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Background: Owing to recurrent aspiration pneumonia and airway stenosis secondary to thoracic deformities, tracheostomy or laryngotracheal separation are often necessary in children with severe motor and intellectual disabilities. However, these procedures are associated with the risks of tracheal stenosis due to tracheal granulation and tracheoinnominate artery fistula formation. We report a case of a child with severe motor and intellectual disabilities treated with an anterior mediastinal tracheostomy., Case Presentation: The patient was a 15-year-old boy with severe motor and intellectual disabilities due to intractable epilepsy. Due to thoracic deformity and tracheomalacia, the patient had a flattened and narrowed trachea. Accordingly, laryngotracheal separation was performed 4 months before admission to avoid aspiration pneumonia. Due to a common cold, the patient required frequent sputum suctioning, which exacerbated the tracheal stenosis. Bronchoscopy revealed tracheal stenosis 4-5 cm caudal to the tracheostomy site, tracheal mucosal ulcers, and pulsation of the innominate artery on the anterior wall of the trachea. We performed an anterior mediastinum tracheostomy to release the tracheal stenosis and prevent tracheoinnominate artery fistula formation., Conclusions: Anterior mediastinal tracheostomy has several advantages. Including sufficient release of bony compression, release of tracheal hyperextension, and relief of tracheal and innominate artery contact ensures a cannula-free tracheostomy, and there is no need to dissect the brachiocephalic artery. It is the procedure of choice in cases of head and neck malignancies requiring extensive tracheal resection and could be a good surgical option for severe tracheal stenosis and tracheoinnominate artery fistula in children with severe motor and intellectual disabilities., (© 2023. The Author(s).)

Purpose: To assess the increase in hospital costs associated with postoperative complications after lower anterior resection (LAR) for rectal cancer., Methods: The subjects of this retrospective analysis were patients who underwent elective LAR surgery between April, 2015 and March, 2017, collected from a Japanese nationwide gastroenterological surgery registry linked to hospital-based claims data. We evaluated total and category-specific hospitalization costs based on the level of postoperative complications categorized using the Clavien-Dindo (CD) classification. We assessed the relative increase in hospital costs, adjusting for preoperative factors and hospital case volume., Results: We identified 15,187 patients (mean age 66.8) treated at 884 hospitals. Overall, 71.8% had no recorded complications, whereas 7.6%, 10.8%, 9.0%, 0.6%, and 0.2% had postoperative complications of CD grades I-V, respectively. The median (25th-75th percentiles) hospital costs were $17.3 K (16.1-19.3) for the no-complications group, and $19.1 K (17.3-22.2), $21.0 K (18.5-25.0), $27.4 K (22.4-33.9), $41.8 K (291-618), and $22.7 K (183-421) for the CD grades I-V complication groups, respectively. The multivariable model identified that complications of CD grades I-V were associated with 11%, 21%, 61%, 142%, and 70% increases in in-hospital costs compared with no complications., Conclusions: Postoperative complications and their severity are strongly associated with increased hospital costs and health-care resource utilization. Implementing strategies to prevent postoperative complications will improve patients' clinical outcomes and reduce hospital care costs substantially., (© 2022. The Author(s).)

The purpose of this study was to prepare clinical practice guidelines for biliary atresia according to the Medical Information Network Distribution Service (MINDS) Handbook for Clinical Practice Guideline Development 2014. The guideline drafting group determined 25 clinical questions (CQs) essential for daily clinical practice. These CQs were grouped into the following four major categories: diagnosis, treatment, complications, and prognosis. A systematic literature search was performed by the systematic review team, and medical evidence was extracted from articles published until July 2014 on PubMed and the Japan Medical Abstracts Society. To reach a consensus, majority voting (>70%) of the panel of the guideline drafting group was adopted according to the Delphi technique. Recommendations for 21 CQs were appropriately provided. However, for four CQs (CQ2, stool color; CQ9, steroid administration; and CQ24 and CQ25, liver transplantation), recommendations could not be determined due to a scarcity of evidence. The first clinical practice guidelines for biliary atresia were successfully created using procedures recommended by the MINDS. It is expected that these guidelines will be used worldwide, and that the standardization of biliary atresia treatment will improve treatment outcomes., (© 2020 Japanese Society of Hepato-Biliary-Pancreatic Surgery.)

