Long-term course of gastrostomy nutritional management in patients with Duchenne muscular dystrophy: A retrospective cohort study.

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease that commonly requires gastrostomy due to dysphagia. This study aimed to investigate the proportion of patients with DMD requiring gastrostomy and to assess the timing and outcomes of gastrostomy in patients with DMD to optimize perioperative care and improve long-term management. We conducted a retrospective cohort study by reviewing the medical records of patients with DMD treated between March 1991 and November 2023 at Kobe University Hospital. To identify risk factors for gastrostomy, Fisher's two-tailed test and logistic analysis were used to compare the gastrostomy (group G) and comparison group without gastrostomy (group C) groups. We identified patients >18 years with DMD. We excluded those without medical records from the last decade and those on nasogastric tube feeding. Among the 135 eligible patients, five (median age, 23 years) underwent percutaneous endoscopic gastrostomy (PEG; uptake rate, 3.7 %), and their data were analyzed for perioperative outcomes. Complications included ventilatory disturbances, aspiration pneumonia, and hemorrhagic shock. Two patients experienced significant postoperative complications, underscoring the high-risk nature of PEG in this population. Participants' postoperative weight changes varied significantly. The presence or absence of ventilatory management, cardiomyopathy treatment, scoliosis, and steroid treatment were examined as risk factors between groups G and C, but no significant differences were observed. This study highlights low gastrostomy usage among patients with DMD, with varying outcomes. Meticulous planning and early consideration of gastrostomy are crucial to enhance the quality of life and nutritional outcomes in these patients.

Enhanced lymphangiogenesis in the left lateral segment of a biopsied liver during portoenterostomy for biliary atresia.

PURPOSE: We investigate the histopathology of the portal vein branches and lymphatic vessels to elucidate the mechanism of atrophy of the left lateral segment (LLS) of the liver in biliary atresia (BA). METHODS: LLS and right anterior segment (RAS) liver biopsy samples obtained during Kasai portoenterostomy (KPE) from ten consecutive patients with BA underwent histopathological investigation of the portal vein and lymphatic vessels using double chromogenic immunostaining for CD31/D2-40 and the hepatitis-like findings (HLF) score. Each parameter and clinical data were compared between prognostic groups. RESULTS: HLF scores in the LLS were always higher than those in the RAS. There was no difference in portal vein and lymphatic vascular morphology, whereas the number of lymphatic vessels was correlated with the fibrotic area of all specimen areas. Left-to-right ratio of the number of lymphatic vessels was correlated with the age at KPE (r = 0.784, p = 0.007) and the pre-KPE CRP value (r = 0.723, p = 0.018). CONCLUSIONS: Lymphangiogenesis on the LLS compared to the RAS was significantly correlated with the degree of fibrosis and the age at KPE. Further investigation is warranted to clarify the causes of LLS atrophy and lymphangiogenesis relevant to immune dysregulation.

Evaluation of the hinotori™ Surgical Robot System for accurate suturing in small cavities.

The hinotori™ Surgical Robot System (hinotori™, Medicaroid, Kobe, Japan) is increasingly being utilized primarily in urology and adult surgery; however, data on its application in pediatric surgery are lacking. This preclinical study aimed to evaluate the limitations of this system for accurate suturing in small cavities designed for pediatric and neonatal applications. Two trained operators performed simple ligature sutures (easy task [ET]) and hepaticojejunostomy sutures (difficult task [DT]) within five differently sized boxes, ranging from 5123 to 125 mL. The suture time, number of internal and external instrument/instrument collisions, instrument/box collisions, and suture accuracy were evaluated. The suture accuracy was assessed using the A-Lap Mini endoscopic surgery skill assessment system. As a result, an increase in the number of collisions and extended suturing times were observed in boxes with volumes smaller than 215 mL. Despite these variations, there were no significant differences between the boxes, and all tasks were precisely performed in all boxes (p = 0.10 for the ET and p = 1.00 for the DT). These findings demonstrate the capability of the hinotori™ system to perform precise suturing techniques within tightly confined simulated neonatal cavities as small as 125 mL. To advance the integration of pediatric robotic surgery utilizing the hinotori™ system, additional trials comparing it with conventional laparoscopic and thoracoscopic techniques using pediatric and animal models are necessary to assess its clinical safety and applicability.

