Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.jpedsurg.2024.06.023

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Language：English   Publisher：Pediatrics International  

Background: In Japan, the administration of extra-hypotonic fluids (approximately 35 mmol/L of sodium) as maintenance fluid is still the mainstream practice, and there have been relatively few reports on maintenance intravenous fluid therapy. Since 2014, our institution has administered maintenance fluids containing 83 mmol/L of Na (HALF) after diuresis is achieved post-Kasai portoenterostomy for biliary atresia (BA). We investigated whether hyponatremia is induced by the administration of half saline during postoperative maintenance of infants with BA. Methods: Patients who underwent surgery for BA at our institution were included. The serum sodium concentration ([Na]) before and after surgery and the incidence of hyponatremia were compared between patients administered fluids with [Na] of 35 mmol/L (exHYPO group, 59 patients) and those with [Na] of 83 mmol/L (HALF group, 20 patients). Results: The median age of patients was 59 days. There were no significant differences in the background or preoperative [Na] between groups. There was a significant decrease in [Na] on postoperative day 3 (POD3) in the exHYPO group compared with the preoperative [Na] value in the exHYPO group and the [Na] value on POD3 in the HALF group. There were no significant differences in [Na] before and after surgery in the HALF group. The odds ratio was 21.0, and the 95% confidence interval was 3.31–130, indicating that the exHYPO group had an increased risk of hyponatremia. Conclusion: Administration of half saline as maintenance fluid can maintain [Na] levels during postoperative care of infants with BA.

DOI： 10.1111/ped.70016

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Language：English   Publisher：Journal of Hepato-Biliary-Pancreatic Sciences  

Background/Purpose: We evaluated the minimally invasive surgery for congenital biliary dilatation (CBD) in adults and children and analyzed the surgical outcomes, especially in children aged <6 years. Methods: Characteristics and surgical outcomes of patients with CBD who underwent minimally invasive surgery at our hospital between 2013 and 2023 were retrospectively reviewed. Results: Overall, 129 patients (89 children aged <6 years, 9 children aged between 6 and 18 years, and 21 adults) were included in this study. Children exhibited more protein plug presence and abnormal biochemical data than adults. Incidence of postoperative pancreatic fistulas was highest in the adult group (3.4%, 11%, and 33%, respectively, p <.01). In children aged <6 years, postoperative bile leakage and pancreatic fistulas occurred in 9.0% and 3.4% of patients, respectively. Multivariate logistic regression analysis revealed that the Todani IVA was associated with a decrease in postoperative bile leakage (odds ratio: −1.7; 95% confidence interval: (−3.3)–(−0.22), p =.03). Conclusion: Adults with CBD required prolonged operative times and had more short-term complications than children with CBD. In children aged <6 years, minimally invasive surgery for CBD can be safely performed; however, a small diameter of the bile duct may be associated with bile leakage.

DOI： 10.1002/jhbp.12069

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Language：English   Publisher：Pediatric Surgery International  

Purpose: Tracheomalacia (TM) is commonly associated with esophageal atresia (EA) and compression by the brachiocephalic artery is a factor for TM. Previous research has focused on the lateral-to-anteroposterior tracheal diameter ratio (LAR). This study aimed to assess the LAR and postoperative outcomes of EA patients. Methods: Patients undergoing thoracoscopic repair for EA between March 2020 and October 2023 were enrolled. Posterior tracheopexy (PT) was performed during thoracoscopic repair of EA on patients with bronchoscopy-confirmed TM; clinical courses and LAR were retrospectively analyzed. Results: Overall, 18 patients were enrolled; 14 patients underwent PT. Their median preoperative and postoperative LARs were 2.26 and 1.50, respectively; this difference was statistically significant. Four patients without TM did not undergo PT and their median LAR was 1.59. Median LAR for patients without PT was lower than that of preoperative patients with PT and no statistical differences were observed from that of postoperative patients with PT. The patients whose LAR improved with PT did not require further surgical intervention for TM. One patient who had a postoperative LAR of 2.25 required external tracheal stenting. Conclusion: LAR is a useful index for determining the severity of TM associated with EA. LAR can reflects the efficacy of PT.