Laparoscopic gastrostomy tube placement has been increasingly adopted by pediatric surgeons. We herein report our experience with the performance of a laparoscopic-assisted Stamm-operation inside the minimal trocar site without the extension of the trocar site incision or mini-laparotomy. We present some technical modifications that facilitate suturing inside the minimal trocar site., Methods: A retrospective chart review was conducted of cases involving patients who underwent laparoscopic-assisted gastrostomy, using a simple extracorporeal method inside the trocar site from April 1998 to March 2018., Results: One hundred five gastrostomy tubes were placed in a laparoscopic-assisted operation. All but two of the cases involved patients with neurological impairment. The mean age was 12.5 years; 28 cases were > 16 years of age. Seventy-five cases underwent gastrostomy during laparoscopic fundoplication. All gastrostomy procedures were completed without intraoperative difficulties; however, 8 cases, which involved a thick abdominal wall, required extension of the trocar site. No cases required conversion to open gastrostomy. No major complications were observed. Two patients developed continuous peristomal cellulitis after surgery, due to the mismatch of the site position and an unsuitable button device size., Conclusion: We demonstrated that laparoscopic gastrostomy with fully extracorporeal suturing within the trocar site is feasible and beneficial, especially for the most neurologically impaired pediatric cases. Technical modification, changing the order of the process, and suturing technique in the minimal space, made it easier to perform the procedures inside the minimal trocar hole.

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety and systematizing treatment, employing evidence-based medicine techniques and aimed at improvement of the outcomes. Clinical questions (CQ) were decided based on the important clinical issues. For document retrieval, key words for published work searches were set for each CQ, and work published from 1980 to the end of September 2014 was searched in PubMed, Cochrane Library and Japana Centra Revuo Medicina databases. The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution System technique. A total of 33 CQ were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, and pathological diagnosis deciding the diagnosis. Thus, the Japanese Clinical Practice Guidelines for Vascular Anomalies 2017 have been prepared as the evidence-based guidelines for the management of vascular anomalies., (This article has been co-published with the permission of The Journal of Dermatology, Pediatrics International and Japanese Journal of Radiology. All rights reserved. © 2020 The Authors. The articles are identical except for minor stylistic and spelling differences in keeping with each journal’s style. Either citation can be used when citing this article.)

Background: Human deciduous pulp stem cells (hDPSCs) have remarkable stem cell potency associated with cell proliferation, mesenchymal multipotency, and immunosuppressive function and have shown beneficial effects in a variety of animal disease models. Recent studies demonstrated that hDPSCs exhibited in vivo anti-fibrotic and anti-inflammatory action and in vivo hepatogenic-associated liver regeneration, suggesting that hDPSCs may offer a promising source with great clinical demand for treating liver diseases. However, how to manufacture ex vivo large-scale clinical-grade hDPSCs with the appropriate quality, safety, and preclinical efficacy assurances remains unclear., Methods: We isolated hDPSCs from human deciduous dental pulp tissues formed by the colony-forming unit-fibroblast (CFU-F) method and expanded them under a xenogeneic-free and serum-free (XF/SF) condition; hDPSC products were subsequently stored by two-step banking including a master cell bank (MCB) and a working cell bank (WCB). The final products were directly thawed hDPSCs from the WCB. We tested the safety and quality check, stem cell properties, and preclinical potentials of final hDPSC products and hDPSC products in the MCB and WCB., Results: We optimized manufacturing procedures to isolate and expand hDPSC products under a XF/SF culture condition and established the MCB and the WCB. The final hDPSC products and hDPSC products in the MCB and WCB were validated the safety and quality including population doubling ability, chromosome stability, microorganism safety, and stem cell properties including morphology, cell surface marker expression, and multipotency. We also evaluated the in vivo immunogenicity and tumorigenicity and validated in vivo therapeutic efficacy for liver regeneration in a CCl 4 -induced chronic liver fibrosis mouse model in the final hDPSC products and hDPSC products in the WCB., Conclusion: The manufacture and quality control results indicated that the present procedure could produce sufficient numbers of clinical-grade hDPSC products from a tiny deciduous dental pulp tissue to enhance clinical application of hDPSC products in chronic liver fibrosis.

Aim: This study aimed to compare the surgical outcomes of patients with the intermediate-type imperforate anus who underwent laparoscopically assisted anorectoplasty (LAARP; L group) with those of patients who underwent sacroperineal anorectoplasty (S group). Materials and Methods: The medical records of patients with intermediate-type imperforate anus at a single institution between April 1983 and April 2017 were retrospectively reviewed. Fecal continence was evaluated using the clinical assessment score for fecal continence developed by the Japanese Study Group of Anorectal Anomalies (maximum score, 8). Results: Twelve cases (rectobulbar urethral fistula, 7; anal agenesis without fistula, 4; and rectovaginal fistula, 1) were included in the L group versus 14 cases (rectobulbar urethral fistula, 11, and anal agenesis without fistula, 3) in the S group. Age and body weight at the time of surgery and rate of associated anomalies did not differ significantly between the two groups. The total scores for fecal continence 3, 5, and 7 years after anorectoplasty were 4, 5, and 4 points in the L group and 4, 5, and 6 points in the S group, respectively, showing no significant intergroup differences. Mucosal prolapse occurred in 50% of the L group and 29% of the S group ( P  = .42), but failed rectocutaneous anastomosis and anal stenosis were not identified in either group. Conclusions: Postoperative fecal continence and the incidence of complications after LAARP were comparable with those after sacroperineal anorectoplasty in patients with intermediate-type imperforate anus.

