Thoracoscopic repair of esophageal atresia with distal tracheoesophageal fistula: is it a safe procedure in infants weighing less than 2000 g?

BACKGROUND: Since Rothenberg first performed thoracoscopic repair for esophageal atresia with distal tracheoesophageal fistula (EA/TEF) successfully in 2000, thoracoscopic repair has achieved status as a routine procedure worldwide. Previously, an international multicenter study reported that this procedure was not inferior to conventional open surgery. However, thoracoscopic surgery is a highly difficult operation for surgeons and anesthesiologists; as a result, the safety and efficacy of the surgery is still under debate. Considering these circumstances, the purpose of this study was to analyze the results of single-center thoracoscopic surgery and to compare the outcomes relative to the patient's weight at the time of surgery. METHODS: We retrospectively analyzed patients with EA/TEF who underwent thoracoscopic surgery in a single center between October 2008 and February 2017. RESULTS: In total, 41 cases of thoracoscopic repair of EA/TEF were performed. Upon subgrouping by over and under 2000 g of body weight at the time of operation, 34 were found to be over 2000 g and seven were under 2000 g. Intraoperative factors and events were not significantly different between the two groups. Additionally, most of the postoperative outcomes, including the rate of postoperative leakage and strictures, showed no difference. On the other hand, the under 2000 g group had more gastroesophageal reflux requiring fundoplication than did the heavier group (P = 0.04). CONCLUSIONS: The results of this center's thoracoscopic repair of EA/TEF were not inferior to other centers' outcomes. Additionally, the intraoperative and postoperative outcomes were similar despite differences in weight at operation. Therefore, thoracoscopic repair might be a feasible surgical option for infants weighing less than 2000 g when performed by a surgeon and anesthesiologist team who are experienced in pediatric thoracoscopic surgery.

Analysis of Pediatric Surgery Using the National Healthcare Insurance Service Database in Korea: How Many Pediatric Surgeons Do We Need in Korea?

BACKGROUND: In the past, general surgeons (GSs) without a pediatric surgical subspecialty often performed surgery on children and, even now, GSs are performing many pediatric surgeries. We aimed to investigate the involvement of pediatric surgeons (PSs) and GSs in pediatric surgery, compare the outcomes of surgery in the neonatal intensive care unit (NICU), and estimate the appropriate PS workforce in Korea. METHODS: We used surgical data from the National Health Insurance Service database that was collected from patients under the age of 19 years in hospitals nationwide from January 2002 to December 2017. In this database, we found 37 hospitals where PSs worked by using the index operation (congenital diaphragmatic hernia, esophageal atresia, hypertrophic pyloric stenosis, Hirschsprung's disease, abdominal wall defect, jejunoileal atresia, malrotation, anorectal malformation, and biliary atresia). It was assumed that the surgery in the 37 hospitals was performed by PS and that the surgery in other hospitals was performed by GS. Mortality was analyzed to compare the outcomes of acute abdominal surgery in the NICU. We estimated the number of PS currently needed in Korea for each situation under the assumption that PS would perform all operations for the index operation, main pediatric diseases (index operation + gastroesophageal reflux disease, choledochal cyst, inguinal hernia, and appendicitis), acute abdominal surgery in the NICU, and all pediatric surgeries. Additionally, we estimated the appropriate number of PS required for more advanced pediatric surgery in the future. RESULTS: The number of pediatric surgeries from 2002 to 2017 increased by 124%. Approximately 10.25% of the total pediatric surgeries were performed by PSs, and the percentage of the surgery performed by PSs increased from 8.32% in 2002 to 15.92% in 2017. The percentage of index operations performed by PSs annually was 62.44% in average. It was only 47.81% in 2002, and increased to 88.79% in 2017. During the last 5 years of the study period, the average annual number of surgeries for main pediatric diseases was approximately 33,228. The ratio of the number of surgeries performed by PS vs. GS steadily increased in main pediatric diseases, however, the ratio of the number of surgery performed by PS for inguinal hernia and appendicitis remained low in the most recent years. The percentage of the number of acute abdominal surgery performed by PS in the NICU was 44% in 2002, but it had recently risen to 89.7%. After 30 days of birth, mortality was significantly lower in all groups that were operated on by PS, rather than GS, during the last 5 years. In 2019, 49 PSs who were under the age of 65 years were actively working in Korea. Assuming that all pediatric surgeries of the patients under the age of 19 years should be performed by PS, the minimum number of PS currently required was about 63 if they perform all of the index operations, the main pediatric surgery was about 209, the NICU operation was about 63, and the all pediatric surgeries was about 366. Additionally, it was determined that approximately 165 to 206 PS will be appropriate for Korea to implement more advanced pediatric surgery in the future. CONCLUSION: The proportion of the pediatric surgery performed by PS rather than GS is increasing in Korea, but it is still widely performed by GS. PSs have better operative outcomes for acute abdominal surgery in the NICU than GSs. We believe that at least the index operation or the NICU operation should be performed by PS for better outcome, and that a minimum of 63 PSs are needed in Korea to do so. In addition, approximately 200 PSs will be required in Korea in order to manage main pediatric diseases and to achieve more advanced pediatric surgery in the future.

