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Objectives: The usefulness of omega-3 lipid emulsions has been extensively studied. The objectives of the present study were to examine the effect of an omega-3 lipid emulsion in reducing oxidative stress in a rat model of intestinal ischemia-reperfusion injury and the underlying mechanism.Methods: A total of 66 rats were divided into three dietary groups (lipid-free, soybean oil, and fish oil groups). Each animal was administered total parenteral nutrition for 3 days, followed by induction of intestinal ischemia for 100 min. Animals subjected to sham surgery served as the controls. Intestinal tissue and blood were harvested 6 and 12 h after the surgery, then, assessment of the histological damage score, plasma-related parameters, and statistical evaluation were performed.Results: The histological damage score in the intestinal tissues was significantly lower in the fish oil group than in the soybean oil group (P = 0.0121). The late-phase urinary level of 8-hydroxy-2-deoxyguanosine was also significantly lower in the fish oil group as compared with that in the other groups (P = 0.0267). Furthermore, the plasma level of high-mobility group box 1 protein was also significantly lower in the fish oil group as compared with that in the lipid-free group (P = 0.0398).Conclusion: It appeared that intravenous administration of an omega-3 lipid emulsion prior to ischemia-reperfusion injury reduced the oxidative stress and severity of tissue damage. Modification of membrane fatty acids may serve as the mechanism underlying this reduction of tissue damage. [ABSTRACT FROM AUTHOR]

Purpose: To assess the impact of the relationship between renal pedicles and tumors on surgical outcomes in patients with non-high-risk abdominal neuroblastoma., Methods: We retrospectively analyzed cases of neuroblastoma without metastasis treated at our hospital between March 2002 and December 2023. Cases in which surgical resection was performed were divided into three groups according to imaging findings at the time of diagnosis and before surgery: Group E (tumor encasing renal pedicles), Group C (tumor in contact with renal pedicles), and Group S (tumor separated from renal pedicles)., Results: Among 256 neuroblastoma cases diagnosed during the study period, 27 non-high-risk cases that underwent surgery for partial abdominal tumor resection or greater were included. The numbers of cases in the S group, C group, and E group, respectively, were 7, 9, and 11 at diagnosis, and 8, 14, and 5 before surgery. Renal complications (combined concurrent renal resection and post-operative renal atrophy) were seen in five E group cases at the time of diagnosis, and two C group cases and three E group cases preoperatively., Conclusion: In non-high-risk abdominal neuroblastomas, tumors encased in the renal pedicles have the highest risk of renal complications, followed by tumors in contact with the renal pedicles., Competing Interests: Declarations. Conflict of interest: The authors have no competing interests to declare that are relevant to the content of this article. Research involving human participants: This retrospective study was approved by the ethics review board of the National Center for Child Health and Development (approval number: 2023-216). Informed consent: The need for informed consent was waived due to the retrospective nature of the study., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background Umbilical mucosal polyps are common, but physicians' unfamiliarity with them can prolong the patient's illness. Furthermore, the details of surgery for umbilical mucosal polyp removal are not well documented. Methods Patients with an umbilical mucosal polyp diagnosed on the basis of the lesion's characteristic appearance were prospectively enrolled. The surgery involved an umbilical scarectomy with the removal of a minimum of the surrounding skin and an exploratory laparotomy to detect any lesions extending into the peritoneal cavity. Results Fourteen patients with a median duration of illness of ten months (range: one month to seven years) were enrolled, and 13 (92.9%) received surgery while one patient whose symptoms resolved following topical steroid treatment did not. Inspection of the intestinal mucosa of all the patients found lesions deep within the umbilicus in four (30.8%) of the 13 surgical patients, including an omphalomesenteric band, umbilical cyst, and umbilical sinus with gastric mucosa in one, one, and two patients, respectively. The postoperative course was uneventful except for one patient who had temporary granulation. Conclusions Umbilical mucosal polyps can be readily diagnosed by their characteristic appearance, thereby preventing the prolongation of illness. An umbilical scarectomy and abdominal exploration may be useful for preventing recurrences and intestinal obstruction., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. The Ethics Committee of Tokyo Metropolitan Children’s Medical Center issued approval H29b-126. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Tomita et al.)

