Pediatric Surgery International最新文献

Objective: To explore the feasibility of open pyeloplasty (OP) for treating pediatric pelviureteric junction obstruction in resource-limited settings in the era of robot assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP).

Methods: A total of 168 patients (56 each) were randomized to receive RALP, LP, or OP, respectively.

Results: The operative time for the RALP was significantly higher (P < 0.001) compared to LP and open OP. The length of stay (LOS) for RALP and LP was substantially lower (P < 0.001) compared to the OP, with average stays of 2.8 ± 1.5 days, 3.1 ± 1.2 days, and 6.4 ± 8.1 days, respectively. In the RALP group, 54 (96.4%) had a non-obstructed drainage pattern post-operatively compared to 52 (92.8%) in the LP group, and 53 (94.6%) patients in the OP group (P = 0.363). Only 2 (3.6%) patients in the RALP group and 4 (7.1%) patients in the LP group, and 3 (5.3%) patients in OP group, exhibited obstructed drainage on dynamic nuclear scan. Grade II complications (urine leakage) occurred in 2 patients in the RALP cohort, and in 3 patients in the LP cohort. The leakage typically resolved within 10 days and did not require further intervention. Higher-grade complications (Clavien Grade 3 and 4a), such as hydronephrosis, were identified in both the RALP and OP groups (one case each).

Conclusion: Due to comparable success rates and minimal complications, OP is a viable alternative to minimally invasive RALP and LP in treating pediatric patients with PUJO, particularly where cost and equipment availability are limiting factors.

Purpose: Congenital pulmonary airway malformation (CPAM) is a developmental lung malformation that seriously endangers children's health. The objective of this study was to investigate the etiology of CPAM by observing changes at the molecular and cellular levels.

Methods: Patient clinical data were collected and analyzed. Tissue samples were collected from CPAM lesions and marginal normal lung tissue during CPAM surgery. The samples were subjected to hematoxylin and eosin (H&E) staining for pathological analysis. Tissue RNA was isolated for RNA sequencing, and the differentially expressed genes (DEGs) were enriched for Gene Ontology (GO) analysis. The cytoskeletal and cell subtypes were subjected to immunofluorescence staining.

Results: RNA sequencing of 7 CPAM patients revealed 1252 DEGs, with 1041 upregulated and 211 downregulated. GO analysis revealed that biological processes related to cilium organization and movement were strongly enriched. Protein-protein interaction (PPI) network analysis highlighted genes such as CCDC65, DNAH5, DNAH11, DNAH12, CFAP43, CFAP70, PIH1D3, RSPH4A and DNAH6 as potentially important in CPAM. Immunofluorescence staining revealed abnormalities in cytoskeleton and cilia, and a reduction in the number of alveolar epithelial type II (AEC II) cells in CPAM lesions compared with normal lung tissue.

Conclusion: Our study revealed a significant increase in cytoskeletal and cilia expression, along with a marked reduction in AEC II. These abnormalities provide potential insights into the etiology of CPAM and may guide the development of improved diagnostic and therapeutic strategies.

Background and objectives: The diagnosis and surgery of congenital lung malformations (CLMs) affect not only children's physical health but also emotional, psychological, and social well-being of their families. This study assessed the long-term quality of life (QoL) and psychological impact in families of children who underwent surgery for CLMs during infancy.

Methods: In May 2024, the national CLM patient association invited parents of children with surgically treated CLMs to complete Parenting Stress Index, Pediatric Quality of Life Inventory (PedsQL), and PedsQL Healthcare Satisfaction Module. Most children had undergone surgery in infancy and were grouped by current age (13-24 months, 2-4 years, 5-7 years, and 8-12 years). The survey assessed parents' current perceptions of their child's QoL, their own stress, and healthcare satisfaction. Maternal and paternal responses were compared, and linear regression explored associations with clinical variables.

Results: Seventy-eight mothers and 47 fathers responded. Overall QoL was high. Lower scores were associated with lack of psychological support and longer hospital stays. Compared to norms, children reported reduced emotional and school functioning. Fathers expressed higher satisfaction, while mothers reported greater distress, especially in the 8-12 year group.

Conclusions: Children generally experience good long-term QoL, but families, particularly mothers, report persistent psychological distress. Integrating psychological care into long-term follow-up is essential.

Purpose: PTTG1 is an oncogene that is highly expressed in various cancers and is involved in regulating the cell cycle in neuroblastoma (NB) cells. However, the specific role of PTTG1 in NB has not been extensively reported. We undertook this study to investigate the expression of PTTG1 in various NB cell lines to identify the gene expression patterns.

