BACKGROUND: Comprehensive VACTERL screening is crucial in children with esophageal atresia (EA), as undetected anomalies can delay diagnosis and treatments for these problems. Screening practices vary widely in the liter...BACKGROUND: Comprehensive VACTERL screening is crucial in children with esophageal atresia (EA), as undetected anomalies can delay diagnosis and treatments for these problems. Screening practices vary widely in the literature, however. This study aimed to assess the proportion of EA patients receiving comprehensive VACTERL screening, examine screening changes over time and determine the prevalence of additional anomalies, VACTERL association and genetic diagnoses. METHODS: A retrospective cohort study was conducted at a single center, including all neonates born with EA between 2000 and 2024. Comprehensive VACTERL screening included vertebral x-ray, cardiac and renal ultrasound, and physical examination for limb deformities and anorectal malformation. VACTERL classification was based on EUROCAT definitions. RESULTS: A total of 240 patients were included. Comprehensive VACTERL screening was conducted in 180 (75%) patients, improving to 100% in the past four years (i.e. 2020-2024). In screened patients, vertebral (32%) and cardiac (28%) anomalies were most commonly identified. VACTERL association was present in 28% of comprehensive screened EA patients: 14% VACTERL-LIKE, 11% STRICT-VACTERL and 3% VACTERL-PLUS. A genetic association was identified in 13% of patients, most commonly trisomy 21. CONCLUSIONS: While 75% of EA patients underwent comprehensive VACTERL screening, a fourth of the patients were not fully assessed, potentially leading to unrecognized anomalies with possible clinical implications. Over the last four years 100% screening rate has successfully been attained. VACTERL association was identified in 28% of patients and a genetic diagnosis was established in 13%. These findings emphasize the importance to pursue systematic and comprehensive VACTERL screening for patients with EA to detect these anomalies.
BACKGROUND: Extrahepatic portal vein obstruction (EHPVO) can result in various complications, including life threatening gastrointestinal bleeding. The Meso-Rex bypass (MRB) restores a physiological portal flow. However...BACKGROUND: Extrahepatic portal vein obstruction (EHPVO) can result in various complications, including life threatening gastrointestinal bleeding. The Meso-Rex bypass (MRB) restores a physiological portal flow. However a history of umbilical vein catheterization (UVC) has sometimes been considered as a contraindication to MRB. OBJECTIVE: To evaluate the feasibility and results of MRB in children with EHPVO secondary to UVC. METHODS: We performed a retrospective, single-center study of 51 children with EHPVO in native (non-transplanted) livers treated between 2009 and 2023. All patients underwent preoperative retrograde portography (RP) to assess intrahepatic portal venous system (IPVS) patency with measurement of transhepatic pressure gradient and a liver biopsy. RESULTS: Of the 51 patients, 23 (45%) had a history of UVC. IPVS was patent in 9/23 (39%) versus 21/28 patients without UVC (75%) (p = 0.0096). None had significant liver fibrosis. Thirty patients underwent MRB. After a median follow-up of 5.7 years, long-term shunt patency was achieved in 29 of 30 patients (96.6%). Two episodes of thrombosis were observed: one early (day 4, managed with surgical thrombectomy) and one late (18 months, managed conservatively). Eleven patients (36%) developed secondary shunt stenosis, successfully treated with radiologic balloon angioplasty in all but one case. The incidence of vascular complications was similar in UVC and non-UVC groups (33% vs. 38%, NS). CONCLUSION: A history of UVC significantly reduces the patency of IPVS but is not a contraindication to MRB when the IPVS is patent on retrograde portography and the liver is not fibrotic.
