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Purpose: This study was performed to clarify the clinical features of cloacal exstrophy (CE) patients who underwent anoplasty and their functional outcomes based on a nationwide survey in Japan. Methods: A questionnaire survey was conducted and data were obtained from 229 CE patients. After the exclusion of non-surviving patients and unknown data, 195 patients were enrolled. We compared the patient characteristics of the no anoplasty group (NAP group) to the anoplasty group (AP group). The defecation function of patients who underwent anoplasty was evaluated. Results: The AP group had significantly lower rates of omphalocele (p = 0.045) and spinal defects (p = 0.003) than the NAP group. Of the 195 patients enrolled in this study, only 17 (8.7%) underwent anoplasty. Of the 17 patients who underwent anoplasty, 7 (41.1%) eventually had a permanent stoma after anoplasty [after ileostomy: n = 3 (60.0%), after colostomy: n = 4 (44.4%)]. Regarding soiling, 4 patients (66.7%) showed the absence of soiling, 2 (33.3%) showed accidental soiling, and no patients showed frequent soiling. Conclusion: In our study, spinal defects may have affected the determination of anoplasty. Anoplasty may improve the quality of life, because defecation can be kept clean, but indications, including colon function, may be carefully considered. [ABSTRACT FROM AUTHOR]

Purpose: After conducting a nationwide survey of persistent cloaca (PC), we assessed whether or not the timing of definitive anorectoplasty affects the long-term bowel function of patients with PC. Methods: Patient information was obtained via questionnaire, and a total of 169 PC patients who underwent posterior sagittal anorectourethrovaginoplasty (PSARUVP) were enrolled in this study. Patients were classified into 2 groups based on their operative period, which was analyzed by the area under the receiver operating characteristic curve: the early group (EG) underwent anorectoplasty at ≤ 18 months old (n = 106), and the late group (LG) underwent anorectoplasty at > 18 months old (n = 63). The bowel function was evaluated using the evacuation score of the Japan Society of Anorectal Malformation Study Group. We also examined the postoperative results of vaginoplasty. Results: The total evacuation score was significantly higher in the EG than in the LG (5.2 ± 1.7 vs. 4.2 ± 1.8, p = 0.003). The frequency of bowel movement and the constipation scores were significantly higher in the EG than in the LG (1.4 ± 0.6 vs. 1.2 ± 0.7, p < 0.05, 2.4 ± 1.0 vs. 2.1 ± 1.0, p < 0.05, respectively). Postoperative vaginal stenosis was observed in 18 cases (10.7%), of which 16 could be reconstructed transperineally. Conclusion: PSARUVP should be performed in early infancy and facilitate vaginal reconstruction. [ABSTRACT FROM AUTHOR]

Purpose: Various prognostic predictors for biliary atresia (BA) have been identified. This study aimed to evaluate the serial changes in the preoperative and postoperative ubiquitous inflammatory biomarkers and their relationship with the outcomes in patients with BA. Patients and methods: Forty-three BA patients were retrospectively reviewed to investigate serial levels of ubiquitous inflammatory biomarkers, including C-reactive protein (CRP) and lymphocyte ratio, and outcomes. The patients with BA were divided based on their outcomes into two prognostic groups: the native liver survivor group (n = 30) and the survivors with living-donor liver transplant group (n = 13). Results: The area under the receiver operating characteristic (ROC) curve analysis showed that a preoperative lymphocyte ratio of < 61% and CRP value > 0.1 mg/dl predicted a poor outcome. In the ROC curve analysis, the timing of reaching the cut-off value of CRP after Kasai portoenterostomy was postoperative day (POD) 57. The third postoperative week, which was the timing of the discontinuation of steroid therapy, was the branchpoint of inflammatory markers between the two prognostic groups. Conclusion: The POD 57 CRP level predicts the surgical outcome of Kasai portoenterostomy. The postoperative anti-inflammatory management of BA can be monitored by the ubiquitous inflammatory biomarkers CRP and the preoperative lymphocyte ratio. [ABSTRACT FROM AUTHOR]

Purpose: Mayer-Rokitansky-Küster-Häuser syndrome (MRKH) usually presents with primary amenorrhea after puberty. The present study clarified the clinical features of MRKH diagnosed at under 16 years old.Methods: We conducted a questionnaire survey to determine the status of MRKH in Japan. Twenty-one MRKH patients were enrolled, and the patient background and clinical features were analyzed.Results: The median age at the diagnosis was 2.6 [1.0-9.0] years. Three patients (14.3%) were diagnosed with type I MRKH, while 18 (85.7%) were diagnosed with type II. The associated anomalies included (some cases overlapped): anorectal malformation (ARM) (n = 13, 72.2%), renal malformation (n = 12, 66.7%), vertebral malformation (n = 11, 61.1%), esophageal atresia (n = 5, 27.8%), and cardiac malformation (n = 4, 22.2%). The comparison of patients with and without ARM revealed that patients with ARM were significantly younger than those without ARM at the time of the diagnosis (1.5 [1-2.6] years vs. 9.1 [8.2-11.7] years, p < 0.05). Associated ARM was the most frequent reason for the diagnosis of MRKH (n = 10, 47.6%).Conclusions: MRKH patients with some associated anomalies, especially ARM, were frequently diagnosed in preoperative imaging examinations or radical operation for ARM. Pediatric surgeons should pay close attention to female patients with ARM to prevent errors in the diagnosis of MRKH. [ABSTRACT FROM AUTHOR]

