Development and validation of nomogram-based models for personalized survival assessment in pediatric hepatoblastoma patients.

Background: Hepatoblastoma (HB) is a prevalent form of liver cancer in pediatric patients, characterized by an embryonal malignant tumor. In the current study, a clinical prediction model was developed; that can effectively assess the likelihood of a patient's survival with HB. Methods: Data from the Surveillance, Epidemiology, and End Results (SEER) database for cases of HB between 2010 and 2019 were used in this retrospective research. Information on clinicopathologic characteristics, therapeutic interventions, and survival outcomes were included in the data. The HB patients were randomly assigned to the training or validation cohort in a 7:3 ratio. Using univariate and multivariate Cox proportional hazards regression models, the prognostic indicators for overall survival (OS) and cancer-specific survival (CSS) were identified. The area under the receiver operating characteristic curve (AUC-ROC), calibration plots, and concordance index (C-index) were used to evaluate the accuracy and calibration of these models. The clinical utility of the models was examined using decision curve analysis (DCA). Results: The multivariate Cox regression analysis revealed multiple autonomous prognostic determinants for the OS and CSS, including age, surgical interventions, and chemotherapy administration. Significantly, tumor size was found to be a strong predictor of OS. AUC values of 0.915, 0.846, and 0.847 for 1-, 3-, and 5-year OS, respectively, indicated that the nomogram-based models were highly accurate at predicting outcomes. Similarly, the AUC values for CSS were 0.871, 0.814, and 0.825. The C-index measurements, which quantify the discriminatory performance of the models, produced CSS values of 0.836 and OS values of 0.864. Furthermore, the calibration plots accurately represented the actual survival rates. Concurrently, the DCA had validated the clinical relevance of the nomogram-based models. Conclusions: The present study successfully developed and validated user-friendly nomogram-based models, allowing for accurate assessment of OS and CSS in pediatric HB patients. These tools enable personalized survival predictions, enhance risk stratification, and strengthen clinical decision-making for managing HB.

Efficacy of peritoneal drainage in very-low-birth-weight neonates with Bell's stage II necrotizing enterocolitis: A single-center retrospective study.

BACKGROUND: Currently, pediatric surgeons are challenged by a lack of consensus on the optimal management strategy (conservative or surgical) for children with Bell's stage II necrotizing enterocolitis (NEC). AIM: To evaluate the clinical efficacy of peritoneal drainage in very-low-birth-weight (VLBW) neonates with modified Bell's stage II NEC. METHODS: This was a retrospective analysis of 102 NEC (modified Bell's stage II) neonates born with VLBW who were treated at the Fujian Children's Hospital (Fujian Branch of Shanghai Children's Medical Center) between January 2017 and January 2020; these included 24 cases in the peritoneal drainage group, 36 cases in the exploratory laparotomy group, and 42 cases in the conservative treatment group. RESULTS: The general characteristics were comparable in the three groups (P > 0.05). Compared with conservative treatment, peritoneal drainage was associated with significantly shorter fasting time, abdominal distension relief time, fecal occult blood (OB) negative conversion time, and reduced hospital length of stay (HLOS) (P < 0.05 for all). Despite some advantages of peritoneal drainage over conservative treatment in terms of cure, conversion to laparotomy, intestinal perforation, intestinal stenosis, and abdominal abscess rates, the differences were not statistically significant (P > 0.05). Compared to exploratory laparotomy, the fecal OB negative conversion time was significantly shorter in the peritoneal drainage group (P < 0.05); similarly, the exploratory laparotomy group showed longer fasting time, abdominal distension relief time, HLOS, and higher complication rate compared to peritoneal drainage group, but the between-group differences were not statistically significant (P > 0.05). CONCLUSION: Peritoneal drainage, an easy-to-operate procedure, can improve the clinical symptoms of VLBW neonates with Bell's stage II NEC and help reduce the HLOS.

Anorectal malformation combined with Hirschsprung's disease: a case report.