INTRODUCTION: Long-term functional outcomes in patients with cloacal malformations remain poorly described. This study reports long-term outcomes from a tertiary referral center. METHODS: Retrospective review of female p...INTRODUCTION: Long-term functional outcomes in patients with cloacal malformations remain poorly described. This study reports long-term outcomes from a tertiary referral center. METHODS: Retrospective review of female patients with cloacal malformations treated between 1980 and 2010. Colorectal, urological, and gynaecological outcomes were obtained from medical records. Sexual function was assessed using a validated female sexual health questionnaire and compared with 15 healthy controls. RESULTS: Eleven women were included, with a mean age at follow-up of 24.77 ± 7.41 years. Associated anomalies were present in 73% of patients, including spinal anomalies in 27%. All patients underwent neonatal stoma creation and closure, the latter at a median age of 2.55 (1.61-3.72) years. Faecal continence was achieved in 73% of patients at a mean age of 10.4 ± 3.1 years; one patient required laxatives and five required enemas. All patients achieved urinary dryness. A Mitrofanoff procedure was required in 36%, and one patient underwent bladder augmentation. End-stage renal disease developed in 36%, with three patients undergoing renal transplantation and one awaiting transplant. Three patients (27%) required vaginoplasty, and four (36%) later underwent external genitoplasty. Müllerian anomalies were present in 73%, and 91% menstruated. Sexual function assessment demonstrated increased fear of sexual activity, avoidance due to perineal appearance, reduced excitation, and greater difficulty with vaginal penetration compared with controls. CONCLUSION: Most patients in our institution with cloacal malformations achieve satisfactory faecal and urinary continence following reconstruction. Renal dysfunction and impaired sexual function remain relevant long-term issues, supporting the need for lifelong multidisciplinary follow-up.
OBJECTIVE: To develop and validate a simple, preoperative risk score based on fundamental patient characteristics to predict the need for reoperation for adhesive small bowel obstruction (ASBO) in neonates undergoing lap...OBJECTIVE: To develop and validate a simple, preoperative risk score based on fundamental patient characteristics to predict the need for reoperation for adhesive small bowel obstruction (ASBO) in neonates undergoing laparotomy. METHODS: In this retrospective cohort study of 410 neonates undergoing laparotomy at a single tertiary center (2008-2019), we identified independent predictors for the primary outcome of "first reoperation for surgically confirmed ASBO" using multivariable Cox regression. Predictors were selected from universally available preoperative variables. The resulting Neonatal Adhesion Risk Score (NARS) was validated on the same cohort. RESULTS: Four independent preoperative predictors were identified: 1) high-risk primary diagnosis (HR 3.8, 95% CI 2.1-6.9), 2) gestational age <34 weeks (HR 2.0, 95% CI 1.2-3.4), 3) presence of major associated congenital anomalies (HR 1.6, 95% CI 1.1-2.4), and 4) birth weight <1500 g (HR 1.4, 95% CI 1.0-2.0). High-risk diagnoses included gastric perforation, jejunal atresia, meconium ileus, and necrotizing enterocolitis (NEC). The NARS stratified patients into low (0-2 points), moderate (3-4 points), and high-risk (≥5 points) groups, with observed reoperation rates for ASBO of <10%, 25-35%, and >50%, respectively. The high-risk group accounted for nearly half of all first reoperations and had a significantly shorter median time to reoperation (7 vs. 24 days, p < 0.001). CONCLUSION: The NARS is a clinically applicable, preoperative tool that effectively identifies neonates at high risk for early and recurrent surgical intervention for ASBO. By utilizing readily available clinical data, it enables targeted surveillance, informed family counseling, and a rational framework for considering adjuvant anti-adhesion strategies in the most vulnerable patients.