DOI： 10.1007/s00383-024-05877-9

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Language：English   Publisher：Asian Journal of Endoscopic Surgery  

The Senhance® robotic system (Senhance [Asensus Surgical Inc., Naderhan, NC, USA]) is a new surgical assistive robot following the da Vinci Surgical System that has been demonstrated to be safe and efficacious. Herein, we report the first case series of pediatric pelvic surgery using Senhance. Two anorectoplasties and one rectal pull-through coloanal anastomosis for rectal stenosis were performed in three children (5–9 months, 7–9 kg) using a 10-mm three-dimensional (3D) 4K camera and 3 and 5 mm forceps operated with Senhance. None of the patients had intraoperative complications or a good postoperative course. Pediatric pelvic surgery with Senhance could be performed precisely and safely with a small body cavity. With its beautiful 3D images, motion of forceps with reduced tremor, and availability of 3-mm forceps, Senhance may be better suited for children compared with other models.

DOI： 10.1111/ases.13379

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Language：English   Publisher：Journal of Pediatric Surgery  

Background: Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes. Methods: The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging. Results: Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P < 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011). Conclusion: Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic. Level of Evidence: Level III.

DOI： 10.1016/j.jpedsurg.2024.05.008

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Language：English   Publisher：Pediatric Surgery International  

Purpose: Biliary atresia (BA) poses a persistent challenge characterized by ongoing liver inflammation and subsequent fibrosis even after the clearance of jaundice (COJ). This study aimed to evaluate the therapeutic potential of eicosapentaenoic acid (EPA) in alleviating liver inflammation and limiting fibrosis during the post-COJ phase of BA. Methods: Among the BA patients undergoing laparoscopic Kasai portoenterostomy (lapKP) between December 2016 and October 2021, EPA (20–40 mg/kg/day) was administered orally to those whose parents consented. The study included patients from January 2014 to October 2021, classifying them into two groups: EPA-treated (Group E) and untreated (Group N). Their liver fibrosis and clinical course at 1 and 2 years post-lapKP were compared. Results: Group E consisted of 25 patients, while Group N comprised 32 patients. Twenty-one patients in Group E and 25 patients in Group N achieved COJ (p = 0.74). Among jaundice-free patients at 1 and 2 years post-lapKP, Group E exhibited significantly lower M2BPGi levels and platelet counts, and Group E showed a significant reduction in Aminotransferase-to-Platelet Ratio Index (APRI) at 2 years post-lapKP. Conclusion: Although EPA administration did not improve COJ, it attenuated the progression of liver fibrosis during the 2 years following lapKP in jaundice-free patients. (200/200Words).

DOI： 10.1007/s00383-024-05800-2

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Language：English   Publisher：Frontiers in Bioengineering and Biotechnology  

The efficacy of drug delivery system (DDS)-type near-infrared (NIR) absorbing agents in enhancing laser photothermal therapy is widely acknowledged. Despite the acknowledged efficacy, the therapeutic advantages of photothermal therapy using DDS-type NIR-absorbing agents over simple photothermal therapy without such agents have not been fully elucidated. This study was designed to investigate two primary objectives: firstly, the ability of DDS-type NIR-absorbing agents to induce cell death at greater depths within tumors, and secondly, their capacity to minimize collateral damage to adjacent healthy organs. To investigate these objectives, we employed a combination of indocyanine green lactosome—a DDS-type NIR-absorbing agent—and a precision-controlled laser hyperthermia system. An orthotopic neuroblastoma tumor model was used to closely simulate clinical conditions. The findings revealed that photothermal therapy using the DDS-type NIR-absorbing agent not only facilitates deeper penetration of cell death within tumors but also significantly mitigates thermal damage to surrounding healthy tissues, when compared to simple phototherapy without the agent. Furthermore, the combined treatment significantly prolonged the survival periods of the animals involved. This study is the first to analyze these therapeutic efficacies using quantitative data from an orthotopic tumor animal model and substantiated the potential of DDS-type NIR-absorbing agents to deepen the therapeutic impact of photothermal therapy while safeguarding vital organs, thereby enhancing overall treatment outcomes.

DOI： 10.3389/fbioe.2024.1444107

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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.

DOI： 10.1007/s00383-024-05786-x

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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.

DOI： 10.1007/s00383-024-05724-x

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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.

DOI： 10.1007/s00595-023-02757-y

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Language：English   Publisher：Cancer Science  

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.

