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Purpose: This study investigated the anti-inflammatory effect of clodronate, a vesicular nucleotide transporter (VNUT) inhibitor, on intestinal-failure-associated liver disease (IFALD) in a rat model of short bowel syndrome (SBS)., Methods: The rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90% small bowel resection. The animals were divided into the following groups: TPN/SBS (Control group), TPN/SBS/intravenous administration of low-dose clodronate (20 mg/kg twice per week; Low group), or TPN/SBS/intravenous administration of high-dose clodronate (60 mg/kg twice per week; High group). On day 7, the rats were euthanized. Hepatic steatosis and hepatocellular injury were also assessed., Results: Hepatic steatosis and lobular inflammation in the liver were observed in all groups. The High group showed histologically reduced hepatic steatosis compared with the Control group. IL-6 and Nlrp3 expression in the High group was significantly suppressed compared to that in the Control group. The expression of other inflammatory cytokines tended to be lower in the High dose group than in the control group. The lipid metabolism gene expression in the liver specimens showed no significant differences among the groups., Conclusion: The high-dose administration of clodronate may, therefore, inhibit hepatic steatosis and inflammation associated with IFALD in patients with SBS., Competing Interests: Declarations Conflict of interest Yudai Tsuruno and the other co-authors have no conflicts of interest to declare. Data availability The datasets generated and/or analyzed during the current study are available from the corresponding author upon reasonable request., (© 2024. The Author(s).)

Purpose: To identify the prognostic factors for pediatric severe intestinal motility disorder (IMD)., Methods: We reviewed the medical records of patients with severe IMD, who required total parenteral nutrition (TPN) for ≥ 60 days at our institution between April, 1984 and March, 2023, examining their characteristics to identify prognostic factors., Results: The types of IMD in the 14 patients enrolled in this study were as follows: isolated hypoganglionosis (IHG, n = 6), extensive aganglionosis (EAG: n = 6), and chronic idiopathic intestinal pseudo-obstruction (CIIP, n = 2). There was no significant difference in mortality among the three types of severe IMD. Weaning-off TPN and the use of the colon were not significant prognostic factors, but cholestasis was a significant prognostic factor (p = 0.005). There was a high mortality rate (50%), with the major causes of death being intestinal failure-associated liver disease (IFALD) following hepatic failure, and catheter-related blood stream infection (CRBSI). One IHG patient underwent small bowel transplantation but died of acute rejection., Conclusion: Severe IMD is still associated with a high mortality rate and cholestasis predicts the prognosis. Thus, preventing or improving IFALD and CRBSI caused by long-term TPN is important for reducing the mortality rate., (© 2024. The Author(s).)

Purpose: Ultrasound-guided supraclavicular catheterization (UGSC) of the brachiocephalic vein (BCV) for long-term tunneled central venous catheter (tCVC) insertion may be safer than the internal jugular vein approach due to its superior field of view. We examined the clinical outcomes of tCVC insertions performed by junior residents through UGSC of the BCV., Patients and Methods: From January 2018 to December 2023, we assessed clinical outcomes and compared the experience levels of surgeons conducting tCVC insertions. Surgeons were categorized into three groups: junior residency (JR), senior residency (SR), and board-certified pediatric surgeons (BCPS)., Results: 177 tCVC insertions were done on 146 patients. Intraoperative complications included 6 cases of arterial puncture, 1 case of pneumothorax, 1 case of over insertion of catheter tip, and 1 case of suspected hemothorax. Distribution across groups was as follows: 28 cases (15.8%) in JR group, 92 (52.0%) in SR group, and 57 (32.2%) in BCPS group. Although the JR group exhibited longer operation times than the BCPS group, no significant differences in intraoperative complications were noted., Conclusion: Junior residents can safely perform UGSC for tCVC insertion. However, careful consideration of complications such as arterial or thoracic puncture is essential and case selection should be based on experience., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis., Methods: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients., Results: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001)., Conclusions: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study aimed to investigate the impact of hepatocyte growth factor (HGF) on colonic morphology and gut microbiota in a rat model of short bowel syndrome (SBS)., Methods: SD rats underwent jugular vein catheterization for total parenteral nutrition (TPN) and 90% small bowel resection [TPN + SBS (control group) or TPN + SBS + intravenous HGF (0.3 mg/kg/day, HGF group)]. Rats were harvested on day 7. Colonic morphology, gut microflora, tight junction, and Toll-like receptor-4 (TLR4) were evaluated., Results: No significant differences were observed in the colonic morphological assessment. No significant differences were observed in the expression of tight junction-related genes in the proximal colon. However, the claudin-1 expression tended to increase and the claudin-3 expression tended to decrease in the distal colon of the HGF group. The Verrucomicrobiota in the gut microflora of the colon tended to increase in the HGF group. The abundance of most LPS-producing microbiota was lower in the HGF group than in the control group. The gene expression of TLR4 was significantly downregulated in the distal colon of the HGF group., Conclusion: HGF may enhance the mucus barrier through the tight junctions or gut microbiome in the distal colon., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Ovarian endometriomas (OEs) are rarely found in the pediatric population, especially before menstruation. We report a 6-year-old girl who was postoperatively diagnosed with OE before menstruation., Case Presentation: A 6-year-old girl presented to a local pediatrician with abdominal pain and vomiting. Abdominal ultrasonography revealed a multilocular cystic lesion to the left of the bladder. Magnetic resonance imaging (MRI) revealed similar findings, with the contents of the cyst showing a low signal on T1-weighted imaging and a high signal on T2-weighted imaging. The patient was referred to our institution for further examination. Enhanced computed tomography (CT) showed a multilocular cystic lesion sized 56 × 44 × 30 mm with partial calcification. The left ovarian vein was dilated, suggesting the origin of the tumor to be the left ovary. Extirpation of the lesion was performed under laparoscopic assistance. Pathological findings indicated an ovarian endometrioma. To our knowledge, this is the youngest report of an OE diagnosed in a patient prior to menstruation., Conclusions: OEs in children before menstruation are extremely rare; thus, the long-term prognosis is yet to be determined., (© 2024. The Author(s).)

