Iodinated Contrast Induced Hypothyroidism in the Infant After Enteral Contrast Enema: A Case Report and Systematic Review.

Background: Excessive iodine intake triggers the Wolff-Chaikoff effect resulting in downregulation of thyroid hormone synthesis to prevent hyperthyroidism. Failure to escape the Wolff-Chaikoff effect can be seen especially in (premature born) infants and may result in prolonged iodine induced hypothyroidism. We describe a rare case of a preterm infant who developed severe iodinated contrast induced hypothyroidism after the use and prolonged stasis of enteral iodinated contrast media (ICM). In addition a systematic literature search was performed to evaluate all available data on this complication. Methods: A systematic literature search was performed in PubMed and Embase. Studies describing the effect of enteral ICM on thyroid function were considered eligible. The primary outcome was to determine the frequency of contrast induced hypothyroidism in infants after administration of enteral ICM. Results: The premature infant in our center developed severe iodinated contrast induced hypothyroidism after enteral ICM. In total, only two studies met our eligibility data, reporting eight patients. Out of these eight patients, four premature infants developed a contrast induced hypothyroidism after enteral administration of ICM. Conclusion: Data on severity, length and frequency of contrast induced hypothyroidism after exposure to enteral ICM is very scarce. The herein reported case and literature search illustrate the potential severity of the complication and underline the necessity of future studies on this topic. We recommend standardized monitoring of thyroid function after exposure to enteral ICM in newborns to prevent delayed diagnosis of severe contrast induced hypothyroidism until evidence based recommendations can be made.

Prerequisites for Clinical Implementation of Whole-Heart 4D-Flow MRI: A Delphi Analysis.

Whole-heart 4D-flow MRI is a valuable tool for advanced visualization and quantification of blood flow in cardiovascular imaging. Despite advantages over 2D-phase-contrast flow, clinical implementation remains only partially exploited due to many hurdles in all steps, from image acquisition, reconstruction, postprocessing and analysis, clinical embedment, reporting, legislation, and regulation to data storage. The intent of this manuscript was 1) to evaluate the extent of clinical implementation of whole-heart 4D-flow MRI, 2) to identify hurdles hampering clinical implementation, and 3) to reach consensus on requirements for clinical implementation of whole-heart 4D-flow MRI. This study is based on Delphi analysis. This study involves a panel of 18 experts in the field on whole-heart 4D-flow MRI. The experience with and opinions of experts (mean 13 years of experience, interquartile range 6) in the field were aggregated. This study showed that among experts in the cardiovascular field, whole-heart 4D-flow MRI is currently used for both clinical and research purposes. Overall, the panelists agreed that major hurdles currently hamper implementation and utilization. The sequence-specific hurdles identified were long scan time and lack of standardization. Further hurdles included cumbersome and time-consuming segmentation and postprocessing. The study concludes that implementation of whole-heart 4D-flow MRI in clinical routine is feasible, but the implementation process is complex and requires a dedicated, multidisciplinary team. A predefined plan, including risk assessment and technique validation, is essential. The reported consensus statements may guide further tool development and facilitate broader implementation and clinical use. LEVEL OF EVIDENCE: NA TECHNICAL EFFICACY: Stage 5.

Identifying Preoperative Clinical Characteristics of Unexpected Gastrointestinal Perforation in Infants-A Retrospective Cohort Study.

