Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Hepato-Biliary-Pancreatic Sciences  

Background: Multicenter study was undertaken to analyze the results of laparoscopic and open Kasai portoenterostomy. Methods: Subjects were infants with type III biliary atresia who underwent open operation (n = 106) or laparoscopic operation (n = 21) between January 2012 and December 2015. Clinical data were compared between open and laparoscopic operations (2016-0534). Propensity score matching was performed to reduce the effect of treatment selection bias. Multivariate analyses were used to estimate the effect of the surgical approach on the jaundice clearance rate and the native liver survival rate. Results: The postoperative jaundice clearance rate and the 1-year native liver survival rate were not significantly different between open and laparoscopic operations. Rates of cholangitis and major complications of laparoscopic operation were comparable to those of open operation. Blood loss, time to resume oral intake, time to drain removal, and duration of analgesic usage of laparoscopic operation were significantly superior to those of open operation. Similar results were observed when analysis was adjusted based on propensity score. Multivariate analyses demonstrated that only age at operation was a poor prognostic factor. Conclusion: Laparoscopic Kasai portoenterostomy was associated with several favorable perioperative outcomes compared with open Kasai portoenterostomy. The difference of surgical approach was not a significant independent predictor.

DOI： 10.1002/jhbp.594

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PubMed

Language：English   Publishing type：Research paper (scientific journal)   Publisher：BMC Pediatrics  

Background: Emergency rescue lung resection is rarely performed to treat congenital lung cysts (CLCs) in neonates. Many reports have described fetal CLC treatment; however, prenatal predictors for postnatal respiratory failure have not been characterized. We hypothesized that fetal imaging findings are useful predictors of emergency surgery. Methods: We retrospectively studied patients with CLC who underwent lung surgery during the neonatal period in our hospital between January 2001 and December 2015. The demographic data, fetal imaging findings, and intra- and postoperative courses of patients who underwent emergency surgery (Em group) were compared with those of patients who received elective surgery, i.e., non-emergency surgery (Ne group). Results: The Em group and Ne group included 7 and 11 patients, respectively. No significant difference was noted in gestational age, time at prenatal diagnosis, birth weight, and body weight at surgery. The volumes of contralateral lung per thoracic volume were significantly smaller in the Em group than in the Ne group (p = 0.0188). Mediastinal compression was more common in the Em group (7/7) than in the Ne group (4/11) (p = 0.0128). Conclusions: This is the report describing neonatal emergency lobectomy in patients with CLC evaluated by fetal MRI using the lung volume ratio and mediastinal shift. In patients with CLC, mediastinal shift and significant decreases in contralateral lung volumes during the fetal stages are good prenatal predictors of postnatal emergency lung resection.

DOI： 10.1186/s12887-018-1085-z

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1186/s12887-017-0826-8

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1053/j.gastro.2006.10.013

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.12659/ajcr.903465

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1097/01.TP.0000081560.16039.67

PubMed

Language：English   Publisher：Surgery Today  

Purpose: To investigate late complications after surgery for congenital biliary dilatation (CBD). Methods: We retrospectively reviewed the patients treated for late postoperative complications of extrahepatic bile duct resection with bilioenteric anastomosis for CBD at our hospital between 1999 and 2019. Results: Twenty-seven complications, including bile duct stenosis with (n = 19) or without (n = 3) hepatolithiasis, remnant intrapancreatic bile duct (n = 2), intestinal obstruction (n = 2), and refractory cholangitis (n = 1) were treated in 26 patients. The median age at radical surgery and the initial treatment of complications was 3 years, 2 months and 14 years, 5 months, respectively. The median period from radical surgery to initial treatment of complications was 7 years, 1 month. Before 2013, bile duct stenosis was initially treated with bile duct plasty (n = 11) or hepatectomy (n = 3), and 71.4% (n = 10) of patients needed further treatment; after 2013, double-balloon endoscopic retrograde cholangiography (DBERC) was used (n = 8), and 25% (n = 2) of patients needed further treatment. Patients with remnant intrapancreatic bile duct, intestinal obstruction, and refractory cholangitis required surgery. Conclusion: Long-term follow-up is necessary after surgery for congenital biliary dilatation. DBERC is thus considered to be useful for bile duct stenosis management.

DOI： 10.1007/s00595-021-02238-0

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p><i>Purpose</i>: The treatment outcomes of patients with laparoscopic surgery for biliary atresia (laparoscopic portoenterostomy) were retrospectively evaluated to determine whether to limit the laparoscopic surgery for biliary atresia to fully experienced surgeons.</p><p><i>Methods</i>: We included 37 patients with laparoscopic portoenterostomy for biliary atresia at our institution from 2014 to 2018. Twelve patients were treated by a surgeon fully experienced in laparoscopic portoenterostomy (Group A) and 23 patients were treated by other surgeons (Group B). Jaundice reduction and jaundice-free survival of patients with the native liver in the two groups were compared.</p><p><i>Results</i>: Differences in age at the time of surgery, body weight, perioperative blood loss, time of drain removal, and time that milk or formula feeding resumed in both groups were not significant. The duration of surgery was significantly shorter in Group A than in Group B. The jaundice-free survival rates of patients with the native liver were 58.3% in Group A and 60.9% in Group B at 6 months after surgery, 66.7% in Group A and 65.2% in Group B at 1 year after surgery, and 58.3% in Group A and 56.5% in Group B at 2 years after surgery. No significant difference was observed.</p><p><i>Conclusions</i>: In this study, no significant difference in the rate of jaundice-free survival of patients with the native liver depending on the amount of surgical experience was observed under the guidance of an operator who was skilled in laparoscopic portoenterostomy.</p>

DOI： 10.11164/jjsps.57.3_596

CiNii Research

Language：English   Publisher：Scientific Reports  

Currently, surgical staples are composed of non–biodegradable titanium (Ti) that can cause allergic reactions and interfere with imaging. This paper proposes a novel biodegradable magnesium (Mg) alloy staple and discusses analyses conducted to evaluate its safety and feasibility. Specifically, finite element analysis revealed that the proposed staple has a suitable stress distribution while stapling and maintaining closure. Further, an immersion test using artificial intestinal juice produced satisfactory biodegradable behavior, mechanical durability, and biocompatibility in vitro. Hydrogen resulting from rapid corrosion of Mg was observed in small quantities only in the first week of immersion, and most staples maintained their shapes until at least the fourth week. Further, the tensile force was maintained for more than a week and was reduced to approximately one-half by the fourth week. In addition, the Mg concentration of the intestinal artificial juice was at a low cytotoxic level. In porcine intestinal anastomoses, the Mg alloy staples caused neither technical failure nor such complications as anastomotic leakage, hematoma, or adhesion. No necrosis or serious inflammation reaction was histopathologically recognized. Thus, the proposed Mg alloy staple offers a promising alternative to Ti alloy staples.

DOI： 10.1038/s41598-019-51123-x

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Language：English   Publisher：Journal of Pediatric Surgery  

Background: Postoperative chylothorax after surgery for esophageal atresia/tracheoesophageal fistula (TEF) is a rare but serious complication, especially in neonates. This study aimed to identify the thoracic duct and ligate chylous leakage sites, using thoracoscopic navigation of an indocyanine-green (ICG)-based near-infrared (NIR) fluorescence imaging system. Methods: From November 2014 to April 2017, thoracoscopic intraoperative ICG-NIR imaging was performed in 10 newborns (11 surgeries) with first TEF operation or with persistent postoperative chylothorax after TEF operation. NIR imaging was performed 1 h after an inter-toe injection of ICG. Thoracoscopic ligations against the NIR-detected leakage sites were performed with sutures. Results: The thoracic duct or lymphatic leakage was directly visualized in each patient. In 8 surgeries with first thoracoscopic TEF operation, one case had suspected minor chylous leakage without postoperative chylothorax. Another case with no chylous leakage at the first operation resulted in chylothorax at postoperative day 11. In three neonates with postoperative chylothorax, leakage points were detected near the ablation site of the azygos vein during the first operation. These points were properly ligated, and postoperative chylous leakage ceased with no adverse events. Conclusions: Thoracoscopic ICG-NIR imaging encourages the repair of refractory chylothorax and seems reliable. Level of Evidence: IV

DOI： 10.1016/j.jpedsurg.2018.01.019

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

DOI： 10.3978/j.issn.2224-4336.2015.02.01

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Language：Japanese  

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Language：English   Publisher：Scientific Reports  

Urine is a complex liquid containing numerous small molecular metabolites. The ability to non-invasively test for cancer biomarkers in urine is especially beneficial for screening child patients. This study attempted to identify neuroblastoma biomarkers by comprehensively analysing urinary metabolite samples from children. A total of 87 urine samples were collected from 54 participants (15 children with neuroblastoma and 39 without cancer) and used to perform a comprehensive analysis. Urine metabolites were extracted using liquid chromatography/mass spectrometry and analysed by Metabolon, Inc. Biomarker candidates were extracted using the Wilcoxon rank sum test, random forest method (RF), and orthogonal partial least squares discriminant analysis (OPLS-DA). RF identified three important metabolic pathways in 15 samples from children with neuroblastoma. One metabolite was selected from each of the three identified pathways and combined to create a biomarker candidate (3-MTS, CTN, and COR) that represented each of the three pathways; using this candidate, all 15 cases were accurately distinguishable from the control group. Two cases in which known biomarkers were negative tested positive using this new biomarker. Furthermore, the predictive value did not decrease in cases with a low therapeutic effect. This approach could be effectively applied to identify biomarkers for other cancer types.