Purpose: We compared the clinical features of patients with biliary atresia (BA) associated with a bleeding tendency (BT) at the time of the diagnosis with those of patients without a bleeding tendency (NBT)., Methods: The patients' background characteristics, age in days at the first visit, Kasai portoenterostomy (KPE), and postoperative course were retrospectively analyzed., Results: Nine of the 93 BA patients (9.7%) showed a BT, including 7 with intracranial hemorrhaging (ICH), 1 with gastrointestinal bleeding, and 1 with a prothrombin time (PT) of 0%. The age at the first visit was 62 ± 12 days old for BT patients and 53 ± 27 days old for NBT patients (p = 0.4); the age at KPE was 77 ± 9 days old for BT patients and 65 ± 24 days old for NBT patients (p = 0.2); the time from the first visit to surgery was 13 ± 7 days for BT patients and 11 ± 10 days for NBT patients (p = 0.5); and the native liver survival rate was 56% for BT patients and 58% for NBT patients (p = 1), with no significant difference in any of the parameters. The neurological outcomes of survivors of ICH were favorable., Conclusions: Appropriate BT correction allowed early KPE even after ICH, resulting in native liver survival rates comparable to those of NBT patients without significant neurological complications., (© 2023. The Author(s) under exclusive licence to Springer Nature Singapore Pte Ltd.)

Background: Transumbilical laparoscopy-assisted appendectomy (TULAA) is the technique of choice for all types of appendicitis. However, the technique is challenging for trainees to learn in comparison with performing conventional three-port laparoscopic appendectomy (CTPLA) in children. We aimed to compare the surgical outcomes of children with appendicitis treated by TULAA versus CTPLA performed by pediatric surgeons in training (PSITs). Materials and Methods: This retrospective study analyzed pediatric patients with acute appendicitis treated with CTPLA or TULAA between April 2016 and December 2022. Operative time (OT: minutes), pneumoperitoneum time (PT: minutes), blood loss (milliliter), length of hospital stay (days), and surgical site infection rate were compared between the two groups. Operative outcomes were also analyzed according to type of appendicitis such as uncomplicated and complicated cases. Results: Two hundred twenty-five laparoscopic appendectomies were performed by CTPLA ( n  = 94) or TULAA ( n  = 131). All cases were performed by PSITs and there was no open conversion cases. TULAA had a shorter OT (67.0 ± 28.4 versus 78.3 ± 21.7; P  < .01) and PT (26.1 ± 17.4 versus 52.5 ± 22.1 min; P  < .01). The surgical site infection rate was slightly higher in the TULAA group, but the difference was not statistically significant. In uncomplicated appendicitis ( n  = 164), significant differences between the CTPLA and TULAA groups were observed in OT (CTPLA versus TULAA: 70.7 ± 14.9 versus 59.1 ± 21.6, P  < .01) and PT (CTPLA versus TULAA: 43.6 ± 13.1 versus 20.4 ± 13.6, P  < .01). With regard to postoperative complications, only surgical site infection was significantly different between the CTPLA and TULAA groups (CTPLA: 0.0% versus TULAA: 8.2%, P  < .05). In complicated cases ( n  = 61), there were significant differences between the groups in PT (CTPLA versus TULAA: 73.4 ± 24.9 versus 42.3 ± 17.2, P  < .01) and length of hospital stay (CTPLA versus TULAA: 7.0 ± 1.3 versus 8.9 ± 4.7, P  < .05). Conclusions: TULAA had a shorter OT and PT than CTPLA. TULAA for PSITs shows similar safety and feasibility to CTPLA for not only uncomplicated cases but also complicated cases.