BACKGROUND: Infants presenting with unexpected pneumoperitoneum upon abdominal X-ray, indicating a gastrointestinal perforation (GIP), have a surgical emergency with potential morbidity and mortality. Preoperative determination of the location of perforation is challenging but will aid the surgeon in optimizing the surgical strategy, as colon perforations are more challenging than small bowel perforations. Therefore, the aim of this study is to provide an overview of preoperative patient characteristics, determine the differences between the small bowel and colon, and determine underlying causes in a cohort of infants with unexpected GIP. METHODS: All infants (age ≤ 6 months) who presented at our center with unexpected pneumoperitoneum (no signs of pneumatosis before) undergoing surgery between 1996 and 2024 were retrospectively included. The differences between the location of perforation were analyzed using chi-squared and t-tests. Bonferroni correction was used to adjust for multiple tests. RESULTS: In total, 51 infants presented with unexpected pneumoperitoneum at our center, predominantly male (N = 36/51) and premature (N = 40/51). Among them, twenty-six had small bowel, twenty-two colon, and three stomach perforations. Prematurity (p = 0.001), birthweight < 1000 g (p = 0.001), respiratory support (p = 0.001), and lower median arterial pH levels (p = 0.001) were more present in patients with small bowel perforation compared with colon perforations. Pneumatosis intestinalis was more present in patients with colon perforation (p = 0.004). All patients with Hirschsprung disease and cystic fibrosis had colon perforation. The final diagnoses were mainly focal intestinal perforations (N = 27/51) and necrotizing enterocolitis (N = 9/51). CONCLUSIONS: Infants with unexpected GIP, birthweight < 1000 g, and prematurity have more risk for small bowel perforation. In case of colon perforation, additional screening (for Hirschsprung and cystic fibrosis) should be considered.

No Pathogenic DICER1 Gene Variants in a Cohort Study of 28 Children With Congenital Pulmonary Airway Malformation.

BACKGROUND: Distinguishing congenital pulmonary airway malformations (CPAMs) from pleuropulmonary blastoma (PPB) can be challenging. Previously diagnosed patients with CPAM may have been misdiagnosed and we may have missed DICER1-associated PPBs, a diagnosis with important clinical implications for patients and their families. To gain insight in potential misdiagnoses, we systematically assessed somatic DICER1 gene mutation status in an unselected, retrospective cohort of patients with a CPAM diagnosis. METHODS: In the Amsterdam University Medical Center (the Netherlands), it has been standard policy to resect CPAM lesions. We included all consecutive cases of children (age 0-18 years) with a diagnosis of CPAM between 2007 and 2017 at this center. Clinical and radiographic features were reviewed, and DICER1 gene sequencing was performed on DNA retrieved from CPAM tissue samples. RESULTS: Twenty-eight patients with a surgically removed CPAM were included. CPAM type 1 and type 2 were the most common subtypes (n = 12 and n = 13). For 21 patients a chest CT scan was available for reassessment by two pediatric radiologists. In 9 patients (9/21, 43%) the CPAM subtype scored by the radiologists did not correspond with the subtype given at pathology assessment. No pathogenic mutations and no copy number variations of the DICER1 gene were found in the DNA extracted from CPAM tissue (0/28). CONCLUSIONS: Our findings suggest that the initial CPAM diagnoses were correct. These findings should be validated through larger studies to draw conclusions regarding whether systematic DICER1 genetic testing is required in children with a pathological confirmed diagnosis of CPAM or not. LEVEL OF EVIDENCE: Level IV.

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.

Pediatric Cardiovascular Computed Tomography: Clinical Indications, Technique, and Standardized Reporting. Recommendations From the Cardiothoracic Taskforce of the European Society of Pediatric Radiology.

Congenital heart diseases affect 1% of all live births in the general population. The prognosis of these children is increasingly improving due to advances in medical care and surgical treatment. Imaging is also evolving rapidly to assess accurately complex cardiac anomalies prenatally and postnatally. Transthoracic echocardiography is the gold-standard imaging technique to diagnose and follow-up children with congenital heart disease. Cardiac computed tomography imaging plays a key role in the diagnosis of children with congenital heart defects that require intervention, due to its high temporal and spatial resolution, with low radiation doses. It is challenging for radiologists, not primarily specialized in this field, to perform and interpret these studies due to the difficult anatomy, physiology, and postsurgical changes. Technical challenges consist of necessary electrocardiogram gating and contrast bolus timing to obtain an optimal examination. This article aims to define indications for pediatric cardiac computed tomography, to explain how to perform and report these studies, and to discuss future applications of this technique.

Four-Dimensional Flow MRI-Derived Hemodynamics in Abdominal Aortic Aneurysms: Reproducibility and Associations With Diameter, Intraluminal Thrombus Volume, and Vorticity.

