 Years of Vacuum Bell Therapy for Pectus Excavatum: Long-term Outcomes and Influencing Factors.

BACKGROUND: Long-term results and factors affecting outcomes of vacuum bell therapy for pectus excavatum are relatively unknown. METHODS: We conducted a retrospective study on patients (<18y) treated with vacuum bell therapy between May 2008 and October 2021. Primary outcome was treatment success; secondary outcomes were analysis of daily time spent on treatment, treatment duration, complications, long-term follow-up, treatment for patients awaiting a Nuss procedure, treatment for female patients, and factors affecting outcomes. RESULTS: Of 259 patients treated with vacuum bell therapy, 18.9% (n = 49/259) were still being treated, 17.4% (n = 45/259) were lost to follow-up and 63.7% (n = 165/259) completed treatment, with a 52.1% (n = 86/165) success rate. Median follow-up was 64.0 months (interquartile range 48.0-87.0). More time spent daily on vacuum bell therapy, total treatment duration, and overnight use led to a higher success rate (P = 0.002, P < 0.001, P < 0.001 resp.). Complications (22.8%, n = 59/259) were minor, recurrence occurred in 2.3% (n = 2/86) of patients. Of the patients treated while awaiting a Nuss procedure, 26.7% (n = 4/15) no longer required the Nuss procedure. Breast growth made 39.3% (n = 11/28) of female patients quit treatment. Deeper deformities (P = 0.02, P = 0.009), flexible chest wall (P = 0.007) and symptomatic pectus excavatum (P = 0.02) resulted in lower success rates. CONCLUSIONS: Vacuum bell therapy is successful in up to 52.1% of patients. Overnight vacuum bell use and treatment while awaiting a Nuss procedure should be encouraged. Older patients with a stiff chest wall can be successfully treated with prolonged treatment. For female patients watchful waiting or early treatment, to prevent challenges during breast growth, is preferred. LEVEL OF EVIDENCE: Level II.

Anatomical Gynecological Anomalies in Girls Born with Anorectal Malformations: A Retrospective Cohort Study of 128 Patients.

STUDY OBJECTIVE: In girls born with an anorectal malformation (ARM), anatomical gynecological anomalies (GA) may be present and might need treatment. Therefore, the aim of this study was to provide an overview of GA in girls born with ARM in our cohort. Additionally, diagnostic timing and methods for GA were assessed. METHODS: A retrospective mono-center study was performed from January 2000 to December 2022. All patients assigned female at birth were eligible for inclusion. GA were classified according to ESHRE/ESGE classification. Outcomes were the number of girls with GA with subsequent screening methods, factors associated with GA, and GA requiring treatment. Uni- and multivariable logistic regression analyses were performed to identify the association between baseline characteristics and the presence of GA. RESULTS: In total, 128 girls were included, of whom 30 (24.1%) had additional GA, with vaginal anomalies being present most often (n = 17). Fifty-six patients (43.8%) underwent full screening, and this number improved over time (37.7% before 2018 vs 72.7% after 2018; P = .003). Thirteen of 30 patients (43.3%) required surgical treatment for their GA, without the occurrence of postoperative complications. CONCLUSION: Additional GA were present in almost a quarter of the girls born with an ARM, with vaginal anomalies most often identified. Despite GA being most often found in patients with cloacal malformations, these anomalies were also identified in patients with other ARM types. Surgical treatment was required in almost half of the girls with GA. Therefore, this study emphasizes the importance of screening for GA in patients with an ARM, regardless of the ARM type.

Improving Quality of Life With Dynamic Compression Bracing in Patients With Pectus Carinatum.

