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.

AFP-L3 as a Prognostic Predictor of Recurrence in Hepatoblastoma: A Pilot Study.

The α-fetoprotein (AFP) level is a sensitive biomarker of active hepatoblastoma (HB). This study aimed to clarify whether the Lens culinaris agglutinin A-reactive fraction of AFP (AFP-L3) after complete resection is a prognostic predictor of HB recurrence. Fourteen HB patients who underwent complete resection of HB were divided into the recurrence group (RG, n=4) and the non-recurrence group (NRG, n=10). The AFP level and AFP-L3 before and after radical surgery were compared between the 2 groups. There was no significant difference in AFP levels in the early postoperative period between the 2 groups (P=0.54), and AFP was not an early prognostic factor for HB recurrence. At 2 months after surgery, the AFP-L3 fell below the detection limit only in the NRG (7/10 cases) (NRG=70.0% vs. RG=0%, P=0.03). In addition, there were some cases of recurrence in those whose AFP level decreased to the normal range, but none in those whose AFP-L3 fell below the detection limit. In conclusion, the AFP-L3 decreased earlier than did the AFP level; thus, the AFP-L3 after complete resection may be a predictor for HB recurrence.

Correction to: Two infant cases of intraperitoneal arterial hemorrhage due to a duplication cyst: a case report.

An amendment to this paper has been published and can be accessed via the original article.

Two infant cases of intraperitoneal arterial hemorrhage due to a duplication cyst: a case report.

BACKGROUND: Intraperitoneal arterial hemorrhage without trauma is extremely rare. We report two infant cases of intraperitoneal arterial hemorrhage due to intestinal duplication. CASE PRESENTATION: In case 1, a 2-month-old girl experienced sudden intraperitoneal hemorrhage from the middle colic artery with no apparent trauma. Hemostasis was achieved with suturing of the hemorrhage point, but the cause of hemorrhage was still unknown. Computed tomography after the first operation revealed a duodenal duplication cyst and a pseudopancreatic cyst. Percutaneous drainage of the pseudopancreatic cyst was performed, and the contents had high pancreatic amylase. As the size of the duodenal duplication cyst also decreased with this drainage, we suspected that the duodenal duplication cyst was connected to the pseudopancreatic cyst and the arterial hemorrhage. We hypothesized that the pancreatic juice inside the duplication cyst leaked into the intraperitoneal cavity and caused rupture of the arterial wall. Therefore, marsupialization of the duodenal duplication was performed to evacuate the pancreatic juice contained in the cyst toward the native duodenum. The postoperative course was uneventful. In case 2, a 6-month-old boy experienced sudden intraperitoneal hemorrhage without trauma. The hemorrhage site was identified as the ileocecal artery, and hemostasis was achieved with sutures. Tissue near the hemorrhage point was biopsied, because the cause of arterial wall rupture was still unknown. The biopsied tissue was found to be intestinal mucosa. The patient had recurrent abdominal pain after the first operation, and computed tomography showed a duplication cyst located near the hemorrhage point. Therefore, we resected the intestinal duplication. Pathology results showed that the intestinal duplication contained intestinal mucosa, ectopic gastric mucosa, and pancreatic tissue. The postoperative course was uneventful. CONCLUSION: Intraperitoneal arterial hemorrhage without trauma is an extremely rare condition, and identifying its cause is difficult. To our knowledge, this is the first report of intraperitoneal arterial hemorrhage due to intestinal duplication. In cases of unexplained intraperitoneal arterial hemorrhage in infants, intestinal duplication near the hemorrhage point should be suspected.

Risk factors for the recurrence of perineal canal.