DOI： 10.1111/cas.16116

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Language：English   Publisher：Hepatology Research  

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.

DOI： 10.1111/hepr.13976

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BACKGROUND: Duodenal duplication cysts (DDC) are rare duplications of the alimentary tract. Their treatment depends on their size and location. A radical treatment is total resection, if possible. However, partial excision, puncture, and marsupialization can be selected to prevent surgical injury to the pancreaticobiliary tract despite the risk of recurrence. There are some reports of pancreaticoduodenectomy for DDC because of the risk of recurrent symptoms and malignancy. However, this is considered excessively invasive for DDC, particularly in pediatric cases, because of its extremely low rate of malignancy and high morbidity and mortality rates. We encountered a case of DDC with a congenital duodenal position anomaly occurring in the second part of the duodenum. Taking advantage of the congenital duodenal position anomaly, the DDC was completely resected without injuring the pancreaticobiliary duct. CASE PRESENTATION: A 6-year-old boy was diagnosed with a duodenal duplication cyst with obstruction. There was a congenital duodenal position anomaly. The distal second part of the duodenum was the dorsal side of the proximal second part of the duodenum and ascended upward from the proximal second part of the duodenum. The third and fourth parts of the duodenum ran downward to the left and posterior parts of the portal vein, forming the ligament of Treitz. Complete laparoscopic resection of the duodenal duplication cyst and the second to fourth parts of the duodenum, and duodenojejunostomy with retrocolic reconstruction was performed because the duodenum was easily mobilized to the ligament of Treitz owing to the duodenal position anomaly. The duodenojejunostomy with retrocolic reconstruction achieved a more physiologically normal appearance compared to what would have been achieved with a Roux-en-Y reconstruction. The patient was discharged on postoperative day 12 without any complications. CONCLUSIONS: The procedure used in this case might not be easily applied in all laparoscopy cases. However, it could be an option for duodenal duplication cysts with congenital duodenal position anomalies.

DOI： 10.1186/s40792-024-01875-0

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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.

DOI： 10.1007/s00383-024-05683-3

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Background: Data on the outcomes of laparoscopic fundoplication (LF) in patients with ventriculoperitoneal (VP) shunts are limited. Materials and Methods: We retrospectively evaluated the demographic characteristics and outcomes of patients who underwent LF at our institutions between 2014 and 2022. Then, we systematically reviewed articles in MEDILINE/PubMed, Cochrane Library, and Web of Science. Results: There was no significant difference in terms of the outcomes between patients with VP shunt (n = 10) and those without (n = 96) at our institutions. None of the patients presented with shunt trouble after LF. The meta-analysis included four retrospective studies and our institutional data. In total, 605 patients (55 with VP shunt) underwent LF. Furthermore, 2 (3.6%) of 55 patients (1 with infection and 1 with occlusion) had shunt troubles. The conversion and complication rates, operative time, and length of hospital stay did not significantly differ between patients with VP shunt and those without. Conclusions: LF can be safely performed on children with VP shunts and is associated with a low risk of shunt troubles. The Clinical Trial Registration number is 2022-387.

DOI： 10.1089/lap.2023.0220

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BACKGROUND: This study aimed to develop a postnatal treatment strategy for infants with prenatally diagnosed congenital biliary dilatation. METHODS: We performed a retrospective study of patients with prenatal diagnosed congenital biliary dilatation (CBD), aged <1 year who underwent surgery at our hospital between 2013 and 2023. We classified the patients into two groups, the "early group," consisting of patients who could not wait for growth, and required early surgery, and the "scheduled group," consisting of patients who were asymptomatic and could undergo scheduled surgery, and compared them. The parameters for early surgical prediction were AST, ALT, TB, DB, and CRP levels at birth, 1 week, 2 weeks, 1 month, 2 months, and 3 months after birth, and immediately before surgery, as well as the cyst diameter, presence of intrahepatic bile duct dilation, and presence of debris in the common bile duct. RESULTS: During the study period, 15 patients were diagnosed prenatally. The cyst diameter was significantly larger at all points in the early group. Patients with a cyst diameter of >30 mm at birth, intrahepatic bile duct dilatation at birth, and postnatal enlargement of the common bile duct to >30 mm are more likely to develop symptoms early. Blood biochemistry tests showed no significant differences between the two groups. CONCLUSIONS: Patients with a cyst diameter >30 mm in the early postnatal period require careful postnatal management and parents should be counseled regarding the high likelihood of their child needing surgery within the first 3 months of life. LEVEL OF EVIDENCE: Level IV.