Background: Most cases of intestinal malrotation appear in neonates with bilious vomiting due to midgut volvulus, whereas in cases that develop beyond infancy, the initial symptoms vary. This study investigated the clinical features of these two populations and identified issues that should be considered in daily practice., Methods: A retrospective chart review was conducted from January 1, 2010, to December 31, 2022. Data on patients with intestinal malrotation were collected in an anonymized fashion from five pediatric surgical hub facilities in the Southern Kyushu and Okinawa areas of Japan., Results: Of the 80 subjects, 57 (71.3%) were neonates (Group N) and 23 (28.7%) were infants and schoolchildren (Group I). The frequencies of initial symptoms, such as abdominal distention (Group N: 19.3% vs. Group I: 13.0%), bilious vomiting (59.6% vs. 43.5%), and hematochezia (8.8% vs. 21.7%), were not skewed by the age of onset (p = 0.535, 0.087, and 0.141, respectively). Midgut volvulus was significantly more frequent in Group N (71.9% [41/57] vs. 34.8% [8/23]; p = 0.005), while the degree of torsion was greater in group I (median 360° [interquartile range: 180-360°] vs. 450° [360-540°]; p = 0.029). Although the bowel resection rate was equivalent (7.0% [4/57] vs. 4.3% [1/23]; p = 1.000), half of the patients in Group N presented with 180° torsion. The neonatal intestine has been highlighted as being more susceptible to ischemia than that in older children., Conclusions: The incidence of midgut volvulus is higher in neonates than in older children. Even relatively mild torsion can cause ischemic bowel changes during the neonatal period., Level of Evidence: LEVEL III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Abstract: We, herein, report a surgical technique for laparoscopy-assisted jejunostomy tube placement in an infant using a loop needle device to fix the jejunum and abdominal wall. A 3-year-old boy with Down's syndrome underwent nutritional management by gastrostomy due to oral feeding difficulty after radical surgery for congenital duodenal stenosis and following bile duct stenosis. However, intractable gastrostomy site leakage emerged; hence, laparoscopy-assisted gastrostomy takedown and simultaneous laparoscopy-assisted jejunostomy tube placement were planned. After laparoscopy-assisted gastrostomy closure was performed, the jejunum was extracted through the umbilical trocar wound. A jejunostomy tube kit was inserted at the left side of the umbilicus. After tube insertion into the jejunum, the jejunostomy tube was wrapped with four interrupted sutures using the Witzel technique. Suture threads were extracted by percutaneous insertion of a loop needle device, and then, the jejunum was fixed to the abdominal wall. The post-operative course was uneventful. Percutaneous insertion of a loop needle device is useful for fixing a jejunostomy tube to the abdominal wall using the Witzel technique. Our technique is safe, secure and expected to reduce the risk of dislodgement of tube and peritonitis associated with jejunostomy tube placement., (Copyright © 2023 Copyright: © 2023 Journal of Minimal Access Surgery.)

Background: We evaluated the effect of recombinant human hepatocyte growth factor (rh-HGF) on intestinal adaptation in a rat model of short-bowel syndrome (SBS)., Methods: Sprague-Dawley rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90 % small bowel resection. The animals were divided into 3 groups: TPN/SBS (control group, n = 7), TPN/SBS/intravenous recombinant human hepatocyte growth factor (HGF) (0.3 mg/kg/day) (HGF group, n = 7), and TPN/SBS/intravenous c-Met inhibitor (0.3 mg/kg/day) (anti-HGF group, n = 5). On day 7, rats were euthanized and histologically evaluated. Serum diamine oxidase (S-DAO) levels were evaluated using an enzyme-linked immunosorbent assay. The nutrient transporter and glucagon-like peptide-2 (GLP-2) receptor expression were evaluated using real-time polymerase chain reaction., Results: The jejunal and ileal villus heights were higher and the S-DAO concentrations significantly higher (p = 0.04) in the HGF group than in the control and anti-HGF groups. The sodium-dependent glucose transporter 1 expression in the HGF group was significantly higher than in the control group and significantly suppressed in the anti-HGF group (p < 0.01). The peptide transporter 1 expression in the jejunum was higher in the HGF group than in the other groups and significantly suppressed in the anti-HGF group (p < 0.01). The GLP-2 receptor expression in the jejunum was higher in the HGF group than the other groups, and it was significantly suppressed in the anti-HGF group (p < 0.01). These jejunal results regarding nutrient transporter an GLP-2 receptor were not found in the ileum., Conclusions: The administration of rh-HGF appears to be more effective in the jejunum than in the ileum., Type of Study: Experimental Research., Level of Evidence: N/A., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: The purpose of this study was to investigate the autophagy associated with apoptosis in hepatic damage in the short bowel syndrome rat model., Methods: SD rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90% small bowel resection. Animals were divided into two groups: TPN plus SBS (Control group) or TPN plus SBS plus intravenous administration of HGF (HGF group). On day 7, the rats were harvested, and hepatocellular injury was evaluated., Results: In an SBS rat model, hepatic steatosis and lobular inflammation were histologically suppressed in the HGF group (p < 0.01). The expression of tumor necrosis factor-α in the HGF group tend to be higher than that in the control group (p = 0.13). The gene expression of transforming Growth Factor-β in the HGF group was suppressed compared to the control group (p < 0.01). HGF treatment may have an antiapoptotic effect via the intrinsic pathway by caspase 9. Protein expressions of Rubicon (p = 0.03) and p62 (p < 0.01) in the HGF group were found to have increased compared to those in the control group., Conclusion: The inhibitory effect of HGF on hepatic steatosis remains unclear, and further studies focusing on the mechanisms of fat accumulation are needed., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Anovestibular fistula (AVF) is the most common type of ARM in female patients. The present study investigated changes over time in the postoperative defecation function of female patients with AVF., Methods: Patient data were collected from 1984 to 2021. Eighty-eight female patients with AVF were enrolled. Patients' characteristics and the long-term outcome of defecation function were reviewed and analyzed retrospectively. The bowel function was evaluated according to the Japan Society of ARM Study Group evacuation score (ES)., Results: Thirty-eight patients underwent anal transposition (AT), and 8 underwent anterior sagittal anorectoplasty (ASARP). The total evacuation score (ES) in AVF patients reached "excellent" at nine years old, regardless of the operative procedure. The constipation scores with AT showed better improvement than those with ASARP, but soiling scores in the ASARP group showed better improvement than those in the AT group. The postoperative complications did not affect the postoperative bowel function in AVF patients., Conclusion: Most patients with AVF eventually achieved a satisfactory total ES. Given the difference in defecation score transition depending on the operative procedure or postoperative complications, it may be important to perform long-term defecation management via surgical procedures., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Surgical management of neuroblastoma (NB) has been performed by open procedures for decades. However, advances in surgical devices and technology have made minimally invasive surgery safe and reproducible. In this study, we compared open and laparoscopic surgery regarding biopsy success and curative resection to determine the safety and feasibility of laparoscopic surgery for pediatric patients with adrenal NB., Methods: We reviewed the clinical data of 22 NB patients who underwent surgery from 2006 to 2021 in our institution. All patients with adrenal NB were diagnosed histologically, and we retrospectively analyzed the data., Results: The male/female ratio was 16/6. The median age was 2.5 (interquartile range: 2-4) years old, and the laterality was on the right in 13 and on the left in 9. A total of 20 patients underwent a tumor biopsy: 14 via laparotomy, five laparoscopically, and one retroperitoneally. Four patients underwent laparoscopic resection and 11 patients underwent open resection after chemotherapy. Two patients with stage LI underwent primary tumor resection laparoscopically. For curative resection in image-defined risk factor (IDRF)-negative patients, laparoscopic surgery resulted in shorter operation time, with less bleeding and an earlier oral intake. The three IDRF-single-positive patients on the liver (laparoscopic surgery: one patient) had a shorter operation time and less bleeding than the IDRF-multiple positive patients., Conclusion: Laparoscopic surgery was performed in a limited number of adrenal NB cases. A laparoscopic biopsy for adrenal NB seems to be safe and feasible to perform. Laparoscopic surgery, in carefully selected cases, permits safe and efficient resection of adrenal NB in pediatric patients., (© 2023 Asia Endosurgery Task Force and Japan Society of Endoscopic Surgery and John Wiley & Sons Australia, Ltd.)