Background: Portal venous gas has been considered as a radiological sign requiring urgent operative intervention; however, the reports concerning portal venous gas associated with favorable outcome are recently increasing., Case Presentation: We describe a 9-month-old boy with acute onset high fever and vomiting. The ultrasonography demonstrated micro-gas bubbles continuously floating in the intrahepatic portal vein. Contrast-enhanced CT, performed 1 h later from echography, revealed a whirlpool sign suggesting an intestinal malrotation with midgut volvulus, but with no signs of residual intrahepatic gas. Operative findings showed a mild volvulus with neither congestion nor ischemic change of the twisted bowel. Detorsion and Ladd's procedure were completed laparoscopically., Conclusions: Transient portal venous gas bubbles may be generated even in the mild intestinal volvulus with no bowel ischemia. Ultrasonography can be a sensitive detector to visualize such small amounts of gas.

Background: Although uncommon and seldom experienced, intestinal perforation is a well-known complication of Hirschsprung's disease (HD). A literature review revealed that the cecum, including the appendiceal base, is a site of perforation. The cecum is not suitable for making an ordinary loop colostomy, and the optimal operative strategy remains to be established., Case Presentation: We present a combination technique composed of tangential cecostomy at the perforated portion and postoperative care with a transanal indwelling tube, which was used in the treatment of a 3-day-old boy with cecal perforation with long-segment Hirschsprung's disease. A temporary simple blowhole stoma and continuous decompression with daily irrigation via a transanal indwelling tube in the distal colon achieved a secure recovery and was followed by a definitive operation in the early period. The combination of tangential cecostomy and transanal indwelling catheter management led to the preservation of the ileocecal valve., Conclusions: We review the Japanese literature and emphasize the usefulness of this combination technique by blowhole tangential cecostomy and transanal tube insertion for neonatal cecal perforation in patients with HD in today's early definitive operation era.

Purpose: Airway obstruction caused by lymphatic malformation (LM) in the head and neck may require a tracheostomy. We present the results of our analysis of a nationwide survey on the indications for tracheostomy in children with head and neck LM., Methods: We analyzed data in relation to tracheostomy based on a questionnaire about 518 children with head and neck LM without mediastinal involvement., Results: Tracheostomy was performed for 43 of the 518 children. Most (32/43) of these children were younger than 1 year of age and the tracheostomy was almost always performed for airway obstruction (40/43). The lesion was in contact with the airway in 32 (72%) of these children, but in only 58 (12%) of the 473 children who were managed without tracheostomy. When the maximum circumferential area of contact was compared, only 20 (27%) of 74 patients with maximum contact of less than a half-circle required tracheostomy, whereas 11 of 13 with maximum contact of more than a half-circle required tracheostomy (P = 0.0001). Six patients without airway contact required tracheostomy because of acute swelling caused by hemorrhage, infection, or both., Conclusions: Children with head and neck LM required tracheostomy to relieve airway obstruction. Tracheostomy should be considered if the lesion is in contact with the airway and surrounds more than a half-circle, and when it causes acute swelling.

Purpose: The aim of this study was to examine the quality of data from the National Clinical Database (NCD) via a comparison with regional government report data and medical charts., Methods: A total of 1,165,790 surgical cases from 3007 hospitals were registered in the NCD in 2011. To evaluate the NCD's data coverage, we retrieved regional government report data for specified lung and esophageal surgeries and compared the number with registered cases in the NCD for corresponding procedures. We also randomly selected 21 sites for on-site data verification of eight demographic and surgical data components to assess the accuracy of data entry., Results: The numbers of patients registered in the NCD and regional government report were 46,143 and 48,716, respectively, for lung surgeries and 7494 and 8399, respectively, for esophageal surgeries, leading to estimated coverages of 94.7% for lung surgeries and 89.2% for esophageal surgeries. According to on-site verification of 609 cases at 18 sites, the overall agreement between the NCD data components and medical charts was 97.8%., Conclusion: Approximately, 90-95% of the specified lung surgeries and esophageal surgeries performed in Japan were registered in the NCD in 2011. The NCD data were accurate relative to medical charts.

