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1.
Cancer Sci ; 115(5): 1634-1645, 2024 May.
Article in English | MEDLINE | ID: mdl-38411285

ABSTRACT

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.


Subject(s)
Biomarkers, Tumor , Homovanillic Acid , Neuroblastoma , Vanilmandelic Acid , Humans , Neuroblastoma/urine , Neuroblastoma/diagnosis , Male , Female , Risk Assessment , Child, Preschool , Biomarkers, Tumor/urine , Infant , Homovanillic Acid/urine , Vanilmandelic Acid/urine , Child , Catecholamines/urine , Case-Control Studies , Dopamine/urine , Dopamine/analogs & derivatives , Chromatography, Liquid
2.
Hepatol Res ; 54(4): 347-357, 2024 Apr.
Article in English | MEDLINE | ID: mdl-37843392

ABSTRACT

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.

3.
Surg Today ; 54(3): 211-219, 2024 Mar.
Article in English | MEDLINE | ID: mdl-36729255

ABSTRACT

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.


Subject(s)
Esophageal Atresia , Esophagoplasty , Tracheomalacia , Humans , Infant , Tracheomalacia/surgery , Tracheomalacia/complications , Esophageal Atresia/surgery , Trachea/surgery , Treatment Outcome
4.
Pediatr Surg Int ; 40(1): 91, 2024 Mar 25.
Article in English | MEDLINE | ID: mdl-38526644

ABSTRACT

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


Subject(s)
Gastroesophageal Reflux , Humans , Gastroesophageal Reflux/complications , Gastroesophageal Reflux/diagnosis , Female , Child , Fundoplication/methods , Male , Child, Preschool , Infant , Diagnosis, Differential , Torticollis
5.
Pediatr Surg Int ; 40(1): 197, 2024 Jul 17.
Article in English | MEDLINE | ID: mdl-39017936

ABSTRACT

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


Subject(s)
Biliary Atresia , Liver Transplantation , Portoenterostomy, Hepatic , Humans , Biliary Atresia/surgery , Biliary Atresia/complications , Biliary Atresia/mortality , Retrospective Studies , Female , Male , Infant , Risk Factors , Portoenterostomy, Hepatic/methods , Survival Rate/trends , Bile , Prognosis , Child, Preschool , Jaundice/etiology , Liver
6.
Pediatr Surg Int ; 40(1): 149, 2024 Jun 03.
Article in English | MEDLINE | ID: mdl-38829446

ABSTRACT

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


Subject(s)
Esophageal Atresia , Thoracoscopy , Tracheoesophageal Fistula , Humans , Tracheoesophageal Fistula/surgery , Tracheoesophageal Fistula/complications , Esophageal Atresia/surgery , Esophageal Atresia/complications , Infant, Newborn , Thoracoscopy/methods , Male , Female , Retrospective Studies , Treatment Outcome , Infant, Low Birth Weight , Anastomotic Leak/surgery
7.
Pediatr Surg Int ; 40(1): 196, 2024 Jul 17.
Article in English | MEDLINE | ID: mdl-39017953

ABSTRACT

PURPOSE: This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE). METHODS: This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve. RESULTS: Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 ± 6.2 years. At the 5th year after KPE, PHT was found in 37.8% of the subjects (n = 121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT + ve subjects had a higher bilirubin level (27.1 ± 11.7 vs 12.3 ± 7.9 µmol/L, p = 0.000) and persistent jaundice conferred a higher risk for PHT (OR = 12.9 [9.2-15.4], p = 0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75). CONCLUSIONS: In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow-up. LEVEL OF EVIDENCE: Level III.


