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To identify prognostic factors of postoperative Hirschsprung-associated enterocolitis (HAEC).A retrospective cohort study of Hirschsprung patients between 2006 and 2021 was conducted. Patients with anorectal malformation and non-definitive surgery were excluded. Associated factors for postoperative HAEC were reported with hazard ratio (HR) and 95% confidence interval (CI).Forty-nine patients were excluded due to concurrent anorectal malformation and incomplete data. Of 274 patients, 75 patients (27.4%) had at least one episode of postoperative HAEC. There were 28 patients (37.3%) who had multi-episodes of HAEC. The total episodes of post-operative HAEC in this study were 121 episodes (36.8%). In multivariable survival analysis, significant factors associated with postoperative HAEC were the aganglionic level above sigmoid colon (HR = 3.47, p = 0.023, 95% CI 1.19-10.09), and total colonic aganglionosis (HR = 14.83, p = 0.004, 95% CI 2.33-94.40). The patients who experienced clinical enterocolitis before 2 weeks after surgery significantly developed more postoperative HAEC (HR = 5.32, p = 0.038, % CI 1.09-25.92). The incidence of postoperative HAEC was increase in patients with postoperative obstructive symptoms (48.0%). One patient died due to severe sepsis from postoperative HAEC, while three others required intensive care.The long involvement of aganglionic segment and early postoperative HAEC was significantly associated with postoperative HAEC. Frequent follow-up, parental education, and early treatment are recommended in these individuals, particularly in the first year after surgery.

This study aimed to validate the “Chiang Mai University Intussusception Failed Score (CMUI)” for intussusception non-operative reduction. Both a 2-year retrospective and a 5-year prospective consecutive review of patients with intussusception were conducted. Data were collected from January 2013 to December 2020. Related retrospective data of a developmental set from two centers from January 2006 to December 2012 were used. Ten prespecified prognostic factors for failed reduction were collected and from these a predictive score was calculated. The actual results of non-operative reduction were collected and set as a reference standard. Altogether, 195 episodes of intussusception were found. Twenty-two patients were excluded due to contraindications; therefore, a total of 173 episodes were included in the validation dataset. The development data set comprised 170 episodes. We found that no statistical significance was found from comparing the areas under the ROC of two datasets (p-value = 0.31), while specificity of the validation set was 93.8% (88.1–97.3). This temporal validation showed a high specificity and a high affinity for prediction of failed reduction as the development dataset despite being in an era of a higher successful reduction rate. The intensive reduction protocols might be introduced among patients with high-risk scores.

The diagnosis of Hirschsprung’s disease (HSCR) relies on history, physical examination, and investigations. Some of investigation modalities could not be done in primary hospital. This study was aimed to develop the clinical score model for diagnosing and early referrals of HSCR, especially in areas where investigations were not available. Overall 483 consecutive suspected HSCR patients who were under 15 years old from January 2006 to December 2020 were included in this study, with 207 (42.86%) patients diagnosed with HSCR and 276 (51.14%) patients in the non-HSCR group. Five clinical parameters were included in the prediction model. The AuROC of clinical parameters, which included having an age younger than one month, male gender, the term infant, history of delayed meconium passage, and history of enterocolitis, was 72%. The prediction score ranged from 0–7, with a score 0–3 meaning a low risk to be HSCR (LHR+ = 0.37). We concluded that patients with suspected HSCR who had clinical score 4–7 had a high probability to be HSCR and, thus, it was suggested that these patients have an early referral for further investigations, which were contrast enema and rectal suction biopsy. In the case of a low probability of HSCR, clinical observation is still warranted. This clinical scoring system can be used as a screening tool to prevent delay diagnosis and complications.