PURPOSE: The aim of this study was to investigate risk factors for recurrence in the perineal canal (PC). METHODS: Patients with PC who underwent operations were enrolled in this study and were divided into recurrence and non-recurrence groups. Preoperative infection, the age at the operation, the presence of colostomy and the treatment procedure for fistula were retrospectively investigated. Regarding the treatment procedure for fistula, either closure of the rectal wall with stitches or ligation of fistula in the rectum was performed. These factors were compared between the two groups. RESULTS: Six of 17 patients with PC who underwent surgical treatment had recurrence. There were no significant differences in the incidence of preoperative infection, age at operation or presence of colostomy (p = 0.60, 0.38, 1.00, respectively). In the recurrence group, all patients were treated by closure of the rectal wall. In the non-recurrence group, five were treated by the closure of the rectal wall with stitches and six by ligation of the fistula. There was a significant association between recurrence and the treatment procedure for fistula (p = 0.04). CONCLUSION: Closure of the rectal wall with stitches is a risk factor for the recurrence of PC.

Repair of type IV laryngotracheoesophageal cleft (LTEC) on ECMO.

PURPOSE: A type IV laryngotracheoesophageal cleft (LTEC) is a very rare congenital malformation. Type IV LTEC that extends to the carina have poor prognosis and are difficult to manage. We present our experience with surgical repair in such a case using extracorporeal membranous oxygenation (ECMO). METHODS: A male infant, who was diagnosed with Goldenhar syndrome, showed severe dyspnea and dysphagia. Laryngoscopy indicated the presence of LTEC. The patient was transferred to our institute for radical operation 26 days after birth. Prior to surgery, a balloon catheter was inserted in the cardiac region of stomach through the lower esophagus to block air leakage, to maintain positive pressure ventilation. We also performed observations with a rigid bronchoscope to assess extent of the cleft, and diagnosed the patient with type IV LTEC. After bronchoscopy, we could intubate the tracheal tube just above the carina. Under ECMO, repair of the cleft was performed by an anterior approach via median sternotomy. RESULTS: The patient was intubated via nasotracheal tube and paralysis was maintained for 2 weeks, using a muscle relaxant for the first 3 days. Two weeks after surgery, rigid bronchoscopy showed that the repair had been completed, and the tracheal tube was successfully extubated without tracheotomy. CONCLUSIONS: Although insertion of a balloon catheter is a very simple method, it can separate the respiratory and digestive tracts. This method allowed for positive pressure ventilation and prevented displacement of the endotracheal tube until ECMO was established. As a result, we safely performed the operation and the post-operative course was excellent.

Clinical features and risk factors of bile duct perforation associated with pediatric congenital biliary dilatation.

PURPOSE: This study aimed to investigate the clinical features and risk factors of bile duct perforation in pediatric congenital biliary dilatation (CBD) patients. METHODS: CBD patients, whose initial symptom was abdominal pain, were enrolled in this study and were divided into perforated and non-perforated groups. The clinical features of the perforated group were investigated. Moreover, the age at operation, sex, and morphologic features of the extrahepatic bile duct were compared between the groups. RESULTS: Fifteen cases of bile duct perforation (10.4%) were identified among the 144 CBD patients who had abdominal pain. Majority of bile duct perforation occurred in patients aged < 4 years. The median duration from onset of abdominal pain to bile duct perforation was 6 (4-14) days. Age at onset [< 4 years old; P = 0.02, OR 13.9, (1.663, 115.3)], shape of extrahepatic bile duct [non-cystic type; P = 0.009, OR 8.36, (1.683, 41.5)], and dilatation of the common channel [P = 0.02, OR 13.6, (1.651, 111.5)] were risk factors of bile duct perforation. CONCLUSIONS: Emergent bile duct drainage might be planned to prevent bile duct perforation if CBD patients have the abovementioned risk factors and experience persistent abdominal pain lasting for a few days from onset.

Secondary Aortoenteric Erosion Followed by Recurrent Lower Extremity Abscesses.

A secondary aortoenteric fistula (SAEF) is a relatively rare complication of aortoiliac reconstructive surgery, often involving relatively fixed duodenal third portion and a vascular anastomosis. We observed a 52-year-old man with a recurrent right lower leg abscess following the erosion between ipsilateral bifurcated prosthetic graft limb and nonfixed jejunum. In situ graft-sparing surgical treatment with aggressive debridement was successfully performed. A SAEF may occur even at a nonvascular anastomosis site, or in nonfixed small bowel, and may become a source of a septic embolus. A high index of suspicion for SAEF is required for early diagnosis and treatment of this life-threatening complication.