Vesicoureteral reflux increases the risk of urinary tract infection prior to corrective surgery in newborn males with anorectal malformation.

PURPOSE: The ideal colostomy type indicated for patients with anorectal malformation (ARM) is disputed. The aim of this study was to analyze the clinical factors associated with urinary tract infection (UTI) prior to corrective surgery in male ARM without perineal fistula having undergone diverting enterostomy. METHODS: A retrospective review of patients diagnosed with ARM and surgically managed at our center from January 2011 to December 2019 was performed. Logistic regression was used to analyze the association between clinical factors and UTI. RESULTS: Eighty boys with ARM without perineal fistula underwent diverting enterostomy and subsequent corrective surgery via laparoscopic-assisted anorectal pull-through. A sigmoid loop colostomy was most often performed (70 patients, 87.5%). Twenty-nine patients (36.3%) were diagnosed with vesicoureteral reflux (VUR), including 14 (48.3%) with febrile UTIs. Six patients had other concomitant genitourinary anomalies excluding VUR. Multivariate logistic regression analysis revealed the presence of VUR as the only independent factor associated with the occurrence of febrile UTI (OR 17.3, 95% CI 3.51-85.26, p < 0.001). CONCLUSION: The development of UTI in newborn males with ARM is associated with the presence of VUR, regardless of stoma type. Voiding cystourethrography should be considered in patients with ARM for early diagnosis of VUR and subsequent antibiotic prophylaxis.

Predicting Survival of Congenital Diaphragmatic Hernia on the First Day of Life.

BACKGROUND: This study aimed to determine perinatal risk factors for 30-day mortality of congenital diaphragmatic hernia (CDH) patients and develop a prognostic index to predict 30-day mortality of CDH patients. Identifying risk factors that can prognosticate outcome is critical to obtain the best management practices for patients. METHODS: A retrospective study was performed for patients who were diagnosed with CDH from November 2000 to August 2016. A total of 10 prenatal risk factors and 14 postnatal risk factors were analyzed. All postnatal variables were measured within 24 h after birth. RESULTS: A total of 95 CDH patients were enrolled in this study, including 61 males and 34 females with mean gestational age of 38.86 ± 1.51 weeks. The overall 30-day survival rate was 63.2%. Multivariate analysis revealed that five factors (polyhydramnios, gestational age at diagnosis <25 weeks, observed-to-expected lung-to-head ratio ≤45, best oxygenation index in 24 h >11, and severity of tricuspid regurgitation ≥ mild) were independent predictors of 30-day mortality of CDH. Using these five factors, a perinatal prognostic index for 30-day mortality was developed. Four predictive models (poor, bad, good, and excellent) of the perinatal prognostic index were constructed, and external validation was performed. CONCLUSIONS: Awareness of risk factors is very important for predicting prognosis and managing patients. Five independent perinatal risk factors were identified in this study. A perinatal prognostic index was developed for 30-day mortality for patients with CDH. This index may be used to help manage CDH patients.

Trans-ethnic meta-analysis of genome-wide association studies for Hirschsprung disease.

