Scoring system for diagnosis and pretreatment risk assessment of neuroblastoma using urinary biomarker combinations.

The urinary catecholamine metabolites, homovanillic acid (HVA) and vanillylmandelic acid (VMA), are used for the adjunctive diagnosis of neuroblastomas. We aimed to develop a scoring system for the diagnosis and pretreatment risk assessment of neuroblastoma, incorporating age and other urinary catecholamine metabolite combinations. Urine samples from 227 controls (227 samples) and 68 patients with neuroblastoma (228 samples) were evaluated. First, the catecholamine metabolites vanillactic acid (VLA) and 3-methoxytyramine sulfate (MTS) were identified as urinary marker candidates through comprehensive analysis using liquid chromatography-mass spectrometry. The concentrations of these marker candidates and conventional markers were then compared among controls, patients, and numerous risk groups to develop a scoring system. Participants were classified into four groups: control, low risk, intermediate risk, and high risk, and the proportional odds model was fitted using the L2-penalized maximum likelihood method, incorporating age on a monthly scale for adjustment. This scoring model using the novel urine catecholamine metabolite combinations, VLA and MTS, had greater area under the curve values than the model using HVA and VMA for diagnosis (0.978 vs. 0.964), pretreatment risk assessment (low and intermediate risk vs. high risk: 0.866 vs. 0.724; low risk vs. intermediate and high risk: 0.871 vs. 0.680), and prognostic factors (MYCN status: 0.741 vs. 0.369, histology: 0.932 vs. 0.747). The new system also had greater accuracy in detecting missing high-risk neuroblastomas, and in predicting the pretreatment risk at the time of screening. The new scoring system employing VLA and MTS has the potential to replace the conventional adjunctive diagnostic method using HVA and VMA.

A new criterion including the aspartate aminotransferase-to-platelet ratio index and liver and spleen stiffness to rule out varices needing treatment in children with biliary atresia: Modification of the Baveno VII criteria.

AIMS: Biliary atresia (BA) is a congestive biliary disease that develops in the neonatal period or early infancy. It may present with portal hypertension and varices needing treatment (VNT) even after successful Kasai portoenterostomy. This study aimed to stratify the risk of VNT in children and adolescents with BA. METHODS: In this prospective cross-sectional study, we measured liver stiffness (LS) and spleen stiffness (SS) by two-dimensional shear wave elastography and checked for VNT endoscopically in 53 patients with BA who attended for follow-up between July 2018 and September 2022. Varices needing treatment were defined as large esophageal varices, esophageal varices of any size with red color signs, and/or gastric varices along the cardia. RESULTS: Twenty-five patients (aged 0-18 years) had VNT. Eighteen patients met the Baveno VI criteria (LS <20 kPa; platelet count >150 000/L) and were deemed to be at low risk of VNT (spared endoscopies) while three had missed VNT (16.7%). Applying the Baveno VII criteria, which combines the SS cut-off value of 40 kPa with the Baveno VI criteria, resulted in five missed VNTs among 22 spared endoscopies (22.7%). A modification of the Baveno VII criteria using the aspartate aminotransferase-to-platelet ratio index (APRI) instead of the platelet count with cut-off values of 25 kPa, 30 kPa, and 1.04 for LS, SS, and APRI, respectively, missed only one VNT (5.0%) among 20 spared endoscopies. CONCLUSIONS: A novel diagnostic criterion that combines LS, SS, and APRI reduced the risk of missing VNT to 5% in children and adolescents with BA.

A systematic review of Sandifer syndrome in children with severe gastroesophageal reflux.