BACKGROUND: Maximum diameter measurements are used to assess the rupture risk of abdominal aortic aneurysms (AAAs); however, these are not precise enough to predict all ruptures. Four-dimensional (4D) flow MRI-derived parameters provide additional information by visualizing hemodynamics in AAAs but merit further investigation before they are clinically applicable. PURPOSE: To assess the reproducibility of 4D flow MRI-derived hemodynamics, to investigate possible correlations with lumen and maximum diameter, and to explore potential relationships with vorticity and aneurysm growth. STUDY TYPE: Prospective single-arm study. POPULATION: A total of 22 (71.5 ± 6.1 years, 20 male) asymptomatic AAA patients with a maximum diameter of at least 30 mm. FIELD STRENGTH/SEQUENCE: A 3.0 T/Free-breathing 4D flow MRI phase-contrast acquisition with retrospective ECG-gating. ASSESSMENT: Patients underwent two consecutive 4D flow MRI scans 1-week apart. Aortic volumes were segmented from time-averaged phase contrast magnetic resonance angiographies. Reproducibility was assessed by voxelwise analysis after registration. Mean flow velocity, mean wall shear stress (WSS), mean lumen diameter, and qualitative vorticity scores were assessed. In addition, Dixon MRI and retrospective surveillance data were used to study maximum diameter (including thrombus), intraluminal thrombus volume (ILT), and growth rate. STATISTICAL TESTS: For reproducibility assessment, Bland-Altman analyses, Pearson correlation, Spearman's correlation, and orthogonal regression were conducted. Potential correlations between hemodynamics and vorticity scores were assessed using linear regression. P < 0.05 was considered statistically significant. RESULTS: Test-retest median Pearson correlation coefficients for flow velocity and WSS were 0.85 (IQR = 0.08) m/sec and 0.82 (IQR = 0.10) Pa, respectively. Mean WSS significantly correlated with mean flow velocity (R = 0.75) and inversely correlated with mean lumen diameter (R = -0.73). No significant associations were found between 4D flow MRI-derived hemodynamic parameters and maximum diameter (flow velocity: P = 0.98, WSS: P = 0.22). DATA CONCLUSION: A 4D flow MRI is robust for assessing the hemodynamics within AAAs. No correlations were found between hemodynamic parameters and maximum diameter, ILT volume and growth rate. LEVEL OF EVIDENCE: 2 TECHNICAL EFFICACY: Stage 2.

A 15-year-old Girl with a Lateral Neck Mass Turning Out to Be Papillary Thyroid Carcinoma-Lateral Ectopic Papillary Thyroid Carcinoma or Lymph Node Metastasis?

Lateral neck lesions in children are common and involve various infectious or inflammatory etiologies as well as embryological remnants such as branchial cleft cysts. Although unusual, ectopic thyroid tissue can also present as a lateral neck mass. Here, we present an unusual case of a 15-year-old girl treated for an asymptomatic lateral neck mass that after surgical removal was found to be papillary thyroid carcinoma (PTC). However, after removal of the thyroid gland, no primary thyroid tumor was found. The question arose whether the lateral neck lesion was a lymph node metastasis without identifiable primary tumor (at histological evaluation) or rather malignant degeneration of ectopic thyroid tissue. Total thyroidectomy was performed with postoperative adjuvant radioactive iodine ablation. Even though PTC in a lateral neck mass without a primary thyroid tumor has been described previously, pediatric cases have not been reported. In this report we share our experience on diagnosis, treatment and follow-up, and review the existing literature.

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.

Transition Zone Pull-through in Patients with Hirschsprung Disease: Is Redo Surgery Beneficial for the Long-term Outcomes?