INTRODUCTION: The incidence of pediatric firearm injuries is increasing. While developed independently, Social Vulnerability Index (SVI) and Child Opportunity Index (COI) quantify socioeconomic disparities in pediatric p...INTRODUCTION: The incidence of pediatric firearm injuries is increasing. While developed independently, Social Vulnerability Index (SVI) and Child Opportunity Index (COI) quantify socioeconomic disparities in pediatric populations although using different variables. There is scant research applying these indices to the incidence of pediatric firearm injuries. We aimed to investigate the association between SVI, COI, and pediatric firearm injuries seen at a state verified pediatric level one trauma center. METHODS: We included children evaluated for firearm injury from 2016 to 2023 in a retrospective cohort study. Patient's residence was linked to concordant year SVI (high indicating higher vulnerability) and state COI (low indicating worse opportunity). SVI and COI quartiles were created; bivariate analysis compared demographics and patient outcomes by quartiles for each index. Incidence rate ratios (IRR) compared SVI and COI quartiles using CDC population variables. RESULTS: 554 children were included. Majority were Black (68.2%) and male (70.4%) with a median (IQR) age of 11.4 years (4.3-15.0). A higher proportion of Black children and patients using government insurance were in higher SVI and lower COI cohorts (p < 0.001). Sex, age, hospital length of stay, and mortality was similar between the SVI and COI quartiles. The IRR increased with increasing SVI and decreasing COI quartiles, compared to the Very Low quartiles, respectively, which persisted when examining the themes of each database. CONCLUSIONS: The incidence of firearm injury was increased in children from areas with increased socioeconomic, racial, ethnic inequities and in those from less advantaged neighborhoods. These data will be useful in identifying areas to target for future interventions to address pediatric firearm injuries.
INTRODUCTION: Despite the broader adoption of minimally invasive liver surgery (MILS) for adults, its application in the pediatric population has been mainly documented in single-center reports. We aimed to summarize the...INTRODUCTION: Despite the broader adoption of minimally invasive liver surgery (MILS) for adults, its application in the pediatric population has been mainly documented in single-center reports. We aimed to summarize the current state of evidence of MILS in children. METHODS: We systematically reviewed PubMed, Scopus, Web of Science, and Cochrane Library databases according to the PRISMA statement for children (<18 years) undergoing MILS (end-of-search date: 03/03/2024). The National Cancer Database (NCDB) was queried for children (<18 years) undergoing MILS (2010-2021). RESULTS: Forty-one articles reporting on 211 patients were included. The approach was laparoscopic in 91.5%, robotic in 5.7%, and hand-assisted 2.8%. The mean age was 5.4 ± 4.4 years, the mean lesion size was 6.2 ± 3.4 cm, and the most common indications were hepatoblastoma (28.9%) and hydatid cyst (27.0%). Most underwent cyst surgery (43.0%), followed by minor liver resection (37.7%). Five (2.4%) had conversion to open. The mean operative time was 162.1 ± 99.1 min and mean length of stay was 6.4 ± 4.9 days. Over a mean follow-up of 22.9 ± 24.6 months, one patient died (0.5%). Forty-six children underwent MILS in NCDB (45 laparoscopic, 1 robotic). The mean age was 4.7 ± 5.7 years and mean tumor size was 1.0 ± 1.3 cm. Most underwent wedge/segmental resection (47.8%). Five (10.9%) had conversion to open and mean length of stay was 6.3 ± 5.0 days. CONCLUSION: This first systematic review and NCDB analysis establishes feasibility benchmarks of MILS in children for both benign and malignant liver lesions. MILS is associated with 2-11% conversion rate and mean length of stay of 6 days. LEVEL OF EVIDENCE: Level III evidence.