Aims: Our aims were to develop a training system for camera assistants (CA), and evaluate participants' performance as CA., Methods: A questionnaire on essential requirements to be a good CA was administered to experts in pediatric endoscopic surgery. An infant-sized box trainer with several markers and lines inside was developed. Participants performed marker capturing and line-tracing tasks using a 5-mm 30° scope. A postexperimental questionnaire on the developed system was administered. The task completion time was measured., Results: The 5-point evaluation scale was used for each item in the questionnaire survey of experts. The abilities to maintain a horizontal line (mean score: 4.5) and to center the target in a specified rectangle on the monitor (4.5) as well as having a full understanding of the operative procedure (4.3) were ranked as highly important. Fifty-two participants, including 5 surgical residents, were enrolled in the evaluation experiment. The completion time of capturing the markers was significantly longer in the resident group than in the nonresident group (244 versus 124 seconds, P = .04), but that of tracing the lines was not significantly different between the groups. The postexperimental questionnaire showed that the participants felt that the line-tracing tasks (3.7) were more difficult than marker-capturing tasks (2.9)., Conclusions: Being proficient in manipulating a camera and having adequate knowledge of operative procedures are essential requirements to be a good CA. The ability was different between the resident and nonresident groups even in a simple task such as marker capturing.

Purpose: Clinical guidelines on lymphatic malformation (LM) influencing the airway have been crafted in the Research Project for Intractable Diseases. We herein report an analysis of a nationwide survey of mediastinal LM and the therapeutic recommendations., Methods: Eighty-seven registered cases with mediastinal involvement were analyzed with a review of the literature., Results: Mediastinal LM was located more often in the upper and anterior mediastinum and was found without any accompanying symptoms in 56/87 cases. Tracheostomy was required in 23/87 cases, mostly < 2 years of age (87%). All patients who needed tracheostomy had a lesion in contact with the airway, while only 55% of those without tracheostomy had contact. Tracheostomy tended to be placed more when the longer segment of the airway was in contact with the LM. Multimodal treatments were performed in 29 patients, but the lesions remained in most cases, and chylothorax, hemorrhaging, nerve palsy, and infections were noted as complications., Conclusions: In patients with mediastinal LM, tracheostomy may be necessary, especially when the lesion is extensive and contacts the airway. Extirpation of the mediastinal LM may be the only therapeutic option, but in cases with few or no symptoms, non-surgical treatment should be considered in light of potential postoperative complications.

Background: Pediatric robot-assisted surgery is increasingly being performed, but it is difficult to perform this procedure in infants. A pediatric thoracoscopic model of a 1-year-old patient was developed in our previous study, and this model was used to evaluate the use of a surgical robot for infant surgery., Methods: Eight pediatric surgeons performed an intracorporeal suturing and knot-tying task using the da Vinci Xi Robotic Surgical System. The task completion time, number of needle manipulations, and force applied during suturing of the robot-assisted thoracoscopic surgery (RATS) group were compared with those of the video-assisted thoracoscopic surgery (VATS) group whose data had been collected from the same 8 surgeons in our previous study., Results: The RATS group showed a significantly shorter completion time than the VATS group in the knot-tying phase (P = .016) and in the total phase (P = .0078). The RATS group showed a significantly smaller number of manipulations than the VATS group in the total phase (P = .039). The RATS group showed a significantly smaller pushing force index than the VATS group in the suturing phase (P = .031), knot-tying phase (P = .031), and in the total phase (P = .031). A seventh rib in the model was dislocated in all RATS group cases., Conclusions: The da Vinci Surgical System might be useful in infants because of fast movement and small pushing force. However, the robotic 8 mm instruments were too large for use in the thoracic cavity of the 1-year-old infant.

Objective: To assess the use of laparoscopic surgeries (LS) and the association between its performance and hospitals' preference for LS over open surgeries., Summary Background Data: LS is increasingly used in many abdominal surgeries, albeit both with and without solid guideline recommendations. To date, the hospitals' preference (LS vs. open surgeries) and its association with in-hospital outcomes has not been evaluated., Methods: We enrolled patients undergoing 8 types of gastrointestinal surgeries in 2011-2013 in the Japanese National Clinical Database. We assessed the use of LS and the occurrences of surgery-related morbidity and mortality during the study period. Further, for 4 typical LS procedures, we assessed the hospitals' preference for LS by modeling the propensity to perform LS (over open surgeries) from patient-level factors, and estimating each institution's observed/expected (O/E) ratio for LS use. Institutions with O/E>2 were defined as LS-dominant. Using hierarchical logistic regression models, we assessed the association between LS preference and in-hospital outcomes., Results: Among 1,377,118 patients undergoing gastrointestinal procedures in 2,336 participating hospitals, use of LS increased in all 8 procedures (35.1% to 44.7% for distal gastrectomy (DG), and 27.5% to 43.2% for right hemi colectomy (RHC)). Those operated at LS-dominant hospitals were at an increased risk of operative death (OR 1.83 [95%CI, 1.37-2.45] for DG, 1.79 [95%CI, 1.43-2.25] for RHC) compared to standard O/E level hospitals (0.5≤O/E<2.0)., Conclusions: LS use widely increased during 2011-2013 in Japan. Facilities with higher than expected LS use had higher mortality compared to other hospitals, suggesting a need for careful patient selection and dissemination of the procedure.