Hirschsprung disease (HSCR) is the most common cause of neonatal intestinal obstruction. It is characterized by the absence of ganglia in the nerve plexuses of the lower gastrointestinal tract. So far, three common disease-susceptibility variants at the RET, SEMA3 and NRG1 loci have been detected through genome-wide association studies (GWAS) in Europeans and Asians to understand its genetic etiologies. Here we present a trans-ethnic meta-analysis of 507 HSCR cases and 1191 controls, combining all published GWAS results on HSCR to fine-map these loci and narrow down the putatively causal variants to 99% credible sets. We also demonstrate that the effects of RET and NRG1 are universal across European and Asian ancestries. In contrast, we detected a European-specific association of a low-frequency variant, rs80227144, in SEMA3 [odds ratio (OR) = 5.2, P = 4.7 × 10-10]. Conditional analyses on the lead SNPs revealed a secondary association signal, corresponding to an Asian-specific, low-frequency missense variant encoding RET p.Asp489Asn (rs9282834, conditional OR = 20.3, conditional P = 4.1 × 10-14). When in trans with the RET intron 1 enhancer risk allele, rs9282834 increases the risk of HSCR from 1.1 to 26.7. Overall, our study provides further insights into the genetic architecture of HSCR and has profound implications for future study designs.

Prevalence of Malnutrition in Hospitalized Patients: a Multicenter Cross-sectional Study.

BACKGROUND: Malnutrition is associated with many adverse clinical outcomes. The present study aimed to identify the prevalence of malnutrition in hospitalized patients in Korea, evaluate the association between malnutrition and clinical outcomes, and ascertain the risk factors of malnutrition. METHODS: A multicenter cross-sectional study was performed with 300 patients recruited from among the patients admitted in 25 hospitals on January 6, 2014. Nutritional status was assessed by using the Subjective Global Assessment (SGA). Demographic characteristics and underlying diseases were compared according to nutritional status. Logistic regression analysis was performed to identify the risk factors of malnutrition. Clinical outcomes such as rate of admission in intensive care units, length of hospital stay, and survival rate were evaluated. RESULTS: The prevalence of malnutrition in the hospitalized patients was 22.0%. Old age (≥ 70 years), admission for medical treatment or diagnostic work-up, and underlying pulmonary or oncological disease were associated with malnutrition. Old age and admission for medical treatment or diagnostic work-up were identified to be risk factors of malnutrition in the multivariate analysis. Patients with malnutrition had longer hospital stay (SGA A = 7.63 ± 6.03 days, B = 9.02 ± 9.96 days, and C = 12.18 ± 7.24 days, P = 0.018) and lower 90-day survival rate (SGA A = 97.9%, B = 90.7%, and C = 58.3%, P < 0.001). CONCLUSION: Malnutrition was common in hospitalized patients, and resulted in longer hospitalization and associated lower survival rate. The rate of malnutrition tended to be higher when the patient was older than 70 years old or hospitalized for medical treatment or diagnostic work-up compared to elective surgery.

Potential association between ITPKC genetic variations and Hirschsprung disease.

Hirschsprung disease (HSCR) is a congenital and complex disorder characterized by intestinal obstruction due to the absence of enteric neurons along variable lengths of the hindgut. Our recent genome-wide association study (GWAS) has revealed regional associations with HSCR at several loci of inositol-trisphosphate 3-kinase C (ITPKC). For fine mapping, we additionally selected and genotyped a total of 12 single nucleotide polymorphisms (SNPs) of ITPKC in 187 HSCR patients and 283 unaffected controls, and performed a further combined imputation analysis based on genotype data from this second stage of fine mapping and our previous GWAS stage, totaling 902 subjects (187 HSCR cases and 715 controls). As a result, several SNPs (minimum P = 0.004) and a haplotype (P = 0.02) were found to be significantly associated with HSCR. In further in silico analyses to ascertain the potential functions of the significant variants, the change from the common allele to the rare allele of the highly conserved nonsynonymous rs76785336 showed a difference in mRNA folding structure. In the case of intronic SNPs, rs2607420 with a high consensus value was predicted to be a new splice site. Although this study has limitations (such as lack of functional evaluations, small number of cases, and further need of replication in other cohorts), our findings suggest that genetic variants of ITPKC may have a potential association with HSCR susceptibility and/or developmental diseases related to enteric nervous system development.

Laparoscopic duodenoduodenostomy with parallel anastomosis for duodenal atresia.