Anorectal malformation (ARM) and Hirschsprungs disease (HSCR) are frequently associated with other congenital malformations, but rarely with one another. We describe the case of a child with intermediate anorectal malformation who underwent ARM correction. This child experienced recurrent postoperative symptoms, including intestinal obstruction, nutrition intolerance, and weight loss. The child was diagnosed with Hirschsprung's disease by colon barium contrast and pathological findings from a rectal biopsy, and subsequently underwent pull -through procedure after conservative treatment failed. After six months of postoperative follow-up, the patient still experiences occasional episodes of enteritis, but the symptoms are substantially less severe than they were before surgery, and the patient's weight is slowly increasing. We described a case of a child who had ARM combined with HSCR. Although the association between ARM and HSCR is uncommon, severe constipation or enteritis following complete correction of ARM in the absence of anal stricture should prompt consideration for HSCR. Before the second stage of ARM surgery, pay close attention to the barium enema examination, as an abnormal shape may indicate the presence of HSCR.

Comparison of Outcomes between Two Surgical Techniques for Patients with Intestinal Neuronal Dysplasia.

Objective: Hirschsprung disease (HSCR) is a serious congenital intestinal disease with a prevalence of 1/5000. HSCR remains one of the most severe congenital malformations of the abdominal organs in children that require complex reconstructive surgery. This study is aimed at investigating the clinical analysis of ileal Santulli stoma and ileal double-lumen stoma in children diagnosed with intestinal neuronal dysplasia (IND). Methods: Retrospective analysis was performed on the children who were admitted to our hospital for intestinal obstruction from January 2014 to January 2019, underwent fistula operation and fistula closure operation, and were diagnosed with IND. According to the different modes of fistula, the children were divided into ileal Santulli stoma group and ileal double-lumen stoma group. The body weight of the children in the two groups during the second stage of fistula closure operation was compared. The number of hospitalizations due to enteritis and dehydration during the two operations was compared. Results: A total of 23 cases (12 males and 11 females) were included in this study, including 10 cases in the Santulli group and 13 cases in the ileal double-lumen stoma group. There were no significant differences in baseline data and fistula location between the two groups. Compared with the ileal double-lumen stoma group, the Santulli stoma group had significantly higher weight of fistula precursor (P < 0.05), the interval between two operations was shorter (P < 0.05), there is less hospitalization for enteritis and dehydration during the two operations (P < 0.05), and there is less economic cost after fistula (P < 0.05). Conclusion: The clinical effect of ileum Santulli fistula is significantly better than double-lumen ileum fistula, which is not only beneficial to the growth and development of children after the first fistula but also can shorten the time of fistula closure, reduce the incidence of dehydration, and reduce the economic burden of family members. Therefore, it is worthy of clinical promotion and application.

Outcomes and comparations of pediatric surgery about choledochal cyst with robot-assisted procedures, laparoscopic procedures, and open procedures: A meta-analysis.

Background: Choledochal cysts (CC) are rare disorders characterized by congenital biliary dilatation of the intrahepatic or extrahepatic bile ducts and always relate to pancreaticobiliary maljunction. Robot-assisted surgery has been able to complete almost all pediatric endoscopic surgery nowadays. But evidence of the post-operative outcomes of robotic-assisted operation is limited, comparing with the laparoscopic operation and traditional open operation. The aim of this meta-analysis was to identify the advantages and deficiencies about robotic-assisted operation for CC. Methods: A meta-analysis of retrospective studies published in PUBMED, MEDLINE, Web of Science and China National Knowledge Infrastructure (CNKI). No date limit was used, with the last search on April 30, 2022. No publication restrictions or study design filters were applied. Results: Nine retrospective cohort studies with 1,395 patients [366 in the robotic-assisted operation group (RG), 532 in the laparoscopic operation group (LG) and 497 in the open operation group (OG)] were enrolled in our study. Subgroup analysis demonstrated the RG had significant longer operative time [standardized mean difference (SMD) = 1.59, 95% CI = (0.02, 3.16), P < 0.05], less blood loss [SMD = -1.52, 95% CI = (-2.71, -0.32), P < 0.05], shorter enteral feeding time [SMD = -0.83, 95% CI = (-1.22, -0.44), P < 0.001], shorter time to stay in the hospital [SMD = -0.81, 95% CI = (-1.23, -0.38), P < 0.001], fewer post-operative complications [Relative risk (RR) =1.09, 95% CI = (1.04, 1.13), P < 0.001] but higher expenses [SMD = 8.58, 95% CI = (5.27, 11.89), P < 0.001] than LG. While a significant older age [SMD = 0.46, 95% CI = (0.26, 0.66), P < 0.001], longer operative time [SMD = 3.96, 95% CI = (2.38, 5.55), P < 0.001] and shorter time to stay in the hospital [SMD = -0.93, 95% CI = (-1.62, -0.25), P < 0.05] than OG. Conclusions: Laparoscopic and robotic-assisted procedure are both safe and minimal invasive operational strategies. Robotic-assisted procedure may slowly surpass and has a trend to replace laparoscopy for its advantages. More experiences in robotic-assisted operation should be accumulated for the unexpected complexities, so as to be more stable in the younger age of children.