BACKGROUND: Patients with pectus carinatum have lower quality of life and self-esteem than their peers. We assessed the impact of dynamic compression system bracing on quality of life in patients with pectus carinatum. METHODS: We conducted a prospective cohort study on patients aged 10-21 years. We assessed quality of life using the Child Health Questionnaire-87, the State-Trait Anxiety Inventory-6, the World Health Organization Quality of Life-BREF, the 36-Item Short Form Survey, and the Single-Step Questionnaire adapted for pectus carinatum. RESULTS: Between March 2013 and March 2016, 225 patients treated with dynamic compression system bracing were included. Patients showed improvements across the overall scores of the 36-Item Short Form Survey (Δ7.7 (2.9-12.4)), Single-Step Questionnaire (Δ4.1 (2.0-6.3)) and three out of four World Health Organization Quality of Life-BREF domains (physical health (Δ8.7 (3.7-13.7)), psychological health (Δ11.8 (6.1-17.5)), environment (Δ5.7 (0.2-11.3))). No changes across the Child Health Questionnaire-87 overall score were observed (Δ5.5 (-0.5-11.5)). Most improvement occurred within six to twelve months after treatment initiation, stabilizing thereafter. Anxiety scores on the State-Trait Anxiety Inventory-6 did not improve (Δ0.5 (-0.1-1.2)). Scores on physical complaints, pain, psychological health and self-esteem/self-image improved across all questionnaires. In contrast to the successfully treated group, the unsuccessfully treated group showed no improvement on any of the questionnaires. Most patients (87.2 %) would choose bracing again, 94.9 % of patients were satisfied with the treatment. CONCLUSIONS: Dynamic compression system bracing improves quality of life, reduces physical complaints and pain and boosts psychological health and self-esteem in patients with pectus carinatum.

Different Analgesia Techniques for Postoperative Pain in Children Undergoing Abdominal Surgery for Intractable Constipation: A Retrospective Cohort Study in a Single Tertiary Children's Hospital.

Functional constipation in the pediatric population is a prevalent issue that is usually well managed. However, in rare cases, conservative treatment fails, and surgical intervention is necessary. This retrospective cohort study aimed to describe and compare different perioperative analgesic techniques in children undergoing major abdominal surgery for intractable constipation. Conducted between 2011 and 2021, this study enrolled patients under 18 years old who underwent initial major abdominal surgery for intractable constipation (i.e., creation of ostomy or subtotal colectomy). Patients were categorized according to the perioperative analgesic technique (i.e., systemic, neuraxial, or truncal block). Of 65 patients, 46 (70.8%) were female, and the median age was 13.5 [8.8-16.1] years during initial major abdominal surgery. Systemic analgesia was used in 43 (66.2%), neuraxial in 17 (26.2%), and truncal blocks in 5 (7.7%) of the surgeries. Patients with neuraxial analgesia reported less postoperative pain (median [interquartile range] numeric rating scale (NRS) 2.0 [0-4.0]), compared to systemic analgesia (5.0 [2.0-7.0], p < 0.001) and to truncal blocks (5.0 [3.0-6.5], p < 0.001). In this preliminary investigation, neuraxial analgesia appears to be the most effective approach to reducing acute postoperative pain in pediatric patients undergoing major abdominal surgery for intractable functional constipation. However, well-designed studies are warranted.

Antegrade Continence Enemas for Pediatric Functional Constipation: A Systematic Review.

BACKGROUND: Despite optimal conservative and medical treatment, some children with functional constipation (FC) continue to experience symptoms. Antegrade continence enema (ACE) surgery has been suggested as the primary surgical option after less invasive pharmacological and non-pharmacological interventions have not been effective. The purpose of this systematic review was to assess the outcomes of ACE for children with FC. METHODS: Electronic databases were searched (inception-March 2024) for studies evaluating ACE surgery performed in children with FC. The primary outcome was treatment success (as defined in the original manuscript), including at least defecation frequency and/or fecal incontinence frequency. Secondary outcomes were cessation of ACE, complications, health-related quality of life (HRQoL) and patient/parent satisfaction. Quality of evidence was evaluated based on tools from the New-Ottawa Scale and Joanna Bridge Institute. RESULTS: Thirteen studies were included, representing 477 children with FC treated with either an appendicostomy or a cecostomy. Reported treatment success rates varied widely, ranging from 32% to 100%. ACE treatment was stopped in 15% due to treatment success and in 8% due to treatment failure, leading to more invasive surgery. Complication rates ranged from 6% to 100%, requiring surgical intervention in 0% to 34%. An improvement in HRQoL following ACE treatment was reported in all three studies that assessed HRQoL. The two studies assessing patient/parent satisfaction, reported high satisfaction rates. CONCLUSION: Reported treatment success and complication rates following ACE surgery for children with FC vary widely. This systematic review highlights the necessity for uniform definitions and treatment guidelines for ACE surgery in children with FC. LEVEL OF EVIDENCE: III.