Backgroud: Recently, laparoscopic percutaneous extraperitoneal closure (LPEC) has gained increased popularity for pediatric inguinal hernia repair. To improve cosmesis, we developed single incision LPEC (SILPEC). The aim of this study was to assess the safety and feasibility of SILPEC compared with traditional open repair (OR)., Methods: This was a single-center retrospective cohort study of 2028 children who underwent inguinal hernia repair between April 2005 and August 2014. Nine hundred and ninety-five patients underwent OR and 1033 patients underwent SILPEC. Medical records were reviewed with respect to operative time, recurrence, incidence of contralateral metachronous inguinal hernia (CMIH), and complications. Patient satisfaction with cosmetic result was also investigated using questionnaires sent by mail., Results: All SILPEC procedures were completed without conversion. Operative time was longer in the SILPEC group than in the OR group for both unilateral and bilateral surgery regardless of sex (unilateral male: p = 0.0006, unilateral female: p < 0.0001, bilateral male: p < 0.0001, bilateral female: p < 0.0001). There was no statistically significant difference in recurrence rate (p = 0.43). The incidence of CMIH was significantly higher in the OR than in the SILPEC group (p < 0.0001). No postoperative testicular atrophy was found in either group. There was no statistically significant difference in ascending testis (p = 0.09), but the frequency of surgical site infection was higher in the SILPEC than in the OR group (p = 0.0013). According to the questionnaire, operative scar was more invisible in the SILPEC than in the OR group (p < 0.0001), but both procedures had equally high levels of satisfaction for cosmetic results (p = 0.58)., Conclusion: SILPEC proved to be a safe and feasible procedure compared with OR with an equally low recurrence rate, more effectiveness for preventing CMIH, and more invisible scar.

Fundoplication is a standard treatment for gastroesophageal reflux disease (GERD) in neurologically impaired children. However, it has a high recurrence rate due to wrap herniation and disruption resulting from the failure or enlargement of the esophageal hiatus suture site. In adult patients with GERD, reinforcement of the sutured esophageal hiatus with a mesh significantly reduces the recurrence rate after surgery for esophageal hiatus hernia. In children, there are no big series of fundoplication with mesh reinforcement. Therefore, we confirmed the safety and feasibility of an expanded polytetrafluoroethylene mesh hiatal reinforcement with fundoplication as an additional procedure for neurologically impaired children. Neurologically impaired children (age, ≥5 years) who underwent laparoscopic fundoplication and gastrostomy at our institution between 2011 and 2013 were included in this study. The operative time, bleeding amount, complications, and recurrence were retrospectively evaluated. Thirteen patients were included in this study. The mean age at operation was 147.2 ± 37.8 months, and mean body weight was 18.5 ± 7.9 kg. The complication rate of epilepsy and scoliosis was 100% and 84.6%, respectively. The operative time was 265.1 ± 38.1 minutes, and mean blood loss was 26.5 ± 34.7 mL. There were no peri- or postoperative complications. None of the patients showed recurrent symptoms such as repeated vomiting and aspiration pneumonia during the mean follow-up period of 2.5 ± 0.6 years. The mesh hiatal reinforcement with fundoplication for neurologically impaired children is safe and feasible.

Isolated hypoganglionosis (IHG) has been proposed as a distinct entity with two subtypes: congenital IHG (CIHG) and acquired IHG (AIHG). However, due to the rarity of the disease and the lack of defining histological criteria, the concept of IHG is not widely accepted. We studied paraffin-embedded intestinal specimens from 79 patients diagnosed with Hirschsprung's disease (HD) (n = 49), CIHG (n = 25), and AIHG (n = 5) collected between January 1996 and December 2015. Histopathological diagnosis of HD, CIHG, and AIHG was confirmed by hematoxylin and eosin staining and immunohistochemical staining using Hu C/D and CD56. We evaluated (immuno)histopathological findings, counted the number of ganglion cells, and measured the size of Auerbach's plexus. Hu C/D labeled neuronal cell bodies, whereas CD56 was detected in all neuronal components. In HD, all ganglion cells in Auerbach's plexus in the normoganglionic segment (NGS) were immunoreactive for Hu C/D, whereas in the aganglionic segment (AGS), these were replaced by CD56-positive extrinsic nerve fibers and bundles. The number of ganglion cells in AIHG and CIHG was significantly lower than in the NGS of HD (p < 0.05). Auerbach's plexus was significantly smaller in CIHG (p < 0.05) but in AIHG equivalent to the NGS in HD. In summary, immunostaining for Hu C/D and CD56 is useful for definitive histopathological diagnosis of IHG.