INTRODUCTION: Transition zone pull-through (TZPT) is incomplete removal of the aganglionic bowel/transition zone (TZ) in patients with Hirschsprung disease (HD). Evidence on which treatment generates the best long-term outcomes is lacking. The aim of this study was to compare the long-term occurrence of Hirschsprung associated enterocolitis (HAEC), requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively to patients with TZPT treated with redo surgery to non-TZPT patients. METHODS: We retrospectively studied patients with TZPT operated between 2000 and 2021. TZPT patients were matched to two control patients with complete removal of the aganglionic/hypoganglionic bowel. Functional outcomes and quality of life was assessed using Hirschsprung/Anorectal Malformation Quality of Life questionnaire and items of Groningen Defecation & Continence together with occurrence of Hirschsprung associated enterocolitis (HAEC) and requirement of interventions. Scores between the groups were compared using One-Way ANOVA. The follow-up duration lasted from time at operation until follow-up. RESULTS: Fifteen TZPT-patients (six treated conservatively, nine receiving redo surgery) were matched with 30 control-patients. Median duration of follow-up was 76 months (range 12-260). No significant differences between groups were found in the occurrence of HAEC (p = 0.65), laxatives use (p = 0.33), rectal irrigation use (p = 0.11), botulinum toxin injections (p = 0.06), functional outcomes (p = 0.67) and quality of life (p = 0.63). CONCLUSION: Our findings suggest that there are no differences in the long-term occurrence of HAEC, requirement of interventions, functional outcomes and quality of life between patients with TZPT treated conservatively or with redo surgery and non-TZPT patients. Therefore, we suggest to consider conservative treatment in case of TZPT.

Short-term outcome after the prenatal diagnosis of right aortic arch.

OBJECTIVES: To determine the proportion of children that require surgery in the first year of life and thereafter in order to improve the counseling of parents with a fetus with a right aortic arch (RAA). METHODS: Fetuses diagnosed with isolated RAA, defined as the absence of intra- or extracardiac anomalies, between 2007 and 2021 were extracted from the prospective registry PRECOR. RESULTS: In total, 110 fetuses were included, 92 with a prenatal diagnosis of RAA and 18 with double aortic arch (DAA). The prevalence of 22q11 deletion syndrome was 5.5%. Six pregnancies were terminated and five cases were false-positive; therefore, the follow-up consisted of 99 neonates. Surgery was performed in 10 infants (10%) in the first year of life. In total, 25 (25%) children had surgery at a mean age of 17 months. Eight of these 25 (32%) had a DAA. Only one child, with a DAA, required surgery in the first week of life due to obstructive stridor. CONCLUSIONS: Children with a prenatally diagnosed RAA are at a low risk of acute respiratory postnatal problems. Delivery in a hospital with neonatal intensive care and pediatric cardiothoracic facilities seems only indicated in cases with suspected DAA. Expectant parents should be informed that presently 25% of the children need elective surgery and only incidentally due to acute respiratory distress.

Assessing Disease Activity in Pediatric Crohn's Disease Using Ultrasound: The Pediatric Crohn Disease Intestinal Ultrasound Score.

OBJECTIVES: Currently, there is no consensus on how to score Crohn disease (CD) activity assessed by intestinal ultrasound (IUS) in children. This study aimed to design an easy-to-use IUS score for disease activity in pediatric CD. METHODS: Children undergoing ileo-colonoscopy for CD assessment underwent IUS the day before ileo-colonoscopy, assessed with simple endoscopic score for CD (SES-CD). IUS features were compared to the SES-CD on segmental level. Multiple regression analyses, separately for terminal ileum (TI) and colon, were done to assess predictors of disease activity and to develop a model. RESULTS: In 74 CD patients (median 15 years, 48% female), 67 TI and 364 colon segments were assessed. Based on receiver operating characteristics curves, bowel wall thickness (BWT) was categorized into low [1 point: 2-3 mm (TI) and 1.6-2 mm (colon)], medium [2 points: 3.0-3.7 mm (TI) and 2.0-2.7 mm (colon)], and high [3 points: >3.7 mm (TI) and >2.7 mm (colon)]. In TI, only BWT was retained in the model [high BWT: odds ratio (OR) 11.50, P < 0.001]. In colon, BWT (high BWT: OR 8.63, P < 0.001) and mesenteric fat (1 point: OR 3.02, P < 0.001) were independent predictors. A pediatric Crohn disease IUS score (PCD-US) cut-off of 1 resulted in a sensitivity of 82% (95% confidence interval, CI: 65%-93%) and 85% (95% CI: 80%-89%) and a cut-off of 3 in a specificity of 88% (72%-97%) and 92% (87%-96%) for TI and colon, respectively. Inter-observer agreement was moderate for TI and colon ( K : 0.42, K : 0.49, respectively). CONCLUSIONS: The PCD-US score is an easy-to-use and reliable score to detect or rule out CD activity on segmental level in children. External validation is needed before applying this score in clinical practice.