Methods: Small interfering RNA (siRNA) targeting PTTG1 was designed and used to transfect NB cells. Cell proliferation levels, wound healing and transwell experiments were undertaken to assess the invasion and migration abilities of transfected and control NB cells. Western blot, PCR, and immunofluorescence experiments were utilized to detect the expression of migration-related proteins, differentiation-related proteins, and autophagy-related proteins in NB cells. Different doses of the autophagy inhibitor 3-methyladenine (3-MA) were used for validating the mechanism.

Results: High expression of PTTG1 was seen in three types of NB cell lines, with the most significant levels observed in SK-N-SH cells. Interference of PTTG1 significantly inhibited the activity of SK-N-SH cells, reducing their proliferation, invasion, and migration abilities, and was accompanied by a decrease in MMP2 and MMP9 protein expression. In addition, there was enhancement of fluorescence intensity of the differentiation marker TUBB3 and the autophagy marker LC3II, and upregulated the protein expression and mRNA levels of GAP43, TH, MEG, TUBB3, LC3II/LC3I, and beclin1, while downregulated the expression levels of P62 and mTOR. After applying the autophagy inhibitor 3-MA, the regulation of SK-N-SH cell proliferation and differentiation by PTTG1 interference was significantly reduced.

Conclusions: PTTG1 is highly expressed in various NB cells. Interfering with PTTG1 induces autophagy, thereby inhibiting SK-N-SH cell proliferation and promoting differentiation.

The historical legacy of using fixed distances from the dentate line (DL) for rectal resection during pull-through for Hirschsprung's disease (HD) has never really been investigated despite widespread continuous use. Swenson initially used 1.5-2.0 cm from the anal verge for resection. In 1962, Bill and Chapman reported an association between residual aganglionic segments, obstructive symptoms, and postoperative enterocolitis. In 1964, Swenson modified his resection to 1.5-2.0 cm from the DL anteriorly and 1.0 cm or less posteriorly. Other master surgeons also explored lines for rectal resection; however almost all were based on anecdotal experience. More recently, possible associations between resection lines and postoperative complications were reported by De la Torre (2017) and a nationwide survey in Japan (2019). In 2007, co-author AY adopted the anorectal line (ARL) exclusively as an alternative to the DL specifically because it was readily identifiable, independent of age/build, functionally relevant as the distal margin of the enteric nervous system, and indispensable for normal anal sensation as the upper border of the anal transitional zone histopathologically. While the anal verge and the DL have been important historically, the ARL objectively enhances the reliability of rectal resection of the aganglionic segment without causing injury to the anal transitional zone.

Objectives: Early identification of pediatric renal tumors improves outcomes. Neighborhood deprivation, a composite measure of social determinants of health, is associated with poor oncological outcomes. We studied whether neighborhood deprivation influenced metastatic presentation or treatment approach.

Study design: Using State Inpatient Databases (SID), we studied children admitted for nephrectomy for a renal tumor in 15 states (2013-2021). Neighborhood deprivation was measured using the Child Opportunity Index 3.0 (COI). Using univariate and multivariate analyses, we examined whether patients presented with metastatic disease and whether they underwent upfront nephrectomy versus nephrectomy after neoadjuvant chemotherapy (delayed nephrectomy).

Results: Out of 1,574 children, 14.2% presented with metastases, and 23.3% underwent delayed nephrectomy. Presentation with metastases and delayed nephrectomy were associated with older age and more complex chronic conditions. Neighborhood deprivation was not significantly associated with presentation with metastases or delayed nephrectomy.

Conclusions: Neighborhood deprivation did not significantly impact the presentation or timing of nephrectomy of pediatric renal tumors; biological factors rather than access to care may be the major drivers of variability. However, administrative databases are limited in their ability to fully inform the study of pediatric renal tumors.

Background: A considerable proportion of pediatric giant hepatoblastoma cases involve the second and third hepatic portals (GHSTP), which were previously considered unresectable. Surgical management of such GHSTP-involved cases is associated with significant technical challenges. This retrospective study was designed to assess the feasibility and surgical approaches for pediatric giant hepatoblastoma with involvement of the second and third hepatic portals.

Methods: From January 2006 to December 2024, 59 pediatric patients with giant hepatoblastoma involving the second and third hepatic portals (GHSTP) underwent partial hepatectomy. The therapeutic outcomes of these cases were retrospectively analyzed.

Results: All 59 patients underwent successful liver tumor resection. The operative duration ranged from 160 to 350 min, with a mean of 270 min. First hepatic portal clamping was performed in all cases. The resection approaches were as follows: 13 cases underwent resection of segments V-VIII; 12 cases, resection of segments IV-VIII; 6 cases, resection of segments I-IV; 10 cases, resection of segments I, IV, and VIII; 8 cases, resection of segments I, IV, V, and VIII; 4 cases, resection of segment I plus segments V-VIII; and 6 cases, resection of segment I plus segments IV-VIII. There was no mortality, and postoperative complications occurred in 8 cases.

