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Purpose: The study aimed to explore and describe the lives of patients with persistent cloaca (PC) from childhood to adulthood., Methods: Semistructured interviews were conducted with nine adult patients with PC. Their experiences and thoughts regarding this disease were analyzed qualitatively and inductively., Results: After classifying the experiences and thoughts of patients with PC, 13 categories were extracted. The following five themes emerged from these categories. (1) Difficulties with excretion and vaginal management because of the disease. (2) The degree of understanding of those around them and society has a huge effect on their way of life. (3) The inferiority of a woman who is not a "normal woman." (4) A "never-ending disease" in which problems continue even after the transition period. (5) Differences in the central point of the narrative depending on the age group., Conclusions: In this study, qualitative and inductive analyses of data from semistructured interviews with patients with PC revealed their experiences and thoughts. The results will provide a guide for young patients and the medical professionals who treat them. Accordingly, monitoring their lives until adulthood is necessary., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study aims to clarify the relationship between changes in skeletal muscle mass during treatment and prognosis of pediatric malignant solid tumors., Methods: Patients with pediatric malignant solid tumors who were treated at Kyushu University Hospital from 2007 to 2017 were divided into two groups: the progression-free survival (PFS) group and the relapse/death (R/D) group; the psoas major muscle volume (PMV) was then compared. We also measured the PMV and psoas muscle area (PMA) of pediatric patients with no complications who underwent surgery for acute appendicitis (control) and compared the values with those of patients with malignant tumors., Results: No significant differences were observed in the PMV and PMA between patients with appendicitis and those with malignant tumors. Significant differences were found in the rate of change in PMV between the PFS (1.424) and R/D groups (1.071) (P = 0.0024). When the cut-off value of the rate of change in the PMV was 1.20, patients whose rate of change in PMV was ≥ 1.20 had longer PFS (P = 0.0231) and overall survival (P = 0.0229) than those whose rate of change was < 1.20., Conclusion: Pediatric patients with malignant solid tumors and increased skeletal muscle mass during treatment have a good prognosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: The number of accessible central veins (CVs) affects the prognosis of patients with intestinal failure (IF). The loss of residual CVs should be avoided. We, therefore, evaluated the efficacy of a new CV catheter-exchange technique using a subcutaneous fibrous sheath (FS) in pediatric IF patients., Methods: We retrospectively collected the CV catheter (CVC) data of pediatric IF patients managed from January 2009 to December 2019. The data were divided into two groups; Groups 1 (CVCs placed with the FS method) and Group 2 (CVCs placed by the primary or another insertion). The main outcome was the CVC indwelling time., Results: Eighty-five CVCs were analyzed. The FS method was attempted in 47 cases and succeeded in 40 (85%). No significant difference was observed between the groups regarding characteristics. A log-rank test revealed an equivalent CVC indwelling time between the two groups (Group 1: 268 [126-588] days vs. Group 2: 229 [126-387] days, p = 0.256)., Conclusions: The FS method is highly recommended for pediatric IF patients, as its attempt showed a high success rate with an indwelling time equivalent to primary insertion. The FS method leads to the prolonged use of a single CV and thereby contributes to improving the outcomes of pediatric IF patients., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Hirschsprung's disease-related short bowel syndrome (HDSBS) is characterized by aganglionosis that extends orally to 75 cm from Treitz's band. The condition is reported be associated with a high mortality rate of 50-80%. This retrospective study aimed to survey the current trends in HDSBS treatment in Japan., Methods: Patient data were extracted from the results of a nationwide survey we conducted, resulting in the retrospective collection of the data of 1087 HD patients from 2008 to 2012 in Japan., Results: A total of 11 (0.9%) cases of HDSBS were noted. All patients underwent jejunostomy as neonates. Radical procedures performed in five patients (A-colon patch method in four, Duhamel's procedure in one). Ziegler's myotomy-myectomy and serial transverse enteroplasty (STEP) were performed in each patient as palliative procedures. No radical operations were performed in 4 of the 11 cases. The mortality rate was 36.4%. Four patients died, 1 patient who underwent STEP and 3 patients who received no radical procedures. The causes of death were sepsis due to enterocolitis or central intravenous catheter infection, and hepatic failure. All patients who underwent radical procedures survived and showed satisfactory outcomes., Conclusion: HDSBS still showed a high mortality rate, although surgical approaches such as the A-colon patch method resulted in satisfactory outcomes.

Purpose: Acquired isolated hypoganglionosis (A-IH) is a late-onset intestinal pseudo-obstruction disorder and shows different pathophysiological findings from congenital isolated hypoganglionosis (C-IH). In this study, we retrospectively examined five cases of A-IH and investigated the features of A-IH., Methods: Five cases of A-IH were extracted from a nationwide retrospective cohort study in 10 years, from which totally 355 cases of Allied Disorders of Hirschsprung's Disease (ADHD) were collected., Results: Ages of onset were between 13 and 17 years in three cases, and 4 years and 4 months in ones. Initial symptoms were abdominal distension and/or chronic constipation in 4 cases, whereas one exhibited intestinal perforation. Affected lesions varied from case to case, extending various length of intestinal tracts. All cases underwent multiple operations (average: 4.6 times), such as enterostomy, resection of dilated intestines, and/or pull-through. Pathological findings showed the decreased numbers of ganglion cells and degeneration of ganglion cells, whereas the size of the plexus was normal. Currently, all cases were alive and almost all eat regular food without requiring parenteral feeding., Conclusion: A-IH is rare, but distinct entity characterized by different clinical courses and pathological findings from those of C-IH. The outcome is considered to be favorable after a resection of affected intestine.