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

Purpose: In general, patients with anorectal malformation (ARM) undergo surgical treatment at around 6 months of age in Japan. We aimed to clarify whether the timing of a definitive operation affects the long-term bowel function. Methods: Patient data were collected from 1984 to 2007. Fifty-two male patients with high- and intermediate-type ARM were enrolled. Patients were classified into two groups based on their operative period: the early group (EG) underwent anorectoplasty at < 5 months of age (n = 22); the late group (LG) underwent anorectoplasty at ≥ 5 months (n = 30). The bowel function was evaluated at 3, 5, 7, 9, and 11 years of age using the evacuation score (ES) of the Japan Society of Anorectal Malformation Study Group. Results: The total score and four functional outcomes improved chronologically with age. The constipation score in the EG showed significantly better improvement in comparison to the LG. The total score and the incontinence score in the EG were significantly higher than those in the LG at 11 years of age. Conclusion: The long-term bowel function in the EG was better than that in the LG. Anorectoplasty at an early age was important for achieving a better postoperative bowel function in ARM patients. [ABSTRACT FROM AUTHOR]

Aim Of the Study: Liver retraction during laparoscopic fundoplication is important for obtaining an optimal space. Several methods have been developed, but the risks and benefits are unclear. We compared three different approaches and evaluated their safety and utility.Methods: Forty-three neurologically impaired patients who underwent laparoscopic fundoplication between 2005 and 2018 were classified into three groups: A, snake retractor method, n = 18; B, crural suture method, n = 13; C, needle grasper method, n = 12. Patients' characteristics and outcomes were reviewed.Main Results: The liver retraction time was significantly shorter in group C than in A or B (p < 0.05). The operative times were shorter in groups B and C than in A. There were no significant differences in the liver enzyme levels. The liver enzyme levels increased temporarily but improved within a week. The C-reactive protein levels were significantly lower in group B than in A or C (p < 0.05).Conclusions: The most convenient method was the needle grasper method, as the other two approaches create conflict with the operator's forceps. The crural suture method damages the liver less, but requires higher surgical skill levels. It is important to select the appropriate method according to the operator's skill and the patient's size and deformity. [ABSTRACT FROM AUTHOR]

Aim Of the Study: We previously showed an increased number of smaller portal vein (PV) branches in the portal areas of liver biopsy specimens of biliary atresia (BA) patients. We evaluated the correlation between this histopathological feature and the prognosis.Patients and Methods: Twenty-five consecutive patients with BA encountered between 2000 and 2012 were classified into three prognostic groups based on their postoperative outcomes: Excellent (n = 11) for native-liver survivors with a normal liver function, Good (n = 6) for native-liver survivors with liver dysfunction, and Poor (n = 8) for survivors after liver transplant or on a waiting list. Data from morphometrical analyses, including the fibrotic portal area, numbers of PVs, diameter and total area of PV branches, were statistically compared among the three groups.Main Results: The number of PV branches per unit area of the whole-liver specimen in the poor prognostic group was significantly lower than that in the excellent group (3.1 ± 0.6 vs. 5.2 ± 2.0/mm2, p = 0.03). There were no significant differences in the other parameters.Conclusions: This is the first report on the relationships between morphometrically analyzed PV branches and the postoperative course in BA patients. The portal venous system is involved as the primary lesion in BA. [ABSTRACT FROM AUTHOR]