Purpose: Recently, children with trisomy 18 have been receiving more active treatment for malignancies. We report herein seven cases complete resection was achieved, and discuss multidisciplinary treatment for hepatoblastoma in patients with trisomy 18., Method: The medical records of children with trisomy 18 who were treated at the study center between 2010 and 2023 were reviewed., Result: Six of 69 patients had hepatoblastoma development, and three of these underwent multidisciplinary treatment. In addition, 6 patients had been referred by another hospital for treatment, and four of these underwent multidisciplinary treatment. Among the seven patients who underwent multidisciplinary treatment, three, two, and two were categorized in Pre-treatment Extent of Disease (PRETEXT) classification group I, II, and III, respectively. Neoadjuvant chemotherapy resulting in tumor reduction was performed in three cases. In all the cases, complete resection was achieved with pathologically safe margins. Perioperative complications included circulatory failure in one case and bile leakage in two cases. Adjuvant chemotherapy was administered in four cases. The postoperative observation period ranged from 3 months to 11 years, and all the patients are recurrence-free., Conclusion: Children with trisomy 18 complicated with hepatoblastoma whose cardiopulmonary conditions are stable may be good candidates for chemotherapy and surgery., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Long-gap esophageal atresia (LGEA) is still a challenge for pediatric surgery. No consensus exists as to what constitutes a long gap, and few studies have investigated the maximum gap length safely repairable by primary anastomosis. Based on surgical outcomes at a single institution, we aimed to determine the gap length in LGEA with a high risk of complications., Methods: The medical records of 51, consecutive patients with esophageal atresia (EA) with primary repair in the early neonatal period between 2001 and 2021 were retrospectively reviewed. Three, major complications were found in the surgical outcomes: (1) anastomotic leakage, (2) esophageal stricture requiring dilatation, and (3) GERD requiring fundoplication. The predictive power of the postsurgical complications was assessed using receiver operating characteristic analysis, and the area under the curve (AUC) and the cutoff value with a specificity of > 90% were calculated., Results: Sixteen patients (31.4%) experienced a complication. The AUC of gap length was0.90 (p < 0.001), and the gap length cutoff value was ≥ 2.0 cm for predicting any complication (sensitivity: 62.5%, specificity: 91.4%)., Conclusion: A gap length ≥ 2.0 cm was considered as defining LGEA and was associated with an extremely high complication rate after primary repair., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: To identify patients with biliary atresia (BA) with extremely poor outcomes of bile drainage surgery using the infant BA liver fibrosis (iBALF) score, a liver fibrosis marker based on standard blood analysis., Background: Although primary liver transplantation is beginning to be considered as an alternative to bile drainage surgery in patients with BA, those most likely to benefit from this procedure have not yet been identified., Methods: The medical records of 380 patients with BA with bile drainage surgery between 2015 and 2019 were collected for retrospective analysis from 60 participating hospitals. To predict native liver survival at age 1 year, a receiver operating characteristic curve was drawn for the iBALF score. The cutoff value was determined as the point indicating >99% sensitivity., Results: The median age at surgery was 56 days (range: 4-183 days), and native liver survival at age 1 year was achieved in 258 (67.9%) patients. An iBALF score of 5.27 was chosen as the cutoff, and 18 patients (4.7%) were found to have an iBALF score >5.27; of these, only 2 (95% CI: 1.4%-34.7%) had native liver survival at age 1 year, indicating a significantly poorer outcome than in the other patients (95% CI: 65.7%-75.4%). Moreover, patients with an iBALF score >5.27 had significantly higher mortality and younger age at salvage liver transplantation., Conclusions: Patients with BA having a preoperative iBALF score >5.27 had extremely poor outcomes of bile drainage surgery and may be considered candidates for primary LTx., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: This study investigates the reduction rate and failure predictors of hydrostatic enema reduction for intussusception. The procedure typically begins with a water-soluble contrast medium at 90 cm above the patient, subsequently elevated to 120 cm. Our focus is on the reduction rate during initial attempts when the contrast medium is positioned at or below 120 cm from the patient., Methods: Hydrostatic enema reductions for intussusception, performed between March 2010 and May 2022 at Tokyo Metropolitan Children's Medical Center, were investigated retrospectively. The initial attempts involved one or more trials. The clinical characteristics, treatment modalities, and outcomes were analyzed. Logistic regression was used to identify the predictors of failure when the reduction was performed with the water-soluble contrast medium at a height of 120 cm or less., Results: Reduction was achieved successfully with the water-soluble contrast medium at a height at or below 120 cm in 77.5% of 351 patients. When reductions performed at heights greater than 120 cm were included, 333 (94.9%) were successful during the initial attempts and were unaccompanied by complications. Predictors of failure of reductions performed at or below 120 cm were age less than 12 months and the presence of trapped fluid., Conclusion: The present study found a successful reduction rate of 77.5% during the initial attempts, suggesting that a height greater than 120 cm may yield an even greater success rate. Children aged below 12 months and those with trapped fluid may have a greater failure risk during the initial attempts., (© 2024 Japan Pediatric Society.)