DOI： 10.1038/s41598-021-83619-w

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Language：English   Publisher：Journal of Pediatric Surgery  

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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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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PubMed

Language：English   Publisher：Journal of Laparoendoscopic and Advanced Surgical Techniques  

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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Language：English   Publisher：Surgery Today  

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：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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Language：English   Publisher：Surgery Today  

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  

DOI： 10.1186/s40792-023-01754-0

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PubMed

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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Language：English   Publisher：Journal of Pediatric Surgery  

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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Publisher：東京医学社  

DOI： 10.24479/ps.0000000514

CiNii Research

Language：English   Publisher：BMC Pediatrics  

Background: Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease and affected individuals typically present with an increased infiltration of IgG4-positive plasma cells in the pancreas, hepatobiliary tract, and liver but rarely in the gastrointestinal tract. Case presentation: A 12-year-old girl presented with vomiting and poor weight gain. Gastroscopy revealed duodenal stenosis and ulceration. Computed tomography revealed edematous duodenal wall thickening and air-fluid levels on the right side of the duodenum, which suggested duodenal perforation or penetration. She underwent pancreaticoduodenectomy, and IgG4-RD was diagnosed via histopathology. Conclusions: This is the first pediatric case of isolated duodenal IgG4-RD resulting in duodenal obstruction after multiple ulcers. Gastrointestinal IgG4-RD should be among the differential diagnoses of unexplained gastrointestinal obstruction or ulceration even in children.

DOI： 10.1186/s12887-023-04190-z

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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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Publisher：東京医学社  

DOI： 10.24479/ps.0000000451

CiNii Research

Language：English   Publisher：Surgery Today  

Purpose: Rectal prolapse after radical surgery for anorectal malformations (ARMs) is a common postoperative complication that causes bleeding and stenosis, and sometimes requires surgical treatment. Different surgical techniques have been reported, but most are associated with high recurrence rates and a long postoperative stay. We performed the hemi-circumferential mucosal resection and anastomosis procedure (HCMR) to preserve anal muscle and anal function. Here, we report the success of our minimally invasive surgical approach to correct rectal prolapse in patients with ARMs. Methods: The subjects of this retrospective review were patients who underwent HCMR for rectal prolapse after radical surgery for ARMs between January, 2014 and August, 2021. HCMR involves rectal mucosal resection without muscle plication. Results: A collective 15 HCMR procedures were performed in 10 patients. The median age at repair was 1.8 years (range, 10 months–18 years). The median operation time and postoperative length of stay were 46 min (range, 17–85 min) and 3 days (range, 1–7 days), respectively. The median postoperative observation period was 3 years 4 months (range, 8 months–7 years 10 months) and no complications or recurrences were observed. Conclusions: HCMR is a safe and effective surgical treatment for rectal prolapse after radical operation for ARMs, which is minimally invasive and preserves rectal muscle and function.

DOI： 10.1007/s00595-022-02611-7

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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：Asian Journal of Endoscopic Surgery  

We report an extremely rare case of a right Bochdalek hernia with a sac, in which the retroperitoneal and intra-abdominal organs prolapsed into the thoracic cavity at the same time. The patient was a 7-month-old female with no comorbidities. She presented with cough and fever, and chest radiography revealed a right diaphragmatic hernia. Computed tomography showed that the right kidney, intestine, colon, and liver had prolapsed into the thoracic cavity. The patient underwent thoracoscopic surgery, which showed that the abdominal and retroperitoneal organs prolapsed into the thoracic cavity through the Bochdalek hernia. The herniated organs were spontaneously reduced using thoracoscopic insufflation. The defect hole was closed with artificial mesh. We adopted a thoracoscopic approach, in terms of easy reduction of herniated organs and accurate evaluation of the hernia orifice, which was useful.

DOI： 10.1111/ases.13139

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PubMed

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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PubMed

Language：English   Publisher：Asian Journal of Endoscopic Surgery  

Bile lake, of the postoperative complications after Kasai portoenterostomy (PE) for biliary atresia, causes cholangitis that may induce progressive fibrosis of the liver. Standard treatment for bile lakes has not yet been established, but there are reports that surgical internal intestinal drainage for bile lakes effectively prevents cholangitis and maintains jaundice-free status. In this case, insertion of the percutaneous transhepatic biliary drainage into the bile lake allowed continuous drainage of large volumes of bile juice. However, reoperation following laparotomy increases the surgical risk of subsequent liver transplantation due to postoperative adhesion. Laparoscopic surgery was selected for the patient who was likely to require liver transplantation in the future. In this case, laparoscopic internal intestinal drainage of bile lakes was performed safely by a Cavitron ultrasonic surgical aspirator for the recurrence of jaundice after laparoscopic revision of PE. Cholangitis and jaundice were rapidly resolved after this surgical procedure.

DOI： 10.1111/ases.13133

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PubMed

Language：English   Publisher：Surgery Today  

Despite improving the survival after repair of esophageal atresia (EA), the morbidity of EA repair remains high. Specifically, tracheomalacia (TM) is one of the most frequent complications of EA repair. Continuous positive airway pressure is generally applied for the treatment of TM. However, surgical intervention is required against an apparent life-threatening event or inability to perform extubation for a long period. According to our review, most cases of TM showed symptom improvement after aortopexy. The ratio of the trachea’s lateral and anterior–posterior diameter at the brachiocephalic artery crossing the trachea, which reflects the compression of the trachea by the brachiocephalic artery, is a good indicator of aortopexy. Our finding suggests that most TM cases associated with EA may not be caused by tracheal fragility alone, but may involve blood vessel compression. Posterior tracheopexy (PT) is also an effective treatment for TM. Recently, open or thoracoscopic PT was able to be performed simultaneously with EA repair. In many cases, aortopexy or PT is a safe and effective surgical treatment for TM with EA. Other surgical procedures, such as external stenting, should be considered for patients with diffuse-type TM for whom aortopexy and PT appear relatively ineffective.

DOI： 10.1007/s00595-023-02652-6

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PubMed

Language：English   Publisher：Journal of Laparoendoscopic and Advanced Surgical Techniques  

Purpose: A laparoscopic approach for malrotation is feasible and safe in hemodynamically stable neonates without intestinal necrosis; however, volvulus is associated with recurrence and conversion. We developed a novel approach using a laparoscopic duodenal-caudal detachment method to perform the Ladd procedure for neonates with volvulus under the limited view of laparoscopy. This study presents the results, effectiveness, and details of the method. Materials and Methods: In the laparoscopic duodenal-caudal detachment method, we first detached the adhesions around the duodenum, including the Ladd's band. After the adhesions were completely removed, the duodenum was freely drawn caudally, leading to the release of torsion. We retrospectively reviewed the medical records of patients who underwent surgery for malrotation of the volvulus at 30 days of age between January 2014 and September 2021. Results: Seven neonates underwent the laparoscopic duodenal-caudal detachment method and 13 underwent the open Ladd procedure. The new technique was performed in all 7 patients, and there were no conversions or recurrences. The operation time was significantly longer in the laparoscopic procedure group (55 minutes versus 111 minutes; P < .01). Conclusions: Our detorsion method, involving an initial incision of the Ladd's band, is safe and effective for neonates and may lead to an improvement in the conversion rates.

DOI： 10.1089/lap.2022.0390

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PubMed

Language：English   Publisher：Archives de Pediatrie  

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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PubMed

Language：English  

DOI： 10.1186/s40792-023-01586-y

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PubMed

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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PubMed

Language：English   Publisher：Pediatrics International  

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.