Laparoscopic-assisted Soave procedure for Hirschsprung disease: 10-year experience with 106 cases.

BACKGROUND: The purpose of this study was to summarize the clinical experience and 10 year follow-up results of laparoscopic assisted Soave procedure for the treatment of long-segment Hirschsprung disease (HD). METHODS: From January 2010 to February 2020, 106 children with long-segment HD participated in this study. The laparoscopic-assisted Soave procedure was performed for the treatment of long-segment HD. The follow-up time was two weeks, one month, and three months after the operation, and then every six months to one year. RESULTS: The operation was successful for all 106 children. All patients were discharged 5-7 days after the operation. The median time in surgery was 150 (100-190) minutes, and the median volume of bleeding was 6 (3-10) ml. The short-term postoperative daily defecation frequency was 4-11 times, 3-7 times within 6 months, and 2-3 times after 6-12 months. Postoperative complications included anastomotic leakage in two cases, perianal dermatitis in 13 cases, anastomotic stenosis in four cases, adhesive bowel obstruction in two cases, enterocolitis in 16 cases, soiling in 11 cases, and constipation recurrence in three cases. CONCLUSIONS: The laparoscopic-assisted Soave procedure is a safe and effective surgical method for treating long-segment HD, and it causes little trauma or bleeding and has a fast postoperative recovery. Yet some complications may occur. Preoperative diagnosis, intraoperative and postoperative standardized processing can reduce the postoperative complications.

Mini-laparoscopic pyeloplasty to treat UPJO in infants.

PURPOSE: The aim of this study was to investigate the safety and clinical efficacy of mini-laparoscopic pyeloplasty in treating ureteropelvic junction obstruction (UPJO) in infants. MATERIAL AND METHODS: We retrospectively analysed the clinical data of 66 infants with UPJO from January 2013 to August 2018 at our hospital. They were divided into the laparoscopic surgery group (group A) and the open surgery group (group B), depending on the surgical method. RESULTS: The bleeding volume, analgesia duration, postoperative hospitalization duration, and incision length in group A were significantly less than those in group B (p < .05). The incidence of incision dehiscence was 0% in group A and 11.7% in group B (p = .045). At the postoperative follow-up, the incidence of anastomotic stenosis was 6.2% in group A and 5.9% in group B (p = .719). The anteroposterior diameter and glomerular filtration rate were significantly improved at the one-year follow-up, but there was no significant difference between the groups (p > .05). CONCLUSIONS: Mini-laparoscopic pyeloplasty to treat UPJO in infants has the same early clinical efficacy and safety as open surgery, and this procedure has the advantages of fewer incisions, less pain, quicker recovery, and better cosmetic outcomes.

Exploration of the thoracoscopic treatment of esophageal atresia under high-frequency ventilation.