Purpose: The present study aimed to review the treatment experience and outcomes of Currarino syndrome (CS) complicated with anorectal stenosis to evaluate the current treatment strategies., Methods: Seven cases of CS complicated with anorectal stenosis, treated at our hospital between 1998 and 2021, were retrospectively investigated. This is a case series article from a single institution., Results: In six and three cases and one case, the presacral mass was a mature teratoma, meningocele, and lipoma, respectively. Resection of the lesion was performed in all six cases of mature teratoma, and duraplasty was performed before resection in all three cases of meningocele. Moreover, surgery for anorectal stenosis was performed simultaneously in four patients. Surgery was performed for six cases of anorectal stenosis, with the remaining case relieved by dilation using a metal bougie. The surgical methods used were a partial resection with end-to-end anastomosis, anorectal strictureplasty, pull-through, posterior sagittal anorectoplasty, and cutback after mass resection. Pathological analysis of the anorectal stenoses revealed disorganized and rough smooth muscle fibers and the replacement of the stroma by an increased quantity of collagen fibers., Conclusions: The clinical outcomes of CS can be improved by establishing a treatment flow chart and understanding the complicated pathophysiology of the disease., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Harada et al.)

Background: The principle of hepatoblastoma (HB) treatment is complete resection. The removal of tumor-bearing section(s) or hemiliver is widely accepted. However, neither the standardized anterior approach for right hepatectomy nor parenchymal sparing anatomical liver resection has been described for HB., Methods: We retrospectively reviewed the clinical course of two pediatric HB patients who underwent extended right hepatectomy using the anterior approach with the liver hanging maneuver and one who underwent parenchymal sparing anatomical liver resection of S4 apical+S8 ventral/dorsal+S7. The critical aspects of surgical techniques are described in detail., Results: In all three patients, R0 resection was achieved without complications and are currently alive without recurrence after an average follow-up of 23 months. Intraoperative cardiac hemodynamics were stable, even in a trisomy 18 patient with cardiac disease., Conclusions: Our findings suggest that these innovative techniques established in adults are safe and feasible for HB in children. These techniques also allow optimal anatomical liver resection to accomplish curative surgery while maintaining the functional reserve of the remnant liver., Competing Interests: All authors have no related conflict of interest to disclose., (© 2023 The Authors.)

No standard diagnostic method or surgical treatment for congenital isolated hypoganglionosis (CIHG) has been established. This study aimed to analyze the clinical outcomes of patients with CIHG and identify the best surgical interventions provided thus far. Data on surgical interventions in 19 patients were collected between 1992 and 2020, including the type of enterostomy, type of revision, and length of the intestines. Ganglion cells in the myenteric plexus were enumerated using Hu C/D staining. The ratio of the length of the small intestine to its height was defined as the intestinal ratio (IR). The outcomes were assessed using the stoma output, growth parameters including the body mass index (BMI), and parenteral nutrition (PN) dependency. All patients required a diverting enterostomy. The IR ranged from 0.51 to 1.75 after multiple non-transplant surgeries. The stoma types were tube-stoma, end-stoma, Santulli-type, and Bishop-Koop (BK)-type. Patients with Santulli- or BK-type stomas had better BMIs and less PN dependency in terms of volume than those with end-stomas or tube-stomas. Two patients with BK-type stomas were off PN, and three who underwent an intestinal transplantation (Itx) achieved enteral autonomy. The management of CIHG involves a precise diagnosis using Hu C/D staining, neonatal enterostomy, and stoma revision using the adjusted IR and Itx if other treatments do not enable enteral autonomy.

Purpose:  The transumbilical approach is widely used for minimally invasive surgery in children. We compared herein the postoperative cosmesis between two types of transumbilical approach: a vertical incision versus periumbilical incision., Methods:  Patients with a transumbilical laparotomy before age one year were prospectively enrolled between January 2018 and December 2020. A vertical incision or periumbilical incision was chosen at the surgeon's discretion. After excluding patients receiving a relaparotomy via another site, a questionnaire about the appearance of the umbilicus was completed by the patients' guardians at postoperative month 6 to assess satisfaction and determine the visual analog scale score. A photograph of the umbilicus was taken while the questionnaire was being administered for later assessment by surgeons blinded to the scar and umbilical shape., Results:  Forty patients were enrolled; 24 patients received a vertical incision while 16 received a periumbilical incision. The incision length was significantly shorter in the vertical incision group (median: 2.0; range: 1.5-3.0 cm vs. median: 2.75; range: 1.5-3.6 cm) (p = 0.001). The patients' guardians reported significantly higher satisfaction (p = 0.002) and higher scores on the visual analog scale (p = 0.046) in the vertical incision group (n = 22) than in the periumbilical incision group (n = 15). The surgeons' evaluation was associated with significantly more patients with a vertical incision than with a periumbilical incision achieving a cosmetically preferable outcome, including an invisible or fine scar and a normal umbilical shape., Conclusion:  A vertical umbilical incision can provide better postoperative cosmesis than a periumbilical incision., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Tomita et al.)

