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.

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.

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.

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.

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.

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.

Treatment guidelines for persistent cloaca, cloacal exstrophy, and Mayer-Rokitansky-Küster-Häuser syndrome for the appropriate transitional care of patients.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH. We were able to publish statements on chronic renal dysfunction, hydrometrocolpos, and pregnancy, based on four CQs about PC, four about CE, and two about MRKH, respectively. However, due to the paucity of proper manuscripts, we were unable to make conclusions about the correct timing and method of vaginoplasty for patients with PC, CE, and MRKH or the usefulness of early bladder closure for patients with CE. These TGs may help clarify the current treatments for PC, CE, and MRKH in childhood, which have been carried out on an institutional basis. To improve clinical outcomes, systematic clinical trials revealing comprehensive clinical data of the urinary and reproductive systems, especially the length of the common channel in PC, are essential.

Experimental validation of laryngotracheal growth and recurrent laryngeal nerve preservation after partial cricotracheal resection in a growing rabbit model.

PURPOSE: The aim of this study was to confirm laryngotracheal growth and recurrent laryngeal nerve (RLN) preservation after partial cricotracheal resection (PCTR) in a growing rabbit model by performing the procedure in pediatric animals. METHODS: Six female Japanese white rabbits, 12 weeks of age, underwent PCTR. The course of the RLN was evaluated during surgery (n = 3). Endoscopic and histologic examinations were performed at 22 weeks of age (n = 6). Four non-operated rabbits, 22 weeks of age, underwent endoscopic and histologic examinations as controls. RESULTS: The RLN was preserved at the esophageal side and entered the larynx behind the cricothyroid joint after PCTR. Endoscopic examination showed normal vocal cord movements and the large reconstructed subglottis. Histologically, sufficient submucosal vessels and cartilage growth were identified at the reconstructed larynx. The median inside luminal area at the anastomotic site in the PCTR group was 24.8 mm2 (range 21.8-29.0 mm2), and that at the cricoid cartilage and trachea in the control group was 23.4 mm2 (range 20.0-26.6 mm2) and 25.6 mm2 (range 22.9-28.8 mm2), respectively. CONCLUSION: No interference with laryngotracheal growth was seen, and RLN preservation was confirmed after PCTR. Use of PCTR in the pediatric age group seems appropriate.

Bronchoscopic assessments and clinical outcomes in pediatric patients with tracheomalacia and bronchomalacia.

BACKGROUND: Tracheomalacia and bronchomalacia (TM/BM) are one of the serious causes of airway obstruction in infants and children. This study reviewed our bronchoscopic assessments and clinical outcomes in pediatric patients with TM/BM, and investigated risk factors of surgical intervention for TM/BM. METHODS: Fifty-seven consecutive patients who were diagnosed as TM/BM by bronchoscopy between 2009 and 2013 were reviewed retrospectively. They were divided into two groups according to the presence (group E, n = 26) or absence (group N, n = 31) of acute life-threatening events and extubation failure (ALTE/EF). The severity of TM/BM was evaluated by Oblateness Index which was obtained from bronchoscopic images. RESULTS: Oblateness Index was significantly higher in Group E than in Group N. Patients in Group E underwent surgical intervention for TM/BM more frequently, and had significantly longer intubation period and hospital stay. Clinical symptoms of ALTE/EF, Oblateness Index ≥ 0.70, and multiple malacic lesions were significant risk factors indicating surgical events in patients with TM/BM. CONCLUSIONS: Patients with TM/BM who had ALTE/EF had more severe malacic lesions indicating surgical intervention, and worse clinical outcomes. Oblateness Index is a simple and semi-quantitative index for bronchoscopic assessment of TM/BM, and can be one of the prognostic tools to predict clinical severity of pediatric TM/BM.

Anterior-posterior cricoid split combined with silastic T-tube stenting for subglottic stenosis in children: a single surgeon's experience.