DOI： 10.1111/ped.15454

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PubMed

Language：English   Publisher：Asian Journal of Endoscopic Surgery  

We report a case of hepatolithiasis 30 years after congenital biliary dilatation (CBD) surgery. A 31-year-old woman presented with fever and epigastric pain. She had a history of radical surgery for type I CBD at the age of 1 year and had no significant symptoms for approximately 30 years after surgery. Laboratory and imaging results showed hepatolithiasis at the common trunk of segments II and III with cholangitis. She was admitted to our hospital for antibiotics and underwent double-balloon endoscopic retrograde cholangiography (DBERC) to treat the hepatolithiasis. Roux-limb jejunum was perforated during DBERC; hence, emergent laparoscopic perforation site closure and simultaneous endoscopic lithotomy through the Roux-limb jejunum with exteriorization via umbilical incision were performed. The postoperative course was uneventful, and the patient was discharged without any complications.

DOI： 10.1111/ases.13116

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PubMed

Language：English   Publisher：Asian Journal of Endoscopic Surgery  

Pheochromocytomas and paragangliomas are rare tumors. A 10 year-old girl was brought to the emergency room with complaints of sudden vomiting and convulsions, and was initially diagnosed with hypertensive encephalopathy. Magnetic resonance imaging and computed tomography scan showed a large mass (6 × 3 × 3 cm) on the dorsal side of the inferior vena cava, surrounded by the right diaphragmatic crus, and closely attached to the aorta. Blood noradrenaline, urinary normetanephrine, and noradrenaline levels were elevated. The final diagnosis was retroperitoneal paraganglioma, then, surgery was contemplated. The location of the liver and great vessels in front of the tumor made the commonly performed transabdominal approach complicated. Therefore, retroperitoneoscopic surgery was preferred for safer resection, with better visualization in a sufficient space with less risk of damaging the surrounding organs. The retroperitoneoscopic approach is a good indication for tumors located behind the great vessels.

DOI： 10.1111/ases.13119

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PubMed

Language：Japanese   Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.46.0_52

CiNii Research

Language：Japanese   Publisher：公益社団法人 日本生体医工学会  

<p>【はじめに】術後成長発達する小児患者にとって、低侵襲手術は非常に重要である。しかし患者数は限られているため、しっかりとした手術を行うためにoff the job-training(OJT)が重要である。さらにOJTでの効率的な手技獲得には、手技を客観的に評価しfeed backを行うシステムが必須である。また安全で効率的な内視鏡手術を行うためには、臓器の位置関係の把握が必要であるため、術中ナビゲーションは重要な要件となる。これらの課題に対して、AIを用いた内視鏡手技評価および手術支援システムの構築に着手しており現状の成果を報告する。【方法と結果】食道閉鎖症モデルを用いた吻合手技を被験者に課し、各被験者の手技を最初に人の目で「check 表」「エラー項目」「時間」を用いて評価した。次にビデオから検出した鉗子の動きと人が判定した手技優劣の関係性をAIで学習させ、上位88％・下位95％の精度で手技優劣が自動判定可能となった。この結果を解析することで今まで必要だった50項目以上の肉眼チェックが、わずか７項目チェックするだけで手技の優劣を判断できることが明らかになった。現在食道閉鎖症の手術画像を用いて、食道・迷走神経・気管を深層学習させ、各種構造物の自動認識を進めている。【まとめ】AI画像解析により内視鏡手技の優劣をビデオで判定可能となった。この結果から新たに効率的な手技判断基準を定めることができた。術中ナビゲーションは現在精度のさらなる向上を目指している。</p>

DOI： 10.11239/jsmbe.annual61.127_2

CiNii Research

Language：English   Publisher：Frontiers in Pediatrics  

A tracheoesophageal fistula (TEF) recurs in approximately 2%–13% of cases of esophageal atresia with TEF that are treated surgically. Currently, there is no consensus on the most effective treatment to prevent recurrent TEF (RTEF). Herein, we present a patient with type C esophageal atresia who underwent thoracoscopic esophago-esophageal anastomosis and TEF repair at 2 days old. However, RTEFs were observed at ages 3, 6, and 11 months, and thoracoscopic TEF repairs using a pleural patch, fascia lata graft, and pectoralis major myocutaneous (PMMC) flap were performed, respectively. A fourth recurrence led to mediastinitis, shock liver, disseminated intravascular coagulopathy, and a compromised respiratory status. Hence, laparoscopic esophageal transection was first performed to improve the respiratory condition by preventing the regurgitation of gastric contents. Once the patient was stable, a subtotal esophageal resection with TEF closure followed by gastric tube reconstruction was performed. In conclusion, we encountered a case of refractory RTEF that was repaired four times using various techniques, including a fascia lata graft and PMMC flap. However, TEF still recurred after these four operations. The final surgical strategy involved an esophageal transection as a palliative therapy, which improved the respiratory condition, followed by closure of the TEF and subtotal esophageal resection. Finally, esophageal reconstruction using a gastric tube after the complete remission of inflammation was effective. This multi-stage surgery was considered the only choice to rescue the patient and effectively prevent another recurrence.

DOI： 10.3389/fped.2022.1053154

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PubMed

Language：English   Publisher：BMC Urology  

Background: The urachus is an embryonic structure that connects the bladder to the allantois during early embryonic development. Occasionally, it fails to disappear at birth, leading to a case of urachal remnant (UR). This study aimed to determine whether our policy for selecting an appropriate UR resection approach is valid. We performed preoperative imaging to examine whether UR continued toward the bladder apex. If so, the UR and bladder apex were excised using the trans-umbilical approach, in addition to laparoscopy, if necessary. If preoperative imaging indicated that the UR ended near the umbilicus, the UR from the umbilicus to the duct end was resected. Pathological evaluations were performed to determine the appropriateness of the surgical approach indicated by preoperative imaging. Methods: We retrospectively reviewed pediatric patients with UR who underwent surgery between 2015 and 2021. Their background characteristics and surgical outcomes were evaluated. Results: Twenty patients with UR were included (median age, 7 [interquartile range, 2–10.25] years). UR continued toward the bladder apex in 10 patients and ended near the umbilicus in 10 patients. Urachus tissue at the bladder site was observed when the UR and bladder apex were excised. When UR was resected from the umbilicus to the duct end, urachus tissue was not pathologically detected at the resection margin. Conclusion: Our policy results in complete resection without excessive surgical invasion.

DOI： 10.1186/s12894-022-01153-x

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PubMed

Language：English   Publisher：Pediatric Surgery International  

Purpose: It is important for patients with biliary atresia (BA) to retain the native liver after Kasai portoenterostomy (PE). Laparoscopic PE (Lap-PE) is standard, whereas laparoscopic revision of PE (Lap-revision) is performed as needed. We report the medium-term outcomes of Lap-revision. Methods: We retrospectively reviewed the demographics and outcomes of 63 patients who underwent Lap-PE between 2013 and 2021. Indications for revision included recurrent jaundice, repeat cholangitis, and persistent jaundice with temporary biliary excretion following the initial PE. We compared liver transplantation (LT) data of patients who underwent Lap-revision with those of patients who did not. Results: Lap-revision was performed in 20 patients. Of those 17 (excluding the two who were jaundice-free before the age of 1, and the one who underwent open conversion for bleeding), 11 (65%) were jaundice-free 1 year after Lap-revision. The surgical parameters of LT did not differ between patients who underwent Lap-revision and those who did not. Conclusion: Lap-revision for patients with BA had a limited but positive effect on native liver survival and did not adversely affect subsequent LT. Therefore, Lap-revision could be second-line standard therapy in patients with BA who have demonstrated biliary drainage at least once after initial PE.

DOI： 10.1007/s00383-022-05235-7

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PubMed

Language：English   Publisher：Pediatric Surgery International  

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.

DOI： 10.1007/s00383-022-05230-y

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PubMed

Language：English   Publisher：Pediatric Surgery International  

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.