Intercostal Nerve Cryoablation or Epidural Analgesia for Multimodal Pain Management after the Nuss Procedure: A Cohort Study.

BACKGROUND: Nuss procedure for pectus excavatum is a minimally invasive, but painful procedure. Recently, intercostal nerve cryoablation has been introduced as a pain management technique. MATERIALS AND METHODS: In this cohort study, we compared the efficacy of multimodal pain management strategies in children undergoing a Nuss procedure. The effectiveness of intercostal nerve cryoablation combined with patient-controlled systemic opioid analgesia (PCA) was compared with continuous epidural analgesia (CEA) combined with PCA. The study was conducted between January 2019 and July 2022. Primary outcome was length of stay (LOS), and secondary outcomes were operation room time, postoperative pain, opioid consumption, and gabapentin use. RESULTS: Sixty-six consecutive patients were included, 33 patients in each group. The cryoablation group exhibited lower Numeric Rating Scale (NRS) pain scores on postoperative day 1 and 2 (p = 0.002, p = 0.001) and a shorter LOS (3 vs. 6 days (p < 0.001). Cryoablation resulted in less patients requiring opioids at discharge (30.3 vs. 97.0%; p < 0.001) and 1 week after surgery (6.1 vs. 45.4%; p < 0.001)). In the CEA group, gabapentin use was more prevalent (78.8 vs. 18.2%; p < 0.001) and the operation room time was shorter (119.4 vs. 135.0 minutes; p < .010). No neuropathic pain was reported. CONCLUSIONS: Intercostal nerve cryoablation is a superior analgesic method compared with CEA, with reduced LOS, opioid use, and NRS pain scores. The prophylactic use of gabapentin is redundant.

Dynamic Compression Therapy for Pectus Carinatum in Children and Adolescents: Factors for Success.

BACKGROUND: Pectus carinatum (PC) is a congenital chest wall deformity. In childhood, it is increasingly treated with dynamic compression therapy. Factors for success for dynamic brace therapy are relatively unknown. METHODS: Between 2013 and 2020, 740 patients treated with the Dynamic Compression System (DCS), were studied. This included the effect of age, gender, pectus height, symmetry and pectus rigidity on treatment time and symptoms with linear multiple regression analyses. RESULTS: Carinatum height and high pressure of initial correction at the start of treatment were associated with a prolonged duration of treatment. For each cm increase in carinatum height, the total treatment duration increased with 1.9 months (p-value= 0.002, 95% CI: 0.70-3.13). An initial correction pressure of ≥7.6 pounds per square inch (psi), increased the treatment duration with 3.5 months (p-value 0.006, 95% CI: 1.04-6.01) compared to an initial correction pressure of ≤5.0 psi. A high initial pressure of correction of ≥7.6 psi increased the odds of having somatic symptoms with 1.19 (p-value= 0.012, 95% CI: 1.04-1.45) and psychosocial symptoms with 1.13 (p-value= 0.04, 95% CI: 1.01-1.27) compared to a low initial pressure of correction of ≤5.0 psi. An initial pressure of correction of 5.1-7.5 psi increased the odds of having somatic symptoms with 1.14 (p-value 0.046, 95% CI: 1.00-1.29) compared to an initial pressure of correction of ≤5.0 psi. Patients with asymmetric chests were more likely to abandon therapy CONCLUSIONS: High carinatum height and high initial pressure of correction are associated with prolonged bracing treatment and a higher failure rate. LEVEL OF EVIDENCE: III.