Diagnosing Hirschsprung Disease in Children Younger than 6 Months of Age: Insights in Incidence of Complications of Rectal Suction Biopsy and Other Final Diagnoses.

BACKGROUND: The gold standard for diagnosing Hirschsprung disease (HD) in patients younger than 6 months is pathological examination of rectal suction biopsy (RSB). The aim of this study was to gain insight into the following: (1) complications following RSB, (2) final diagnosis of patients referred for RSB, and (3) factors associated with HD. METHODS: Patients suspected of HD referred for RSB at our center were analyzed retrospectively. Severity of complications of RSB was assessed using Clavien-Dindo (CD) grading. Factors associated with HD were tested using multivariate logistic regression analysis. RESULTS: From 2000 to 2021, 371 patients underwent RSB because of infrequent defecation, at a median age of 44 days. Three patients developed ongoing rectal bleeding (0.8%) graded CD1. Most frequent final diagnoses were: HD (n = 151, 40.7%), functional constipation (n = 113, 31%), idiopathic meconium ileus (n = 11, 3%), and food intolerance (n = 11, 3%). Associated factors for HD were male sex (odds ratio [OR], 3.19; confidence interval [CI], 1.56-6.53), presence of syndrome (OR, 7.18; CI, 1.63-31.69), younger age at time of RSB (OR, 0.98; CI, 0.85-0.98), meconium passage for more than 48 hours (OR, 3.15; CI, 1.51-6.56), distended abdomen (OR, 2.09; CI, 1.07-4.07), bilious vomiting (OR, 6.39; CI, 3.28-12.47), and failure to thrive (OR, 8.46; CI, 2.11-34.02) (model R 2 = 0.566). CONCLUSION: RSB is a safe procedure with few and only minor complications. In the majority of patients referred for RSB under the age of 6 months, HD was found followed by a functional cause for the defecation problems. RSB should be obtained on a low threshold in all patients under the age of 6 months with the suspicion of HD.

Comparing Smartphone Virtual Reality Exposure Preparation to Care as Usual in Children Aged 6 to 14 Years Undergoing Magnetic Resonance Imaging: Protocol for a Multicenter, Observer-Blinded, Randomized Controlled Trial.

BACKGROUND: A magnetic resonance imaging (MRI) procedure can cause preprocedural and periprocedural anxiety in children. Psychosocial interventions are used to prepare children for the procedure to alleviate anxiety, but these interventions are time-consuming and costly, limiting their clinical use. Virtual reality (VR) is a promising way to overcome these limitations in the preparation of children before an MRI scan. OBJECTIVE: The objective of this study is (1) to develop a VR smartphone intervention to prepare children at home for an MRI procedure; and (2) to examine the effect of the VR intervention in a randomized controlled trial, in which the VR intervention will be compared to care as usual (CAU). CAU involves an information letter about an MRI examination. The primary outcome is the child's procedural anxiety during the MRI procedure. Secondary outcomes include preprocedural anxiety and parental anxiety. We hypothesize that the VR preparation will result in a higher reduction of the periprocedural anxiety of both parents and children as compared to CAU. METHODS: The VR intervention provides a highly realistic and child-friendly representation of an MRI environment. In this randomized controlled trial, 128 children (aged 6 to 14 years) undergoing an MRI scan will be randomly allocated to the VR intervention or CAU. Children in the VR intervention will receive a log-in code for the VR app and are sent cardboard VR glasses. RESULTS: The VR smartphone preparation app was developed in 2020. The recruitment of participants is expected to be completed in December 2022. Data will be analyzed, and scientific papers will be submitted for publication in 2023. CONCLUSIONS: The VR smartphone app is expected to significantly reduce pre- and periprocedural anxiety in pediatric patients undergoing an MRI scan. The VR app offers a realistic and child-friendly experience that can contribute to modern care. A smartphone version of the VR app has the advantage that children, and potentially their parents, can get habituated to the VR environment and noises in their own home environment and can do this VR MRI preparation as often and as long as needed. TRIAL REGISTRATION: ISRCTN Registry ISRCTN20976625; https://www.isrctn.com/ISRCTN20976625. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/41080.