Hepatoblastoma (HB) is the most common malignant liver tumor in children. Although the development of treatment strategies with advances in chemotherapy has greatly improved the prognosis of HB, surgical resection and liver transplantation still play a vital role in the treatment of HB. In recent years, technological innovations have led to the development of new surgical approaches for HB. In this review, we describe the latest research on the surgical management of HB, including new imaging technologies, minimally invasive approaches, and the application of associating liver partition portal vein ligation for staged hepatectomy. We also discuss the current role of liver transplantation, use of ante-situm or ex-situ liver resection with auto-transplantation, and management of metastatic HB., (© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Background: The effects of disease classification and the patient's preoperative condition on the difficulty of performing a laparotomy for pediatric congenital biliary dilatation (CBD) have not been fully elucidated., Methods: The present study retrospectively analyzed 46 pediatric CBD laparotomies performed at the study center between March 2010 and December 2021 and predictors of operative time. The patients were separated into a short operative time group (SOT) (≤ 360 min, n = 27) and a long operative time group (LOT) (> 360 min, n = 19)., Results: The preoperative AST and ALT values were higher, and the bile duct anastomosis diameter was larger, in the LOT. Correlation analysis demonstrated that the maximum cyst diameter, preoperative neutrophil-to-lymphocyte ratio, AST, ALT, AMY, and bile duct anastomosis diameter correlated positively with operative time. Multivariate analysis identified the maximal cyst diameter, preoperative AST, and bile duct anastomosis diameter as significant factors affecting surgical time. Postoperatively, intrapancreatic stones and paralytic ileus were observed in one patient each in the SOT, and mild bile leakage was observed in one patient in the LOT., Conclusions: The maximum cyst diameter, preoperative AST, and bile duct anastomosis diameter have the potential to predict the difficulty of performing a pediatric CBD laparotomy., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The slide tracheoplasty (STP) is the standard treatment for severe congenital tracheal stenosis (CTS). Understanding the features of the tracheal stenosis in each case and choosing an appropriate incision design are very important for successfully executing the procedure. The present study aimed to evaluate the advantages of three-dimensional (3D) printed models of the trachea for improving CTS., Methods: Three-D tracheal models were created using computed tomography (CT) data from ten patients undergoing STP for CTS. Simulated surgery was performed using the hollow models after reinforcing with them with a coating of gum spray. Clinical outcomes, including patient survival, postoperative surgical interventions, and time required for STP, were compared with the corresponding values in the last ten patients before the introduction of 3D model simulations., Results: All ten patients for whom simulated surgery using a 3D tracheal model were conducted achieved good airway patency after their STP. The surgeons reported feeling that the 3D model simulations were highly effective although there was no significant difference in the clinical outcomes of the groups with or without simulated STP. The models were useful not only for surgical planning but also for sharing important information among the multidisciplinary team and the patients' family., Conclusion: Our experience using 3D tracheal models demonstrated several features enabling improvement in the surgical treatment of CTS., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Radical surgery for congenital tracheal stenosis (CTS) is technically demanding. CTS combined with tracheal bronchus (TB) and pulmonary artery (PA) sling is a particularly challenging condition. We herein report our successfully modified surgical techniques for CTS combined with TB and PA sling., Methods: Nine patients treated at our institution from July 2010 to December 2020 for CTS with TB and PA sling were enrolled. The patients' characteristics, operative results, and clinical outcomes were reviewed and analyzed retrospectively., Results: The mean age at the operation and body weight were 8.0 ± 4.4 months old and 6.5 ± 0.8 kg, respectively. The mean tracheal diameter and length of the stenotic lesion were 3.2 ± 1.0 mm (mean stenosis rate 46.2%) and 25.4 ± 4.9 mm, respectively. All cases were complicated with PA sling at bifurcation stenosis with tracheobronchomalacia. All patients underwent modified posterior-anterior slide tracheoplasty with an inverted Y-shaped incision at the bifurcation and repositioning of the PA. The mean postoperative intubation period was 25.0 ± 32.1 days. There were no major intraoperative or postoperative complications, including hypoxic-ischemic encephalopathy. The mean hospital stay was 92.2 ± 73.4 days. All patients were discharged home without tracheostomy or oxygen support., Conclusion: Our slide tracheoplasty technique for CTS with TB and PA sling achieved excellent outcomes., Level of Evidence: Level IV., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The application of slide tracheoplasty (STP) in the treatment of congenital tracheal stenosis (CTS) has improved patient outcomes over the past few decades. We reviewed our experiences with the procedure, elucidated risk factors, and discussed important aspects of perioperative management to improve outcomes., Method: Patients with CTS undergoing STP between July 1998 and December 2020 were enrolled. Patient characteristics, perioperative condition, management, operative details, and outcomes, including mortality and postoperative intervention, were collected from medical records., Results: Eighty patients