Outcomes after Surgical Treatment for Rectal Atresia in Children: Is There a Preferred Approach? A Systematic Review.

Rectal atresia (RA) affects only 1 to 2% of all children with anorectal malformations. No consensus on optimal treatment strategy is yet achieved. Therefore, the aim of this systematic review is to summarize all surgical interventions for RA and outcomes described in the current literature. A literature search was conducted in PubMed, Embase, Web of Science, and Cochrane Library on January 24, 2022. All studies describing treatment for RA in children (< 18 years) were included. Operation technique and postoperative complications were listed. Only descriptive analysis was anticipated. Quality of the studies was assessed using Johanna Briggs Institute critical appraisal checklist for case reports and series. The search yielded 6,716 studies of which, after duplicate removal, 4,028 were excluded based on title and abstract screening. After full-text assessment, 22 of 90 studies were included, yielding 70 patients. Posterior sagittal anorectoplasty (PSARP) and pull-through were most performed (43/70 and 18/70 patients, respectively). Four patients experienced postoperative complications: anal stenosis (n = 1), anastomotic stenosis (n = 2), and death due to a pulmonary complication (n = 1). In the low-quality literature available, most patients with RA are treated with PSARP or pull-through technique. A low complication rate of both has been described but follow-up was often not mentioned. Larger well-designed studies should be performed to determine optimal treatment strategy for children with RA. This study reflects level of evidence V.

Routine contrast enema prior to stoma reversal seems only required following treatment for necrotizing enterocolitis: An evaluation of the diagnostic accuracy of the contrast enema.

INTRODUCTION: Contrast enemas are often made prior to stoma reversal in order to detect distal intestinal strictures distal of the stoma. If untreated these strictures can cause obstruction which might necessitate redo-surgery. However, the value of contrast enemas is unclear. Therefore, we aim to evaluate the contrast enema's diagnostic accuracy in detecting strictures in children with a stoma. METHODS: Young children (≤3 years) treated with a stoma between 1998 and 2018 were retrospectively included. The STARD criteria were followed. Patients treated for anorectal malformations and those that died before stoma reversal were excluded. Surgical identification of strictures during reversal or redo-surgery within three months was used as gold standard. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and area under the curve (AUC) reflected diagnostic accuracy. RESULTS: In 224 included children, strictures were found during reversal in 10% of which 95% in patients treated for necrotizing enterocolitis. Contrast enema was performed in 68% of all patients and detected 92% of the strictures. In the overall cohort, the sensitivity was 100%, specificity 98%, PPV 88% and NPV 100% whilst the AUC was 0.98. In patients treated for NEC, the sensitivity was 100%, specificity 97%, PPV 88% and NPV 100% whilst the AUC was 0.98. CONCLUSION: Strictures prior to stoma reversal seem to be mainly identified in patients treated for NEC and not in other diseases necessitating a stoma. Moreover, the contrast enema shows excellent diagnostic accuracy in detecting these strictures. For this reason we advise to only perform contrast enemas in patients treated for NEC. LEVEL OF EVIDENCE: II.

The Importance of Screening for Additional Anomalies in Patients with Anorectal Malformations: A Retrospective Cohort Study.