DOI： 10.1016/j.jpedsurg.2023.10.045

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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.

DOI： 10.1007/s00595-023-02687-9

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Language：English   Publisher：BMC 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.

DOI： 10.1186/s12893-023-02232-y

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Language：English   Publisher：Journal of Hepato-Biliary-Pancreatic Sciences  

Purpose: Preoperative comprehension of the anatomical variations of the hepatic artery and bile duct is essential for safe laparoscopic surgery for pancreaticobiliary maljunction (PBM). This study aimed to investigate the impact of anatomical variations of the hepatic artery and bile duct on surgical technique and postoperative complications. Methods: We conducted a retrospective review of patients with PBM who underwent laparoscopic surgery at our institution between January 2014 and December 2022 to investigate anatomical variations in the hepatic artery and bile duct, surgical technique, and postoperative complications. Results: We included 112 patients with PBM, with a median age of 4 years (interquartile range, 0–55). Overall, 29 of 112 patients had an aberrant right hepatic artery (ARHA) running ventral to the common hepatic duct (CHD), and they underwent hepaticojejunostomy on the ventral side of the ARHA. Additionally, eight of 112 patients had an aberrant posterior hepatic duct (APHD), which was joined to the CHD in all but one case. The presence of APHD was associated with postoperative bile leak occurrence. Conclusion: Performing hepaticojejunostomy ventral to the ARHA is important to prevent complications. Furthermore, APHD may be a risk factor for postoperative bile leak and requires careful bile duct plasty.

DOI： 10.1002/jhbp.1381

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Language：English   Publisher：Pediatric Surgery International  

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.

DOI： 10.1007/s00383-023-05548-1

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BACKGROUND: Congenital chylothorax (CCT) and postoperative chylothorax (POCT) are rare and difficult to treat. We report our treatment strategy and outcomes for chylothorax, including thoracoscopic surgery with indocyanine-green (ICG) near-infrared fluorescence lymphangiography. METHODS: A retrospective review of patients with CCT and POCT from 2014 to 2021 was performed. After definitive diagnosis, conservative treatments with octreotide, followed by intravenous steroids as needed, were performed. Patients who were refractory to conservative treatment were transferred to surgical treatment, consisting of thoracoscopic lymphatic leak ligations using ICG intraoperative lymphangiography. The effectiveness of conservative and surgical treatment was then examined. RESULTS: We included 19 cases of CCT and 31 cases of POCT. The 31 POCT patients included 23 of 84 postoperative patients with congenital diaphragmatic hernia (CDH), 7 of 54 postoperative patients with esophageal atresia (EA), and 1 of 3 postoperative patients with lymphatic malformation. The efficacy of conservative treatment was 12/19 for CCT, 22/23 for CDH, and 4/7 for EA. Surgical intervention was performed in 10 patients, and the rate of resolution of chylothorax within 3 weeks after surgery was 90%. CONCLUSION: Thoracoscopic lymphatic leak ligations with intraoperative ICG lymphangiography are feasible and useful in patients with chylothorax refractory to conservative treatment. LEVEL OF EVIDENCE: Level IV.

DOI： 10.1016/j.jpedsurg.2022.11.018

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Language：English   Publisher：Frontiers in Pediatrics  

Congenital duodenal atresia with situs inversus is occasionally accompanied by a preduodenal portal vein (PDPV), which is incidentally diagnosed during surgery. Duodenoduodenostomy is the most common and effective treatment. However, some patients require other anastomoses. Here, we present two cases of laparoscopic gastrojejunostomy for congenital duodenal atresia with situs inversus and PDPV and describe the reason for selecting gastrojejunostomy. The optimal surgical strategy is patient specific and should be determined based on the patient's general and physical condition.