PURPOSE: Sandifer syndrome (SS), which combines gastroesophageal reflux (GER) and a neurological or psychiatric disorder, is an uncommon condition that often takes a long time to diagnosis. We aimed to systematically review available papers regarding SS. METHODS: After presenting our two cases of SS, we systematically reviewed articles published in MEDILINE/PubMed, Cochrane Library, and Web of Science. RESULTS: The meta-analysis included 54 reported cases and 2 of our own cases. Our results showed that all cases achieved symptom improvement with appropriate treatment for GER. Notably, 19 of the 56 cases exhibited anatomical anomalies, such as hiatal hernia and malrotation. Significantly more patients with than without anatomical anomalies required surgery (p < 0.001). However, 23 of the 29 patients without anatomical anomalies (79%) achieved symptom improvement without surgery. Patients who did not undergo surgery had a median (interquartile range) duration to symptom resolution of 1 (1-1) month. CONCLUSION: The primary care providers should keep SS in the differential diagnosis of patients presenting with abnormal posturing and no apparent neuromuscular disorders. Fundoplication may be effective especially for patients with anatomical anomalies or those whose symptoms do not improve after more than 1 month with nonsurgical treatment.

Thoracoscopic primary repair is useful for esophageal atresia with tracheoesophageal fistula in neonates with low body weight.

PURPOSE: The surgical indication of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula is under debate. The current study aimed to investigate the outcome of thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula in patients weighing < 2000 g and those who underwent emergency surgery at the age of 0 day. METHODS: The surgical outcomes were compared between patients weighing < 2000 g and those weighing > 2000 g at surgery and between patients who underwent surgery at the age of 0 day and those who underwent surgery at age ≥ 1 day. RESULTS: In total, 43 patients underwent thoracoscopic primary repair for esophageal atresia with tracheoesophageal fistula. The surgical outcomes according to body weight were similar. Patients who underwent surgery at the age of 0 day were more likely to develop anastomotic leakage than those who underwent surgery at the age of ≥ 1 day (2 vs. 0 case, p = 0.02). Anastomotic leakage was treated with conservative therapy. CONCLUSION: Thoracoscopic primary repair is safe and useful for esophageal atresia with tracheoesophageal fistula even in newborns weighing < 2000 g. However, emergency surgery at the age of 0 day should be cautiously performed due to the risk of anastomotic leakage.

Bile lakes in patients with biliary atresia who presented with jaundice-free native liver survival indicating the risk of subsequent liver transplantation due to various factors.

PURPOSE: The prognostic factors of subsequent liver transplantation (LT) in patients with biliary atresia (BA) who presented with jaundice-free native liver survival were investigated. METHODS: This study retrospectively reviewed patients who underwent portoenterostomy (PE) for BA. Patients with jaundice-free native liver survival at 1 year postoperatively were divided into the autologous liver survivor and liver transplant recipient groups. Peri- and postoperative data were compared between the two groups. RESULTS: Among 97 patients with BA, 29 who received LT within 1 year after PE were excluded from the analysis. Further, 48 patients currently living with native liver and 20 who received LT after 1 year postoperatively were compared. Bile lake (BL) was the strongest risk factor of LT. The risk score was 2.38 ∗ B L s c o r e + 0.00466 ∗ T B A , and the area under the receiver operating characteristic curve was 0.83. Patients with BL and those without significantly differed in terms of the native liver survival rate. Patients with BL who presented with not only cholangitis but also gastrointestinal hemorrhage and hepatopulmonary syndrome received LT. CONCLUSION: BL can cause different pathologies. Moreover, it is an evident risk factor of subsequent LT in patients with BA who are living with native liver at 1 year after PE.

The presence of high-risk varices after sclerotherapy in biliary atresia.

BACKGROUND: Esophagogastric varices (EGVs) may develop as a result of portal hypertension in children with biliary atresia (BA). Although endoscopic injection sclerotherapy (EIS) with ethanolamine oleate (EO) is reported useful for children, risk factors associated with the presence of high-risk EGVs after treatment remain unknown. METHODS: The subjects were BA patients under 15 years of age who underwent EO-EIS. We retrospectively reviewed a total of 28 treatment sessions of EGVs with red signs and those larger than F2, which were considered to be at high risk of bleeding. Survival analysis was performed for the presence of high-risk EGVs at the time of follow-up endoscopy as the occurrence of an event. RESULTS: Univariate analysis showed a significantly increased risk of the presence of high-risk EGVs post-EO-EIS in patients with increased liver stiffness (LS) and Mac-2 binding protein glycan isomer (M2BPGi), with hazard ratios of 1.48 and 1.15, respectively. The median presence-free period was significantly shorter in the LS ≥ 2.8 m/s patients than in those with LS <2.8 m/s (189 vs. 266 days). Similarly, the median presence-free period was significantly shorter in patients with M2BPGi ≥ 4.0 than in those with M2BPGi < 4.0 (182 vs. 203 days). The results of multivariate analysis revealed that the risk of the presence of high-risk EGVs was significantly higher only in the high-LS group, with a hazard ratio of 2.76. CONCLUSIONS: Increased LS is associated with risk of the presence of high-risk EGVs following EO-EIS in children with BA.