DOI： 10.1007/s00383-022-05243-7

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PubMed

Language：English   Publisher：BMC Pediatrics  

Background: Omphalocele is a congenital abdominal wall defect of the umbilical cord insertion site. A giant omphalocele, with a fascial defect > 5 cm in diameter and/or containing > 50% of the liver within the hernia sac, can be challenging for pediatric surgeons. Recently, negative pressure wound therapy has been reported as an effective management for giant omphaloceles; however, it is not recommended for an infected wound with necrotic tissue as it may exacerbate infection. We adopted negative pressure wound therapy with irrigation and dwell time (NPWTi-d) for a case of a ruptured giant omphalocele. Artificial membranes, followed by artificial dermis, were used to promote fibrous capsule formation, and then NPWTi-d was used to promote granulation while controlling infection. However, studies have not been conducted regarding NPWTi-d for ruptured giant omphaloceles; hence, we present our treatment experience with NPWTi-d for a giant omphalocele. Case presentation: The patient was a boy born at 38 weeks and 3 days of gestation, weighing 1896 g. He was diagnosed with a ruptured giant omphalocele with a total liver and intestine defect hole of 10 cm × 10 cm. The patient underwent silo placement using an artificial mesh, followed by plicating the artificial mesh at 4 days of age. The herniated viscera were gradually reduced into the abdominal cavity; however, the defect size was still large. Hence, a collagen-based artificial dermis was patched on the defect hole. After creating a fresh and smooth granulated tissue, NPWTi-d was applied at 33 days of age to promote granulation and control infection. We used the 3 M™ V.A.C.® Ulta Therapy Unit with 3 M™ VeraFlo™ therapy. NPWTi-d was stopped at 60 days of age when the granulation tissue was well formed including at the artificial dermis site. The wound was managed with prostandin ointment and appropriate debridement, resulting in complete epithelialization at 5 months of age. Conclusions: Artificial membranes followed by artificial dermis were used to promote a fibrous capsule and artificial dermis granulation, which protects against organ damage. NPWTi-d achieved better control of infection and promoted wound healing. NPWTi-d combined with artificial dermis can effectively treat ruptured giant omphaloceles.

DOI： 10.1186/s12887-022-03755-8

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Language：English   Publisher：Frontiers in Pediatrics  

Hernioplasty for giant inguinal hernias can cause abdominal compartment syndrome (ACS) in adults but rarely does in infants. We encountered a case of a giant bilateral inguinal hernia in infancy complicated by ACS after hernioplasty. Silo placement via a skin incision effectively treated ACS, after which the abdominal wall was safely closed. Hernioplasty performed early in the clinical course can help expand the abdominal cavity and avoid ACS. Thus, hernioplasty should be performed earlier if the hernia size in the flank space gradually increases.

DOI： 10.3389/fped.2022.1030934

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PubMed

Language：English   Publisher：Frontiers in Pediatrics  

Background: The prognosis of BA is known to be poor if definitive surgery is performed too late. Therefore, excluding BA as a diagnosis at an early stage is crucial. Conventional cholangiography requiring cannulation through the gallbladder may be unnecessarily invasive for patients, especially when ruling out BA. Therefore, a less invasive alternative such as indocyanine green (ICG) cholangiography, which does not require cannulation, should be established. In this study, we focused on excluding BA and confirmed the usefulness of intravenous ICG fluorescence cholangiography. To the best of our knowledge, this is the first preliminary study to report the use of intravenous ICG cholangiography for BA exclusion. Methods: The study participants were patients who underwent liver biopsy and intraoperative cholangiography after they were suspected to have BA, between 2013 and 2022. ICG fluorescence cholangiography was performed on all patients who provided informed consent. Results: During the study period, 88 patients underwent a laparoscopic liver biopsy and cholangiography. Among them, 65 (74%) were diagnosed with BA and underwent a subsequent laparoscopic Kasai portoenterostomy. BA was ruled out intraoperatively in 23 patients. Of the 23 patients in whom BA was ruled out, 14 underwent ICG cholangiography, 11 had gallbladder (GB) fluorescence, and 9 had both GB and common bile duct (CBD) fluorescence. Conventional cholangiography was very difficult in 2 of 23 cases: in 1 case, cannulation of the atrophic gallbladder was impossible, and cholecystectomy was indicated after multiple attempts; in 1 case, upstream cholangiography was not possible. In both cases, ICG fluorescence cholangiography successfully imaged the CBD and the GB. Conclusions: In conclusion, intravenous ICG fluorescence cholangiography might be a useful and less invasive diagnostic procedure that can rule out BA in infants.

DOI： 10.3389/fped.2022.1005879

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PubMed

Language：English   Publisher：Nagoya Journal of Medical Science  

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.

DOI： 10.18999/nagjms.84.4.716

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PubMed

Publisher：東京医学社  

DOI： 10.24479/ps.0000000254

CiNii Research

Language：English   Publisher：Surgical Endoscopy  

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.

DOI： 10.1007/s00464-022-09132-x

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Publisher：東京医学社  

DOI： 10.24479/ps.0000000238

CiNii Research

Publisher：東京医学社  

DOI： 10.24479/ps.0000000234

CiNii Research

Language：English   Publisher：Surgical Endoscopy  

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.

DOI： 10.1007/s00464-022-09185-y

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PubMed

Language：English   Publisher：International Journal of Hematology  

Central venous catheters (CVCs) are essential devices in the treatment of pediatric patients with hematological and oncological disorders; however, the most suitable type of CVC for these patients remains unclear. We retrospectively compared risk factors for unplanned removal of two commonly used CVCs, peripherally inserted central catheters (PICCs) and tunneled CVCs, to propose which is the better device. We followed 89 patients fitted with a tunneled CVC (total 21,395 catheter-days) and 84 fitted with a PICC (total 9177 catheter-days) between January 1, 2013 and December 31, 2015, until catheter removal. Patients with a PICC had a significantly higher 3-month cumulative incidence of catheter occlusion (5.2% vs. 0%, p = 4.08 × 10−3) and total unplanned removals (29.0% vs. 6.9%, p = 0.0316) than those with tunneled CVCs. However, the cumulative incidence of central line-associated bloodstream infection did not differ significantly by CVC type. Multivariable analysis identified younger age (< 2 years) [sub-distribution hazard ratio (SHR) 2.29; 95% confidence interval (CI) 1.27–4.14] and PICC (SHR 2.73; 95% CI 1.48–5.02) as independent risk factors for unplanned removal. Thus, our results suggest that tunneled CVCs are preferable in pediatric patients with hematological and oncological disorders requiring long-term, intensive treatment.

DOI： 10.1007/s12185-022-03346-4

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PubMed

Language：English   Publisher：BMC Surgery  

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.

DOI： 10.1186/s12893-022-01738-1

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PubMed

Language：English   Publisher：Familial Cancer  

Hereditary leiomyomatosis and renal cell cancer (HLRCC) is caused by heterozygous germline variants in the fumarate hydratase (FH) gene and is associated with increased susceptibility to cutaneous leiomyomas, uterine leiomyomas, and renal cell carcinoma (RCC). HLRCC-associated RCC usually occurs in the middle age, with the median age being 40–44 years. This report describes a seven-year-old (84-month-old) male who developed a large right kidney tumor with multiple cystic lesions that contained enhanced solid components. There was no evidence of distant metastasis. The male patient underwent right nephrectomy and has been recovering well without metastasis or recurrence. Pathological examination revealed that tumor cells with relatively prominent nucleoli and surrounded by halos, were located in a limited area. Immunohistochemical staining was negative for FH. Whole-exome sequencing identified his germline variant in the FH gene and its loss of heterozygosity in the tumor. At nine years (114 months) of age, the male patient showed no recurrence of the tumor. This was the youngest-onset case of HLRCC-associated RCC to date. This report may affect the starting age for future RCC-surveillance programs for patients with HLRCC.

DOI： 10.1007/s10689-021-00268-8

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Language：English   Publisher：Journal of Minimal Access Surgery  

Background: Laparoscopic definitive surgery for choledochal cyst (CC) in infants requires advanced skills because of their small size. If patients with a prenatal diagnosis of CC have any biliary symptoms, they need semi-emergency definitive surgery. This study aimed to estimate whether laparoscopic definitive surgery for CC can be performed safely and effectively in infants, especially when emergency surgery is required. Patients and Methods: From January 2006 to December 2019, 21 patients under 1 year of age underwent laparoscopically or open definitive surgery, and 16 patients aged 3-5 years underwent laparoscopic surgery for CC at our institution. In cases of prenatal diagnosis, elective surgery (EL) was performed at about 6 months of age for patients with no biliary symptoms; the semi-emergency surgery (EM) was performed when patients had any biliary symptoms. Surgical outcomes were retrospectively compared between the Lap <1 y and Op <1 y groups and between the Lap <1 y and Lap 3-5 y groups. In addition, the surgical outcomes of those who underwent EM were also evaluated. Results: Operative time was significantly longer, and blood loss was significantly lower in the Lap <1 y group than in the Op <1 y group. All surgical outcomes were similar between the Lap <1 y and Lap 3-5 y groups and between the EM and EL groups. Conclusion: Laparoscopic definitive surgery for CC in infants under 1 year of age is safe and feasible. Even semi-emergency laparoscopic surgery can be performed safely and effectively in small infants.

DOI： 10.4103/jmas.JMAS_98_21

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PubMed

Language：English   Publisher：Pediatric Surgery International  

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.