DOI： 10.3389/fped.2023.1220393

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Language：English   Publisher：Frontiers in Pediatrics  

Retropancreatic fascia hernia is a novel internal hernia originating from the retropancreatic fascial defect, which subsequently expands toward the dorsal aspect of the pancreatic body and migrates into the retroperitoneal space. We encountered a rare case of concomitant retropancreatic fascia and Bochdalek hernias. Here, we describe the imaging characteristics of this hernia type and its surgical strategies.

DOI： 10.3389/fped.2023.1149515

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Language：English   Publisher：American Journal of Case Reports  

Objective: Congenital defects/diseases Background: Kluth demonstrated that esophageal atresia/tracheoesophageal fistula (EA/TEF) has several anatomical variations and thus requires a preoperative imaging study to determine the surgical strategy. We routinely perform a contrast examination with iodixanol to assess the location of the TEF and the upper end of the esophageal pouch to determine the most appropriate approach. We herein present two cases of type C EA/TEF who successfully underwent radical surgery by a cervical approach based on the information from the contrast examination. Case Reports: Case 1 was a Japanese boy suspected of type C EA/TEF after birth. A contrast examination with iodixanol showed that a TEF was at the second thoracic vertebra (Th2), as was the upper end of the esophageal pouch. Thus, the patient underwent esophago-esophageal anastomosis and TEF ligation using a cervical approach; the postoperative course was uneventful. Case 2 was also a Japanese boy suspected of type C EA/TEF. A contrast examination showed that the TEF was at Th1-2, as was the upper end of the esophageal pouch. Thus, the patient underwent esophago-esophageal anastomosis and TEF ligation using a cervical approach. The patient suffered from congenital tracheal stenosis and required tracheoplasty. However, there were no apparent complications after the surgery. Conclusions: Here, we used the imaging information to adopt the cervical approach in type C EA/TEF cases and concluded that routine preoperative contrast examinations helped assess the TEF location and upper end of the esophageal pouch without significant complications.

DOI： 10.12659/AJCR.938723

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Language：English   Publisher：Pediatric Surgery International  

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.

DOI： 10.1007/s00383-023-05461-7

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Language：English   Publisher：Journal of Hepato-Biliary-Pancreatic Sciences  

Background/Purpose: Congenital biliary dilatation (CBD) is a disease associated with pancreaticobiliary maljunction. The most frequent postoperative complication is intrahepatic stones, which are caused by hilar bile duct stenosis (HBDS). Methods: We retrospectively reviewed the records of patients who underwent primary surgery for CBD between 2013 and 2021. We evaluated images and videos of HBDS, the laparoscopic technique of releasing the stenosis, and its occurrence rate and compared intraoperative bile duct findings with those of preoperative magnetic resonance cholangiopancreatography (MRCP). Results: There were 87 CBD cases in this study. HBDS occurred in 52% and preoperative MRCP for HBDS showed a 96% sensitivity and 74% specificity in this study. Bile duct plasty was performed in 45% of the cases and videos demonstrated typical methods of laparoscopic bile duct plasty. The mid- to long-term complications were hepatolithiasis in three patients, anastomotic site stricture in three, and postoperative obstruction in two. Conclusions: Our study revealed that preoperative imaging studies are useful for the prediction of HBDS. Our laparoscopic meticulous probing method for finding BDS reveals more intrahepatic BDS through magnification. Therefore, this may reduce the incidence of intrahepatic stones. Preoperative imaging and intraoperative findings can be complemented to plan management that reduces long-term complications.

DOI： 10.1002/jhbp.1235

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BACKGROUND: Like esophageal varices, cardiac varices are often treated with endoscopic variceal ligation (EVL). However, we previously reported that EVL for cardiac varices may be associated with a high risk of rebleeding from the ulcer if the O-ring spontaneously drops off early. The efficacy and safety of para-variceal endoscopic injection sclerotherapy (EIS) with polidocanol for the treatment of cardiac varices in children and adolescents were evaluated. METHODS: Eleven patients under 18 years of age with portal hypertension who underwent para-variceal EIS with polidocanol for cardiac varices with red signs, which were considered to be at high risk of bleeding, were retrospectively reviewed. RESULTS: One session of para-variceal polidocanol-EIS was performed for each of the 11 patients. One patient experienced temporary hypoxia due to aspiration of saliva when the tracheal intubation tube was removed after the procedure but recovered by endotracheal suctioning; there were no other adverse events. In six of the eight cases in which efficacy could be evaluated, eradication of cardiac varices was achieved. CONCLUSION: Para-variceal polidocanol-EIS may be considered instead of EVL for small cardiac varices with red signs in pediatric patients with cardiac varices.