Objective: Explore the feasibility and safety of thoracoscopy in the treatment of esophageal atresia under high-frequency oscillatory ventilation (HFOV) mode. Methods: This was a single-center retrospective analysis. A total of 24 children were divided into the HFOV and the No-HFOV group. The demographic information, surgical results and relevant experience were analyzed. Results: All patients in the HFOV group underwent thoracoscopic esophageal atreplasty with a mean operation duration of 165.8 ± 33.9 min. Two patients had postoperative anastomotic leakage, which was cured after conservative treatment. One child had a recurrent tracheoesophageal fistula, which was closed after endoscopic cauterization. The mean postoperative mechanical ventilation time was 8.83 ± 8.02 days. There was no return of anastomotic leakage or r-TEF after oral feeding. Furthermore, there was no significant difference between the NO-HFOV and the HFOV groups except for the operation time where the HFOV group was shorter than that of the NO-HFOV group. Conclusion: Thoracoscopic esophageal atresia anastomosis under HFOV ventilation is feasible for patients with severe pulmonary infection, heart malformation, such as patent ductus arteriosus, ventricular septal defect, and poor anesthesia tolerance, but the long-term prognosis still needs further study in a large sample size.

Clinical Efficacy of an Indwelling Transanal Tube for the Prevention of Anastomotic Leakage After Hirschsprung's Disease: A Single Center Experience with Chinese Patients.

Background: The aim of this study was to investigate the clinical effectiveness of an indwelling transanal tube for the prevention of anastomotic leakage (AL) after a radical operation for Hirschsprung's disease (HD). Methods: We retrospectively analyzed the clinical data from 158 patients who had undergone laparoscopic-assisted Soave procedures for HD at our hospital from May 2015 to May 2019. Patients were divided into two groups depending upon whether the anal drainage tube was retained or not retained: an indwelling group (group A, n = 86) and a no-indwelling group (group B, n = 72). Results: All 158 children had a successful operation by a laparoscopic technique. There was no significant difference in the duration of the operation, the length of the incision, the amount of bleeding, or the postoperative hospitalization time between the two groups. Compared with the no-indwelling group, maintaining the transanal tube had significant advantages for preventing incidences of AL (P < .05). The 4-year follow-up showed that the incidence of postoperative enterocolitis with the indwelling transanal tube was significantly lower than in the group without the drainage tube (P < .05). Conclusions: The laparoscopic-assisted Soave procedure with an indwelling transanal tube is a safe and feasible method for the treatment of HD in children. This method can not only drain intestinal contents but also reduce the occurrence of AL.

Using Elevated Cholesterol Synthesis as a Prognostic Marker in Wilms' Tumor: A Bioinformatic Analysis.

BACKGROUND: Wilms tumor is the most common renal malignancy of children. Identifying factors that could predict the prognosis of patients with Wilms tumor is clinically meaningful. Many studies found tumors with elevated cholesterol synthesis that are featured with dismal prognosis. Even in some clinical trials, people with excessive dietary cholesterol intake and high plasma low-density lipoprotein levels are observed to have increased risk for cancer. However, the role of cholesterol biosynthesis in Wilms tumor has not yet been well clarified. METHODS: RNA sequencing transcriptome data and all corresponding clinicopathological information used in our study were downloaded from the TARGET database. High-throughput sequencing (Fragments Per Kilobase of transcript per Million fragments mapped) data sets of 130 tumor samples and 6 normal samples were obtained for further analysis. RESULTS: Wilms tumor samples with higher activity of cholesterol synthesis are characterized with worse overall survival (P < 0.05). In addition, Wilms tumor samples with mitigated activity of cholesterol synthesis are featured with better dendritic cell (DC) function and cytolytic activity (P < 0.05). Furthermore, we constructed a prognosis model based on differential expressed cholesterol synthesis-related genes (DECSG), which could predict the OS of patients with Wilms tumor accurately. KEGG and GO analysis of differential expressed genes between tumor samples with high and low cholesterol synthesis indicated that DECSGs are highly enriched in "mitosis nuclear division," "nuclear division," "chromosome segregation," "cell cycle," "Spliceosome," and "RNA transport." CONCLUSIONS: In conclusion, our study reported increased cholesterol synthesis in Wilms tumor predicts a worse prognosis and mitigated cytolytic activity, DC function, and MHC I signature in the tumor microenvironment. We also constructed a prognosis model for predicting the OS of patients with good accuracy, which is promising in clinical translation. Future studies should focus on the detailed mechanism that caused increasing cholesterol which promotes tumor progression and undermines patients' survival.