Background: The repair of large abdominal wall defects that cannot be closed primarily is quite challenging. The component separation technique (CST) is a surgical approach using autologous tissue to close large abdominal wall defects. The CST requires extensive dissection between the abdominal skin and the anterior sheath of the rectus abdominis muscle. Subsequently, incisions are made at both sides of the external oblique aponeurosis, releasing the external oblique muscle from the internal oblique muscle, and then the right and left rectus abdominis muscles are brought together in the midline for defect closure. However, impairment of blood flow in the abdominal wall skin and necrotic changes are recognized as potential complications., Case Presentation: The CST was performed in a 4-year-old boy with a large ventral hernia who had undergone skin closure with abdominal wall relaxing incisions for the primary treatment of giant omphalocele in the neonatal period. Given his history of incisions on the abdominal wall, he was speculated to be at high risk for postoperative skin ischemia. Dissection was therefore kept to a minimum to preserve the blood supply from the superior and inferior epigastric arteries and perforating branches of those arteries through the rectus abdominis muscle. In addition, care was taken to adjust the muscle relaxant dosage while monitoring the intravesical pressure, ensuring that it did not exceed 20 mmHg to avoid impaired circulation in the abdominal wall caused by abdominal compartment syndrome. He was discharged 23 days after the surgery without any complications, and neither recurrence of the ventral hernia nor bowel obstruction was observed in 4 years., Conclusions: A giant omphalocele with primary skin closure was treated by applying the CST. The procedure can be performed safely while preserving the blood flow to the abdominal wall, even in patients with a history of relaxing incisions on the abdominal skin. The CST is expected to be effective for repairing the large abdominal wall defects seen in giant omphalocele when primary closure is not possible., (© 2023. The Author(s).)

Purpose: Severely neurologically impaired patients sometimes require anti-reflux surgery with preceding gastrostomy. We apply a traction technique for laparoscopic fundoplication (LF) without gastrostomy takedown (GTD) in such cases. We conducted a multicenter review to assess the feasibility of our approach. Materials and Methods: In brief, the traction technique involves left-lateral-traction of the stomach body, right-lateral-traction of the round ligament of the liver, and elevation of the left liver lobe to create a sufficient field for manipulating the forceps. Patients who underwent LF with Nissen's procedures in 2010-2022 were retrospectively reviewed. Data were analyzed by a one-way analysis of variance. Results: The operative approaches included the traction technique ( n  = 16; Group 1), GTD and reconstruction ( n  = 5; Group 2), and LF followed by gastrostomy ( n  = 92; Group 3). In comparison with Group 1, significant differences were only found in pneumoperitoneum time (Group 1 versus Group 2 versus Group 3: 174.4 minutes versus 250.4 minutes versus 179.5 minutes; P  = .0179). Operating time (222.7 minutes versus 303.0 minutes versus 239.7 minutes; P  = .0743), duration to full-strength enteral nutrition (10.4 days versus 17.2 days versus 11.0 days; P  = .0806), and length of hospital stay (17.2 days versus 31.0 days versus 18.5 days; P  = .3247) were equivalent. No re-fundoplication was required in Group 1. Conclusion: The traction technique secures the operative quality and outcome of LF without GTD.