A preconception lifestyle intervention in women with obesity and cardiovascular health in their children.

BACKGROUND: Maternal obesity during pregnancy is associated with poorer cardiovascular health (CVH) in children. A strategy to improve CVH in children could be to address preconception maternal obesity by means of a lifestyle intervention. We determined if a preconception lifestyle intervention in women with obesity improved offspring's CVH, assessed by magnetic resonance imaging (MRI). METHODS: We invited children born to women who participated in a randomised controlled trial assessing the effect of a preconception lifestyle intervention in women with obesity. We assessed cardiac structure, function and geometric shape, pulse wave velocity and abdominal fat tissue by MRI. RESULTS: We included 49 of 243 (20.2%) eligible children, 24 girls (49%) girls, mean age 7.1 (0.8) years. Left ventricular ejection fraction was higher in children in the intervention group as compared to children in the control group (63.0% SD 6.18 vs. 58.8% SD 5.77, p = 0.02). Shape analysis showed that intervention was associated with less regional thickening of the interventricular septum and less sphericity. There were no differences in the other outcomes of interest. CONCLUSION: A preconception lifestyle intervention in women with obesity led to a higher ejection fraction and an altered cardiac shape in their offspring, which might suggest a better CVH. IMPACT: A preconception lifestyle intervention in women with obesity results in a higher ejection fraction and an altered cardiac shape that may signify better cardiovascular health (CVH) in their children. This is the first experimental human evidence suggesting an effect of a preconception lifestyle intervention in women with obesity on MRI-derived indicators of CVH in their children. Improving maternal preconception health might prevent some of the detrimental consequences of maternal obesity on CVH in their children.

Determining the Correct Resection Level in Patients with Hirschsprung Disease Using Contrast Enema and Full Thickness Biopsies: Can the Diagnostic Accuracy be Improved by Examining Submucosal Nerve Fiber Thickness?

BACKGROUND: Intraoperative resection level in patients with Hirschsprung disease (HD) is determined by contrast enema, surgeon's intraoperative judgement and full thickness biopsy (FTB) identifying ganglia. This study aims to evaluate diagnostic accuracy of contrast enema and FTB in determination of resection level and whether this can be improved by measuring submucosal nerve fiber diameter. METHODS: We retrospectively analyzed contrast enema and intraoperative FTBs obtained in our center, determining diagnostic accuracy for level of resection. Gold standard was pathological examination of resection specimen. Secondly, we matched transition zone pull-through (TZPT) patients with non-TZPT patients, based on age and length of resected bowel, to blindly compare nerve fibers diameters between two groups using group comparison. RESULTS: From 2000-2021, 209 patients underwent HD surgery of whom 180 patients (138 males; median age at surgery: 13 weeks) with 18 TZPTs (10%) were included. Positive predictive value of contrast enema was 65.1%. No caliber change was found in patients with total colon aganglionosis (TCA). Negative predictive value of surgeon's intraoperative judgement and FTB in determining resection level was 79.0% and 90.0% (91.2% single-stage, 84.4% two-stage surgery) respectively. Mean nerve fiber diameter in TZPT was 25.01 µm (SD= 5.63) and in non-TZPT 24.35 µm (SD= 6.75) (p = 0.813). CONCLUSION: Determination of resection level with combination of contrast enema, surgeon's intraoperative judgement and FTB results in sufficient diagnostic accuracy in patients with HD. If no caliber change is seen with contrast enema, TCA should be considered. Resection level or transition zone cannot be determined by assessment of submucosal nerve fiber diameter in FTB. TYPE OF STUDY: clinical research paper.

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.

Intestinal Ultrasound to Assess Ulcerative Colitis Disease Activity in Children: External Validation and Comparison of 2 Intestinal Ultrasound Activity Indices.