Significance of Serum Leucine-rich Alpha-2 Glycoprotein as a Diagnostic Marker in Pediatric Inflammatory Bowel Disease.

Serum leucine-rich alpha-2 glycoprotein (LRG) has been utilized for adult inflammatory bowel disease (IBD); however, its efficacy in pediatric IBD remains unknown. The aim of this study was to compare the diagnostic accuracy of serum LRG for pediatric IBD with that of current inflammatory markers, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). This retrospective case-control study included pediatric patients, aged <16 years, who underwent colonoscopy and/or esophagogastroduodenoscopy between April 2017 and March 2022. All eligible patients were divided into two groups: patients with IBD, diagnosed with ulcerative colitis and Crohn's disease, and non-IBD controls. The optimal cut-off value of serum LRG for IBD diagnosis was determined from receiver operating characteristic analysis, and diagnostic accuracy of serum LRG was compared to serum ESR and CRP. A total of 53 patients (24 with IBD and 29 non-IBD controls) met the inclusion criteria. The cut-off value of serum LRG for IBD diagnosis was determined to be 19.5 µg/ml. At this cut-off value, serum LRG had a positive predictive value (PPV) of 0.80 and negative predictive value (NPV) of 0.88. In contrast, PPV and NPV were 0.78 and 0.70 for serum ESR and 0.82 and 0.72 for serum CRP, respectively. Serum LRG can be a potential diagnostic marker for pediatric IBD, with higher diagnostic accuracy than that of the conventional serum markers ESR and CRP.

Endosurgery workshop to improve the confidence of novice pediatric surgeons in performing laparoscopic hepaticojejunostomy.

PURPOSE: This study assessed the efficacy of a high-impact, short-term workshop in honing the laparoscopic hepaticojejunostomy technical skills and self-confidence of novice pediatric surgeons, focusing on vertical needle driving and knot tying. METHODS: Lectures, hands-on sessions, pre- and post-workshop evaluations, and training using porcine models were conducted to refine basic and advanced skills. The "hepaticojejunostomy simulator" was used for comparative analysis of precision in pre- and post-workshop vertical needle driving and knot tying. Participants self-evaluated their skills and confidence on a 5-point scale. RESULTS: After the workshop, eight inexperienced pediatric surgeons demonstrated a significant improvement in hepaticojejunostomy suturing task completion rates and needle-driving precision at the jejunum and hepatic duct. However, the A-Lap Mini Endoscopic Surgery Skill Assessment System indicated no significant improvements in most assessed parameters, except for the full-layer closure score (p = 0.03). However, a significant increase in participants' confidence levels in performing laparoscopic hepaticojejunostomy was observed. CONCLUSION: The workshop augmented technical proficiency and confidence in young pediatric surgeons. The combination of lectures, practical exposure, and model training is an effective educational strategy in pediatric surgical instruction.

Peroral Endoscopic Myotomy in Pediatric Patients with Achalasia up to 12 Years of Age: A Pilot Study in a Single-Center Experience in Japan.

INTRODUCTION: Peroral endoscopic myotomy (POEM) is a minimally invasive endoscopic procedure for achalasia; its indication has expanded from adults to children. We aimed to evaluate the postoperative efficacy and antireflex status of POEM in young children with achalasia aged 12 years or younger. PATIENTS: AND METHODS: Pediatric patients with achalasia aged 18 years or younger who underwent POEM in our hospital between 2016 and 2021 were included and divided into two age groups: group A (≤ 12 years) and group B (13-18 years). The success rate (Eckardt score ≤ 3), endoscopic reflux findings, and antiacid use at 1 year postoperatively were compared between the groups. RESULTS: Ten patients (four boys and six girls; Chicago classification type I: five, type II: four, and unclassified: one) were included. Mean age and preoperative Eckardt scores in groups A (n = 4) and B (n = 6) were 9.2 ± 3.0 versus 15.6 ± 0.6 years (p = 0.001) and 5.5 ± 3.9 versus 7.2 ± 3.7 (p = 0.509), respectively, and mean operative time and myotomy length were 51.3 ± 16.6 versus 52.5 ± 13.2 minutes (p = 0.898) and 10.8 ± 4.6 versus 9.8 ± 3.2 cm (p = 0.720), respectively. The 1-year success rate was 100% in both groups. Mild esophagitis (Los Angeles classification B) was endoscopically found in one patient in each group (16.7 vs. 25.0%, p = 0.714), and antiacid use was required in three patients (group A, two; group B, one; 50.0 vs. 16.7%, p = 0.500). CONCLUSION: The success rate of POEM within 1 year in young children with achalasia aged 12 years or younger was equal to that in adolescent patients. However, young children tended to require antiacids 1 year postoperatively; therefore, long-term follow-up is necessary.