BACKGROUND: Total Colonic and Small Bowel Aganglionosis (TCSA) is a severe form of Hirschsprung Disease (<10 % of cases). Surgical gold standard is Pull-Through (P-T) procedure. After P-T, in selected cases, an unplanned...BACKGROUND: Total Colonic and Small Bowel Aganglionosis (TCSA) is a severe form of Hirschsprung Disease (<10 % of cases). Surgical gold standard is Pull-Through (P-T) procedure. After P-T, in selected cases, an unplanned ileostomy could be required to cope with poor bowel control. Aim of this study is to investigate unplanned ileostomies in TCSA. METHODS: A retrospective observational cohort study was performed. Charts of all TCSA patients admitted between 2017 and 2025 were reviewed. Patients requiring an unplanned ileostomy were identified. Data regarding clinical features, surgical management and outcomes before and after stoma fashioning were collected. RESULTS: Twelve percent (8/69) of TCSA patients required an unplanned ileostomy; 6(6/8,75 %) had a syndrome with intellectual disability (ID). Considering the entire cohort, all patients with Down Syndrome (5/5) and 50 %(1/2) of patients with Mowat-Wilson required an ileostomy. Median age at ileostomy was 9.3 (IQR 6.75) years. Seven patients (87.5 %) required an ileostomy due to a severe perineal rash, one to treat a sever Crohn-like ileitis. ID was significantly associated with ileostomy at univariate and multivariate analysis (OR respectively 105 and 48, p < 0.001).All patients with ID and 50 % of those without ID did report complete symptoms resolution after ileostomy fashioning. CONCLUSION: This is the first study quantifying and investigating the causes of unplanned ileostomies in TCSA. ID is a major risk factor for an unplanned ileostomy. An exhaustive counseling should be given to parents of patients with syndromic ID, emphasizing a considerable probability of a potentially definitive ileostomy and the uselessness of a P-T.
BACKGROUND: Ethanolamine oleate (EO) is a sclerosing agent commonly used to treat slow-flow vascular malformations (SFVMs). Macroscopic hemoglobinuria is the most frequent complication of EO sclerotherapy and may occasio...BACKGROUND: Ethanolamine oleate (EO) is a sclerosing agent commonly used to treat slow-flow vascular malformations (SFVMs). Macroscopic hemoglobinuria is the most frequent complication of EO sclerotherapy and may occasionally progress to acute renal failure. Because EO was originally developed for adult esophageal and gastric varices, data on its pediatric use remain limited. This study aimed to identify risk factors for macroscopic hemoglobinuria and to develop a practical scoring system to support the safe use of EO in children. METHODS: This retrospective study included patients aged ≤15 years who underwent EO sclerotherapy for venous or lymphatic-venous malformations between January 2019 and March 2025. Associations between macroscopic hemoglobinuria and patient characteristics, magnetic resonance imaging findings, and intraoperative factors, including the weight-adjusted EO dose, were analyzed. Based on significant factors, a risk-scoring system was developed and its diagnostic performance evaluated. RESULTS: A total of 100 procedures with 51 patients were analyzed, and macroscopic hemoglobinuria occurred in 34.6% of cases. Hemoglobinuria was not observed in patients with lesions confined to subcutaneous tissue, or those without drainage outflow on contrast imaging. Four factors were identified: lesion size, morphology, drainage outflow, and weight-adjusted EO dose (>0.158 mL/kg). A 8-point scoring system demonstrated good discriminative performance (area under the curve, 0.828), with a cutoff score ≥4 providing optimal diagnostic balance (Youden index, 0.44). All cases resolved with hydration and haptoglobin administration, and no patient developed acute renal impairment. CONCLUSION: This study presents the first pediatric-focused risk scoring framework for hemoglobinuria after EO sclerotherapy. The proposed scoring system enables objective clinical risk stratification and may contribute to safer and more individualized treatment strategies for children with SFVMs.