BACKGROUND: There is currently no consensus on the definition of an abnormal intestinal ultrasound (IUS) for children with ulcerative colitis (UC). This cross-sectional study aimed to externally validate and compare 2 existing IUS indices in children with UC. METHODS: Children undergoing colonoscopy for UC assessment underwent IUS the day before colonoscopy, assessed with the Mayo endoscopic subscore. The UC-IUS index and the Civitelli index were compared with the Mayo endoscopic score in the ascending, transverse, and descending colon. The area under the receiver-operating characteristic curve for detecting a Mayo endoscopic score ≥2 of both scores was compared and sensitivity and specificity were calculated. RESULTS: A total of 35 UC patients were included (median age 15 years, 39% female). The area under the receiver-operating characteristic curve was higher for the UC-IUS index in the ascending colon (0.82 [95% confidence interval (CI), 0.67-0.97] vs 0.76 [95% CI, 0.59-0.93]; P = .046) and transverse colon (0.88 [95% CI, 0.76-1.00] vs 0.77 [95% CI, 0.60-0.93]; P = .01). In the descending colon, there was no difference (0.84 [95% CI, 0.70-0.99] vs 0.84 [95% CI, 0.70-0.98]). The optimal cutoff for the UC-IUS was <1 point to rule out a Mayo endoscopic score ≥2 (sensitivity: 88%, 100%, and 90% in the ascending, transverse, and descending colon, respectively) and a Mayo endoscopic score ≥2 could be detected using a cutoff of >1 (specificity: 84%, 83%, and 87%, respectively). For the Civitelli index, in our cohort, the optimal cutoff was <1 to rule out a Mayo endoscopic score ≥2 (sensitivity 75%, 65%, and 80%, respectively) and a cutoff >1 to detect a Mayo endoscopic score ≥2 (specificity 89%, 89%, and 93%, respectively). CONCLUSIONS: In this cohort, the UC-IUS index performed better than the Civitelli index. The UC-IUS index had both a high sensitivity and specificity in this cohort, when using 1 point as cutoff for a Mayo endoscopic score ≥2.

In this prospective study, we validated and compared 2 intestinal ultrasound indices to score pediatric ulcerative colitis: the UC-IUS index and the Civitelli index. In our cohort, the UC-IUS index was more accurate.

The Management of Post-appendectomy Abscess in Children; A Historical Cohort Study and Update of the Literature.

Introduction: Recent studies have shown that specific cases of post-appendectomy abscess (PAA) in children could be treated conservatively. However, due to the lack of high-quality evidence, choice of treatment still depends on preferences of the treating surgeon, leading to heterogeneity in clinical practice. Therefore, we aimed to provide an update of recent literature on the management of PAA in children and subsequently evaluate the outcomes of a large multicenter cohort of children treated for PAA. Methods: A literature search was performed in Pubmed and Embase, selecting all randomized controlled trials, prospective and retrospective cohort studies, and case series published from 2014 and onward and reporting on children (<18 years) treated for a PAA. Subsequently, a historical cohort study was performed, including all children (<18 years) treated for a radiologically confirmed PAA between 2014 and 2021 in a tertiary referral center and two large peripheral centers. Medical charts were reviewed to compare non-invasive (i.e., antibiotics) and invasive (i.e., drainage procedures) treatment strategies. Primary outcome was the success rate of treatment, defined as no need for further interventions related to PAA or its complications. Results: The search yielded 1,991 articles, of which three were included. Treatment success ranged between 69-88% and 56-100% for non-invasive and invasive strategies, respectively. Our multicenter cohort study included 70 children with a PAA, of which 29 (41%) were treated non-invasively and 41 (59%) invasively. In the non-invasive group, treatment was effective in 21 patients (72%) compared to 25 patients (61%) in the invasive group. Non-invasive treatment was effective in 100% of unifocal small (<3 cm) and 80% of unifocal medium size PAA (3-6 cm), but not effective for multiple abscesses. Conclusion: Non-invasive treatment of especially unifocal small and medium size (<6 cm) PAA in children seems to be safe and effective. Based on these results, a standardized treatment protocol was developed. Prospective validation of this step-up approach-based treatment protocol is recommended.