Subject(s)
Biliary Atresia , Cholestasis , Hypertension, Portal , Portoenterostomy, Hepatic , Humans , Biliary Atresia/surgery , Biliary Atresia/complications , Portoenterostomy, Hepatic/methods , Male , Female , Hypertension, Portal/etiology , Infant , Cholestasis/etiology , Postoperative Complications/epidemiology , Prospective Studies , Follow-Up Studies , Survivors/statistics & numerical data , Infant, Newborn , Child, Preschool
8.
Surg Endosc ; 37(8): 6408-6416, 2023 08.
Article in English | MEDLINE | ID: mdl-36947222

ABSTRACT

BACKGROUND: Pediatric endoscopic surgery has become an alternative to conventional techniques with the development of medical equipment. However, there is no formal, standardized curriculum for pediatric endoscopic surgery, and its requirement remains elusive. The purpose of this study is to determine the baseline knowledge of pediatric surgeons that is considered essential for pediatric laparoscopic and thoracoscopic surgery. METHODS: Sixteen web-based multiple-choice questions based on the fundamental cognitive knowledge of pediatric endoscopic surgery were administered. The questions were created based on the fields covered by the Fundamentals of Laparoscopic Surgery ™ (FLS) certification examination blueprints and eight specific diseases of pediatric surgery. Pediatric surgeons and pediatric surgical trainees participated in this study voluntarily. RESULTS: A total of 122 surgeons participated through the Japanese Society of Pediatric Surgeons. The response rate was 95% (122/128). The total mean examination score of all participants was 79.4% (77.3-81.4%). There were no significant differences in total scores between the board-certified pediatric surgeons without an endoscopic surgical skill qualification and the non-board-certified pediatric surgeons (80.4% vs. 77.1%, p = 0.12). The endoscopic surgical skill-qualified surgeons had significantly higher percentages of correct responses in specific subjects than board-certified pediatric surgeons and surgeons without pediatric board certification (94.3% vs. 82.9%, p = 0.02; 94.3% vs. 77.5%, p = 0.0002). The FLS original subjects' scores were not significantly different among them. The mean score of surgeons who had experienced more than 200 cases of endoscopic surgery, including adult cases, was 83.2% (80.4-85.9%). CONCLUSIONS: A knowledge gap exists between surgeons, board-certified pediatric surgeons, and endoscopic surgical skill-qualified surgeons in Japan. In the field of pediatric surgery, an effective formal curriculum, such as FLS, is required to help address this vast knowledge gap for the safe conduct of endoscopic surgeries.


Subject(s)
Comprehension , Laparoscopy , Adult , Humans , Child , Cross-Sectional Studies , Japan , Clinical Competence , Laparoscopy/education
9.
BMC Pediatr ; 23(1): 376, 2023 07 25.
Article in English | MEDLINE | ID: mdl-37491193

ABSTRACT

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.


Subject(s)
Duodenal Obstruction , Immunoglobulin G4-Related Disease , Female , Humans , Child , Immunoglobulin G4-Related Disease/diagnosis , Immunoglobulin G4-Related Disease/pathology , Duodenal Obstruction/diagnostic imaging , Duodenal Obstruction/etiology , Ulcer , Liver/pathology
10.
Pediatr Int ; 65(1): e15454, 2023 Jan.
Article in English | MEDLINE | ID: mdl-36541813

ABSTRACT

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.


Subject(s)
Biliary Atresia , Esophageal and Gastric Varices , Varicose Veins , Child , Humans , Sclerotherapy/adverse effects , Sclerotherapy/methods , Sclerosing Solutions/adverse effects , Biliary Atresia/therapy , Biliary Atresia/complications , Retrospective Studies , Endoscopy, Gastrointestinal/methods , Varicose Veins/complications , Varicose Veins/drug therapy , Esophageal and Gastric Varices/etiology , Esophageal and Gastric Varices/therapy , Gastrointestinal Hemorrhage/therapy , Gastrointestinal Hemorrhage/complications
11.
Surg Today ; 53(5): 628-632, 2023 May.
Article in English | MEDLINE | ID: mdl-36282326

ABSTRACT

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.


Subject(s)
Anorectal Malformations , Rectal Prolapse , Humans , Infant , Rectal Prolapse/surgery , Rectal Prolapse/etiology , Anorectal Malformations/surgery , Follow-Up Studies , Rectum/surgery , Treatment Outcome , Anastomosis, Surgical/adverse effects , Postoperative Complications/etiology , Recurrence
12.
Surg Today ; 2023 Oct 13.
Article in English | MEDLINE | ID: mdl-37831144

ABSTRACT

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.

13.
Surg Today ; 53(12): 1363-1371, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37087700

ABSTRACT

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.