Anastomotic time was associated with postoperative complications: a cumulative sum analysis of thoracoscopic repair of tracheoesophageal fistula in a single surgeon's experience.

PURPOSE: This study aimed to evaluate the learning curve of thoracoscopic repair of tracheoesophageal fistula (TEF) by a single surgeon using a cumulative sum (CUSUM) analysis. METHODS: Prospective clinical data of consecutive Gross type-C TEF repairs performed by a pediatric surgeon from 2010 to 2020 were recorded. CUSUM charts for anastomosis and operating times were generated. The learning curves were compared with the effect of accumulation based on case experience. RESULTS: For 33 consecutive cases, the mean operative and anastomosis times were 139 ± 39 min and 3137 ± 1110 s, respectively. Significant transitions beyond the learning phase for total operating and anastomosis times were observed at cases 13 and 17. Both the total operating time and anastomosis time were significantly faster in the proficiency improvement phase than in the initial learning phase. Postoperative complications significantly decreased after the initial anastomosis learning phase but not after the initial total operating learning phase. CONCLUSIONS: Thoracoscopic repair of TEF is considered safe and feasible after 13 cases, where the surgeon can improve their proficiency with the total operation procedure, and 17 cases, which will enable the surgeon to achieve proficiency in anastomosis. Postoperative complications significantly decreased after gaining familiarity with the anastomosis procedure through the learning phase.

Safe thoracoscopic repair of recurrent congenital diaphragmatic hernia after initial open abdominal repair.

PURPOSE: The optimal surgical approach for recurrent congenital diaphragmatic hernia (CDH) remains controversial. We compared the surgical outcomes of a thoracoscopic approach versus an open abdominal approach for recurrent CDH after initial abdominal open repair. METHOD: The subjects of this comparative study were patients who underwent open abdominal or thoracoscopic surgery for recurrent CDH following an initial open abdominal repair. RESULTS: Among 166 patients with Bochdalek-type CDH, 15 underwent reoperation for recurrent CDH following an open abdominal repair. Seven patients underwent open abdominal surgery (group O) and eight underwent thoracoscopic surgery (group T). The operative duration was similar for the two groups, with less blood loss (17.2 ml/kg vs. 1 ml/kg, P = 0.001) and fewer intraoperative complications in the T group (n = 6 vs. n = 0 cases, P = 0.001). There was no significant difference in the number of postoperative complications (n = 1 vs. n = 1, P = 1.0) or in the number of patients with a second CDH recurrence (n = 2 vs. n = 1, P = 0.569) between the two groups. CONCLUSION: Thoracoscopic surgery is preferable to the open surgical approach for recurrent CDH following an initial abdominal open repair.

Preoperative management comprising tube irrigation using a trans-anal indwelling tube for infants with hirschsprung disease can allow single-stage radical surgery.

BACKGROUND: Preoperative management of Hirschsprung's disease (HD) is currently being conducted with the goal of performing single-stage radical surgery without ileostomy. METHODS: We retrospectively reviewed HD cases between 2013 and 2022, as well as their outcomes related to preoperative management. RESULTS: Thirty-nine patients with HD were included in this study, including short-segment HD (30 cases), long-segment HD (4 cases), and total colonic aganglionosis (5 cases). Among these 39 patients, 95% (37 of 39 patients) underwent single-stage radical surgery after management with glycerin enema use (n = 13), irrigation with tube insertion each time irrigation was performed (n = 13), and irrigation using a tube placed in the bowel (n = 11). CONCLUSIONS: Preoperative management of patients with HD allowed for single-stage surgery of long-segment HD and total colonic aganglionosis. Cases that could be managed without performing an emergency enterostomy during the neonatal period were managed with irrigation until radical surgery was performed.