Incidence of congenital tracheal stenosis in left pulmonary artery sling diagnosed by bronchoscopy.

PURPOSE: Congenital tracheal stenosis (CTS) has been reported to occur in 50-65% of cases of left pulmonary artery sling (LPAS), but the exact incidence rate is unknown. This study aimed to determine the actual rate using bronchoscopy and to elucidate morphological features in computed tomography (CT) diagnosis. METHODS: We performed a single institutional retrospective review of all patients with LPAS between January 2010 and March 2022. The percentage of complete tracheal rings in patients with LPAS was evaluated using bronchoscopy. The anteroposterior/lateral diameter ratios at the smallest and largest diameters of each CTS patient's trachea were measured on CT. The Wilcoxon signed-rank test was used to analyze the differences between the two parts. RESULTS: Fifty-two patients with LPAS were enrolled. All patients had complete tracheal rings on bronchoscopy. CT analysis of 32 patients with CTS was performed. The median anteroposterior/lateral diameter ratio at the smallest diameter was 1.05 (interquartile range [IQR] 0.95-1.15); the median ratio at the largest diameter was 0.94 (IQR 0.89-0.99). There was a significant difference between the two parts (p = 0.013). CONCLUSION: CTS might be universally associated with LPAS. The circular tracheal cross-section on CT might imply the existence of a complete tracheal ring.

CYP1A1 Is a Useful Diagnostic Marker for Angiofibroma of Soft Tissue.

Angiofibroma of soft tissue (AFST) is a recently described benign fibroblastic neoplasm composed of uniform bland spindle cell proliferation in fibrous and fibromyxoid stroma with prominent thin-walled small branching vessels. A major recurrent genetic abnormality in AFST is t(5;8)(p15;q13), which results in the rearrangement of AHRR and NCOA2 . Owing to a lack of discriminatory IHC markers and potential overlap with other mesenchymal neoplasms, it may be difficult to confirm the diagnosis of AFST in some cases. Triggered by a recent gene expression profile study of AFST, which showed the significant upregulation of AhR/AHRR/ARNT downstream genes (including CYP1A1 ), we used a mouse monoclonal antibody to explore the diagnostic significance of CYP1A1 expression in histologically confirmed AFST cases along with 224 control cases, consisting of 221 neoplastic mimickers and 3 non-neoplastic lesions. We found moderate to strong cytoplasmic expression of CYP1A1 in 13 of 16 AFST cases (sensitivity, 81.3%). In contrast, the vast majority of other examined histologic mimickers exhibited no expression of CYP1A1 (specificity, 97.3%), except for 3 myxofibrosarcomas (3/31), 2 solitary fibrous tumors (2/22), and 2 neurofibroma (1/27). Our results indicate that CYP1A1 immunohistochemistry may aid in the diagnosis of AFST by distinguishing among various kinds of tumors, particularly those harboring prominent vasculature.

Dienogest Use for Recurrent Pyosalpinx as a Long-Term Complication of Cloacal Exstrophy: A Case Report.