Background Wilms tumor is the most common renal malignancy in childhood. It occurs primarily between the ages of 2 and 5 years. The usual manifestations are abdominal mass, hypertension, and hematuria. The case presented here had an unusual presentation, with dilated cardiomyopathy and hypertension secondary to the Wilms tumor. Case summary A 3-year-old boy presented with a 5-d history of irritability, poor appetite, and respiratory distress. His presenting clinical symptoms were dyspnea, tachycardia, hypertension, and a palpable abdominal mass at the left upper quadrant. His troponin T and pro-B-type natriuretic peptide levels were elevated. Echocardiography demonstrated a dilated hypokinetic left ventricle with an ejection fraction of 29%, and a suspected left renal mass. Computed tomography scan revealed a left renal mass and multiple lung nodules. The definitive diagnosis of Wilms tumor was confirmed histologically. The patient was administered neoadjuvant chemotherapy and underwent radical nephrectomy. After surgery, radiotherapy was administered, and the adjuvant chemotherapy was continued. The blood pressure and left ventricular function normalized after the treatments. Conclusion Abdominal mass, dilated cardiomyopathy and hypertension can indicate Wilms tumor in pediatric patients. Chemotherapy and tumor removal achieve successful treatment.

Gastroschisis is the most common congenital abdominal wall defect. Due to the exposure of midgut to amniotic fluid, the recovery of bowel function is often delayed. This study aimed to identify the factors associated with the successful early enteral feeding in gastroschisis and to develop further guidelines of treatment.A retrospective cohort study of gastroschisis babies from January 2006 to December 2015 was done. Exclusion criteria were incomplete data and death. Successful early enteral feeding was defined when full feeding was achieved within 21 days of life.One hundred and five gastroschisis patients were divided into a successful early-feeding group (n = 56, 53%) and a non-successful early-feeding group (n = 49, 46%). In multivariable analysis, significant factors for successful feeding clustered by primary treatment were female (RR = 1.38, P value 0.001), gestational age 36 weeks (RR = 1.23, P value 0.001), age at surgery less than 10 h (RR = 1.15, P value 0.001), postoperative extubation time 4 days (RR = 1.39, P value 0.001), and age when feeding started less than 10 days (RR = 35.69, P value 0.001).Several factors were found to be associated with successful early enteral feeding. The modifiable factors found in this study were surgery within 10 h, early postoperative extubation within 4 days, and feeding started before 10 days of life. These will guide the management of gastroschisis to achieve successful early enteral feeding.

Hirschsprung-associated enterocolitis (HAEC) is a common life-threatening complication of Hirschsprung disease (HSCR). It has been proposed that gut microbiota, which have an essential role in gut-homeostasis, are associated with HAEC. Recent studies demonstrated an increase in alpha diversity of fecal microbiota over time in HSCR mice and a decrease in diversity after surgery. In addition, clinical studies have reported a reduction in bacterial richness in HSCR children after surgery. Some studies revealed a difference in microbiota between the proximal ganglionic and distal aganglionic intestine and found a difference in bacterial character between fecal and colonic specimens. HAEC studies found an increase in Proteobacteria, especially Escherichia and Enterobacteriaceae, with a decrease in Firmicutes and Bifidobacterium in HAEC patients. However, the direction of alpha diversity in HAEC patients is still controversial. The self-comparison of microbiota in treatment periods suggested that probiotics might improve gut dysbiosis and decrease the frequency of enterocolitis, but some reported contradictory findings. This review comprehensively summarizes and discusses key findings from animal and clinical data of the distinct microbiome associated with HCSR and the association of gut dysbiosis with the development of HAEC. This information should be useful in the establishment of novel interventions to improve gut dysbiosis and prevent enterocolitis in HSCR patients.