Background: The prokinetic agent cisapride is effective for the treatment of gastroesophageal reflux disease (GERD) in infants and children, but is no longer used for this purpose because of safety concerns. Therefore, other pharmacological agents need to be investigated for efficacy in GERD treatment. In this study, we examined the effectiveness and safety of mosapride for the treatment of neurologically impaired children and adolescents with GERD., Methods: Mosapride (0.3 mg/kg/day) was administered to 11 neurologically impaired patients with GERD (five male; median age, 12.3 years). Esophageal acid exposure was measured using esophageal pH monitoring before and at >5 days after the start of mosapride treatment. The pressure and length of the lower esophageal sphincter were compared before and after mosapride treatment., Results: In the 11 patients, median reflux index (percentage of the total monitoring period during which recorded pH was <4.0) was 17.5% (range, 4.4-59%) before and 8.2% (range, 2.8-20.7%) after mosapride treatment (P = 0.02). Median esophageal clearance was 1.0 min/reflux (range, 0.5-2.1 min/reflux) before and 0.7 min/reflux (range, 0.4-1.2 min/reflux) after treatment with mosapride (P = 0.02). The median number of reflux episodes before (219) and after (122) drug treatment did not differ significantly., Conclusion: The decreased reflux index in neurologically impaired patients with GERD is due to mosapride, therefore mosapride may be a candidate for GERD treatment., (© 2016 Japan Pediatric Society.)

Purpose: For 30 years, we have consecutively performed rectal mucosal punch biopsy to diagnose Hirschsprung's disease. The aim of this study was to evaluate the safety of our technique., Methods: Patients with suspected Hirschsprung's disease who underwent punch biopsy, including our original "K-PUNCH" method using an S-moid forceps and non-specific blood-collecting tube at our department and branch hospital between April 1986 and March 2016 were included in the present study. Our punch biopsy technique is characterized by excellent visibility and a direct grasping sensation. The backgrounds and complications of the patients were retrospectively investigated., Results: During this period, 954 patients (median age 4 months; range 1 day-73 years) underwent punch biopsy. Although there were no cases of severe complications (i.e., rectal perforation, infection or full-thickness biopsy), one (0.1%) of the 954 cases in the early period showed liver dysfunction and required transfusion due to bleeding. In addition, inappropriate specimens were obtained in 37 patients (3.9%)., Conclusion: Punch biopsy including the "K-PUNCH" method is considered safe and feasible and is associated with a low rate of complications and inappropriate specimen harvesting among patients of all ages. Comorbidities, including the potential for hemorrhage, should always be considered.

Purpose: Thoracoscopic esophageal atresia/tracheoesophageal fistula (EA/TEF) repair in neonates is technically difficult because of the small working space and fragility of tissues. This study aimed to show the construct validity of the neonatal EA/TEF simulator by video-based assessment of endoscopic suturing skill., Materials and Methods: A rapid-prototyped neonatal chest model with an artificial esophagus model similar to the actual neonatal esophagus was developed. Forty pediatric surgeons performed an endoscopic intracorporeal suturing task and a knot-tying task using the model, and a questionnaire survey was administered. Each task was video recorded and assessed using two skill assessment methods (the 29-point checklist method and the error assessment sheet method). The task completion time and the number of manipulations were measured., Results: With regard to experience in performing thoracoscopic TEF repair, the experienced surgeons (ESs, ≥3 EA/TEF repair experiences, n = 6) were significantly superior to the inexperienced surgeons (ISs, <3 experiences, n = 34) in all metrics. Upon comparison by the pediatric Endoscopic Surgical Skill Qualification (ESSQ) status, there were no significant differences in all metrics between the ESSQ-qualified (n = 15) and nonqualified (n = 25) surgeons. The qualified ESs (n = 6) were significantly superior to the qualified ISs (n = 9) in all metrics., Conclusion: Video-based endoscopic surgical skill assessment using the neonatal EA/TEF simulator could differentiate ESs from ISs. The construct validity of the simulator was demonstrated, and the simulator would be useful especially for practicing thoracoscopic EA/TEF procedures.