BACKGROUND: Cloacal exstrophy (CE) is a rare congenital disease that requires multiple surgeries for complex gastrointestinal and genitourinary anomalies. Long-term complications are not uncommon; however, they are poorly reported. Pyosalpinx is sometimes encountered during CE management in adolescents and young adults. CASE: A 28-year-old woman with a history of CE presented with fever, lower abdominal pain, and vomiting and was diagnosed with left pyosalpinx. Computed tomography-guided drainage and intravenous antibiotic administration were successful; however, she had 2 readmissions for recurrent pyosalpinx 1 week after discharge and again 4 months later. She was administered Dienogest, a synthetic progestin, to prevent recurrent pyosalpinx and had no recurrence for 8 months. SUMMARY AND CONCLUSION: Dienogest is a conservative treatment choice for preventing the recurrence of pyosalpinx for patients with CE.

Encouraging Young Pediatric Surgeons and Evaluation of the Effectiveness of a Pediatric Endosurgery Workshop by Self-Assessment and an Objective Skill Validation System.

Background: Japanese pediatric endosurgery experts conducted a workshop for young pediatric surgeons in Russia in collaboration with Russian expert pediatric surgeons. This study was aimed to develop a contributive workshop program and evaluate its impact on young pediatric surgeons. Methods: A 2-day pediatric endosurgery workshop was held in Moscow in February 2020. After conducting a needs assessment survey, Japanese and Russian faculties developed the workshop contents, including pre- and postworkshop skills assessments, lectures, and hands-on training. Skills assessments were performed using the objective skill validation system, the "A-Lap Mini," mimicking intestinal anastomosis. The trainees self-evaluated their knowledge and skills using a five-point scale. Results: Fifteen novice trainee participated and 14 (93.3%) completed the workshop program. The completion rate for the suturing task before and after the workshop was 40.0% (6/15) and 85.7% (12/14), respectively. The following five skill evaluation criteria, which were objectively evaluated: performance time changed from 751.6 ± 247.1 seconds to 780.0 ± 313.3 seconds (P > .05), number of full-thickness sutures improved from 1.0 ± 1.41 to 2.64 ± 0.84 (P = .003), area of wound-opening changed from 0.42 ± 0.83 mm2 to 0.53 ± 1.13 mm2 (P > .05), suture tension improved from 55.48% ± 19.51% to 61.95% ± 23.91% (P > .05), and maximum air leakage pressure improved from 3.76 ± 2.11 kPa to 8.42 ± 7.68 kPa (P > .05). Regarding the self-assessed questionnaire administered before and after the workshop, the confidence in endosurgery skills significantly improved as follows: forceps manipulation ability improved from 2.7 to 3.7 (P < .05), and suturing performance improved from 2.5 to 3.6 (P < .05). The usefulness of the workshop for clinical surgery was scored at 4.3. Conclusions: Quantitative skill evaluation with an automatic feedback function was useful for endosurgery training. Delivering feedback concerning the assessment results to the trainee helps them to determine the specific training requirements needed for clinical endosurgery.

The perioperative transition of serum biomarkers of a 1.5-year-old boy with very-long-chain acyl-CoA dehydrogenase deficiency.

Very long-chain acyl-coenzyme A dehydrogenase deficiency (VLCADD, OMIM 201475) is a congenital fatty acid oxidation disorder. Individuals with VLCADD should avoid catabolic states, including strenuous exercise and long-term fasting; however, such conditions are required when undergoing surgery. The perioperative management of VLCADD in infants has rarely been reported and details regarding the transition of serum biomarkers reflecting catabolic status have not been disclosed. Herein, we present the perioperative clinical and biological data of cryptorchidism in a 1.5-year-old boy with VLCADD. The patient was diagnosed through newborn screening and his clinical course was very stable. Genetic testing of ACADVL revealed compound heterozygous variants c.506 T > C (p.Met169Thr) and c.606-609delC (p.L216*). The enzyme activity of the patient with VLCAD was only 20% compared to that of healthy control. Left orchiopexy for the pediatric cryptorchidism was planned and performed at 1 and a half year of age. Induction anesthesia involved thiopental, fentanyl and rocuronium. The glucose infusion rate was maintained above 6.6 mg/kg/min starting the day before surgery until the operation was completed. Anesthesia was maintained with sevoflurane at approximately 2%. The serum concentration of tetradecenoylcarnitine were stable during the operation, ranging between 0.08 and 0.19 µM (cutoff <0.2 µM), and never deviated from the reference range. Concentration of other serum biomarkers including free fatty acid, 3-OH-butyrate, and creatine kinase, remained similarly unchanged. In this report, we describe the uneventful perioperative management of unilateral orchiopexy for left cryptorchidism in a 1.5-year-old boy with VLCADD using sufficient glucose infusion and volatile anesthesia.