DOI： 10.1007/s00383-022-05125-y

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Language：English   Publisher：BMC Pediatrics  

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.

DOI： 10.1186/s12887-022-03207-3

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PubMed

Publisher：東京医学社  

DOI： 10.24479/pm.0000000090

CiNii Research

Language：English   Publisher：Nagoya Journal of Medical Science  

We reviewed the outcomes of meconium peritonitis and evaluated the safety and feasibility of primary radical surgery for meconium peritonitis. A total of 21 cases of meconium peritonitis between 2006 and 2020 were retrospectively reviewed. The patients were classified into two groups based on the type of surgery: group I (primary radical surgery, n = 16) and group II (multistage surgery; drainage only or ileostomy, followed by elective surgery, n = 5). Patient backgrounds and surgical outcomes were compared between the two groups. The term of prenatal diagnosis, preoperative white blood cell count, and preoperative catecholamine use were not significantly different between the two groups. Group I included more mature neonates than group II (gestational age at birth, 35w1d vs 30w1d, p = 0.02; birth weight, 2.5 kg vs 1.1 kg, p < 0.01). Preoperative C-reactive protein was significantly lower in group I (0.37 mg/dL vs 2.8 mg/dL, p < 0.05). Operation time, blood loss, time to enteral feeding, and complication rates were not significantly different between the two groups. The surgical outcomes of primary radical surgery were comparable to those of multistage surgery, although the patients’ backgrounds were different. Our strategy of selecting one-stage or multiple-stage surgery for treatment of meconium peritonitis, depending on the patient’s general condition and degree of intestinal ischemia, was reasonable.

DOI： 10.18999/nagjms.84.1.148

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.4240/wjgs.v14.i1.56

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PubMed

Language：Japanese   Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.45.0_74

CiNii Research

Language：Japanese   Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.45.0_77

CiNii Research

Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2022081278

CiNii Research

Language：English   Publisher：Pediatric Surgery International  

Purpose: The features of right-sided congenital diaphragmatic hernias (RCDHs) are quite different from those of left-sided CDHs (LCDHs). We have summarized the features of RCDHs experienced in our institution. Methods: This retrospective study analyzed the cases of patients with CDH registered at our institution between 2011 and 2020. Defects on each side were compared based on prenatal diagnosis, medical treatment, type of surgery, and outcomes. Results: A total of 101 patients underwent surgery at our institution during the neonatal period, and 11 had RCDHs. RCDHs and LCDHs were significantly different in terms of extracorporeal membrane oxygenation (36% vs. 6%, p = 0.002), patch repair (81% vs. 28%, p < 0.001), recurrence rate (36% vs. 11%, p = 0.022), and length of hospital stay (117 days vs. 51 days, p = 0.012). The severity of the fetal diagnosis did not reflect postnatal severity. All patients with RCDH survived to discharge, and there was no significant difference in survival rate between the right and left sides. Conclusion: Neonates with RCDH required more intensive treatments; however, the survival rate was comparable between RCDH and LCDH. RCDH was significantly different from LCDH and an optimal treatment strategy for RCDHs should be established.

DOI： 10.1007/s00383-021-04999-8

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PubMed

Language：English   Publisher：Pediatric Surgery International  

Purpose: Tracheomalacia (TM) is a frequent complication after esophageal atresia (EA) repair. This study aimed to review patients who underwent aortopexy for TM after EA repair and to compare their imaging features. Methods: The patients who underwent thoracoscopic EA repair and contrast-enhanced computed tomography (CECT) at our hospital between 2013 and 2020 were retrospectively reviewed. The ratio of the lateral and anterior–posterior diameter of the trachea (LAR) where the brachiocephalic artery (BCA) crosses the trachea was defined. The LAR of the patients who underwent CECT for asymptomatic pulmonary disease was set as a normal reference. The Z-score of each LAR was calculated and compared between the patients that did or did not undergo aortopexy. Results: A total of 51 patients represented the controls, 5 patients underwent aortopexy, and 12 patients were discharged without surgery. The mean LARs in the patients who underwent aortopexy, did not undergo aortopexy, and controls were 3.54, 1.54, and 1.15, respectively. The mean Z-score of the aortopexy group was 21.2. After successful aortopexy, each patient’s LAR decreased to < 1.5. Conclusion: Aortopexy was preferred if the trachea was compressed by the BCA. The LAR is a useful indicator for predicting the therapeutic effect of aortopexy.

DOI： 10.1007/s00383-021-04985-0

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PubMed

Language：English   Publisher：Pediatric Surgery International  

Purpose: Minimally invasive examinations are particularly important in pediatric patients. Although the significance of urinary N1,N12-diacetylspermine (DiAcSpm) as a tumor marker (TM) has been reported in many types of adult cancers, its usefulness in pediatric cancers has not been reported. This may be due to urinary DiAcSpm level variations with age. This study aims to measure the normal levels of urinary DiAcSpm in healthy individuals and investigate its usefulness as a TM in childhood cancer. Methods: Urinary samples were collected from pediatric patients with and without cancer. The urinary DiAcSpm levels were measured, and the values were compared. Results: A total of 32 patients with cancer and 405 controls were enrolled in the study. Of the 32 patients, 13 had neuroblastoma, 9 had malignant lymphoma (ML), and 10 had leukemia. In the control group, the urinary DiAcSpm values markedly fluctuated among those with young age, especially infants; meanwhile, the values converged among those aged roughly 10 years and above. The sensitivity of DiAcSpm was significantly different among the three types of cancers: neuroblastoma (30.8%), ML (77.8%), and leukemia (40%). Conclusion: The urinary DiAcSpm value is a useful TM for both screening and follow-up of ML.

DOI： 10.1007/s00383-021-04987-y

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Language：English   Publisher：Nagoya Journal of Medical Science  

Massive splenomegaly and hypersplenism in patients with biliary atresia after Kasai portoenterostomy were treated with partial splenic embolization or total splenectomy. We performed partial splenectomy to reduce the complications of partial splenic embolization and avoid overwhelming post-splenectomy infection. This study aimed to evaluate the long-term effects of partial splenectomy for hypersplenism on postoperative liver and spleen function in patients with biliary atresia. Among jaundice-free patients with biliary atresia who underwent Kasai portoenterostomy between January 1992 and December 2012, 15 underwent partial splenectomy for massive splenomegaly and hypersplenism at our institution. Changes in the laboratory data 10 years post partial splenectomy were retrospectively investigated, and these along with the latest data were measured. A total of four patients (27%) required living-donor liver transplantation after partial splenectomy, a proportion similar to those who did not undergo partial splenectomy. Compared to the preoperative baseline, the platelet counts were significantly higher at 1 and 3 years after surgery (p < 0.05). Aspartic aminotransferase-to-platelet ratio index was significantly lower at 1, 7, and 10 years after partial splenectomy (p < 0.05). No further surgeries were required for hypersplenism after partial splenectomy over 10 years, and there were no cases of overwhelming post-splenectomy infection after partial splenectomy. Partial splenectomy is safe and effective for the treatment of hypersplenism with biliary atresia over a long time period. It could be considered as an alternative to partial splenic embolization as it can suppress hypersplenism for a long time and induces fewer postoperative complications.

DOI： 10.18999/nagjms.83.4.765

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PubMed

Language：English   Publisher：Pediatrics International  

Background: Endoscopic variceal ligation (EVL) is a widely accepted treatment for esophagogastric varices in patients with portal hypertension (PHT). It is used for urgent treatment and prophylactic treatment of esophagogastric varices in pediatric as well as adult patients. However, major life-threatening adverse events such as early rebleeding can occur. Although early rebleeding after EVL among children and adolescents has been reported, the risk factors remain obscure. This study evaluated the risk factors for early rebleeding after EVL in children and adolescents. Methods: The subjects were children and adolescents (<18 years) with PHT who underwent EVL for esophagogastric varices. Early rebleeding was defined as hematemesis, active bleeding, or blood retention in the stomach, confirmed by esophagogastroduodenoscopy from 2 h to 5 days after EVL. Results: A total of 50 EVL sessions on 22 patients were eligible for this study. There were four episodes of early rebleeding. No other major adverse event has occurred. Multivariate analysis showed that EVL implemented at cardiac varices just below the esophagogastric junction (EGJ), within 5 mm from the EGJ, is the independent factor for a higher risk of early rebleeding: odds ratio 18.2 (95% confidence interval: 1.40–237.0), P = 0.02. Conclusions: Children and adolescents who undergo EVL for cardiac varices just below the EGJ have a higher risk of early rebleeding than those who do not.