BACKGROUND: In children with anorectal malformations (ARM), additional anomalies can occur within the VACTERL-association. Routine screening is of great importance for early identification and potential treatment. However, uniformity in screening protocols is lacking and only small cohorts have been described in literature. The aim of this study was to assess and describe a unique large cohort of ARM patients who underwent VACTERL screening in the neonatal period. METHODS: A retrospective mono-center cohort study was performed. Included were all neonates born between January 2000 and December 2020 who were diagnosed with ARM and screened for additional anomalies. Full screening consisted of x-ray and ultrasound of the spine, cardiac and renal ultrasound, and physical examination for limb deformities, esophageal atresia, and ARM. Criteria for VACTERL-classification were predefined according to the EUROCAT-definitions. RESULTS: In total, 216 patients were included, of whom 167 (77.3%) underwent full VACTERL-screening (66% in 2000-2006 vs. 82% in 2007-2013 vs. 86% in 2014-2020). Median age at follow-up was 7.0 years (IQR 3.0-12.8). In 103/167 patients (61.7%), additional anomalies were identified. Some 35/216 patients (16.2%) fulfilled the criteria of a form of VACTERL-association. In 37/216 patients (17.1%), a genetic cause or syndrome was found. CONCLUSIONS: The majority of ARM patients underwent full screening to detect additional anomalies (77%), which improved over time to 86%. Yet, approximately a quarter of patients was not screened, with the potential of missing important additional anomalies that might have severe consequences in the future. Forms of VACTERL-association or genetic causes were found in 16% and 17% respectively. This study emphasizes the importance of routine screening. LEVEL OF EVIDENCE: III.

Impact of Presence, Level, and Closure of a Stoma on Growth in Young Children: A Retrospective Cohort Study.

INTRODUCTION: A stoma will cause nutrients loss which could result in impaired growth. Impaired growth can negatively impact long-term development. This study aims to evaluate: (1) the effect of stomas on growth comparing small bowel stoma versus colostomy and (2) if early closure (within 6 weeks), proximal small bowel stoma (within 50 cm of Treitz), major small bowel resection (≥ 30 cm), or adequate sodium supplementation (urinary level ≤ 30 mmol/L) influences growth. METHODS: Young children (≤ 3 years) treated with stomas between 1998 and 2018 were retrospectively identified. Growth was measured with weight-for-age Z-scores. Malnourishment was defined using the World Health Organization's definition. Comparison between changes in Z-scores at creation, closure, and a year following closure was done by Friedman's test with post hoc Wilcoxon's signed rank test or Wilcoxon's rank-sum test when necessary. RESULTS: In the presence of a stoma in 172 children, 61% showed growth decline. Severe malnourishment was seen at the time of stoma closure in 51% of the patients treated by small bowel stoma and 16% of those treated by colostomy. Within a year following stoma closure, 67% showed a positive growth trend. Having a proximal small bowel stoma and undergoing major small bowel resection led to significantly lower Z-scores at closure. Adequate sodium supplementation and early closure did not lead to significant changes in Z-scores. CONCLUSION: Stomas have a negative impact on growth in the majority of children. This impact might be decreased by preventing small bowel stomas when possible, specifically proximal stomas, and limiting small bowel resection. Since stoma closure is essential in reversing the negative effect on growth, we opt that early closure might result in an early shift to catch-up growth.

Children with an Anorectal Malformation Going to Primary School: The Parent's Perspective.

BACKGROUND: Continence problems occur often in children with anorectal malformations (ARM). The aim of this study was to evaluate parental experiences with toilet facilities at Dutch primary schools and their experience with how schools deal with ARM children. METHODS: This survey was developed in collaboration with the national patient advocacy group (PAG). Recruitment for participation was conducted by the PAG (email listing and social media) and one expertise center for ARM. Participants were parents of school-attending ARM children aged 3 to 12 years. RESULTS: Sixty-one participants (31.9%) responded to the survey. The median age of the children was 7.0 years (IQR 5.0-9.0). Schools were often located in a village (63.9%) and encompassed 100-500 children (77.0%). In total, 14 parents (23.0%) experienced difficulties in finding a primary school. Experiences with the school were described as solely positive (37.7%), solely negative (9.8%), positive and negative (34.4%), and neither positive nor negative (16.4%). Regarding school toilet facilities, 65.6% of the toilets were reported clean and 78.7% were easily accessible. CONCLUSIONS: About 25% of parents reported difficulties in enrolling their children into primary school, and 45% reported negative experiences. This highlights the need for improved guidance and the optimization of education in schools when dealing with ARM children.