Background: Although nonoperative treatment (non-OPT) for complicated appendicitis is performed widely, the long-term outcomes and merits of interval appendectomy (IA) need to be evaluated., Methods: Between April 2007 and December 2013, all appendicitis patients with well-circumscribed abscess or phlegmon were required to select either laparoscopic surgery (OPT) or non-OPT with optional IA on admission. Optional IA was planned at ≥3months after non-OPT. For non-OPT, intravenous injection of antibiotics was continued until the serum C-reactive protein concentration decreased to <0.5mg/dL, with occasional drainage of abscesses., Results: Thirty-three patients chose OPT, and 55 chose non-OPT. Among non-OPT patients, 16 selected IA. The success rate of non-OPT was 98.2%. Recurrence occurred in 13 (34.2%) of the 38 non-IA group patients. Although the non-IA group patients frequently had perforated appendicitis at recurrence, they visited the hospital earlier than at the initial appendicitis and had less inflammation. Readmission rate or complications in patients undergoing IA were not different compared with those of the patients in the non-IA group, who had recurrence at ≥3months, or with those of patients in the OPT group., Conclusion: Although many patients experienced recurrent appendicitis after successful nonoperative treatment, IA may not be necessary after non-OPT., Level of Evidence: Prospective comparative study, level II., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Aim: Scoliosis, which is often associated with neurological impairment in children, sometimes makes it difficult to perform laparoscopic procedures. This study assessed the impact of scoliosis on performing laparoscopic Nissen fundoplication., Methods: Medical records and radiographic examinations of patients who underwent laparoscopic Nissen fundoplication at a single institution from 2006 to 2015 were reviewed retrospectively. Patients' data on age at surgery, height, weight, duration of pneumoperitoneum, and amount of bleeding were collected. The Cobb angle was measured using X-rays, and the direction (right or left) of the scoliotic curve was recorded. The chest compression ratio was calculated using computed tomography axial images., Results: Eighty-five patients were included and analyzed in this study, of which 89% were neurologically impaired. Median age, height, and weight were 120 months, 110 cm, and 17 kg, respectively. A positive correlation between age and the Cobb angle (ρ = 0.64) and a negative correlation between age and the chest compression ratio (ρ = -0.56) were observed. The right-curved scoliotic group showed significantly more bleeding than the nonscoliotic (<10°) group (P = .01; nonscoliotic, 0 mL; right curved, 7.5 mL; left curved, 0 mL). The severe scoliotic group (≥45°) showed more bleeding than the nonscoliotic group (P = .02). Neither the direction of the scoliotic curve nor scoliotic severity showed a significant difference in the duration of pneumoperitoneum., Conclusions: The older the patient, the more severe their scoliosis and chest compression were. Right-curved or severe scoliosis could be risk factors for intraoperative bleeding in laparoscopic Nissen fundoplication.

Purpose: Minimally invasive surgery (MIS) has become widely accepted as a technique for abdominal neuroblastoma resection. However, the indications for MIS are still controversial. The aim of this study was to evaluate image-defined risk factors (IDRFs), complications, and oncologic outcomes in patients with abdominal neuroblastomas treated with MIS., Methods: Between August 1998 and February 2016, MIS was planned for 20 children with abdominal neuroblastomas. Clinical data were retrospectively reviewed and compared between the IDRF-negative and IDRF-positive patients., Results: On the basis of the latest IDRF guidelines, five patients were classified as IDRF-positive and four of them had operative complications; namely, partial infarction of the ipsilateral kidney or open conversion. Concerning the two patients who needed open conversion, the primary reason for open conversion was difficulty in dissection of the tumor from the vena cava. Preoperative images of these cases showed either deformation or subtotal encasement of the vena cava. Relapse occurred in three high-risk patients and in none of the low/intermediate-risk patients. No complication occurred in the IDRF-negative cases., Conclusions: IDRF-negative might be a good indication for MIS for abdominal neuroblastoma. However, deformation or subtotal encasement of the vena cava should be considered as IDRF-positive for MIS.

Purpose: We previously developed a pediatric thoracoscopic surgical simulator and showed its construct validity. In this study, the same skill assessment experiments were conducted in an additional 31 surgeons and the results of 53 surgeons in total were analyzed., Methods: A suture pad with force sensors was placed in a rapid-prototyped pediatric chest model of a 1-year-old patient. Participants completed the endoscopic intracorporeal suturing and knot-tying task, both in the pediatric chest model setup and in a box trainer setup. The task was evaluated using eight metrics: the 29-point checklist score, error score, number of manipulations, task completion time, force index, width of the pad's slit after suturing, and right and left tool paths., Results: The 53 participants included skilled surgeons certified by the Endoscopic Surgical Skill Qualification (ESSQ) system (n = 8) and unskilled surgeons without the certification (n = 45). The skilled surgeons showed significantly better performance than the unskilled surgeons in six metrics in the pediatric chest model setup. In contrast, no significant differences between the two groups were observed in the box trainer setup. Upon comparison between the setups, the unskilled surgeons showed worse results in six metrics in the pediatric chest model than in the box trainer, whereas the skilled surgeons showed equivalent performance in both setups., Conclusions: Our pediatric thoracoscopic surgical simulator was superior to the conventional box trainer for identifying skilled surgeons. The skilled surgeons showed excellent performance even in the intricate pediatric chest model, providing quantified targets for young pediatric surgeons' training.