DOI： 10.1111/ped.14614

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>A 21-month-old girl with chief complaints of vomiting and fever was admitted to a hospital and diagnosed as having congenital biliary dilatation. Magnetic resonance imaging performed on the sixth day of hospitalization revealed bile duct perforation, and the patient was transferred to our department for further treatment. A perforation approximately 2 cm in diameter was found at the confluence of the common bile duct and cystic duct. We concluded that biliary drainage and repair of the perforation would not improve her pathophysiology. Therefore, laparoscopic one-stage radical surgery was performed. In addition, bile duct plasty was performed to treat the severe intrahepatic bile duct membranous stenosis that were observed in the right and left bile ducts of the hepatic hilum. The patient was discharged without complications on the tenth postoperative day.</p><p>Two-stage surgery is the recommended treatment method for congenital biliary dilatation with biliary perforation. However, in recent years, there have been few reports showing that one-stage open radical surgery is useful. In this case, we successfully performed laparoscopic one-stage radical surgery for congenital biliary dilatation with biliary perforation. If laparoscopic radical surgery for congenital biliary dilatation is established at the institution, and the patient is in good general condition, laparoscopic one-stage radical surgery may be an effective modality for treating this condition.</p>

DOI： 10.11164/jjsps.57.5_855

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Language：Japanese  

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Language：English   Publisher：Pediatric Surgery International  

A correction to this paper has been published: https://doi.org/10.1007/s00383-021-04873-7.

DOI： 10.1007/s00383-021-04873-7

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Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2021229976

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Language：Japanese  

DOI： 10.1200/JCO.2021.39.15_suppl.e22008

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Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>The patient was a newborn female with colonic atresia and gastroschisis. A silo was placed immediately after birth and surgery was scheduled the following day. Adhesive detachment was performed by washing the intestinal tract with saline. The oral and anal segments of the colonic atresia were identified, and functional end-to-end anastomosis was performed. Severe edema precluded the immediate return of the intestines to the abdominal cavity and required ongoing treatment with a silo. The intestines gradually returned to the abdominal cavity during the following week. The abdominal wall was closed on postnatal day 9 by a sutureless method. The patient’s postoperative course was good, and full feeding was initiated on postnatal day 19. The patient was discharged from the hospital on postnatal day 32 and recovered without complications. In cases of gastroschisis with colonic atresia, a two-stage surgical procedure is commonly employed. However, functional end-to-end anastomosis using a linear stapler is also an effective method because anastomoses can be performed without regard to differences in intestinal caliber.</p>

DOI： 10.11164/jjsps.57.1_22

CiNii Research

Language：English   Publisher：Pediatric Surgery International  

Purpose: One of the main causes of stricture at hepaticojejunostomy site after surgery for congenital biliary dilatation is inflammation or infection associated with bile leak. The aim of this study was to determine the risk factors and outcomes of bile leak after laparoscopic surgery. Methods: We retrospectively reviewed the demographics and outcomes of patients who underwent laparoscopic surgery for congenital biliary dilatation between September 2013 and December 2019. Data from patients with bile leak were compared to data from patients without bile leak. Results: Fourteen of 78 patients had bile leak. Hepatic duct diameter at anastomosis was the only risk factor of bile leak. Patients with the diameter ≤ 10 mm had higher incidence of bile leak than in patients with the diameter > 10 mm (P = 0.0023). Among them, bile leak occurred more frequently in patients operated on by non-qualified surgeons based on the Japan Society for Endoscopic Surgery endoscopic surgical skill qualification system than by qualified surgeons (P = 0.027). However, none of the patients with bile leak developed anastomotic stricture afterwards. Conclusion: Although good technical skill is necessary to avoid bile leak in narrow hepatic duct cases (≤ 10 mm), slight bile leak may not result in anastomotic stricture.

DOI： 10.1007/s00383-020-04791-0

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Language：English   Publisher：Pediatric Surgery International  

Purpose: This study aimed to investigate the negative effects of intestinal obstruction for jaundice-free native liver survival after Kasai portoenterostomy (PE) for biliary atresia (BA). Methods: We retrospectively reviewed the records of patients who underwent PE for BA between 2006 and 2019. We evaluated the postoperative morbidity of intestinal obstruction for up to 2 years after PE and the effects of intestinal obstruction on jaundice-free native liver survival. On the basis of their initial operation, patients were divided into open portoenterostomy (Open-PE) and laparoscopic portoenterostomy (Lap-PE) groups, and morbidity was compared. Results: Of the 87 patients reviewed, 6 (6.9%) patients developed postoperative intestinal obstruction and underwent surgery to relieve the obstruction. The morbidity of early postoperative intestinal obstruction was 1.68 per 10,000 person days. The jaundice-free native liver survival rate among patients who once achieved jaundice-free status after PE was significantly lower in the patients with intestinal obstruction compared to in those without intestinal obstruction (0% vs. 73.8%; RR = 3.81, p = 0.007). No significant differences were seen in postoperative intestinal obstructions between the Open-PE and Lap-PE groups (p = 0.242). Conclusions: Intestinal obstruction negatively impact jaundice-free native liver survival, even in patients who once achieved jaundice-free status after PE for BA.

DOI： 10.1007/s00383-020-04807-9

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Language：English   Publisher：Pediatric Surgery International  

Purpose: Although we generally perform thoracoscopic lobectomy for congenital lung cysts (CLCs), we recently began performing thoracoscopic-limited pulmonary resection (segmentectomy or small partial lung resection) on relatively small lesions and on lesions involving multiple lobes. Our study aimed to determine the therapeutic outcomes of thoracoscopic CLC surgery. Methods: We retrospectively reviewed patients aged ≤ 18 years who underwent their first CLC surgery at our facility between 2013 and 2020. Results: A comparison between patients < 4 months old and those ≥ 4 months old showed no significant difference in operating time or incidence of complications. Blood loss volume (mL/kg) was significantly greater in patients < 4 months old and in patients who had undergone semi-urgent or urgent surgery. Operating time and postoperative complications were not increased in semi-urgent or urgent surgeries. There was no significant difference in operating time, blood loss volume, or postoperative complications between patients with a preoperative history of pneumonia and patients with no such history. Conclusion: In most patients, thoracoscopic surgery for CLC was safely performed. Limited pulmonary resection is considered difficult to perform thoracoscopically in children, but can be safely performed using new devices and navigation methods. Level of evidence: III.

DOI： 10.1007/s00383-020-04793-y

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Language：Japanese   Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.44.0_50

CiNii Research

Language：Japanese   Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.44.0_72

CiNii Research

Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2021085587

CiNii Research

Language：English   Publisher：BMC Surgery  

Background: In cases of Hirschsprung disease, complete and reproducible resection of the aganglionic bowel is ideal to achieve good postoperative bowel function. Reliable identification of the upper margin of the surgical anal canal, which is the squamous-columnar junction, is necessary during transanal pull-through. Here, we describe a novel staining technique using Lugol’s iodine stain to visualize the upper margin of the surgical anal canal. Methods: Lugol’s iodine staining was performed in five patients with Hirschsprung disease treated using a single-stage laparoscopic transanal pull-through modified Swenson procedure. In two of these patients, endocytoscopic observation with ultra-high magnification was performed using methylene blue and crystal violet to mark the border of the squamous epithelium at 1 week before surgery. The alignment between the incisional line, which was revealed using Lugol’s iodine staining and endocytoscopic marking, was evaluated. Complications, including postoperative bowel dysfunction, were evaluated. Results: In all cases, Lugol’s iodine staining produced a well-demarcated line. The endocytoscopic marking of the upper margin of the surgical anal canal was aligned with the line revealed by Lugol’s iodine staining. There were no complications associated with the transanal pull-through procedure, including postoperative bowel dysfunction. Conclusions: Lugol’s iodine staining could be a safe and practical method to visualize the upper margin of the surgical anal canal intraoperatively. This finding may be useful for surgeons to make a consistent removal of the aganglionic bowel during surgery for Hirschsprung disease.

