Journal of pediatric surgery最新文献

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.

Background: Patients with esophageal atresia with or without tracheoesophageal fistula (EA-TEF) frequently require fundoplication to manage gastroesophageal reflux disease (GERD). Data is limited on whether fundoplication improves GERD-related outcomes and if outcomes vary by type of fundoplication.

Methods: This is a retrospective cohort study of patients <18 years-old who underwent EA-TEF repair and fundoplication at a single institution from January 2013 to September 2025. In the first aim, we compared GERD symptoms, medications, nutrition, and esophageal stricture dilation rate before and after fundoplication. In the second aim, we compared outcomes between partial and complete fundoplication at 12 months after surgery.

Results: Of 226 children with EA-TEF repair, 36 (15.9%) underwent fundoplication, with 14 (38.8%) complete and 22 (61.1%) partial fundoplication. The median age at fundoplication was 7.3 [3.7, 15.9] months. Most patients (69.4%) had Type C EA-TEF. The most common indication was refractory GERD (88.9%). There were no statistical differences in GERD-related symptoms - oral aversion, dysphagia, retching, and vomiting - and medication use when comparing pre- to post-fundoplication. Fundoplication significantly reduced the need for post-pyloric feeds (67% vs 11%, P<0.001) and improved weight-for-age z-scores (-1.94 vs -1.03, P<0.001). Fundoplication also decreased the need for stricture dilation (3.07 vs. 1.36 dilations/year, P=0.001). There were no differences in outcomes between partial and complete fundoplication. Eight patients required fundoplication revision, four from each group.

Conclusion: In our EA-TEF cohort, fundoplication facilitated conversion from post-pyloric to gastric feeds, decreased stricture burden, and improved nutritional status. Outcomes were not impacted by the type of fundoplication performed.

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.

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.