Subject(s)
Esophageal Atresia , Surgeons , Tracheoesophageal Fistula , Child , Humans , Tracheoesophageal Fistula/surgery , Tracheoesophageal Fistula/complications , Esophageal Atresia/surgery , Prospective Studies , Anastomosis, Surgical , Postoperative Complications/epidemiology , Learning Curve , Thoracoscopy/methods , Retrospective Studies
14.
BMC Surg ; 23(1): 333, 2023 Nov 01.
Article in English | MEDLINE | ID: mdl-37915052

ABSTRACT

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.


Subject(s)
Enterostomy , Hirschsprung Disease , Infant, Newborn , Infant , Humans , Hirschsprung Disease/surgery , Retrospective Studies , Anal Canal/surgery , Ileostomy
15.
Pediatr Surg Int ; 39(1): 271, 2023 Sep 09.
Article in English | MEDLINE | ID: mdl-37684432

ABSTRACT

PURPOSE: To ensure the safe spread of pediatric endoscopic surgery, it is essential to build a training curriculum, and a survey of the current situation in Japan is necessary. The present study assessed an efficient training curriculum by clarifying instructor class pediatric surgeons' experiences, including autonomy when performing advanced endoscopic surgeries. METHODS: An online nationwide questionnaire survey was conducted among pediatric surgeons who had Endoscopic Surgical Skill Qualification (ESSQ) and board-certified instructors who had skills comparable to ESSQ. We assessed participants' training experience, opinions concerning the ideal training curriculum, and the correlation between surgical experience and the level of autonomy. The Zwisch scale was used to assess autonomy. RESULTS: Fifty-two participants responded to the survey (response rate: 86.7%). Only 57.7% of the respondents felt that they had received sufficient endoscopic surgery training. Most respondents considered an educational curriculum for endoscopic surgery including off-the-job training essential during the training period. Autonomy had been acquired after experiencing two to three cases for most advanced endoscopic surgeries. CONCLUSION: This first nationwide survey in Japan showed that instructor class pediatric surgeons acquired autonomy after experiencing two to three for most advanced endoscopic surgeries. Our findings suggest that training, especially off-the-job training, has been insufficient.


Subject(s)
Specialties, Surgical , Surgeons , Humans , Child , Japan , Curriculum , Endoscopy
16.
Pediatr Surg Int ; 39(1): 261, 2023 Sep 03.
Article in English | MEDLINE | ID: mdl-37660350

ABSTRACT

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.


Subject(s)
Choledochal Cyst , Laparoscopy , Robotic Surgical Procedures , Humans , Child , Retrospective Studies , Treatment Outcome
17.
Pediatr Surg Int ; 39(1): 179, 2023 Apr 11.
Article in English | MEDLINE | ID: mdl-37041419

ABSTRACT

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.


Subject(s)
Enterocolitis, Necrotizing , Signal Transduction , Infant, Newborn , Humans , Enterocolitis, Necrotizing/pathology , Proto-Oncogene Proteins c-myc/genetics , Proto-Oncogene Proteins c-myc/metabolism , Intestines/pathology , Inflammation , Sequence Analysis, RNA
18.
Hepatol Res ; 52(5): 479-487, 2022 May.
Article in English | MEDLINE | ID: mdl-35106887

ABSTRACT

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

19.
Surg Endosc ; 36(10): 7352-7359, 2022 Oct.
Article in English | MEDLINE | ID: mdl-35226163

ABSTRACT

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.


Subject(s)
Choledochal Cyst , Cholestasis , Laparoscopy , Lithiasis , Liver Diseases , Bile , Bile Ducts/surgery , Child , Choledochal Cyst/surgery , Cholestasis/surgery , Humans , Lithiasis/surgery , Liver Diseases/surgery , Retrospective Studies
20.
Surg Endosc ; 36(8): 6035-6048, 2022 08.
Article in English | MEDLINE | ID: mdl-35312850

ABSTRACT

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.


Subject(s)
Esophageal Atresia , Tracheoesophageal Fistula , Child , Esophageal Atresia/surgery , Humans , Infant, Newborn , Minimally Invasive Surgical Procedures , Pilot Projects , Thoracoscopy/methods , Tracheoesophageal Fistula/surgery
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