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.

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.

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.

Epithelial and ganglionic distribution at the distal rectal end in anorectal malformations: could it play a role in anastomotic adaptation?

PURPOSE: In anorectal malformations (ARMs), the epithelium of the distal rectal end is not well described. We histomorphologically evaluated epithelial and ganglionic distribution in the distal rectal end of ARMs resected during anorectoplasty to assess similarities and differences with normal anal canal structure. METHODS: In this single-center retrospective study, specimens from 60 ARM patients (27 males, 33 females) treated between 2008 and 2019 were evaluated. RESULTS: Epithelium type and alignment sequence as well as ganglionic distribution were similar in the distal rectal end and in a normal anal canal. Stratified columnar epithelium (anal transitional zone, ATZ) was seen in 49/60 (81.7%) cases and in all ARM types, including the no-fistula type. Anal crypts were identified in the stratified columnar epithelium (ATZ) of 46/49 (93.9%) patients. Regarding distal rectal end-resecting anorectoplasty, in 90% of patients, resection was performed distal to the Herrmann line. Ganglion cell distribution was exclusively proximal to the Herrmann line. CONCLUSION: Epithelial and ganglionic distribution was similar in the distal rectal end of ARMs and in a normal anal canal. The ATZ is the epithelial boundary between the rectum and skin in a normal anal canal. ATZ preservation could reproduce anal canal structure in ARM reconstruction.

Laparoscopic Ladd's procedure in neonates: A simple landmark detorsion technique.

BACKGROUND: The aim of this study was to assess the efficacy of our simple landmark technique for laparoscopic detorsion and the Ladd's procedure (lap-Ladd) for malrotation with midgut volvulus in neonates and to identify the risk factors for reoperation after the lap-Ladd. METHODS: We conducted a retrospective chart review of 42 patients after lap-Ladd for malrotation between April 2017 and June 2019. Information regarding patient status and intraoperative and postoperative data were analyzed. RESULTS: Thirty-one patients had volvulus (73.8 %), while 11 patients did not (26.2%). The median age and weight between the two groups at operation were 9 days (range, 3-28 days), 3.2 kg (range, 2-8 kg) and 6 days (range, 2-11), 2.9 kg (range, 2-3.8 kg), respectively. The operative time was significantly shorter in patients with volvulus compared to those without (60 vs 105 min, P = 0.002). Two cases were converted to open surgery because of ischemic changes of the total small intestine during surgery. Reoperation was required in two patients with volvulus (due to adhesive small bowel obstruction and recurrent volvulus). There was no significant predictive factor for reoperation after the lap-Ladd procedure. CONCLUSION: Our simple landmark lap-Ladd procedure demonstrated feasibility and good short-term outcomes in neonates with malrotation, regardless of the presence or absence of volvulus.

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.

Robotic-assisted surgery for choledochal cyst in children: early experience at Vietnam National Children's Hospital.

PURPOSE: We aimed to describe our robotic-assisted surgery (RAS) techniques and assess the early results of RAS for choledochal cysts in children. METHODS: We conducted a retrospective chart review of children who underwent RAS for a congenital choledochal cyst at our institution between February 2013 and August 2016. We analyzed patient characteristics, operative data, and postoperative outcomes. RESULTS: Thirty-nine patients underwent RAS for a choledochal cyst (female 30). The operation was performed with four robotic ports and one laparoscopic port for the assistant. The Roux loop was fashioned extracorporeally. Twenty patients (51.3%) had a Todani Type I cyst and the others had Type IV. The mean patient age and weight and choledochal cyst diameter at the time of the operation were 40.2 months (range 5-108 months), 13.4 kg (range 6.5-29 kg), and 27.2 mm (range 9-112 mm), respectively. The mean operating time was 192.7 min (range 150-330 min). There were no intraoperative complications; no conversions to laparoscopic or open surgery; and no postoperative complications, including cholangitis, cholelithiasis, or anastomotic stenosis. CONCLUSION: Pediatric RAS CC resection is safe and feasible. The robot-assisted technique overcame technical difficulties. However, in pediatric cases, a skilled robotic surgical team and procedural modifications are needed.

Fetal Primary Small Bowel Volvulus Associated with Acute Gastric Dilatation Detected by Ultrasonography.

Fetal intestinal volvulus is a rare condition, and fetal diagnosis of this disease is still challenging, especially in primary cases not accompanied by other comorbidities, such as intestinal malformations. Herein, we report a case of fetal primary small bowel volvulus associated with acute gastric dilatation detected by ultrasonography. We speculate that the mechanism of acute gastric dilatation in our case was peristatic malfunction of the whole intestine caused by a strangulated ileus resulting from fetal intestinal volvulus. In conclusion, acute gastric dilatation detected by fetal ultrasound can indicate the fetal intestinal volvulus.

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.