Purpose: Midgut volvulus is an urgent disease often occurring in neonates. This study clarified the clinical features of midgut volvulus and evaluated predictors to avoid bowel resection., Methods: This bi-center retrospective study enrolled 48 patients who underwent surgery for intestinal malrotation between 2010 and 2022. Patients' background characteristics and preoperative imaging findings were reviewed., Results: Midgut volvulus was recognized in 32 patients (66.7%), and 6 (12.5%) underwent bowel resection. Based on a receiver operating curve analysis of bowel resection, the cut-off value of the body weight at birth and at operation were 1984 g [area under the curve (AUC) 0.75, 95% confidence interval (CI) 0.52-0.99] and 2418 g (AUC 0.70, 95% CI 0.46-0.94), respectively. The cut-off value of intestinal torsion was 540° (AUC 0.76, 95% CI 0.57-0.95), and that of the time from the onset to the diagnosis was 12 h (AUC 0.85, 95% CI 0.72-0.98). For midgut volvulus with an intestinal torsion > 540°, the most sensitive preoperative imaging test was ultrasonography (75%) Patients with bloody stool tended to undergo bowel resection., Conclusions: Patients with a low body weight and bloody stool should be confirmed to have whirlpool sign by ultrasonography and scheduled for surgery as soon as possible., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: In this study, we used a near-infrared ray catheter (NIRC) to visualize the urethral line. We herein report our intraoperative visualization technique of the urethra using an illuminating catheter in laparoscopy-assisted anorectoplasty (LAARP) for imperforated anus., Patient and Surgical Technique: A 3.0-kg term male neonate with anorectal malformation was delivered. An invertogram revealed the type as intermediate. Transverse colostomy was performed at the left upper abdomen. A recto-bulbar urethral fistula (RBUF) was diagnosed via distal colostogram and voiding cystourethrogram. LAARP was planned at 6 months of age. We performed the operation with four trocars. A 45° 5-mm scope was used to clearly view the deep pelvic space. Before starting rectal dissection, a 6-Fr pig-tail-type NIRC was inserted through the external opening of the urethra to visualize the urethra during the laparoscopic procedure. The catheter tip was placed in the bladder, and excretion of urine was maintained through the NIRC during the procedures. While dissecting the deep pelvic space between the posterior wall of the urethra and anterior wall of the rectum, the exact line of the urethra was clearly confirmed by overlay images of the NIRC. The RBUF was dissected safely using this innovative image-guided technique. Anoplasty was performed between the rectal stump and perineal skin. The postoperative course was uneventful. Oral intake was started on postoperative day 1. Postoperative dynamic urography showed no complications., Conclusion: An NIRC is useful for detecting the urethra during LAARP., (© 2022 Asia Endosurgery Task Force and Japan Society of Endoscopic Surgery and John Wiley & Sons Australia, Ltd.)

Introduction: Management of neonates with long gap esophageal atresia (LGEA) is one of the most challenging situations facing pediatric surgeons. Delayed anastomosis after internal traction for esophageal lengthening was reported as a useful technique for long gap cases. Additionally, the use of near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in pediatric surgery, especially for blood perfusion validation. We report a novel technique for safe and secure anastomosis for LGEA in the neonatal period using internal traction and ICG-guided NIR fluorescence., Patient and Surgical Technique: A pregnant woman with polyhydramnios was admitted to the department of obstetrics in our hospital. At 29 weeks of gestation, ultrasound showed mild polyhydramnios and absence of the fetal stomach. A male neonate was born at 38 weeks of gestation with 21 trisomy. EA (Gross type A) was diagnosed based on an X-ray study that showed the absence of gastric bubble with a nasogastric tube showing the "coil-up" sign. Thoracoscopic internal traction and laparoscopic gastrostomy were performed on day 4 after birth. We confirmed the distance between the upper pouch and lower pouch on X-ray. On day 16 after birth, thoracoscopic anastomosis was performed. We successfully performed esophageal anastomosis without tearing the esophageal wall. Blood perfusion of the upper and lower pouch was validated after anastomosis using ICG-guided NIR fluorescence., Conclusion: Delayed anastomosis for LGEA in the neonatal period using internal traction and ICG-guided NIR fluorescence is safe and feasible., (© 2022 Asia Endosurgery Task Force and Japan Society of Endoscopic Surgery and John Wiley & Sons Australia, Ltd.)