DOI： 10.1186/s12893-020-00986-3

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Language：English   Publisher：BMC Pediatrics  

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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Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2020230886

CiNii Research

Language：Japanese   Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.43.0_84

CiNii Research

Language：Japanese   Publisher：日本膵・胆管合流異常研究会  

DOI： 10.34410/jspbm.43.0_36

CiNii Research

Language：Japanese  

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Publisher：(株)東京医学社  

DOI： 10.24479/j00645.2020041175

CiNii Research

Language：English   Publisher：Pediatric Surgery International  

Purpose: High-dose postoperative steroid therapy after Kasai portoenterostomy is reported to improve jaundice clearance and a strong anti-inflammatory activity might prevent fibrous tissue formation which is often observed at the porta hepatis in revision surgery. We started steroid pulse therapy for the patients with cessation of decrease in jaundice and aimed to evaluate the efficacy in this study. Methods: The demographics and outcomes of patients who underwent laparoscopic Kasai portoenterostomy and received steroid pulse therapy within 2 months postoperatively between September 2014 and December 2018 were retrospectively reviewed; the therapy was determined successful when the serum total bilirubin level decreased to or below two-thirds of the pre-therapy level after 2 weeks. Patient data in the successful group were compared with those in the unsuccessful group. Results: Steroid pulse therapy was successful in seven of 16 patients (43.8%). The percentage of patients whose serum total bilirubin level decreased to normal was significantly higher in the successful group at 3 months (85.7% vs. 11.1%, P = 0.0028) and after all (100% vs. 33.3%, P = 0.011). Conclusions: Steroid pulse therapy was effective for some patients. Unsuccessful cases may have little chances of jaundice clearance; revision Kasai portoenterostomy would be a good option.

DOI： 10.1007/s00383-019-04537-7

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Language：Japanese  

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Language：English   Publisher：Hepatology Research  

Aim: Esophagogastric variceal hemorrhage is a cause of poor prognosis in patients with biliary atresia (BA). To prevent variceal hemorrhage, simple and reliable screening methods for high-risk esophagogastric varices (HR-EGV) are needed. We evaluated the efficacy of liver stiffness (LS) and spleen stiffness (SS) as measured by 2-D shear wave elastography (2D-SWE), which was reported to be more accurate than transient elastography, for detecting HR-EGV in children with BA. Methods: Thirty-four children with BA were enrolled. Both LS and SS were measured by 2D-SWE. The presence of large esophageal varices or esophageal varices of any size with red wale marking and/or the presence of gastric varices along the cardia were defined as HR-EGV. Clinical data were collected and previously reported prediction indices for varices were calculated. Results: Liver stiffness and SS were obtained from all patients. Fourteen patients showed HR-EGV. Significantly different variables between patients with and without HR-EGV were as follows: spleen diameter (116 mm vs. 95 mm), clinical prediction rule (104.7 vs. 124.7), King's variceal prediction score (78.8 vs. 99.4), aspartic aminotransferase-to-platelet ratio index (2.03 vs. 0.98), LS (2.63 m/s vs. 1.87 m/s), and SS (4.44 m/s vs. 3.69 m/s). The highest area under the receiver operating characteristic curve for detecting HR-EGV was that for SS (0.900), and the cut-off SS of 4.12 m/s yielded 92.9% sensitivity and 90% specificity. The intraclass correlation coefficient for intra-observer reproducibility was 0.828. Conclusions: Spleen stiffness from 2D-SWE offered the most accurate predictor of HR-EGV in BA children.

DOI： 10.1111/hepr.13381

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Language：English   Publisher：Journal of Plastic, Reconstructive and Aesthetic Surgery  

Background: The aim of this study was to demonstrate the feasibility and safety of the Nuss procedure for patients with pectus excavatum (PE) with a history of intrathoracic surgery. Patients: From April 2010 to December 2013, we performed 6 cases of PE repair in patients with a history of intrathoracic surgery. The causes of previous operations were congenital cystic adenomatoid malformation in 4 patients and congenital diaphragmatic hernia in 2. The patients’ median age was 5 years (range, 4–9 years) and median preoperative pectus severity index was 4.63 (range, 3.42–10.03). Their intraoperative and postoperative courses were reviewed retrospectively. Results: The mean overall operation time was 127.5 ± 17.0 minutes, and the mean operation time for endoscopic pneumolysis was 28.8 ± 12.3 minutes. Intraoperative exploration for pleural adhesion revealed that the endoscopic approach in the previous operation was associated with low pleural adhesion, and the open thoracotomy or laparotomy approach was associated with low to high pleural adhesion. One patient developed a pneumothorax on the first postoperative day. All the other patients had uneventful postoperative courses. All the patients received bar removal 2–3 years after bar insertion. One patient developed atelectasis after bar removal. All the other patients had an uneventful postoperative course. The mean postoperative follow-up time after bar removal was 20.1 ± 14.7 months. Conclusions: History of intrathoracic surgery seems not a contraindication for the Nuss procedure. However, perioperative complications should be carefully monitored in both the bar insertion and removal operations.

DOI： 10.1016/j.bjps.2018.12.027

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PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1080/08941939.2017.1366603

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PubMed

Language：English   Publisher：Clinical Radiology  

Aim: To present the technique and the diagnostic accuracy of the air test to diagnose Hirschsprung's disease (HD). Materials and methods: Children who attended hospital for chronic constipation (CC) between January 2012 and December 2016 for whom the air test was performed were enrolled. The test was conducted during contrast enema under fluoroscopic observation using 20–50 ml injections of air into the rectum through a 10 F Nelaton catheter. The demographics, results of the air test, and additional examinations, as well as the outcomes of subsequent treatments were analysed retrospectively. Results: The air test was conducted in 179 patients (median: 3 years, range: 0–14 years), and was positive in 150 and negative in 29 cases. Of the 29 patients with negative results, four were diagnosed with HD by rectal suction biopsy (RSB). Of the remaining 25 patients, RSB was conducted in seven and HD was excluded in all cases. In all 150 patients with positive air test results, CC was adequately controlled with conservative treatment. The sensitivity and specificity of the air test were 100% (4/4) and 85.7% (150/175), respectively. Conclusions: The air test can be used as a new non-invasive screening method for HD, performed simultaneously with contrast enema.

DOI： 10.1016/j.crad.2018.08.008

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Language：English   Publisher：Pediatric Surgery International  

Purpose: We previously showed that meticulous probing and resection of the intrahepatic bile duct stenosis (IHBDS)-causing membrane or septum was effective in preventing hepatolithiasis after choledochal cyst excisions in open surgeries. Then, we introduced this maneuver into laparoscopic choledochal cyst excisions in 2014 and performed routine resections since then. The aim of this study was to show the feasibility of this method in laparoscopic surgery. Methods: We retrospectively reviewed the demographics and outcomes of patients who underwent laparoscopic choledochal cyst excisions at our hospital between January 2014 and December 2017. The patients who underwent surgical treatment for IHBDS-causing membrane or septum were compared with those who did not undergo the procedure. The outcomes of the patients with IHBDS were also compared between patients who were ≥ 3 years of age and those < 3 years at operation. Results: Seventeen of 35 patients underwent laparoscopic resection of IHBDS-causing membrane or septum. There were no complications related to the procedure although the operative time and intraoperative bleeding amount increased in the patients with IHBDS who were ≥ 3 years of age. Conclusions: Meticulous probing and excision of the IHBDS-causing membrane or septum is safe and feasible during laparoscopic choledochal cyst excision.

DOI： 10.1007/s00383-018-4320-7

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Language：English   Publisher：Pediatric Surgery International  

Purpose: Biliary atresia (BA) is characterized by progressive inflammation of the biliary system. This inflammation persists and causes liver fibrosis, although jaundice disappears after Kasai portoenterostomy (KP). We aimed to confirm whether the oral administration of eicosapentaenoic acid (EPA) suppresses liver fibrosis in postoperative patients with BA. Methods: We reviewed patients who underwent laparoscopic KP (lapKP) between January 2014 and September 2017. From December 2016, 30 mg/kg/day of EPA was orally administered to patients who opted to take medicine (EPA group). Patients who did not receive EPA were assigned to the non-EPA group. Mac-2 binding protein sugar chain modified isomer (M2BPGi) and hyaluronic acid (HA) levels were compared between the two groups in patients showing disappearance of jaundice at 6 months after the first lapKP. Results: Seventeen patients in the non-EPA group and 11 in the EPA group were enrolled. At 6 months after the first lapKP, 10 patients in the non-EPA group and six in the EPA group were without jaundice. M2BPGi and HA levels were significantly lower in the EPA group. Conclusions: Liver fibrosis was suppressed in patients without jaundice 6 months after lapKP, who were administered EPA. We believe that periductular inflammation was alleviated by EPA supplementation.