Purpose: The diagnosis and surgical treatments of Hirschsprung's disease (HD) have undergone various changes in the last few decades because of establishment of laparoscopic procedures. A retrospective nationwide survey for 4 decades was performed to study the changing profile of HD in Japan., Methods: The patient data were collected in 4 phases: Group 1, between 1978 and 1982; Group 2, between 1988 and 1992; Group 3, between 1998 and 2002; and Group 4, between 2008 and 2012., Results: The incidence and the male/female ratio remained almost the same over time (1/4, 895 in newborns and 2.9:1 in Group 4). The patients with a family history increased to 7.1% in Group 4, in comparison to 2.8-6.0% in other groups. Regarding the extent of aganglionosis, sigmoid colon increased to 63.1% in Group 4, compared to 51.9% in Group 3. Manometry was performed less frequently in Group 4 (45.8%) than in Group 3 (66.1%). Transanal endorectal pull-through (TAEPT) was the most popular operation in Group 4 (49.6%). In addition, laparoscopy-assisted operations increased to 46.9% in Group 4, in comparison to 29.7% in Group 3. The incidence of preoperative enterocolitis and the mortality rate in Group 4 were 17.2% and 2.4%, respectively, and were markedly decreased in comparison to Group 1 (29.2% and 6.5%, respectively). The mortality rate decreased over time to 2.4% in Group 4. Over the last decade, there has been remarkable improvement in the mortality rate associated with the small intestine (aganglionosis extending orally to more than 30 cm of the terminal ileum). The rates were 25.5% in Group 4, 53.6% in Group 1, 33.3% in Group 2, and 35.5% in Group 3. In addition, the mortality rates of the remaining aganglionosis subgroups also improved., Conclusion: Primary operations without laparotomy, including TAEPT and laparoscopy-assisted operations, have become the first choice for the definitive surgical treatment of HD in Japan. The mortality rate has decreased over time. However, the mortality rate of small intestinal aganglionosis is still relatively high. The development of new treatment strategy for small intestinal aganglionosis is called for.

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: We verified the appropriate port location for laparoscopic hepaticojejunostomy using a comprehensive laparoscopic training simulator., Methods: We developed a hepaticojejunostomy model, consist of common hepatic duct and intestine and participants required to place two sutures precisely using two different port locations (A: standard port location, B: modified port location). The order of tasks was randomly determined using the permuted block method (Group I: Task A → Task B, Group II: Task B → Task A). The time for task completion and total number of errors were recorded. In addition, we evaluated the spatial paths and velocity of both forceps. Statistical analyses were performed using a statistical software program., Results: The time for the task, the total error score, and the spatial paths and velocity of both forceps were not significantly different between groups I and II. Furthermore, the port location and order of tasks (group I or group II) did not significantly affect the results. In contrast, there were significant differences in the performance between experts and novices, who were classified as such based on the total number of experienced endoscopic surgeries., Conclusion: Preoperative port simulation in advanced surgery using our artificial simulator is feasible and may facilitate minimally invasive surgery for children.

Purpose: Pediatric surgeons require highly advanced skills when performing endoscopic surgery; however, their experience is often limited in comparison to general surgeons. The aim of this study was to evaluate the effectiveness of endoscopic surgery training for less-experienced pediatric surgeons and then compare their skills before and after training., Methods: Young pediatric surgeons (n = 7) who participated in this study underwent a 2-day endoscopic skill training program, consisting of lectures, box training and live tissue training. The trainees performed the Nissen construction tasks before and after training using our objective evaluation system. A statistical analysis was conducted using the two-tailed paired Student's t tests., Results: The time for task was 984 ± 220 s before training and 645 ± 92.8 s after training (p < 0.05). The total path length of both forceps was 37855 ± 10586 mm before training and 22582 ± 3045 mm after training (p < 0.05). The average velocity of both forceps was 26.1 ± 3.68 mm/s before training and 22.9 ± 2.47 mm/sec after training (p < 0.1). The right and left balance of suturing was improved after training (p < 0.05)., Conclusion: Pediatric surgery trainees improved their surgical skills after receiving short-term training. We demonstrated the effectiveness of our training program, which utilized a new laparoscopic fundoplication simulator.

Purpose: Endoscopic surgery is performed under a horizontal view in comparison to the vertical view that is associated with open surgery. We developed an endoscopic pseudo-viewpoint alternation system with out any scope action. We investigate the effect of this novel system on forceps manipulation among expert pediatric surgeons., Methods: Six expert pediatric surgeons performed a Nissen wrap in a fundoplication simulator either with or without this system. The constructed Nissen wrap was evaluated. The total path length and the average velocity of the forceps were also analyzed., Results: The times required either with or without this system were 587.5 ± 122.7 and 634.0 ± 212.4 s (p = 0.45), respectively. The total path lengths of right and left forceps either with or without this system were 12,309 ± 2495.5 and 15,726 ± 5649.6 mm (p = 0.07), 10,091 ± 2439.2 and 12,575 ± 5511.1 mm (p = 0.11), respectively. The average velocity of the right and left forceps with or without this system were 26.9 ± 5.29 and 31.6 ± 1.62 mm/s (p = 0.04), 21.6 ± 2.48 and 25.5 ± 6.48 mm/s (p = 0.15), respectively. There was no significance in the suture balance and suture interval., Conclusion: The endoscopic pseudo-viewpoint alternation system thus made it possible for expert pediatric surgeons to carry out slow and economical forceps manipulation. These effects make it possible for surgeons to perform safe and precise surgery, thus leading to a shortening of operation time.