Conclusion: Resection of giant hepatoblastoma involving the second and third hepatic portals remains technically challenging; however, with meticulous and accurate preoperative assessment coupled with a thorough understanding of hepatic anatomy, such tumor resection is feasible and can be performed with acceptable safety. Moreover, complete resection of this subset of tumors may effectively improve the long-term survival outcomes of patients with hepatoblastoma.

Purpose: Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma. FOXO1 fusion indicates poor prognosis and lead to dysregulation of transcriptioanal network. This study aims to investigate clinical characteristics and therapeutic targets concerning FOXO1 fusion status.

Method: 65 pediatric RMS patients were enrolled. Clinical data were analyzed using Kaplan-Meier estimates and Cox regression. Surgically resected tumor tissues were subject to single-cell RNA sequencing (scRNA-seq). Patient-derived xenograft (PDX) was establish and dissociated to cells for high-throughput drug screening.

Results: Among the 65 patinets (36 patients with embryonal RMSs (ERMSs), 15 patients with alveolar RMSs (ARMSs) and 14 patients with other types of RMSs), 73.3% of ARMSs were defined as fusion positive (FP) while 6 ERMS (ERMS)s were also FP. Cox regression analysis identified FOXO1 fusion as a risk factor alone and combined with pathologic subtype, sex and age or metastasis status. scRNA-seq revealed distinct transcription factor networks between FP and FN RMS, showing up-regulated activity of OLIG2, NHLH1, SNAI1, TFF3 and other TFs related to neural development and differentiation. MAPK, PI3K-Akt, and mTOR pathways were enriched in FP-RMS tumor cells. High-throughput drug screening of PDX-derived cells identified sensitive drugs targeting FP-RMS specific signatures. AMG-337 was selected and validated for its anti-tumor effect.

Conclusion: FOXO1 fusion status influences RMS clinical outcomes, including rare FP-ERMS cases. scRNA-seq combined with drug screening identified MET as a promising therapeutic target in FP-RMS.

Purpose: Assess the safety of single-stage laparoscopic proctocolectomy with ileal pouch-anal anastomosis (LPC-IPAA) in children with familial adenomatous polyposis (FAP).

Methods: Medical records for all patients with FAP who underwent LPC-IPAA between 1/1/2013 and 8/1/2024 at a pediatric center were reviewed. Baseline characteristics, postoperative complications, and functional outcomes were assessed.

Results: Thirty-nine patients met inclusion criteria. Mean age was 14.9 years (range 9.4-21.5) and mean BMI was 23.0 (range 15.3-47.0). Follow-up averaged 2.8 years (SD 2.9). Mean operative time was 228.4 min (SD 44.5) in the stapled group (n = 32) and 316.1 min (SD 43.1) in the hand-sewn group (n = 7) (p < 0.001). There were no anastomotic leaks. One patient developed a postoperative abscess. Six (15%) developed presumed pouchitis, 4 of whom were in the hand-sewn group and all successfully treated with antibiotics. Six patients (15%) developed an anastomotic stricture - all in the stapled group - and responded well to anal dilation. No patient required treatment for fecal incontinence.

Conclusion: LPC-IPAA in adolescents and young adults with FAP can safely be performed in a single operation and is associated with favorable functional outcomes.

Purpose: Increasing evidence has indicated a role of oxidative stress in the pathogenesis of biliary atresia (BA). This study aimed to identify key oxidative stress-related biomarkers in BA and explore their therapeutic potential.

Methods: BA datasets were obtained from the GEO database. Differentially expressed genes (DEGs) were identified using the GSE46960 dataset. These DEGs were intersected with oxidative stress-related genes from GeneCards to obtain the oxidative stress-related DEGs (ORDEGs). Functional enrichment (GO, KEGG) and gene set enrichment analyses were performed. Hub genes were identified using machine learning and protein-protein interaction (PPI) network analysis. Their expression was validated via qRT-PCR in BA patient liver samples and rhesus rotavirus (RRV)-induced mouse models. Potential transcription factors (TFs), microRNAs, and drugs targeting hub ORDEGs were predicted using TRRUST, TarBase, and DSigDB, respectively.

Results: Six hub ORDEGs (CCL2, CXCL8, SPP1, EDN1, TGFB3, and F3) were ultimately identified. Their mRNA levels were significantly upregulated in both BA patients and mice. A total of 52 TFs and 13 microRNAs were identified. Among the predicted drugs, simvastatin may hold therapeutic potential.

Conclusion: This study identified six key ORDEGs (CCL2, CXCL8, SPP1, EDN1, TGFB3, and F3) to be important pathogenic factors and potential therapeutic targets in BA.