Purpose: The intravenous administration of acetaminophen (IAA) has become standard postoperative analgesic management for pediatric surgery. However, the most effective methods of IAA for postoperative acute appendicitis are unclear. We evaluated the analgesic efficacy of scheduled IAA vs on-demand IAA for postoperative acute appendicitis., Methods: Ninety-four patients who underwent laparoscopic appendectomy in our institution between January 2017 and December 2020 were enrolled. The patients were divided into two groups based on the postoperative pain control protocols. The scheduled IAA group (SA group, n = 42) was managed by scheduled IAA and additional on-demand use of pentazocine as rescue therapy. The on-demand IAA group (ODA group, n = 52) was managed by on-demand IAA as the first choice, with pentazocine as the second choice for pain control. The patients' background characteristics, operative results and postoperative outcomes were reviewed., Results: The number of times pain complaints were made per patient per day (NPPD) on postoperative days (POD) 1 and 2 was significantly lower in the SA group than in the ODA group (POD 1; 1.12 ± 1.21 vs 2.62 ± 1.89, p < 0.01; POD 2; 0.45 ± 0.86 vs. 1.31 ± 1.69, p < 0.01). According to pathological findings, NPPD was lower in the SA group than in the ODA group for both phlegmonous appendicitis (0.71 ± 1.01 vs. 2.10 ± 2.13, P < .05) and gangrenous appendicitis (1.33 ± 1.50 vs 2.94 ± 1.68, P < .01). On POD 2, the incidence of gangrenous appendicitis was significantly lower in the SA group than in the ODA group (0.57 ± 0.93 vs 1.78 ± 1.86, P < .01)., Conclusions: Scheduled IAA may have favorable efficacy for postoperative pain control after emergency surgery for acute appendicitis in pediatric patients., (© 2022 Asia Endosurgery Task Force and Japan Society of Endoscopic Surgery and John Wiley & Sons Australia, Ltd.)

Background: Reduction en masse (REM) is a rare condition following manual inguinal hernia (IH) reduction in which a hernia sac is reduced back into the preperitoneal space with a loop of the bowel incarcerated at the neck of the sac. It resembles successful manual reduction and may thus be overlooked easily. We herein report an infantile case of REM of an IH that was successfully treated laparoscopically., Case Presentation: A 10-month-old boy with a surgical history of bilateral open IH repair at 4 months old presented with a bulge in his left groin and vomiting. A left incarcerated recurrent IH was suspected, and manual reduction was performed. The hernia was apparently reduced successfully, but abdominal distention and vomiting persisted. He was admitted for further observation due to the symptoms. On day 2 after admission, abdominal X-ray showed extensive small bowel obstruction (SBO). Enhanced computed tomography (CT) revealed protrusion of the small bowel with a closed-loop in the left groin. A closed-loop SBO due to postoperative adhesion or an internal hernia was suspected. To assess the etiology of SBO, emergent laparoscopic exploration with hernia repair was planned. Laparoscopy revealed REM of the left incarcerated IH with a thickened peritoneum at the neck of the sac. Laparoscopic reduction was performed, and the incarcerated small bowel showed no signs of ischemia. The hernia sac was not associated with the previously ligated processes vaginalis, which had been closed by a previous Potts' procedure. It was located at the inside of the processes vaginalis. The sac was successfully closed by laparoscopic percutaneous extraperitoneal closure procedures, and iliopubic tract repair was also performed via the previous inguinal incision. The postoperative course was uneventful., Conclusion: Pediatric IH is due to the patent processes vaginalis, and REM is extremely rare. Laparoscopic surgery for REM is a relatively common and useful approach for the diagnosis and treatment of adults. In our infantile case, the laparoscopic approach was similarly effective for both investigating the cause of SBO and performing high ligation of the sac for this rare condition with IH., (© 2022. The Author(s).)

Purpose: Representative neonatal surgical diseases are often complicated by congenital heart disease (CHD). We reviewed our decade of experience from the perspective of the prognosis and report on the management of infants with CHD., Methods: Cases with and without CHD between 2011 and 2020 were retrospectively compared. Qualitative data were analyzed using a chi-square test with Yates' correction, and quantitative data were compared using Student's t-test., Results: Of the 275 neonatal surgical cases, 36 had CHD (13.1%). Ventricular septal defect was the most common cardiac anomaly, followed by atrial septal defect. Esophageal atresia showed the highest complication rate of CHD (43.8%, 14/32) followed by duodenal atresia (38.5%, 10/26). The mortality rates of patients with and without CHD (22.2% [8/36] vs. 1.3% [3/239]) were significantly different (χ 2  = 30.6, p < 0.0001). Of the eight deaths with CHD, six patients had cyanotic complex CHD. Notably, four of these patients died from progression of inappropriate hemodynamics in the remote period after definitive non-cardiac surgery., Conclusion: Considering its high-mortality, the presence of CHD, especially cyanotic heart disease, is an important issue to consider in the treatment of neonatal surgical diseases. Pediatric surgeons should be alert for changes in hemodynamics after surgery, as these may affect mortality., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Severely neurologically impaired patients often require the creation of a gastrostomy when they develop difficulty with oral intake followed by antireflux surgery. We use a traction technique for laparoscopic fundoplication without removing the gastrostomy in such cases. Right lateral traction of the round ligament of the liver and left lateral traction of the stomach body are performed using a needle device. The left liver lobe is elevated with a liver retractor inserted via the epigastrium. This traction technique allows a sufficient surgical field for manipulating forceps to be created in cases with a gastrostomy, enabling fundoplication to be performed safely without removing the gastrostomy., (© 2022 Asia Endosurgery Task Force and Japan Society of Endoscopic Surgery and John Wiley & Sons Australia, Ltd.)