underwent STP. Sixty-five patients (81.3%) had an associated cardiovascular anomaly. Thirteen patients (16.3%) had unilateral lung agenesis or hypoplasia. Preoperative mechanical ventilation was necessary in 54 (67.5%) patients, and extracorporeal membrane oxygenation (ECMO) was required in eight patients (10.0%). An endotracheal tube was placed before the stenotic entrance to avoid granulation. During STP, the trachea was dissected as little as possible to maintain the blood supply. The one-year survival rate was 88.8% (nine patients died). One patient (1.3%) required postoperative balloon dilation, and none required stenting or granulation removal. Of the survivors, 62 (92.5%) achieved successful extubation without tracheostomy. Multivariable analysis revealed complex cardiovascular anomaly (P = 0.05) and preoperative ECMO (P = 0.019) to be adverse predictors of survival., Conclusion: Although STP can be performed successfully in CTS patients, surgeons and families should be aware of factors that may lead to a more difficult postoperative course or increase the mortality. Meticulous, perioperative positioning of the endotracheal tube and preserving the tracheal blood flow can minimize the need for postoperative intervention., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: The optimal management method for pediatric pancreatic trauma is controversial. Moreover, the efficacy of stent placement via endoscopic retrograde pancreatography (ERP) remains poorly documented., Methods: The present, retrospective review of pediatric patients with pancreatic trauma was conducted from 2010 to 2020 at a single institution., Results: Ten, male children with the median age of 9.5 years (range 4-14 years) with a grade I (n = 2), II (n = 4) or III (n = 4) pancreatic injury were identified. Of six of these patients in whom ERP was performed, four had a pancreatic duct injury (PDI). Pancreatic stent placement was performed in all the patients with ERP at a site proximal to the injury in four patients and across the injury in two patients. A pseudocyst or pancreatic fluid collection was detected in five patients, of these, two with a grade II injury were managed successfully with conservative therapy while three with PDI required surgery. In the four patients with PDI, only one in whom the stent was placed across the PDI was able to avoid surgery., Conclusion: Therapeutic ERP might be effective even if a patient has a PDI, therefore, early ERP should be considered as a treatment option., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Minimally invasive surgery (MIS) is appropriate for the treatment of some neuroblastomas (NBs); however, the indications and technical issues are unclear. This study aimed to clarify the current status of MIS for mediastinal NB in Japan., Methods: Preliminary questionnaires requesting the numbers of neuroblastoma cases in which MIS was performed from 2004 to 2016 were sent to 159 Japanese institutes of pediatric surgery. Secondary questionnaires were sent to institutions with MIS cases to collect detailed data., Results: One hundred thirty-four (84.2%) institutions returned the preliminary questionnaire and 83 institutions (52.2%) reported a total of 1496 operative cases. MIS was performed for 175 (11.6%) cases. Among the 175 cases, completed forms of 140 patients were returned and 40 (male, n = 28; female, n = 12) cases had mediastinal NB. Fourteen patients received thoracoscopic biopsy, none were converted to thoracotomy. Twenty-eight patients received MIS for radical resection, none were converted to thoracotomy. Perioperative complications (Horner's syndrome) were recognized after radical resection in one (2.5%) case., Conclusions: MIS was performed in a limited number of mediastinal NB cases. A thoracoscopic approach would be feasible for mediastinal NB., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Patients with severe motor and intellectual disabilities often suffer from tracheal stenosis due to chest deformation and brachiocephalic artery compression, which sometimes leads to serious complications, such as dying spell and tracheobrachiocephalic artery fistula. We herein described our experience of performing a novel and simple thoracoplastic procedure combined with brachiocephalic artery transection in two patients with severe chest deformation and tracheal stenosis., Case Presentation: The patients were a 12-year-old female with cerebral palsy due to periventricular leukomalacia and a 21-year-old male with subacute sclerosing panencephalitis stage IV in the Jabbour classification following a laryngotracheal separation. Both patients showed severe chest deformation and symptoms of airway stenosis resulting in dying spells. The sternum was laterally transected between the manubrium and the sternal body, and a manubriotomy was performed longitudinally, ending with an inverse T-shaped sternotomy. Since the clavicle and the first rib remained attached to the halves of the divided manubrium, the sternum was allowed to be left open, resulting in improvement of the mediastinal narrowing and tracheal stenosis. Postoperative computed tomography (CT) showed that the distance between the halves of the manubrium was maintained at 10-11 mm, and that the mediastinal narrowing in both patients improved; the sternocervical spine distance increased from 20 mm to 22  and 13 mm to 16 mm, respectively. The patients' tracheal stenosis below the sternal end of the clavicle and the manubrium and respiratory symptoms improved, and the patients are currently at home in a stable condition with no chest fragility and no upper limb movement disorder 1 year after surgery., Conclusions: Our observations suggested that the inverse T-shaped sternotomy combined with brachiocephalic artery transection may relieve symptoms of tracheal stenosis due to severe chest deformation in patients with severe motor and intellectual disabilities., (© 2021. The Author(s).)