Colonic diverticulitis in children: A retrospective study of 16 patients.

BACKGROUND: Pediatric colonic diverticulitis (CD) is a rare entity. This study aimed to investigate the clinical features of CD in children. METHODS: We performed a retrospective chart review of children aged ≤15 years who were diagnosed with CD in our institution from May 2006 to November 2016. RESULTS: Sixteen patients were diagnosed with CD. All CD cases were observed to be solitary cecal diverticulitis; 14 cases were detected using ultrasound and the other two cases were diagnosed by computed tomography. Five patients were male (31.3%), and the median age was 12 years (range, 8-15 years). Initial symptoms were fever (temperature >38°C) in six (37.5%) patients, right lower quadrant abdominal pain in 16 (100%), anorexia in eight (50%), and nausea / vomiting in five (31.3%). A patient experienced persistent constipation; however, diarrhea was not observed as a clinical symptom in any patient. The median duration from symptom onset to admission was 1 day (range, 0-4 days), and the median length of hospital stay was 6 days (range, 4-10 days). All CD cases were treated with intravenous antibiotics. The median follow-up period was 90 months (range, 37-163 months), and during this period, recurrence of CD was observed in three (18.8%) patients. At recurrence, antibiotics were administered in all cases. CONCLUSIONS: In this study, all cases of CD were solitary cecal diverticulitis, and ultrasound was useful for the diagnosis of cecal diverticulitis in children. Non-operative treatment should be recommended as an initial treatment for CD in children.

An anterior cricoid split and long-term silastic T-tube stenting for children with severe subglottic stenosis.

PURPOSE: The management of subglottic stenosis (SGS) remains challenging. Although laryngotracheal reconstruction with a costal cartilage graft (LTR) has been widely performed, restenosis with cicatricial tissue may require long-term stenting, especially in patients with severe SGS. An anterior cricoid split (ACS) with long-term stenting has been shown to be useful for patients with mild SGS. Thus, we evaluated the clinical outcomes of patients, including severe SGS, who underwent ACS compared to those with LTR. METHODS: A retrospective chart review was conducted in 25 patients with severe SGS (Grades III and IV) who underwent initial laryngoplasty (ACS or LTR) in our hospital from January 2009 to April 2018. RESULTS: 17 patients (8 with Grade III and 9 with Grade IV) underwent ACS, and 8 (6 with Grade III and 2 with Grade IV) underwent LTR. The median duration of stenting was 11 months (range: 0.8-50) in the ACS group and 12 months (range: 0.4-29) in the LTR group. Thirteen of 17 patients (76.5%) in the ACS group were decannulated, whereas 4 of 8 patients (50%) in the LTR group were decannulated (p = 0.2). CONCLUSION: ACS might be useful even for children with severe SGS. The optimal duration of stenting should be investigated further.

Point mutagenesis in mouse reveals contrasting pathogenetic effects between MEN2B- and Hirschsprung disease-associated missense mutations of the RET gene.

Missense mutations of the RET gene have been identified in both multiple endocrine neoplasia (MEN) type 2A/B and Hirschsprung disease (HSCR: congenital absence of the enteric nervous system, ENS). Current consensus holds that MEN2A/B and HSCR are caused by activating and inactivating RET mutations, respectively. However, the biological significance of RET missense mutations in vivo has not been fully elucidated. In the present study, we introduced one MEN2B-associated (M918T) and two HSCR-associated (N394K and Y791F) RET missense mutations into the corresponding regions of the mouse Ret gene by genome editing (RetM919T , RetN396K and RetY792F ) and performed histological examinations of Ret-expressing tissues to understand the pathogenetic impact of each mutant in vivo. RetM919T/+ mice displayed MEN2B-related phenotypes, including C-cell hyperplasia and abnormal enlargement of the primary sympathetic ganglia. Similar sympathetic phenotype was observed in RetM919T/- mice, demonstrating a strong pathogenetic effect of the Ret M918T by a single-allele expression. In contrast, no abnormality was found in the ENS of mice harboring the Ret N394K or Y791F mutation. Most surprisingly, single-allele expression of RET N394K or Y791F was sufficient for normal ENS development, indicating that these RET mutants exert largely physiological function in vivo. This study reveals contrasting pathogenetic effects between MEN2B- and HSCR-associated RET missense mutations, and suggests that some of HSCR-associated RET missense mutations are by themselves neither inactivating nor pathogenetic and require involvement of other gene mutations for disease expressivity.