Robotic versus laparoscopic radical surgery for pediatric congenital biliary dilatation: a comparison of surgical outcomes of a single surgeon's initial experience.

PURPOSE: This study aimed to clarify the validity of robot-assisted surgery (RAS) for pediatric patients with congenital biliary dilatation (CBD). METHODS: We retrospectively compared RAS and laparoscopic surgery (LS) for pediatric CBD performed by the same certified surgeon between 2016 and 2022. RESULTS: We included 6 RAS and 12 LS cases in this study. One case of RAS with laparotomy was excluded from the analysis. The patients in the two groups had comparable ages and body weights. The median surgery duration, the suture time per stitch, and the time to drain removal were 385 min, 145 s, and 5 days in the RAS group and 370 min (p = 0.28), 177 s (p = 0.03), and 6 days (p = 0.03) in the LS group, respectively. The time to create the Roux-en-Y limb was significantly longer in the RAS group. Postoperative complications occurred in one RAS case and in four LS cases. CONCLUSIONS: Less anastomotic time per stitch and less time to drain removal suggest that RAS may contribute to accurate suturing and fine intra-pancreatic bile duct dissection. In addition, RAS requiring large movements of forceps in a large surgical field, such as Roux-en-Y creation, is inferior to LS.

Single-cell RNA sequencing of intestinal immune cells in neonatal necrotizing enterocolitis.

PURPOSE: Necrotizing enterocolitis (NEC) causes fatal intestinal necrosis in neonates, but its etiology is unknown. We analyzed the intestinal immune response to NEC. METHODS: Using single-cell RNA sequencing (scRNA-seq), we analyzed the gene expression profiles of intestinal immune cells from four neonates with intestinal perforation (two with NEC and two without NEC). Target mononuclear cells were extracted from the lamina propria of the resected intestines. RESULTS: In all four cases, major immune cells, such as T cells (15.1-47.7%), B cells (3.1-19.0%), monocytes (16.5-31.2%), macrophages (1.6-17.4%), dendritic cells (2.4-12.2%), and natural killer cells (7.5-12.8%), were present in similar proportions to those in the neonatal cord blood. Gene set enrichment analysis showed that the MTOR, TNF-α, and MYC signaling pathways were enriched in T cells of the NEC patients, suggesting upregulated immune responses related to inflammation and cell proliferation. In addition, all four cases exhibited a bias toward cell-mediated inflammation, based on the predominance of T helper 1 cells. CONCLUSION: Intestinal immunity in NEC subjects exhibited stronger inflammatory responses compared to non-NEC subjects. Further scRNA-seq and cellular analysis may improve our understanding of the pathogenesis of NEC.

Serum matrix metalloproteinase-7 in biliary atresia: A Japanese multicenter study.

BACKGROUND: Biliary atresia (BA) is among the commonest indications for liver transplantation (LT) in children. We examined whether serum matrix metalloproteinase-7 (MMP-7) is useful for diagnosis of BA in Japanese infants, and whether serum MMP-7 concentrations before and after Kasai portoenterostomy (KP) predicted LT within a year. METHODS: Subjects under 6 months old at eight pediatric centers in Japan were enrolled retrospectively, including patients with cholestasis and normal controls (NC) without liver disease. Patients with cholestasis were divided into groups representing BA versus cholestasis from other causes (non-BA). Serum samples were collected from patients with BA at diagnosis and 1 and 4 weeks after KP, as well as from non-BA and NC. RESULTS: Serum MMP-7 concentrations were significantly higher in BA at diagnosis (median, 89.1 ng/ml) than in non-BA (11.0; p < 0.001) or NC (10.3; p < 0.001). Receiver operating characteristic (ROC) analysis of MMP-7 for BA versus non-BA yielded an area under the ROC curve of 0.99 (95% confidence interval, 0.96-1.00). An optimal cut-off value of 18.6 ng/ml for serum MMP-7 in diagnosing BA demonstrated sensitivity and specificity of 100% and 90%, respectively. Serum MMP-7 before and 1 week and 4 weeks after KP did not differ significantly between BA requiring only KP and BA requiring LT after KP. CONCLUSION: Serum MMP-7 is a useful marker for diagnosis of BA in Japanese infants, but it could not predict LT within a year.