DOI： 10.1016/j.arcped.2022.11.016

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BACKGROUND: We encountered two cases of a new type of retroperitoneal hernia. We herein report the unique features of these cases. CASE PRESENTATION: Case 1: A Japanese girl was born at a gestational age of 37 weeks, weighing 2550 g. She underwent laparotomic left diaphragmatic hernia repair for a left Bochdalek hernia at the age of one day. The postoperative course was uneventful; however, chest radiography at the age of 35 days revealed bowel gas in the mediastinum, while computed tomography exhibited intestinal prolapses from the medial side of the mesh into the thoracic cavity. Reoperation was performed at the age of 77 days, showing that the defect hole was not at the diaphragm but in the absence of retropancreatic fascia, which was connected to the posterior mediastinum from the supramesocolic space. The mediastinum space was closed with a suturing spine and artificial mesh, and the defect hole in the pancreatic body was sutured. Case 2: A Japanese boy was born at a gestational age of 40 weeks, weighing 3502 g. He was diagnosed with a left diaphragmatic hernia at birth and underwent laparotomy at the age of two days. Operative findings showed no defect hole in the diaphragm, and no intestine was observed in the abdominal cavity. After close observation of the abdominal cavity, the intestine was found around the pancreatic body, and manual reduction of the intestine was performed. The defect hole existed in the absence of the retropancreatic fascia, which was connected to the extra-pleural space. The defect hole in the pancreatic body was sutured and closed with a non-absorbable thread. CONCLUSIONS: We assumed that our cases were a new type of retroperitoneal hernia, which we named "retropancreatic fascia hernia".

DOI： 10.1186/s40792-023-01586-y

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Language：English   Publisher：Frontiers in Pediatrics  

Background: We present the surgical technique and outcomes of reduced-port laparoscopic restorative proctocolectomy with ileal-J-pouch anal canal anastomosis (IPACA) without diverting ileostomy for total colonic and extensive aganglionosis (TCA+). Methods: We retrospectively reviewed TCA+ cases between 2014 and 2022. Preoperative ileostomy was performed when transanal bowel irrigation was ineffective. Radical surgery for TCA+ was performed at approximately 6 kg. The surgery was performed using laparoscopy through a multi-channel trocar with or without an additional 3-mm trocar and IPACA reconstruction with indocyanine green fluorescence angiography (ICG) to assess anastomotic perfusion and Lugol's iodine staining to visualize the surgical anal canal. Results: Ten patients with TCA+ were included. Ileostomy was performed in seven cases. The median operation time and blood loss were 274.5 min and 20 ml, respectively. No significant postoperative complications were found. All patients experienced frequent liquid stools and perianal excoriation in the early postoperative period, requiring anti-flatulence or codeine. The median follow-up period was 3.5 years. Three patients required irrigation management 1 year postoperatively, and the others defecated a median of 3.5 times per day. The median Kelly's clinical score was 5 in 5 patients aged >4 years. Conclusion: Reduced-port surgery, combined with Lugol's iodine staining and ICG, was safe, feasible, and had cosmetically and clinically acceptable mid-term outcomes.

DOI： 10.3389/fped.2022.1090336

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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/sec patients than in those with LS < 2.8 m/sec (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. (246 words).

DOI： 10.1111/ped.15454

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DOI： 10.1007/s00383-022-05243-7

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DOI： 10.1007/s00383-022-05230-y

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This study aimed to evaluate the safety and effectiveness of circumumbilical incision (CUI) for neonates requiring intestinal anastomosis. Seventy neonates requiring intestinal anastomosis at our institution between 2003 and 2020 were included in this retrospective case-control study. Patients were classified into the CUI (25 patients: 36%) and transverse incision (TI) groups (45 patients: 64%). Postoperative complications and surgical outcomes were compared between the two groups. Intestinal perforation at the non-anastomotic site occurred significantly more often in the CUI group than in the TI group (3 patients: 12%, and 0 patients: 0%, respectively (p = 0.042)). There were no between-group differences regarding anastomotic leakages, anastomotic strictures, time to enteral feeding, operative time, and blood loss. Neonatal intestinal surgery employing CUI might be associated with increased intestinal perforation at the non-anastomotic site. Hesitating to enlarge the skin incision to maintain favorable cosmetic outcomes might cause severe injury to the delicate neonatal intestine during the surgical procedure owing to the restricted surgical field. When performing CUI, we suggest that the skin incision should be extended without hesitation whenever there is difficulty in manipulating the intestine.