DOI： 10.1007/s00383-018-4313-6

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Language：English   Publisher：Pediatric Surgery International  

Purpose: Laparoscopic-assisted anorectoplasty (LAARP) is a minimally invasive procedure in which the levator ani muscle is left to potentially improve postoperative fecal continence. This study aimed to retrospectively evaluate postoperative complications and long-term outcomes of fecal continence after LAARP and compare them to those after posterior sagittal anorectoplasty (PSARP). Methods: Forty-five male patients with high and intermediate anorectal malformation (aged ≥ 4 years) who underwent LAARP or PSARP (LAARP 29, PSARP 16) from 1999 to 2013 were included. Postoperative complications and postoperative fecal continence were retrospectively evaluated and compared. Postoperative complications were also compared before and after introducing a urethroscope during fistula resection. Results: Complications after LAARP and PSARP were seen in 12 vs. 2 cases (p = 0.09) of mucosal prolapse and in 9 vs. 1 case (p = 0.07) of posterior urethral diverticulum (PUD), respectively. The incidence of PUD decreased after introduction of a urethroscopy from 40% in 8/20 cases to 11% in 1/9 cases. No significant difference was found in terms of fecal continence in both groups. Conclusion: Through our study, greater improvement of postoperative fecal continence after LAARP has not been shown. LAARP was at higher risk for mucosal prolapse and PUD. However, precise dissection of the urethral fistula could be performed after the introduction of urethroscopy.

DOI： 10.1007/s00383-018-4323-4

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Pediatric Surgery International  

Purpose: Anovestibular fistula (AVF) is the most common type of anorectal malformation in females. Delayed anorectoplasty with fistula dilatation is commonly performed during infancy; however, we have been actively performing anorectoplasty in neonates. We report the surgical complications and postoperative defecation function associated with single-stage anorectoplasty performed in neonates. Methods: Patients who underwent surgery for AVF between 2007 and 2017 at two institutions were retrospectively studied. The operation time, amount of bleeding, time to start oral intake, perioperative complications, and Kelly’s score were compared among patients who underwent surgery as neonates and those who underwent surgery as infants. Results: Eighteen neonates and 17 infants underwent anterior sagittal anorectoplasty. The median operation time and time to start oral intake were significantly shorter in the neonatal group (72 min; 3 days, respectively) than in the infant group (110 min, p = 0.0002; 5 days, p = 0.0024, respectively). Postoperative wound disruption was significantly more frequent in the infant group. Of the ten patients each in the neonatal and infant groups, there was no significant difference in Kelly’s score at age ≥ 4 years. Conclusion: Single-stage anorectoplasty in neonates with AVF can be feasibly performed and does not impair postoperative defecation function. Levels of evidence: III.

DOI： 10.1007/s00383-018-4324-3

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Language：English   Publisher：Pediatric Surgery International  

Purpose: The Soave procedure (SO) is performed most commonly for Hirschsprung disease. SO reduces the risk of injury to the pelvic structures; however, a residual aganglionic muscle cuff could interfere with bowel movement and lead to obstructive enterocolitis. The Swenson procedure is considered ideal in terms of peristalsis. Currently, laparoscopic surgery provides better visualization and facilitates precise dissection, possibly leading to feasible performance of the laparoscopic modified Swenson procedure (SW). We present our operative technique and the efficacy of the SW compared with that of SO. Methods: We retrospectively reviewed the records of 16 and 27 patients who underwent SW and SO, respectively, between 2012 and 2017. Results: Operative time, blood loss, length of stay, and frequency of bowel movements showed no significant difference between the two groups. In the SW group, temporary dysuria occurred in one patient, postoperative enterocolitis in two, wound infection in one, and severe perianal excoriation in four, whereas in the SO group, obstructive symptoms occurred in three patients, small-bowel obstruction in one, and severe perianal excoriation in three. The complications and outcomes were comparable between both groups. Conclusion: Laparoscopic SW was safe and feasible for the short-term follow-up outcomes.

DOI： 10.1007/s00383-018-4318-1

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Publisher：金原出版  

DOI： 10.18888/op.0000000707

CiNii Research

Language：Japanese   Publisher：The Japanese Society of Pediatric Surgeons  

<p>The patient was a 5-year-old boy who had no medical history. He was referred to us with suspected acute appendicitis and complaints of fever and lower abdominal pain. Although his abdominal pain was intense, rebound tenderness and muscular protection were negative. There were no symptoms of problems in the digestive tract such as vomiting or diarrhea. His blood examination revealed an increase in inflammatory reactions, as indicated by the increased white blood cell count and C-reactive protein levels. Computed tomography (CT) revealed wall thickening of the ascending colon; therefore, we started antibiotic administration after acute enterocolitis was diagnosed. After admission, we performed CT again and found a fatty and dense mass with a swirling structure inside the mass; therefore, we diagnosed the patient as having idiopathic omental torsion. Because the symptoms were relieved, conservative management was continued and the patient was discharged on the fourth day after hospitalization. Omental torsion is a rare condition, particularly seen in children; its classical treatment option is surgery. Although conservative management was successful in this case, there are few reports on it. There are many cases where surgery is performed after an initial diagnosis of acute appendicitis is made owing to the presence of similar symptoms. However, in recent years, because of advances in imaging diagnostic techniques such as CT, more patients are being accurately diagnosed. We believe that if patients are accurately diagnosed, surgery will always be required; however, conservative management is sometimes possible.</p>

DOI： 10.11164/jjsps.54.2_302

CiNii Research

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.jpedsurg.2017.08.056

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DOI： 10.1007/s00383-017-4140-1

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.18999/nagjms.79.3.415

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1089/lap.2016.0207

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.4103/0972-9941.181771

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.18999/nagjms.78.4.447

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1007/s00383-016-3939-5

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Language：English   Publishing type：Research paper (scientific journal)  

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1155/2015/173014

PubMed

Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1016/j.jpedsurg.2005.12.036

PubMed

Event date： 2014

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

A 12-year-old boy was referred to our hospital with suspected lymphoma when a splenic tumor was detected incidentally on a computed tomography (CT) scan following a fall. Magnetic resonance imaging (MRI) showed a round mass, 5 cm &times; 4.5 cm &times; 4.5 cm in size, in the splenic hilum that was hypointense on T1-weighted and hyperintense on T2-weighted imaging. On contrast-enhanced MRI, the mass was strongly enhanced almost homogeneously from the early phase, and the contrast effect was prolonged. A preoperative diagnosis of hemangioma or vascular tumor was made, and laparotomic partial splenectomy was performed. Doppler echocardiography was used to confirm the presence of blood flow between the tumor and healthy tissue, and peripheral branches of the splenic artery were sequentially clamped to identify the vessels feeding the tumor. Indigo carmine was injected via the tumor feeding vessels, and the incision line was determined so as to minimize hemorrhage during the partial resection. The remaining spleen was fixed to the greater omentum to prevent torsion. The patient's postoperative course was uneventful, and he was discharged from hospital five days postoperatively. The pathological diagnosis was cord capillary hemangioma.

Event date： 2010

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2014

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Objective : Patients with hemophilia may show only a slight prolongation of the activated partial thromboplastin time (APTT) and the diagnosis may be overlooked during conventional preoperative testing. In the present study, an attempt was made to ensure identification of patients with latent hemophilia by setting the APTT cutoff value at 35.1 s (reference cutoff value at our hospital, 40.0 s) during testing prior to planned surgery.<BR>Method : The subjects comprised 445 patients who underwent testing of the coagulation profile prior to planned surgery between April 1, 2011 and November 30, 2013. A detailed medical history was obtained from the patients with APTT &ge;35.1 s ; those who gave no history of coagulation abnormalities were excluded. All the remaining patients were subjected to further testing (prothrombin time, repeat APTT, fibrinogen, antithrombin III, double-filtration plasmapheresis, D-dimer, and factor VIII, IX, and vW activity) to confirm the presence or absence of hemophilia.<BR>Results : The APTT was &ge;35.1 s in 40 patients (9.0%), of which 10 were excluded based on their negative medical history. The final diagnoses of the remaining 30 patients were as follows : no coagulation abnormalities, n=28 ; normalization during follow-up, n=1 ; hemophilia A, n=1. The patient with hemophilia A had no family history and the initial APTT value was within the reference cutoff value at 39.5 s.<BR>Conclusion : Efforts must be made to ensure preoperative identification of patients who may have undiagnosed latent hemophilia. Screening using an APTT cutoff value lower than the current reference value is required in order to ensure identification during preoperative testing.

Event date： 2009

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2008.7

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2008

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2007.7

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2007.5

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

We report a case of a patient with mesenteric lymphangioma who presented with small-bowel volvulus. The patient was a 6-year-old boy who, from the age of 3 years, had presented with abdominal pain and vomiting but had not been diagnosed. He presented to our Unit with a 5-day history of intermittent abdominal pain and vomiting. CT of abdomen showed not only the mass but also a &ldquo;whirlpool sign&rdquo; suggestive of small-bowel volvulus. At surgery the bowel was found to be loosely twisted with mesenteric lymphangioma.

Event date： 2007

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan  

Event date： 2006

Language：Japanese   Presentation type：Oral presentation (general)  

Country：Japan