Background: Our group previously proved that the human enteric nervous system can be visualized with confocal laser endomicroscopy after topical application of cresyl violet using surgically resected intestine specimens. The present report documents the first in vivo visualization of the human enteric nervous system with confocal laser endomicroscopy using local cresyl violet staining. The aim of this study was to evaluate the technical feasibility and clinical efficiency of confocal laser endomicroscopy in patients with Hirschsprung's disease and allied disorders in vivo., Methods: Confocal laser endomicroscopy was performed in vivo in two patients to confirm the presence of the enteric nervous system during surgery in patients with Hirschsprung's disease and allied disorders. Cresyl violet was gently injected from the serosal side into the muscular layer of the intestine, and scanning was performed within 30 min. Then, the scanned intestines were resected, and the visualized area of the specimens was pathologically evaluated., Results: The ganglion cell nuclei and the enteric nervous system network were clearly visualized intraoperatively in both cases. The morphological findings were similar to the pathological findings of the enteric nervous system in both cases although the period of visibility was brief., Conclusion: This study demonstrated the first, real-time observation of the enteric nervous system in humans using confocal laser endomicroscopy and suggest the potential to identify the enteric nervous system intra-operatively during surgery for Hirschsprung's disease and allied disorders., (© 2021. The Author(s).)

Background: Evidence has been published on the successful applications of the anti-tumor necrosis factor alpha antibody infliximab, such as induction therapy, salvage treatment for acute cellular rejection, and treatment for chronic ulcerative inflammation, in intestinal transplant recipients. However, the optimal protocol for the effective use of infliximab remains largely undetermined due to scarcity of available clinical data. We report a continuative application of infliximab as maintenance therapy for recurrent chronic ulcerative ileitis in a recipient of isolated intestinal transplantation (ITx)., Case Summary: The patient was a 11-year-old boy with intestinal motility disorder classified as a hypogenic type of intestinal dysganglionosis. The patient underwent living-donor related intestinal transplant. His immunosuppression regimen consisted of daclizumab, tacrolimus, and steroids. Although he did not show rejection while on tacrolimus monotherapy, routine screening endoscopy showed several ulcerative lesions in the distal end of the graft 2 years after the intestinal transplant. Endoscopic work up to evaluate the progression of anemia revealed stenosis with ulcerative inflammatory changes and multiple longitudinal ulcers in the graft. Since the endoscopic findings suggested ulcerative lesions in Crohn's disease, infliximab treatment was considered. Treatment with infliximab and a small dose of oral prednisolone afforded successful withdrawal of total parenteral nutrition and maintenance of a well-functioning graft without infectious complications for 5 years since the administration of the first dose of infliximab., Conclusion: Infliximab is effective as maintenance therapy for recurrent chronic ulcerative ileitis in an isolated ITx patient., Competing Interests: Conflict-of-interest statement: The authors declare that they have no conflicts of interest., (©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.)

Background: A previous report proposed ultrasonography-based classification as a promising means of predicting pediatric spontaneously resolving appendicitis. The present study investigated the long-term prognosis of supportive care for low-grade appendicitis identified by ultrasonography, defined as an appendix with a smooth submucosal layer irrespective of blood flow or an appendix with an irregular layer and increased blood flow., Methods: The present, retrospective cohort study enrolled patients under 16 years of age with acute appendicitis at a children's hospital between October 2010 and September 2016. The inclusion criteria were ultrasonography findings showing an appendix with (1) full visualization, (2) a diameter ≥6 mm, (3) a smooth submucosal layer or an irregular layer with increased blood flow, and (4) no appendiceal mass, abscess, or perforation. The exclusion criteria were: (1) a history of acute appendicitis, (2) antibiotic administration within 72 hours before diagnosis, and (3) antibiotic administration or surgery before supportive care. The primary outcome was the event-free duration, defined as a period of supportive care alone with no additional intervention or recurrence of appendicitis., Results: One hundred and eighty-two patients were enrolled. The median Alvarado score was 7 (interquartile range, 6-8), and the median follow-up duration in event-free cases was 1,922 days (interquartile range, 1,347-2,614 days). The event-free rate was 75.0%, 67.0%, and 62.5%, at 1, 2, and 5 years, respectively., Conclusion: The long-term, event-free rate exceeded 60% in patients with low-grade appendicitis defined by ultrasonography who received neither surgery nor antibiotic treatment. Most recurrences occurred within 2 years of the initial diagnosis., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Etilefrine, a sympathomimetic agent, is reportedly effective against postoperative chylothorax. However, its effectiveness in treating congenital chylothorax was unknown. We report herein a case of refractory congenital chylothorax treated with etilefrine in a late preterm neonate with massive fetal chylous pleural effusion. The chylothorax was unresponsive to previous treatments, including dietary and pharmacological treatment and thoracic duct ligation. The pleural effusion decreased after intravenous etilefrine was begun on day of life (DOL) 84 and resolved after the addition of chemical pleurodesis with OK-432 on DOL 90. This combination therapy may be a viable treatment option for cases of congenital chylothorax that are unresponsive to other treatments., Competing Interests: The authors have no conflicts of interest to declare., (Copyright © 2020 by S. Karger AG, Basel.)