INTRODUCTION: Congenital esophageal stenosis (CES) related to tracheobronchial remnant (TBR) is an extremely rare malformation, typically diagnosed in infants and young children. Endoscopic dilatation or resection and an...INTRODUCTION: Congenital esophageal stenosis (CES) related to tracheobronchial remnant (TBR) is an extremely rare malformation, typically diagnosed in infants and young children. Endoscopic dilatation or resection and anastomosis are both accepted therapeutic options, but the superiority of one versus the other remains controversial. METHODS: We performed a comprehensive literature search in PubMed and MEDLINE using standardized search terms and subject headings from 1969 onwards. Inclusion criteria were patient age 0-18 years, confirmed diagnosis of CES due to TBR, English language, intervention by balloon dilatation or surgical resection, and reporting of outcome. RESULTS: A total of 215 articles were identified, of which 33 met the inclusion criteria. These consisted of 11 retrospective observational studies, 10 case series, and 12 single case reports. In total, 183 patients were included in our analysis, of which 103 patients were initially treated with resection/anastomosis, and 80 with primary dilatation. Patients who underwent primary resection required between 1 and 3 additional dilations. Patients who underwent initial dilatation required between 1 and 7 re-dilations. The resection/anastomosis group group demonstrated a significantly lower mean complication rate (2%) compared to those who underwent primary dilatation (13.8%, p < 0.01). CONCLUSION: Both resection and balloon dilatation are viable options to treat CES associated with TBR. While those undergoing upfront dilatation are more likely to require further interventions, some may be spared a more invasive operative procedure.
Children living in remote and Indigenous communities in Canada often face significant barriers to timely surgical care due to geographic isolation, limited local resources, and financial pressures within a publicly funde...Children living in remote and Indigenous communities in Canada often face significant barriers to timely surgical care due to geographic isolation, limited local resources, and financial pressures within a publicly funded healthcare system. These challenges frequently create ethical dilemmas for clinicians and families, who must choose between delaying care for transfer to a tertiary center or proceeding locally with limited expertise. This paper explores three illustrative cases including emergent neonatal volvulus with no possibility of transfer, pyloric stenosis with family-declined transfer, and elective hernia repair with family-requested transfer. The purpose is to examine the ethical tensions that arise when access to specialized pediatric surgery is constrained. Applying the principles of beneficence, non-maleficence, justice, and respect for autonomy, each scenario highlights the complex interplay between individual patient needs, professional responsibility, systemic equity, and fiscal stewardship. Through these cases, we argue that ethical decision-making under constraint demands not only clinical judgment but also an understanding of the healthcare system and a nuanced approach. Solutions such as enhanced rural surgical training, telemedicine support, culturally informed care, and robust infrastructure investment are essential to ensure that no child is left behind due to geography or resource scarcity.
OBJECTIVE: To develop and validate a deep learning-based multi-instance learning (MIL) model that integrates CT imaging and clinical data to improve the accuracy of discriminating between strangulated small bowel obstruc...OBJECTIVE: To develop and validate a deep learning-based multi-instance learning (MIL) model that integrates CT imaging and clinical data to improve the accuracy of discriminating between strangulated small bowel obstruction (StSBO) from simple small bowel obstruction (SiSBO) in pediatric patients. MATERIALS AND METHODS: This multicenter retrospective study, conducted between January 2018 and June 2024, enrolled hospitalized pediatric patients aged 1-14 years with a diagnosis of small bowel obstruction. We developed the clinical, multi-instance learning, and combined models based on CT and clinical features. Model performance was evaluated using receiver operating characteristic (ROC) analysis, while SHapley Additive exPlanations (SHAP) interpreted feature contributions. We further assessed whether MIL-assisted diagnosis could enhance physician accuracy in diagnosing StSBO. RESULTS: The study sample comprised 168 patients (mean age, 6.36 ± 3.97, 118 men). Ascites and closed-loop sign were identified as independent predictors of StSBO on multivariate analysis (both p< 0.05). The MIL model achieved the area under the curve (AUC) of 0.86 (95%CI 0.70-1.00), p= 0.01 in the external test cohort. The combined model showed the highest diagnostic performance (AUC 0.87, 95%CI 0.72-1.00, p= 0.01) in the external test cohort, with MIL-derived features showing predominant importance in SHAP analysis. Both junior and experienced radiologists and surgeon demonstrated improved diagnostic performance with MIL assistance, showing AUC increases of 16%, 2%, and 20%, respectively. CONCLUSIONS: The MIL model performed well in diagnosing StSBO, and clinical data integration improved its performance. As a decision support tool, the model may aid risk stratification and facilitate timely escalation of care in pediatric StSBO management.