Purpose: Short bowel syndrome (SBS) patients require total parenteral nutrition (TPN) following massive small bowel resection (SBR), which may cause intestinal failure-associated liver disease (IFALD), a life-threatening complication. Hepatocyte growth factor (HGF) acts as a potent hepatocyte mitogen with anti inflammatory and antioxidant actions. The present study evaluated the effect of recombinant human HGF (rh-HGF) on SBR and subsequent IFALD using a parentally fed rat model of SBS., Methods: Rats underwent jugular vein catheterization for continuous TPN and 90% SBR. They were divided into 2 groups: TPN alone (SBS/TPN group: n = 7) or TPN plus the intravenous administration of rh-HGF (0.3 mg/kg/day) (SBS/TPN+HGF group: n = 7). On day 7, their tissues and stool were harvested to evaluate the effects of HGF., Results: Regarding the histological findings, based on the nonalcoholic fatty liver disease (NAFLD) activity score, the SBS/TPN+HGF group showed significantly less hepatic steatosis and inflammatory cell infiltration than the SBS/TPN group (NAFLD activity score, 4.00 ± 1.83 vs. 1.00 ± 0.82; p < 0.01). The SBS/TPN+HGF group showed a higher expression of Farnesoid X receptor in the liver and lower expression of Toll-like receptor 4 in the ileum than the SBS/TPN group. Regarding the composition of the bacterial gut microbiota, Actinobacteria, Bacteroidetes and Proteobacteria were decreased in the SBS/TPN+HGF group compared with the SBS/TPN group., Conclusion: In our SBS with TPN rat model, rh-HGF administration had a preventive effect against hepatic steatosis and dysbiosis. rh-HGF may therefore be a potentially effective therapeutic agent for SBS and subsequent IFALD., Type of Study: Experimental research., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest in association with the present study., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Enteric duplication is a congenital disease that occurs throughout the entire gastrointestinal tract. Although it may sometimes cause intestinal volvulus, a few reports have described cases of enteric duplication twisted on itself. We experienced a rare pediatric case of long-segment tubular ileal duplication showing torsion. Torsion of enteric duplication is extremely rare. We herein report a pediatric case showing unusual torsion of ileal duplication requiring emergency surgery., Case Presentation: A 3-year-old boy presented with abdominal pain and vomiting. Contrast-enhanced computed tomography (CT) revealed a cystic luminal structure with a blind end and fluid collection in the pelvic cavity. CT also showed no findings of ileus or intestinal dilatation except for a cystic luminal structure. The preoperative diagnosis was torsion of Meckel's diverticulum. The patient underwent emergent explorative diagnostic laparoscopy. As a result, a necrotic luminal structure and bloody ascites were recognized, and small-scale laparotomy was performed. Long-segment ileal duplication was recognized. The long-segment tubular ileal duplication shared the anti-mesenteric side of the intestinal wall along one-third of its length. The residual two-thirds of its length was free from the ileum and its blind end was twisted in a manner that looked similar to Meckel's diverticulum. Normal ileum and the duplication, including the twisted necrotic portion, were resected, and ileal anastomosis was performed. The postoperative course was uneventful. A pathological examination confirmed the definitive diagnosis of enteric duplication., Conclusions: We reported the unusual presentation of intestinal duplication mimicking torsion of Meckel's diverticulum. Enteric duplication shows various clinical symptoms and presentations. We must understand that the classification of digestive enteric duplication is diverse with a variety of associated clinical symptoms., (© 2022. The Author(s).)

Purpose: Retroperitoneal teratomas (RPTs) are rare in infants. We report our experience of treating pediatric patients with RPTs over many years at a single institution, with the aim of developing a safe and secure operative strategy for RPTs in infants., Methods: We reviewed the medical records of patients who underwent treatment for RPTs in our institution between April, 1984 and December, 2017, to analyze their background and clinical data. The diagnosis of RPT was confirmed histologically in all patients., Results: The subjects of this retrospective analysis were 14 pediatric patients (female, n = 11; male, n = 4), ranging in age from 6 days to 12 years, 11 (73.3%) of whom were under 1 year of age. Complete surgical resection was performed in all patients. The tumor ruptured during surgery in four (26.7%) patients and perioperative vessel injuries occurred in six (40.0%) patients, resulting in nephrectomy in one (6.7%). Three (20.0%) patients suffered unilateral renal dysfunction as a surgical complication. Only one patient received postoperative chemotherapy. All patients were free of disease at the time of writing., Conclusion: Perioperative complications are not uncommon during surgery for RPTs, despite their benign nature. Preoperative imaging evaluation is important and operative management may be challenging. Because of the favorable prognosis and the frequency of adverse events in surgery, partial resection or split excision is sometimes unavoidable. Meticulous follow-up for recurrence is required for such patients., (© 2021. Springer Nature Singapore Pte Ltd.)