Clinical efficacy of transverse preputial island flap urethroplasty for single-stage correction of proximal hypospadias: a single-centre experience in Chinese patients.

BACKGROUND: This study was designed to summarize the clinical outcomes of transverse preputial island flap urethroplasty for single-stage correction of proximal hypospadias in our hospital. METHOD: This study retrospectively analysed the clinical data, including the preoperative general information, intraoperative and postoperative data, and follow-up data, of 155 children with proximal hypospadias who were admitted to our hospital from January 2009 to January 2019. RESULTS: During follow-up, a total of 92 postoperative complications occurred, and 41 patients underwent reoperation. There were 49 patients with urinary fistula, 26 patients with urethral stricture, 9 patients with urethral diverticulum and 8 patients with urinary tract infection. Regarding the family members' satisfaction with the cosmetic appearance of the penis, the satisfaction rate with the urinary meatus was 85.2%, the satisfaction rate with the glans appearance was 87.7%, the satisfaction rate with the the appearance of the foreskin of the penis was 92.3%, and the satisfaction rate with the overall penis shape was 89.0%. CONCLUSION: Proximal hypospadias is a serious condition that is often combined with severe chordee, and transverse preputial island flap urethroplasty for single-stage correction is an effective surgical procedure for treating this condition.

The Value of Thoracic Lavage in the Treatment of Anastomotic Leakage After Surgery for Type III Esophageal Atresia.

BACKGROUND The aim of this study was to define whether the addition of thoracic lavage to chest drainage was more efficient than the use of chest drainage alone in the treatment for anastomotic leaks in type III esophageal atresia. MATERIAL AND METHODS The clinical data of 42 patients with anastomotic leakage treated with the addition of thoracic lavage from January 2012 to March 2019 in our hospital were analyzed retrospectively. The clinical data of 50 patients with anastomotic leakage treated without thoracic lavage from March 1999 to December 2011 in our hospital were selected as controls. RESULTS The duration of fistula healing, mechanical ventilation, hospitalization in intensive care unit, and gastric tube intubation in the thoracic lavage group were significantly shorter than those in the non-lavage group. The cost of hospitalization and the incidence of severe pneumonia were significantly lower in the thoracic lavage group than in the non-lavage group. The diameter of the anastomotic opening after anastomotic fistula healing was wider in the thoracic lavage group than in the non-lavage group. CONCLUSIONS The technique of thoracic lavage is simple, economical and convenient and can effectively promote the healing of anastomotic fistulas, accelerate postoperative recovery in children and reduce the cost of treatment.

Clinical Analysis of Azygos Vein Preservation Under Thoracoscope in the Operation of Type III Esophageal Atresia.

Purpose: The aim of this study was to investigate the clinical effectiveness of azygos vein preservation when using a thoracoscope in the operation for type III esophageal atresia for children. Materials and Methods: We retrospectively analyzed the clinical data of 34 patients (group B) who underwent the thoracoscopic operation of type III esophageal atresia with azygos vein preservation from January 2017 to April 2018. The clinical data of 36 patients (group A) who underwent the thoracoscopic operation of type III esophageal atresia with azygos vein mutilation from April 2015 to December 2016 were selected as a control group. Results: All patients were successfully operated on. There was no significant difference in the operation time, amount of bleeding, mechanical ventilation time, and intensive care time between the azygos vein mutilation group and azygos vein preservation group. Compared with the operation severing the azygos vein, preservation of azygos vein has obvious advantages in postoperative hospitalization time, the incidence of anastomotic leakage (P < .05). The follow-up results for 1 year after the operation showed that the incidence of anastomotic stricture with preservation of azygos vein was significantly lower than that with azygos vein transection (P < .05). Conclusions: The thoracoscopic operation of type III esophageal atresia with azygos vein preservation can not only improve the edema of esophageal tissue around the esophagus, but also reduce the occurrence of esophageal anastomotic leakage and accelerate the postoperative recovery of children, and it has the same safety as the operation with azygos vein mutilation.