Background: Hirschsprung's disease is a congenital abnormality of the enteric nervous system (ENS) presenting severe constipation soon after birth due to the lack of ganglion cells in the distal gut. Surgery for Hirschsprung's disease requires an intraoperative histopathological diagnosis to assess the extent of aganglionosis. Confocal laser endomicroscopy (CLE) is a novel endoscopic technique allowing real-time, in vivo analysis of cellular details during ongoing endoscopy. In this study, we evaluated the possibility of a new application of CLE to provide real-time observations of the ENS in patients with Hirschsprung's disease. In this preclinical feasibility study, we assessed the visualization of the ENS by CLE using surgically resected intestines., Methods: The subjects were nine patients who underwent pull-through surgery for Hirschsprung's disease between September 2014 and March 2016. The colon specimens were stained with 0.1% cresyl violet and evaluated using CLE. We compared the CLE findings with those of the histopathological examination., Key Results: The ENS was clearly visualized as a ladder-like structure in the ganglionic segment but was not observed in the aganglionic segment. Of the 69 samples, corresponding positive and negative results for both CLE and the histopathology were obtained in 61 (88%). In addition, CLE was able to visualize unique, wavy structures comprising thick nerve bundles characteristic of the aganglionic/transition zone in Hirschsprung's disease., Conclusions and Inferences: As a novel tool for visualizing the human ENS, CLE has the potential to revolutionize how pediatric surgeons identify the level of ganglionosis during surgery for Hirschsprung's disease and may be a superior alternative to intraoperative histopathological diagnosis., (© 2020 John Wiley & Sons Ltd.)

Background: Detecting a neoplasm in pediatric patients with acute abdominal pain is difficult, especially when there is no palpable mass. Ihara's maneuver, which allows the physician to apply manual pressure directly on the pancreatic body, is a useful palpation method of diagnosing acute pancreatitis in children. We report a case of solid pseudopapillary neoplasm of the pancreas (SPNP) detected by Ihara's maneuver., Case Report: An otherwise healthy, 15-year-old male visited our emergency department with acute abdominal pain and nausea. His vital signs were appropriate for his age. A physical examination denied peritoneal signs, but Ihara's maneuver induced strong tenderness. His serum amylase and lipase were normal. A contrast-enhanced computed tomography scan revealed a well-defined, 2.2-cm, nonenhanced mass in the pancreatic tail. Laparoscopic distal pancreatectomy was performed, and the diagnosis of SPNP was confirmed. The patient was well postoperatively without any metastasis. SPNP is a rare neoplasm with low malignant potential. Although it typically occurs in young females, it has also been reported in children. The early diagnosis of SPNP is usually challenging because most patients do not have specific symptoms or laboratory findings. In the present case, the SPNP was difficult to detect by palpation because of its size, but Ihara's maneuver induced strong tenderness of the pancreas and led to a diagnosis. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Ihara's maneuver has the potential to enable early diagnosis not only of pancreatitis but also of pancreatic tumors, such as SPNP., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objectives: To identify the imaging features peculiar to congenital tracheal stenosis (CTS) complicated with left pulmonary artery sling (LPAS) with the aim of presenting a hypothesis of tracheal stenosis embryology in LPAS., Methods: We retrospectively reviewed CTS patients (with complete cartilaginous rings) admitted between April 2010 and July 2018. All the patients were classified into the LPAS or non-LPAS group, and their clinical characteristics and qualitative variables on computed tomography (CT) imaging were compared., Results: Of the 72 patients enrolled, 61 had bilateral lungs. Among the bilateral lung patients, 26 (43%) had LPAS. The tracheal bifurcation was significantly deeper, the stenotic region was longer, and the bronchial angle (especially in the right) was wider, in the LPAS group. The cut-off values for the thoracic vertebral level at the tracheal bifurcation (>4.8), subcarinal angle (>118.1), and right bronchial angle (>61.9) were useful for diagnosing suspected cases of LPAS. In the time-dependent course, LPAS complicated with a congenital heart defect was a statistically significant risk factor of respiratory symptoms (hazard ratio, 3.01; 95% confidence interval, 1.23-7.37; P = .02)., Conclusions: The CT findings described here should immediately raise suspicion of LPAS on chest X-ray and also suggest tracheal "squeezing and milking" by the surrounding vessels in the embryo. Patients with LPAS complicated with a heart defect should be followed carefully to determine the optimal timing of intervention., (© 2020 Wiley Periodicals, Inc.)