Laparoscopic definitive surgery for congenital biliary dilatation with aggressive hilar bile ductoplasty and complete resection of the intrapancreatic bile duct in pediatric patients is safe and effective, comparable to open surgery.

BACKGROUND: After surgery for congenital biliary dilatation (CBD), hilar and hepatic bile duct stenosis and intrapancreatic bile duct remnants can cause many postoperative complications. We investigated the efficacy of laparoscopic surgery with aggressive bile ductoplasty and complete excision of the intrapancreatic bile duct in CBD patients compared to open surgery. METHODS: Pediatric patients who underwent surgery for CBD at our institution between 2006 and 2020 were divided into two groups: laparoscopic surgery (Lap group) and open surgery (Op group). Surgical outcomes were compared between the two groups. RESULTS: We enrolled 160 patients (85 in the Lap group and 75 in the Op group). Hilar bile ductoplasty was performed in 52 (61%) patients from the Lap group and 40 (53%) patients from the Op group. The operative time was significantly longer, the amount of blood loss was significantly lower, and the length of hospital stay was significantly shorter in the Lap group than in the Op group. Postoperative early and late complications occurred at a similarly low rate in both groups. When the surgical outcomes were compared between the group who underwent laparoscopic bile ductoplasty and the group that did not, postoperative early and late complications occurred similarly in both groups. CONCLUSIONS: Laparoscopic surgery for CBD with aggressive hilar bile ductoplasty and complete excision of the intrapancreatic bile duct is safe and effective, comparable to open surgery. However, it is necessary to verify whether long-term occurrence of hepatolithiasis similar to open definitive surgery can be obtained in the future.

Evaluation of minimally invasive surgical skills training: comparing a neonatal esophageal atresia/tracheoesophageal fistula model with a dry box.

BACKGROUND: Pediatric surgeons require highly advanced minimally invasive surgical skills to perform rare and complex surgeries in a very vulnerable population. We developed a neonatal esophageal atresia (EA) model to improve thoracoscopic surgical skills. This study aimed to evaluate the concurrent validity of the model by undertaking pre- and post-training skills assessments in two groups of students with no prior experience performing minimally invasive surgery, using the EA model and a dry box (DB). METHODS: A pilot study was performed. The participants were randomly divided into two groups: one trained using the DB and one trained using the EA model. Both groups practiced a minimally invasive surgical suture task. The task completion time, 29-point checklist score, modified suturing error sheet score, and three-dimensional forceps movement in both groups were compared pre-and post-training by video analysis. RESULTS: The EA model task was significantly more difficult than that of the DB. Both groups showed significant improvement in the task time, 29-point checklist score, and modified suturing error sheet score; however, the EA model training was more efficient in improving each error item. Regarding forceps movement, the EA model training significantly decreased wasted motion, whereas the DB was limited in this regard. CONCLUSIONS: Short-term training on the EA model, which was more technically demanding than the DB, decreased technical error and wasted motion, and allowed learners to acquire surgical skills more efficiently than training with the DB model. These facts revealed the concurrent validity of the EA model.

Single-incision laparoscopic percutaneous extraperitoneal closure (SILPEC) for inguinal hernia with prolapsed ovary.