Background: Laparoscopic Kasai portoenterostomy has been performed in infants with biliary atresia at several institutions, but laparoscopic anastomosis requiring multi-directional suturing on a vertical plane of the liver remains a challenge. To assist multi-directional suturing, we developed a multi-degree-of-freedom (DOF) needle driver whose tip length was 15 mm and shaft diameter was 3.5 mm. The tip of the multi-DOF needle driver has three DOFs for grasp, flection and rotation. The aim of this study was to evaluate the performance of the multi-DOF needle driver in two kinds of in vivo experiments., Methods: Surgeons were asked to perform four-directional laparoscopic suturing on a vertical plane of the liver in six rabbits using the multi-DOF needle driver or a conventional needle driver. The needle grasping time, the needle handling time, the number of needle insertions, the number of liver lacerations, the suturing width and depth, and the area of necrotic tissues were analyzed and compared. Additionally, one surgeon was asked to perform laparoscopic hepato-jejunostomy in four rabbits to assess the feasibility of Kasai portoenterostomy using the multi-DOF needle driver., Results: The suturing depth using the multi-DOF needle driver was significantly larger than that using the conventional needle driver in both the right and downward suturing directions. No statistically significant differences were found in other metrics. Liver lacerations were observed only when suturing was performed using the conventional needle driver. The experimental laparoscopic hepato-jejunostomy using the multi-DOF needle driver was successful., Conclusions: Using the multi-DOF needle driver, uniform multi-directional suturing on a vertical plane of the liver could be performed. The short distal tip of the multi-DOF needle driver demonstrated its advantages in multi-directional suturing in a small body cavity. The multi-DOF needle driver may be able to be used to perform complex tasks in laparoscopic Kasai portoenterostomy.

Owing to recent advances in minimally invasive surgery (MIS), laparoscopic and thoracoscopic surgery have been gradually introduced for use in neonates and infants. This review focuses on two popular MIS procedures for diaphragmatic diseases in neonates and infants: congenital diaphragmatic hernia (CHD) repair and plication for diaphragmatic eventration. While several advantages of MIS are proposed for CDH repair in neonates, there are also some concerns, namely intraoperative hypercapnia and acidosis and a higher recurrence rate than open techniques. Thus, neonates with severe CDH, along with an unstable circulatory and respiratory status, may be unsuitable for MIS repair, and the use of selection criteria is, therefore, important in these patients. It is generally believed that a learning curve is associated with the higher recurrence rate. Contrary to CDH repair, no major disadvantages associated with the use of MIS for diaphragmatic eventration have been reported in the literature, other than technical difficulty. Thus, if technically feasible, all pediatric patients with diaphragmatic eventration requiring surgical treatment are potential candidates for MIS. Due to a shortage of studies on this procedure, the potential advantages of MIS compared to open techniques for diaphragmatic eventration, such as early recovery and more rapid extubation, need to be confirmed by further studies.

The National Clinical Database (NCD) of Japan was established in April, 2010 with ten surgical subspecialty societies on the platform of the Japan Surgical Society. Registrations began in 2011 and over 4,000,000 cases from more than 4100 facilities were registered over a 3-year period. The gastroenterological section of the NCD collaborates with the American College of Surgeons' National Surgical Quality Improvement Program, which shares a similar goal of developing a standardized surgical database for surgical quality improvement, with similar variables for risk adjustment. Risk models of mortality for eight procedures; namely, esophagectomy, partial/total gastrectomy, right hemicolectomy, low anterior resection, hepatectomy, pancreaticoduodenectomy, and surgery for acute diffuse peritonitis, have been established, and feedback reports to participants will be implemented. The outcome measures of this study were 30-day mortality and operative mortality. In this review, we examine the eight risk models, compare the procedural outcomes, outline the feedback reporting, and discuss the future evolution of the NCD.

Background: Laparoscopic pyloromyotomy (LP) is a well-established approach for treating hypertrophic pyloric stenosis. Although grasping the pylorus with forceps is important in LP, it requires a high level of surgical skill. To make this approach easier, in 2008 we introduced the method of Dozier and Kim, in which a vascular clamp (VC) is used for grasping the pylorus (LP with VC). Here, we retrospectively compared results between traditional LP (TLP) and LP with VC., Materials and Methods: Forty-three patients underwent LP with VC, whereas 194 patients underwent TLP. Two ports in the umbilicus and the left flank are common in both procedures. Instead of a right flank port, however, in LP with VC, we directly insert a VC to grasp the pylorus., Results: Patients' characteristics were not different between the two groups. There was only one wound infection and no major complication associated with LP with VC, whereas there were three major complications in TLP. Our hospital is a pediatric teaching institution in Japan where many surgeons perform their first LP. Twenty-eight surgeons performed their first LP in our hospital in the past 17 years, and LP with VC was the first LP for nine. The operative time for surgeons' first LP was significantly shorter with LP with VC than with TLP, based on Welch's t test (42.8 versus 54.4 minutes; P = .048)., Conclusions: Our results suggest that LP with VC is an easy and safe technique, and it is recommended for beginners in laparoscopic surgery.

We report a 14-year-old boy with alpha-fetoprotein-producing gastric cancer (AFPGC) who was found with ruptured metastatic tumor in the liver. AFPGC is exceedingly rare in pediatric age. It often shows metastases to the liver and should be included in differential diagnoses of liver tumors with increased serum AFP.