Background: Image-guided surgery with an open magnetic resonance imaging (MRI) system is applied for brain tumors in the neurosurgery field, but has rarely been reported in pediatric surgery. We report our initial experience of intraoperative confirmation of precision rectal pull-through during laparoscopically assisted anorectoplasty (LAARP) in an open MRI operating theater for pediatric patients with anorectal malformation (ARM)., Case Presentation: A 3.0 kg term male neonate was delivered with anorectal malformation. An invertogram revealed the intermediate type. Transverse colostomy was made on the left upper abdomen. The recto-bulbar urethral fistula (RBUF) was diagnosed by a distal colostogram and voiding cystourethrogram. LAARP was planned at 6 months of age. Because this was the first procedure in which the pediatric abdomen had been scanned in an open MRI operating theater in our institution, we scanned his pelvic floor under sedation 3 weeks before the operation using the open MRI system in our operation room. We performed the operation with 4 trocars. The peritoneal reflection was carefully incised and the rectum was dissected. The RBUF was resected. The center of the muscle complex was detected at the perineal skin with an electrical nerve stimulator, and a 7-mm longitudinal skin incision was made on the perineal lesion for anoplasty. The muscle complex and the pubo-rectal sling were confirmed laparoscopically using a 3.5-mm bipolar forceps connected to the electrical nerve stimulator. Anoplasty was performed between the rectal stump and perineal skin. After anoplasty, the patient was scanned with open MRI under general anesthesia. We attached the quadrature-detection (QD) head coil around the patient's pelvis and inserted him in the gantry. A 0.45-T open MRI clearly revealed that the pulled through rectum was located in the center of the muscle complex on T2-weighted images. The postoperative course was uneventful. Oral intake was started on post-operative day 1. Postoperative dynamic urography showed no complication (e.g., leakage or residual fistula)., Conclusions: We successfully performed LAARP for ARM, with intraoperative confirmation of precision rectal pull-through in an open MRI operating theater. Further cases are required to evaluate the application of open MRI systems in pediatric surgery., (© 2021. The Author(s).)

Background: Accessory liver lobe (ALL) is a rare liver malformation. An ALL develops due to malformation of the endodermal caudal foregut and segmentation of the hepatic bud in the third week of gestation. Most ALLs are asymptomatic and are detected incidentally during abdominal surgery. The incidence of ALL is < 1% in patients who undergo abdominal surgery. However, some ALLs twist and cause acute abdomen. We experienced a pediatric case of ALL torsion in a patient who underwent elective laparoscopic surgery., Case Presentation: The 5-year-old girl had a 3-month history of epigastralgia and vomiting, which occurred every 2 weeks. Abdominal ultrasonography with color Doppler imaging revealed an 11.8 × 13.6 mm nonvascular lesion with mixed echogenicity near the round ligament of the liver. Enhanced computed tomography confirmed a 14 × 16 × 20 mm low-attenuation mass surrounded by a hyperdense line and disproportionate fat stranding on the right side of the round ligament of the liver. There was no ascites or hemorrhage. These findings suggested an abscess of the round ligament of the liver. Her symptoms improved with the administration of oral antibiotics; thus, we planned to perform elective exploratory laparoscopy and subsequent resection. Two trocars (5 mm) were inserted through a multichannel port device at the umbilicus and one trocar (3 mm) was inserted at the right lateral abdomen. Upon observation of the abdominal cavity, the omentum was observed adhering to the round ligament of the liver. Macroscopic observation revealed no apparent mass lesions. We performed adhesiolysis of the omentum from the round ligament of the liver using a vessel sealing system. We performed resection at the site at which adhesion had formed between the round ligament of the liver with the surrounding tissue using a vessel sealing system and the resected specimen was extracted through the umbilical wound. The postoperative course was uneventful. A pathological examination revealed necrotic liver tissue. The resected tissue was founded to be an ALL with ischemic change., Conclusions: The recurrent abdominal pain was induced by torsion of the ALL. Pediatric surgeons should consider ALL torsion as a differential diagnosis for epigastralgia of unknown etiology.

Nutritional disorders in Duchenne muscular dystrophy (DMD) worsen the medical condition. In particular, obesity is a serious problem that increases the risk of cardiomyopathy and affects nursing care. However, it is often difficult to evaluate body fatness in the advanced stages of DMD. Skinfold thickness measurement is a classical method to evaluate body fatness and is easily performed, even for bed-bound patients at home. We aimed to investigate the utility of skinfold thickness measurement in non-ambulatory DMD patients. Twenty-two patients with non-ambulatory, steroid-naive DMD ranging in age of 12-47 years were evaluated by body mass index (BMI), blood tests, measurement of triceps skinfold thickness (TSF), and abdominal computed tomography (CT) measurement of the areas of both subcutaneous and visceral fat. TSF showed good correlation with BMI (r = 0.80; p < 0.001), serum triglycerides (r = 0.67; p < 0.01), area of subcutaneous fat (r = 0.85; p < 0.0001), and area of visceral fat (r = 0.76; p < 0.0001). These results indicate the skinfold thickness measurement may be applicable as a screening tool in clinical practice where CT and magnetic resonance imaging assessment is often difficult in patients with advanced DMD., (Copyright © 2016 Elsevier B.V. All rights reserved.)