BACKGROUND: Most studies reporting the outcomes of laparoscopic ovarian prolapsed hernia operations with large sample sizes are based on intracorporeal closure, while studies on extraperitoneal closure have limited sample sizes. We proactively used the single-incision laparoscopic percutaneous extraperitoneal closure (SILPEC) technique and obtained favorable outcomes, which we report in this paper. METHODS: We retrospectively reviewed patients who had undergone laparoscopic surgery for inguinal hernia at our institution. They were retrospectively classified into two groups based on the pre- or intraoperative diagnosis of hernia with a prolapsed ovary, namely the prolapse group and the non-prolapse group, respectively. The data were statistically analyzed and p < 0.05 was considered statistically significant. RESULTS: A total of 771 subjects underwent SILPEC during the study period, including 400 girls. Among them, 63 girls were diagnosed with an ovarian prolapsed hernia. SILPEC was successfully performed through a single port in all cases, with a single exception, in whom the forceps was inserted directly through the right lower quadrant to pull up the ovary. The duration of surgery in the prolapse group was not higher than that in the non-prolapse group. During the SILPEC surgery, the ovaries were successfully reverted into the abdominal cavity by external compression of the inguinal area alone in 38 of the 63 patients. In the remaining 25 cases, the ovaries were reverted into the abdominal cavity by external compression of the inguinal area and traction of the round ligament with forceps. None of these cases failed to return to the ovaries. CONCLUSION: Our study results indicate that SILPEC may be performed safely for the treatment of ovarian prolapsed inguinal hernia. Since the ovary and fallopian tube are close to the internal inguinal ring due to the short round ligament, the procedure requires careful suturing with traction of the round ligament.

Utility of endoscopic retrograde cholangiopancreatography in management of pediatric pancreaticobiliary disease.

BACKGROUND: The purpose of this study was to evaluate the utility of endoscopic retrograde cholangiopancreatography (ERCP) in pediatric patients with pancreaticobiliary diseases. METHODS: A retrospective review was performed on patients who underwent ERCP for the treatment of biliary tract disease and detailed examination of pancreatitis at our institution from January 1999 to December 2020. RESULTS: ERCP was performed for congenital biliary dilatation (CBD) (n = 42), choledocholithiasis (n = 9), common bile duct stenosis (n = 1), and several types of pancreatitis (n = 13). The only severe complication of ERCP was common bile duct injury. Three (5.8%) of 52 biliary diseases failed to be treated by ERCP. All patients with pancreatic disease were correctly diagnosed and treated. CONCLUSIONS: Endoscopic biliary drainage with a temporary stent was adequate for symptomatic relief in CBD. Stenting of the pancreatic duct was useful for improving the angulation and drainage of the pancreatic duct. ERCP was useful for understanding the anatomy of the pancreatic duct and revealing potential treatments. Therefore, ERCP and transendoscopic therapy are sufficiently feasible in pediatric patients and should be actively introduced for the investigation and treatment of pancreaticobiliary diseases.

Thoracoscopic posterior tracheopexy during primary esophageal atresia repair ameliorate tracheomalacia in neonates: a single-center retrospective comparative cohort study.

BACKGROUND: Esophageal atresia (EA) is often associated with tracheomalacia (TM). The severity of TM symptoms varies widely, with serious cases requiring prolonged respiratory support and surgical treatment. Although we performed thoracoscopic posterior tracheopexy (TPT) during primary EA repair to prevent or reduce the symptoms of TM, few studies have investigated the safety and effectiveness of TPT during primary EA repair. Therefore, this study aimed to evaluate the safety and efficacy of TPT in neonates. METHODS: We retrospectively reviewed the records of all patients diagnosed with TM who underwent primary thoracoscopic EA repair between 2013 and 2020 at the Nagoya University Hospital. Patients were divided into two groups: TPT (TPT group) and without TPT (control group). TPT has been performed in all patients with EA complicated by TM since 2020. We compared patient backgrounds, surgical outcomes, postoperative complications, and treatment efficacy. RESULTS: Of the 22 patients reviewed, eight were in the TPT group and 14 were in the control group. There were no statistically significant differences in the surgical outcomes between the groups (operation time: p = 0.31; blood loss: p = 0.83; time to extubation: p = 0.30; time to start enteral feeding: p = 0.19; time to start oral feeding: p = 0.43). Conversion to open thoracotomy was not performed in any case. The median operative time required for posterior tracheopexy was 10 (8-15) min. There were no statistically significant differences in postoperative complications between the groups (chylothorax: p = 0.36; leakage: p = 1.00; stricture: p = 0.53). The respiratory dependence rate 30 days postoperative (2 [25%] vs. 11 [79%], p = 0.03) and the ratio of the lateral and anterior-posterior diameter of the trachea (LAR) were significantly lower in the TPT group (1.83 [1.66-2.78] vs. 3.59 [1.80-7.70], p = 0.01). CONCLUSIONS: TPT during primary EA repair for treatment of TM significantly lowered respiratory dependence rate at 30 days postoperative without increasing the risk of postoperative complications. This study suggested that TPT could improve TM associated with EA.