Purpose: Many kinds of operative procedures have been proposed for anorectal malformation (ARM) patients. At our institution, sacroperineal or sacroabdominoperineal anorectoplasty (SP-SAP) have been performed from 1984 to 2007. The aim of this study is clarify the change over the time in the postoperative bowel function in male ARM patients.Methods: Patient data were collected from 1984 to 2007. Fifty-two male patients with high- and intermediate-type ARM were enrolled. The patients' characteristics and bowel function were reviewed and analyzed retrospectively. The bowel function was evaluated according to the evacuation score (ES) of the Japan Society of ARM Study Group.Results: The operative procedures were SP-SAP in 52 male patients. The total ES improved chronologically and significantly until 11 years of age. Regarding the clinical stratification of the ES, the ratio of "excellent" and "good" results was over 91.9% at 11 years of age. A satisfactory bowel movement score was achieved by 9 years of age. The constipation, incontinence and soiling scores improved slowly but continuously until 11 years of age.Conclusion: The ES showed continuous improvement after a definitive operation. An understanding of the characteristics of improvement is very important in managing the postoperative bowel function in ARM patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: Cloacal exstrophy (CE) patients may need bladder reconstruction after initially undergoing surgery to obtain continence and improve their quality of life. This study attempts to clarify the clinical features of CE patients who underwent bladder augmentation (BA) and their urinary functional outcomes based on a nationwide survey in Japan. Methods: A questionnaire survey was conducted, and 150 CE patients were enrolled. Their clinical characteristics and urinary outcomes were reviewed. Results: BA was performed in 52 patients (34.7%). Most cases underwent early bladder closure at initial surgery in neonate period. The age at the BA was performed 6.4 [6–9.0] years. Among them, the most used organ for BA was ileum (n = 30, 57.7%). Regarding the outcomes, the age when the renal function was evaluated was 14.0 [10.0–20.5] years and the serum creatinine level was 0.44 [0.36–0.60] (mg/dl). Clean intermittent catheterization was required in 37 (71.2%) patients. On the other hand, no dialysis or kidney transplantation was necessary in any of these patients. Conclusion: The renal function and conditions of patients who underwent BA were relatively well preserved. Individualized management with a stepwise surgical approach for CE patients should thus be considered in the future. [ABSTRACT FROM AUTHOR]

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 (χ2 = 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. [ABSTRACT FROM AUTHOR]

Purpose: Total parental nutrition (TPN) causes gastrointestinal mucosal atrophy. The present study investigated the effects of hepatocyte growth factor (HGF) on the intestinal mucosal atrophy induced by TPN. Methods: Rats underwent jugular vein catheterization and were divided into four groups: oral feeding (OF), TPN alone (TPN), TPN plus low-dose HGF (0.3 mg/kg/day; TPNLH), and TPN plus high-dose HGF (1.0 mg/kg/day; TPNHH). On day 7, rats were euthanized, and the small intestine was harvested and evaluated histologically. The expression of c-MET, a receptor of HGF, and nutrition transporter protein were evaluated using quantitative polymerase chain reaction. Results: The jejunal villus height (VH) and absorptive mucosal surface area in the TPNHH group were significantly higher than in the TPN group (p < 0.05). The VH in the ileum showed the same trend only in the TPNHH group, albeit without statistical significance. The crypt cell proliferation rate (CCPR) of the jejunum in both HGF-treated groups was significantly higher than in the TPN group (p < 0.01). The expression of c-MET and transporter protein in all TPN-treated groups was decreased compared with that in the OF group. Conclusion: HGF attenuated TPN-associated intestinal mucosal atrophy by increasing the villus height, which was associated with an increase in CCPR. [ABSTRACT FROM AUTHOR]

Purpose: Total parenteral nutrition (TPN) sometimes induces parenteral nutrition-associated liver disease (PNALD). Hepatocyte growth factor (HGF) acts as a potent hepatocyte mitogen anti-inflammatory and antioxidant actions. We aimed to evaluate the effect of HGF on PNALD in a rat model of TPN. Methods: A catheter was placed in the right jugular vein for 7-day continuous TPN. All rats were divided into three groups: TPN alone (TPN group), TPN plus intravenous HGF at 0.3 mg/kg/day [TPN + HGF (low) group], and TPN plus HGF at 1.0 mg/kg/day [TPN + HGF (high) group]. On day 7, livers were harvested and the histology, inflammatory cytokines and apoptosis were evaluated. Results: Histologically, lipid droplets were apparent in the TPN group, but decreased in the TPN + HGF (low) and TPN + HGF (high) groups. The histological nonalcoholic fatty liver disease activity scores in the TPN + HGF (low) and TPN + HGF (high) groups were significantly lower than that in the TPN group (p < 0.01). There were no significant differences in the inflammatory cytokine levels of the three groups. The caspase-9 expression levels in the TPN + HGF (low) and TPN + HGF (high) groups were significantly decreased in comparison to that in the control group (p < 0.05). Conclusion: The intravenous administration of HGF attenuated hepatic steatosis induced by 7-day TPN dose dependently. [ABSTRACT FROM AUTHOR]