Background Ilioinguinal/iliohypogastric nerve block is commonly performed to control postherniotomy pain. The posterior quadratus lumborum block has been recently described as an effective analgesic technique for pediatric low abdominal surgery. No data were found regarding the use of posterior quadratus lumborum block in comparison with the traditional ilioinguinal/iliohypogastric nerve block in pediatric inguinal surgery. Aim This randomized assessor-blinded study compared postoperative analgesic effects between ultrasound-guided posterior quadratus lumborum block and ilioinguinal/iliohypogastric nerve block in pediatric inguinal herniotomy. Methods One- to seven-year-old children scheduled for unilateral open herniotomy were randomly assigned to receive either ultrasound-guided posterior quadratus lumborum block with 0.25% bupivacaine 0.5 mL/kg or ultrasound-guided ilioinguinal/iliohypogastric nerve block with 0.25% bupivacaine 0.2 mL/kg after induction of general anesthesia. The primary outcome was the proportion of patients who received postoperative oral acetaminophen. The required fentanyl in the recovery room, 24-hour acetaminophen consumption, success rate of regional blocks, block performance data, block-related complications, postoperative pain intensity, and parental satisfaction were assessed. Results This study included 40 patients after excluding four cases who were ineligible. The number of patients who required postoperative oral acetaminophen was significantly lower in the posterior quadratus lumborum block group (15.8% vs 52.6%; OR: 5.9; 95% CI: 1.3, 27.3; P = .022). The pain scores at 30 minutes, 1, 2, 6, 12, and 24 hours were similar between groups. There was no evidence of between-group differences in block performance time, the number of needle passes, block-related complications, and parental satisfaction. Conclusion The posterior quadratus lumborum block with 0.25% bupivacaine 0.5 mL/kg provided better pain control than the ilioinguinal/iliohypogastric nerve block with 0.25% bupivacaine 0.2 mL/kg after open herniotomy in children. The ultrasound guidance technique for the posterior quadratus lumborum block is safe and as simple as the ultrasound-guided ilioinguinal/iliohypogastric nerve block for pediatric patients.

Tamgal J, Damrongmanee A, Khorana J, Tepmalai K, Ukarapol N. Clearance of jaundice after the modified Kasai`s operation predicts survival outcomes in patients with biliary atresia. Turk J Pediatr 2019; 61: 7-12. The aim of this study was to assess the probability of survival with native liver (SNL) and the rate of esophageal variceal bleeding (EVB) as well as their potential risk factors, in patients diagnosed with Biliary Atresia (BA), who underwent the hepaticoportoenterostomy (HPE) by retrospectively reviewing medical records between 2007 and 2016. The subjects were classified as poor outcomes if they died or a liver transplant (LT) was performed. A total of 73 cases were enrolled. The average age at HPE was 106.2 +/- 58.5 days. Poor outcome was observed in 27.4%, 54.8% survived with native liver and 17.8% were lost to follow-up. The principal cause of death was sepsis, followed by massive upper GI hemorrhage. The overall 10-year SNL was 66.8%. Only total bilirubin (TB) > 3 mg/dL at 3, 6 months after HPE and presence of associated anomalies negatively affected SNL (p=0.0155, 0.0042 and 0.001, respectively). Most of the patients experienced EVB within 3 years of age, in which TB > 9 mg/dL at 12 months after HPE was significantly associated with probability of the EVB outcome. Any interventions to improve jaundice clearance after HPE should be strongly pursued in order to improve outcomes in BA patients, particularly in centers where liver transplantation (LT) is not available. Surveillance esophagogastroduodenoscopy around the age of 1.5 years in patients having TB > 9 mg/dL may be beneficial to identify large varices having potential fatal bleeding.

Improvements in the endoscopic submucosal dissection (ESD) technique have made circumferential ESD in the rectum possible. However, little is known about the clinical course after extensive ESD in the rectum. The aim of this study was to determine the stricture risk in the rectum after total or subtotal circumferential ESD.A total of 69 patients with 69 rectal tumors that required ≥ 75 % circumferential resection were identified at Kobe University Hospital and an affiliated hospital between April 2005 and May 2014. Among the patients, 61 were available for evaluation of stricture development, either by follow-up colonoscopy or by surgical specimens. The rate and possible risk factors of post-ESD strictures were investigated.Post-ESD rectal strictures developed in 12 patients (19.7 %). Patients who underwent total circumferential ESD developed a stricture (5/7, 71.4 %) more frequently than those with subtotal (≥ 90 %) ESD (7/16, 43.8 %). Patients undergoing an ESD procedure that involved 90 % of the circumference did not develop strictures. The strictures were membranous or 10 mm long in all cases. Of the patients with stricture, 11 received endoscopic balloon dilation and one received bougie with short-caliber-tip transparent hood; all strictures improved following dilation therapy. Statistical analysis revealed that ≥ 90 % circumferential resection was an independent risk factor for stricture, whereas morphology and size were not.Patients who underwent total or subtotal circumferential ESD of a rectal tumor had a high risk of stricture formation. Dilation helped to alleviate the stenosis.Study registered at University Hospital Medical Information Network (UMIN 000016559).