Background: Congenital tracheal stenosis (CTS) is a rare and life-threatening disease in children. Although pulmonary artery sling (PA sling) complicated by CTS sometimes occurs, there are few reports detailing the management of CTS with PA sling. The purpose of this retrospective study was to determine the appropriate indications for surgical intervention for CTS complicated by PA sling., Methods: We evaluated 42 patients (19 males and 23 females) with the median age of 9.9±3.3 months (range, 5-34 months) with CTS complicated by PA sling who were treated at our hospital between 2005 and 2018. Twenty-eight patients received both a slide tracheoplasty and PA re-implantation, and 14 patients were managed conservatively for CTS. Among the latter, nine patients received PA re-implantation only, and five were managed conservatively without any surgery. We determined the surgical indications by retrospectively comparing the DLR value [tracheal diameter (mm)/stenotic length ratio], history of ventilator respiration, mortality rate, and post-operative course of patients at a single institution., Results: The cut-off value for the DLR was determined to be 5.9 (sensitivity: 0.929, specificity: 0.714) by using the ROC curve (AUC 0.89, P<0.05)., Conclusions: A DLR value under 5.9 may serve as a new surgical indication for CTS complicated by PA sling., Competing Interests: Conflicts of Interest: The authors have no conflicts of interest to declare., (2019 Journal of Thoracic Disease. All rights reserved.)

Purpose: Lingual thyroglossal duct cysts (L-TGDCs) are rare and sometimes lethal owing to their association with asphyxia. We aimed to analyze our single institutional experience with L-TGDCs., Methods: Twelve L-TGDC cases treated at our institution between January 2010 and December 2017 were investigated., Results: The male/female ratio was 6/6. The age at the diagnosis was 2 ± 1.4 months (7 days to 6 months), and 3 patients were diagnosed in the neonatal period. The patients presented with stridor (n = 12; 100%), growth retardation (n = 5; 42%), apnea (n = 3; 25%), and vomiting (n = 1; 8.3%). Lateral X-rays were obtained in 8 cases (66.7%); a lingual mass was suspected in 7 (87.5%). Transoral marsupialization of the cyst was performed under direct vision in all cases. All cases were nasally and orally intubated using a laryngoscope, bronchoscope, or airway scope. The mean operative time was 18 ± 2.9 min. The mean cyst size was 10.5 ± 1.8 mm. No recurrence was observed during the follow-up period (37.5 ± 18 months)., Conclusion: L-TGDC requires a precise diagnosis and rapid intervention because of the risk of asphyxia resulting in sudden death. Transoral marsupialization under direct vision is an effective and secure approach. L-TGDC should be considered when patients younger than six months of age present with respiratory distress., Type of Study: Retrospective Study., Level of Evidence: Level IV., (Copyright © 2019 Elsevier Inc. All rights reserved.)

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., (© 2018 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Background: Despite the presence of ganglion cells in the rectum, some patients have symptoms similar to those of Hirschsprung's disease. A consensus has yet to be established regarding the terminology for these diseases. We defined this group of diseases as "allied disorders of Hirschsprung's disease" and compiled these guidelines to facilitate accurate clinician diagnosis and provide appropriate treatment strategies for each disease., Methods: These guidelines were developed using the methodologies in the Medical Information Network Distribution System (MINDS). Of seven allied disorders, isolated hypoganglionosis; megacystis-microcolon-intestinal hypoperistalsis syndrome; and chronic idiopathic intestinal pseudo-obstruction were selected as targets of clinical questions (CQ). In a comprehensive search of the Japanese- and English-language articles in PubMed and Ichu-Shi Web, 836 pieces of evidence related to the CQ were extracted from 288 articles; these pieces of evidence were summarized in an evidence table., Results: We herein outline the newly established Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease. Given that the target diseases are rare and intractable, most evidence was drawn from case reports and case series. In the CQ, the diagnosis, medication, nutritional support, surgical therapy, and prognosis for each disease are given. We emphasize the importance of full-thickness intestinal biopsy specimens for the histopathological evaluation of enteric ganglia. Considering the practicality of the guidelines, the recommendations for each CQ were created with protracted discussions among specialists., Conclusions: Clinical practice recommendations for allied disorders of Hirschprung's disease are given for each CQ, along with an assessment of the current evidence. We hope that the information will be helpful in daily practice and future studies., (© 2018 Japan Pediatric Society.)