Purpose: Composite lipid emulsion (CLE) has been used for intestinal failure-associated liver disease (IFALD) to compensate for the disadvantages of soybean oil lipid emulsion (SOLE) or fish oil lipid emulsion (FOLE). However, the influence of its administration is unclear. We evaluated the effects of these emulsions on IFALD using a rat model of the short-bowel syndrome. Methods: We performed jugular vein catheterization and 90% small bowel resection in Sprague–Dawley rats and divided them into four groups: control (C group), regular chow with intravenous administration of saline; and total parenteral nutrition co-infused with SOLE (SOLE group), CLE (CLE group) or FOLE (FOLE group). Results: Histologically, obvious hepatic steatosis was observed in the SOLE and CLE groups but not the FOLE group. The liver injury grade of the steatosis and ballooning in the FOLE group was significantly better than in the SOLE group (p < 0.05). The TNF-α levels in the liver in the FOLE group were significantly lower than in the SOLE group (p < 0.05). Essential fatty acid deficiency (EFAD) was not observed in any group. Conclusion: Fish oil lipid emulsion attenuated hepatic steatosis without EFAD, while CLE induced moderate hepatic steatosis. The administration of CLE requires careful observation to prevent PN-induced hepatic steatosis. [ABSTRACT FROM AUTHOR]

Purpose: Short bowel syndrome (SBS) patients require long-term parenteral nutrition following massive bowel resection, which causes intestinal failure-associated liver disease (IFALD). Previous reports have shown that glucagon-like peptide-2 (GLP-2) resulted in the bowel adaptation for SBS. The aim of this study was to evaluate the effect of GLP-2 for IFALD in a parenterally fed rat model.Methods: Using rat, a catheter was placed in the jugular vein, and 90% small bowel resection (SBR) was performed. Animals were divided into three groups: SBR and total parenteral nutrition (TPN) (SBS/TPN group), SBR and TPN plus GLP-2 at 1 µg/kg/h [SBS/TPN/GLP-2 (low) group], and SBR and TPN plus GLP-2 at 10 µg/kg/h [SBS/TPN/GLP-2 (high) group]. On day 13, the liver was harvested and analyzed by using nonalcoholic fatty liver disease (NAFLD) score.Results: Histologically, hepatic steatosis in the SBS/TPN group and SBS/TPN/GLP-2 (high) group was observed. Both steatosis and lobular inflammation score in the SBS/TPN/GLP-2 (low) group were significantly lower compared with those in the other two groups (p < 0.05). Active NAFLD score in the SBS/TPN/GLP-2 (low) group was significantly lower compared with that in the SBS/TPN/GLP-2 (high) group (p < 0.01).Conclusion: Low-dose GLP-2 intravenous administration improves hepatic steatosis of IFALD following in an SBS parenterally fed rat model. [ABSTRACT FROM AUTHOR]

Purpose: This study assessed the impact of 2D and 3D environments by comparing pediatric surgeons (PS) and gastrointestinal surgeons (GIS) using a laparoscopic hepaticojejunostomy simulator.Methods: We developed a high-fidelity simulator of laparoscopic hepaticojejunostomy. Thirty-five participants (19 PS and 16 GIS) performed hepaticojejunostomy in both 2D and 3D environments. We evaluated the required time, total path length, and average velocities of bilateral forceps in both situations using the para-axial port layout.Results: Regarding the participants' characteristics, the performance history of laparoscopic hepaticojejunostomy differed significantly between PS and GIS. In PS, the 3D environment did not markedly affect compared with 2D. In GIS, however, the 3D environment affected the time and movement of the right forceps. There were no significant differences in the time between PS and GIS in either environment. In both environments, the right-hand movement of the PS group was shorter and slower than that of the GIS group, but the left-hand movement was the opposite.Conclusion: There were significant differences in forceps movement characteristics between the PS and GIS. The effects of a 3D environment could not be clarified in this study, because it may depend on the port layout used and the operative procedures. [ABSTRACT FROM AUTHOR]

Purpose: Portal hypertension in patients with biliary atresia (BA) is generally thought to result from portal vein (PV) narrowing secondary to hepatic fibrosis. To test the hypothesis, we morphometrically analyzed the PVs and hepatic arteries (HAs).Methods: Morphometrical analyses of 25 BA and 26 non-BA liver biopsy specimens from patients treated from 2000 to 2014. The total specimen area, the fibrotic portal area, vessel diameter and medial thickness of the HAs were measured.Results: The PV diameter in BA patients was significantly smaller than that in non-BA patients. In BA, the numbers of normal-sized PVs and capillaries were decreased and increased, respectively. The PV diameter was not significantly correlated with the degree of fibrosis. We newly found that medial hypertrophy and the HA diameter increased with the number of endothelial cells in BA. The PV diameter was not significantly correlated with the medial thickness and was positively correlated with the HA diameter in BA.Conclusions: The narrowing of the PV is unlikely to occur secondarily to liver fibrosis. The medial hypertrophy of the HA is not correlated with the decrease in the PV blood flow. These findings seem to be unique to the primary vascular lesions of BA. [ABSTRACT FROM AUTHOR]