Patients with gastric volvulus recurrence have high incidence of wandering spleen requiring laparoscopic gastropexy and splenopexy.

PURPOSE: Though gastric volvulus in neonates and infants resolves by conservative therapy and aging, some cases require surgical intervention. This study aimed to review the cases of gastric volvulus requiring surgical intervention and evaluate their characteristics. METHODS: We retrospectively reviewed gastric volvulus cases requiring surgical intervention. Surgical indication was persistent acute gastric volvulus and repeated hospitalization for gastric volvulus. We evaluated the characteristics of those cases requiring surgical intervention and the surgical results of laparoscopic gastropexy. RESULTS: The median age of patients included was 4 years (range: 1-6 years). All eight cases of gastric volvulus requiring sugery had congenital spleen diseases. Six of the eight cases suffered from a wandering spleen, while two cases presented with situs inversus with asplenia. Both splenopexy (preperitoneal distension balloon [PDB] or blunt separaion methods) and gastropexy were performed in cases with wandering spleen. No postoperative complications were reported in any of the eight cases, except the recurrence of gastric volvulus due to suture shedding in one case. CONCLUSION: Laparoscopic gastropexy for gastric volvulus and splenopexy for cases concomitant with wandering spleen were found to be effective surgical approaches. Both PDB and blunt separation methods for making extraperitoneal pockets for the spleen were employed successfully.

Adding a spur valve to laparoscopic portoenterostomy for patients with biliary atresia can achieve a high jaundice clearance rate and lower the number of episodes of cholangitis.

PURPOSE: This study aimed to evaluate the efficacy of adding a spur valve to laparoscopic portoenterostomy for patients with biliary atresia. METHODS: We retrospectively reviewed the records of all patients diagnosed with biliary atresia (BA) who underwent laparoscopic portoenterostomy (Lap-PE) between 2013 and 2021. The patients were divided into two groups: Lap-PE with a spur valve (spur group) and without it (control group). Perioperative management was the same in both groups. We compared patient backgrounds and clinical outcomes, including jaundice clearance and the number of postoperative cholangitis episodes. RESULTS: Of 63 patients reviewed, 16 received a spur valve. There were no statistically significant differences in the patient backgrounds between the groups. All patients in the spur group achieved jaundice clearance. The number of postoperative cholangitis episodes one year after surgery was significantly lower in the spur group than in the control group (1 [0-3] vs. 3 [0-9], p = 0.04). The jaundice-free survival rate with the native liver at one year after surgery was significantly higher in the spur group (100% vs. 53%, p = 0.01). CONCLUSIONS: Adding a spur valve during Lap-PE significantly lowered the number of cholangitis episodes 1 year after surgery.

Biochemical markers to predict the development of gastrointestinal bleeding and esophageal varices after portoenterostomy in biliary atresia.

PURPOSE: Gastrointestinal bleeding (GIB) due to esophageal varices (EV) is one of the factors that negatively impact native liver survival of patients with biliary atresia (BA). Gastrointestinal fibroscopy (GIF) is usually used to determine the presence of EVs; however, it requires general anesthesia. The aim of this study is to search for markers in blood tests obtained during routine check-ups that can predict the development of GIB. METHODS: Data of patients with BA who underwent portoenterostomy at our hospital from 2014 to 2020 were retrospectively reviewed. The patients' data were assigned to three groups according to specific time points: Group B, which included data at GIB; Group NB-T, which included data at GIF and EV treatment; and Group NB-NT, which included data at GIF without treatment. The data in Group B were compared to those of other groups. RESULTS: In our study, GIB occurred in 11 patients, and 12 cases and 8 cases were classified into Groups NB-NT and NB-T, respectively. Compared with the other groups, only ChE and M2BPGi in Group B showed statistically significant differences. CONCLUSIONS: ChE and M2BPGi are useful for predicting GIB.