Analysis of predictive factors for perforated appendicitis in children.

BACKGROUND: The aim of this research was to investigate the diagnostic value of objective factors present at admission for identifying predictive markers of perforated appendicitis in children. METHODS: We performed a retrospective case review of 319 children aged ≤15 years who underwent treatment for acute appendicitis at our institution over a 6-year period from January 2011 to December 2016. Univariate and multivariate analyses were performed to identify risk factors for perforation of acute appendicitis in children. RESULTS: In the 6-year period, 319 patients underwent treatment for acute appendicitis, of whom 72 (22.6%) had perforated appendicitis. Multivariate analysis revealed five independent factors predicting perforated appendicitis at admission: longer symptom duration (≥2 days), fever (axillary temperature ≥38.0 °C), elevated C-reactive protein level (≥3.46 mg/dL), appendiceal fecalith on imaging, and ascites on imaging. Among patients with all five risk factors, 93.3% had perforated appendicitis. None of the patients without any of these factors had a perforated appendicitis. CONCLUSIONS: Longer symptom duration (≥2 days), fever (axillary temperature ≥38.0 °C), elevated C-reactive protein level, and the presence of appendiceal fecalith and ascites on imaging are independent and objective factors predicting perforated appendicitis at admission. These risk factors have the potential to be helpful as an ancillary index for physicians determining the severity of appendicitis.

Laparoscopic fundoplication for a child with abdominal intrathecal Baclofen pump.

This is the first case report describing a laparoscopic fundoplication in a child with an intrathecal Baclofen pump which was inserted because of severe spasticity secondary to cerebral palsy. The child had symptoms of gastroesophageal reflux with recurrent episodes of aspiration pneumonia. These were managed with a gastrostomy and conservative therapy with no success. The presence of an intrathecal Baclofen pump makes abdominal surgery challenging and carries the risk of pump infection with its associated sequelae. However, we performed a successful laparoscopic fundoplication with no intraoperative complications and the child was asymptomatic at 18 months follow-up.

Insulin therapy for hyperglycemia in neonatal sepsis using a preterm mouse model.

BACKGROUND: Stress-induced hyperglycemia is a frequent complication of neonatal sepsis. Hyperglycemia induces oxidative stress and immunosuppression. We investigated the glucose kinetics and effect of insulin administration during stress-induced hyperglycemia in a neonatal sepsis mouse model. METHODS: A stock cecal slurry (CS) solution was prepared from adult cecums and 3.0 mg of CS/g (LD40 ) was administered intraperitoneally to 4-day-old FVB mouse pups. Blood glucose levels were measured at 1.5, 3, 6, and 9 h post-sepsis induction and compared with basal levels. Two different doses of ultrafast-acting insulin were administered subcutaneously, and blood glucose levels and survival rates were monitored. RESULTS: Blood glucose levels were significantly higher than those of baseline levels with a peak at 3 h, which progressively decreased from 6 to 9 h post-sepsis induction. Insulin treatment reduced post-sepsis-induced hyperglycemia at 1.5 and 3 h. The mortality rate of CS-only pups (39%) was similar to that of CS + 1 U/kg insulin pups (60%). However, the mortality rate of CS + 5 U/kg insulin pups (82%) was significantly higher than that of CS-only pups. CONCLUSIONS: Marked hyperglycemia was induced immediately after post-sepsis induction, and the high-dose insulin treatment increased mortality post-induction. Stress-induced hyperglycemia could therefore be a physiological and protective response for preterm sepsis, and aggressive treatment of this hyperglycemia might be contraindicated.