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DOI： 10.1007/s00464-022-09132-x

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DOI： 10.1111/ped.14614

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DOI： 10.1038/s41598-021-90550-7

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DOI： 10.1200/JCO.2021.39.15_suppl.e22008

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DOI： 10.1007/s00464-019-07108-y

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

Abstract

Background

The fundamental treatment for patients with pediatric malignant mediastinal tumors is chemotherapy. Therefore, accurate diagnosis is essential for selecting the appropriate chemotherapeutic regimen. However, malignant mediastinal tumors occasionally cause respiratory distress, and biopsies under general anesthesia are dangerous for such patients as invasive mechanical ventilation can aggravate airway obstruction caused by mass effect. In this study, we reviewed our 10-year diagnostic experience to evaluate the efficacy of our practices and confirm a safe diagnostic protocol for future patients.

Methods

We retrospectively reviewed medical records of children with malignant mediastinal tumors diagnosed at Nagoya University Hospital from 2007 to 2018 who demonstrated respiratory distress. Respiratory distress included dyspnea, massive pleural effusion, wheezing, and hypoxemia owing to tumors. Data on sex, age at onset, primary symptoms, location of tumor, management strategy (especially the method of diagnosis and definitive diagnosis), clinical course, prognosis during the acute phase (within 3 months from the onset of respiratory symptoms), and long-term outcome were collected.

Results

Twelve pediatric patients met the review criteria. There were seven anterior mediastinal tumors and five posterior mediastinal tumors. All anterior mediastinal tumors were diagnosed via bone marrow smear, thoracentesis, or core needle biopsy while maintaining spontaneous breathing. Regarding posterior tumors, two patients were diagnosed via a core needle biopsy and lymph node excisional biopsy under spontaneous breathing. Two cases were initially diagnosed solely using tumor markers. One patient with severe tracheal compression underwent tumor resection with extracorporeal membrane oxygenation stand-by. No patient died of diagnostic procedure-related complications.

Conclusions

In 11 of the 12 cases reviewed, safe and accurate tumor diagnosis was accomplished without general anesthesia. A diagnostic strategy without general anesthesia considering the tumor location proved to be useful.

DOI： 10.1186/s12887-020-02183-w

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Other Link： https://link.springer.com/article/10.1186/s12887-020-02183-w/fulltext.html

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DOI： 10.1016/j.jpedsurg.2017.08.056

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DOI： 10.1007/s00383-017-4140-1

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DOI： 10.18999/nagjms.79.3.415

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DOI： 10.1016/j.braindev.2016.11.012

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DOI： 10.1186/s12871-017-0336-0

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DOI： 10.1089/lap.2016.0207

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A right aortic arch (RAA) is found in 5% of neonates with tracheoesophageal fistulae (TEF) and may be associated with vascular rings. Oesophageal repairs for TEF with an RAA via the right chest often pose surgical difficulties. We report for the first time in the world a successful two-stage repair by left-sided thoracoscope for TEF with an RAA and a vascular ring. We switched from right to left thoracoscopy after finding an RAA. A proximal oesophageal pouch was hemmed into the vascular ring; therefore, we selected a two-stage repair. The TEF was resected and simple internal traction was placed into the oesophagus at the first stage. Detailed examination showed the patent ductus arteriosus (PDA) completing a vascular ring. The subsequent primary oesophago-oesophagostomy and dissection of PDA was performed by left-sided thoracoscope. Therefore, left thoracoscopic repair is safe and feasible for treating TEF with an RAA and a vascular ring.

DOI： 10.4103/0972-9941.181771

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.18999/nagjms.78.4.447

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DOI： 10.1111/ped.13040

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Responsible for pages：128-130   Language：Japanese Book type：Textbook, survey, introduction

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Responsible for pages：303-304   Language：Japanese Book type：Textbook, survey, introduction