PURPOSE: Treatment strategies and clinical outcomes of subglottic stenosis (SGS) in children are varied due to the degree and range of stenotic lesions. The optimal surgical procedure for SGS in children is still under debate. The aim of this study was to evaluate the clinical outcomes of our anterior-posterior cricoid (APC) split technique combined with long-term T-tube stenting for grade II or III SGS in children. METHODS: A retrospective chart review of children with SGS between January 2011 and December 2016 was conducted. APC split was performed via open procedure under rigid bronchoscopy. After splitting, a silastic T-tube was inserted as a stent and removed 6 months postoperatively. RESULTS: Seven children underwent APC split during the period. All children had undergone previous tracheostomy, and APC split was performed when the children were 3-9 years old without any intraoperative complications. Median duration of T-tube stenting was 11 months, and all children were decannulated successfully. There were T-tube-related complications, including two tube-tip granulation that required intervention and one accidental T-tube removal. CONCLUSION: APC split is a technically simple and reproducible procedure, and it could be employed as an optimal procedure for SGS in children.

Evaluation of the intraoperative risk factors for esophageal anastomotic complications after primary repair of esophageal atresia with tracheoesophageal fistula.

PURPOSE: The aim of this study is to identify the risk factors for esophageal anastomotic stricture (EAS) and/or anastomotic leakage (EAL) after primary repair of esophageal atresia with tracheoesophageal fistula (EA/TEF) in infants. METHODS: A retrospective chart review of 52 patients with congenital EA/TEF between January 2000 and December 2015 was conducted. Univariate and multivariate analyses were performed to identify the risk factors for anastomotic complications. RESULTS: Twenty-four patients were excluded from the analysis because they had insufficient data, trisomy 18 syndrome, delayed anastomosis, or multi-staged operations; the remaining 28 were included. Twelve patients (42.9 %) had anastomotic complications. EAS occurred in 12 patients (42.9 %), and one of them had EAL (3.57 %). There was no correlation between anastomotic complications and birth weight, gestational weeks, sex, the presence of an associated anomaly, age at the time of repair, gap between the upper pouch and lower pouch of the esophagus, number of sutures, blood loss, and gastroesophageal reflux. Anastomosis under tension and tracheomalacia were identified as risk factors for anastomotic complications (odds ratio 15, 95 % confidence interval (CI) 1.53-390.0 and odds ratio 8, 95 % CI 1.33-71.2, respectively). CONCLUSION: Surgeons should carefully perform anastomosis under less tension to prevent anastomotic complications in the primary repair of EA/TEF.

Severe acquired subglottic stenosis in children: analysis of clinical features and surgical outcomes based on the range of stenosis.

PURPOSE: The present study analyzed the clinical features and surgical outcomes of laryngotracheal reconstruction (LTR) in pediatric patients with severe acquired subglottic stenosis (SGS) based on the range of stenosis. The aim was to clarify the indications for LTR in severe acquired SGS. METHODS: The medical records of 33 pediatric patients with severe acquired SGS (Myer-Cotton grade III or IV) at our institution between January 1994 and December 2013 were retrospectively reviewed. RESULTS: Nine patients had stenosis localized at the subglottis (localized SGS), and twenty-four patients had stenosis extending to the glottis or supraglottis from the subglottis (extended SGS). 66.7 % (6/9) of localized SGS patients were intubated after infancy, and 95.8 % (22/23) of extended SGS patients were intubated in the neonatal period. The duration of intubation was significantly shorter with localized than with extended SGS. Sixteen patients underwent LTR. The operation-specific decannulation rate was 80.0 % (4/5) in the localized SGS group and 14.3 % (1/7) in the extended SGS group. CONCLUSION: The range of stenosis was affected by the period and duration of endotracheal intubation. Surgical outcomes of LTR tended to differ between localized SGS and extended SGS. LTR can be effective for localized SGS.

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.

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.