BACKGROUND: Currently, a diamond-shaped anastomosis is preferred for the surgical repair of duodenal atresia (DA) in both open and laparoscopic surgery. We report the results of laparoscopic duodenoduodenostomy with parallel anastomosis (LDPA) in DA. METHODS: We retrospectively reviewed 22 patients who underwent laparoscopic duodenoduodenostomy from February 2005 to May 2015 in Samsung Medical Center. All patients underwent operation within the first month after birth. Patients who were transversely anastomosed after duodenotomy and patients who underwent simultaneous operation on combined anomalies were excluded. Parallel anastomosis was used in all surgeries. Four trocars were used in laparoscopic repair. After mobilization of both proximal and distal ends, the proximal end was incised transversely and the distal end was incised longitudinally. Duodenoduodenostomy with parallel anastomosis using a 5-0 glyconate monofilament was performed with interrupted sutures. RESULTS: Eleven patients (50 %) were male. Median gestational age was 36 + 6 weeks (32 + 7-40 + 6). Median age at the time of operation and median body weight were 3 days (1-12) and 2.53 kg (1.63-3.18), respectively. All patients were diagnosed prenatally and 16 patients (72.7 %) had associated anomalies. Median operation time was 142 min (96-290) and median postoperative day to start oral feeding was 5 days (3-9) and median postoperative day of reaching full feeding was 11 days (6-19). Median postoperative day was 13 days (10-60). There was no anastomotic leakage or stenosis. Median follow up was 3.5 months (1-21). Currently, there is no late complication. CONCLUSIONS: LDPA can be performed easily to patients who have DA in neonatal period. It is anatomically natural and the risk of leakage or stenosis does not seem significant. Therefore, parallel anastomosis should be considered as a safe procedural option for laparoscopic duodenoduodenostomy in DA.

Intraoperative Assessment of Facial Nerve Trunk Width in Early Childhood With Cervicofacial Lymphatic Malformation.

BACKGROUND: Facial nerve damage during head and neck surgery has long been an important issue. However, few publications on the gross anatomy of the facial nerve are available in the young population. The aim of this study was to provide in vivo measurements of the facial nerve trunk during lymphatic malformation (LM) resection and to determine the association between the trunk width and patient- and disease-related variables. METHODS: We conducted a retrospective analysis of 11 consecutive pediatric patients (11 facial nerve trunks) who underwent cervicofacial LM resection. The facial nerve of the affected side was dissected, and its trunk width at bifurcation was measured using calipers under a microscope during the operation. RESULTS: Eleven patients younger than 6 years were enrolled. The median width of the facial nerve in patients younger than 1 year was 1.15 mm; it was 2.5 mm in those older than 1 year. Trunk width was significantly greater in patients older than 1 year than those younger than 1 year, whereas no statistical significance was found when comparing other age groups. Patient weight was positively correlated with trunk width, whereas LM grade and diameter showed no significant correlation. CONCLUSIONS: The significantly greater width of the facial nerve trunk in LM patients older than 1 year than those younger than 1 year suggests that the age of 1 may be a threshold for facial nerve hypertrophy and growth acceleration. This study provides informative in vivo data to help understand facial nerve characteristics in young patients.

Association Analysis of SLC6A20 Polymorphisms With Hirschsprung Disease.

PURPOSE: Hirschsprung disease (HSCR) is a congenital and heterogeneous disorder, which is caused by no neuronal ganglion cells in part or all of distal gastrointestinal tract. Recently, our genome-wide association study has identified solute carrier family 6, proline IMINO transporter, member 20 (SLC6A20) as one of the potential risk factors for HSCR development. This study performed a replication study for the association of SLC6A20 polymorphisms with HSCR and an extended analysis to investigate further associations for subgroups and haplotypes. METHODS: For the replication study, a total of 40 single nucleotide polymorphisms (SNPs) of SLC6A20 were genotyped in 187 HSCR subjects composed of 121 short-segment HSCR, 45 long-segment HSCR (L-HSCR), 21 total colonic aganglionosis, and 283 unaffected controls. Imputation was performed using genotype data from our genome-wide association study and this replication study. RESULTS: Imputed meta-analysis revealed that 13 SLC6A20 SNPs (minimum P = 0.0002 at rs6770261) were significantly associated with HSCR even after correction for multiple comparisons using false discovery rate (FDR) (minimum PFDR =  .005). In further subgroup analysis, SLC6A20 polymorphisms appeared to have increased associations with L-HSCR. Moreover, haplotype analysis also showed significant associations between 2 haplotypes (BL3_ht2 and BL4_ht2) and HSCR susceptibility (PFDR <  .05). CONCLUSIONS: Although further replications and functional evaluations are required, our results suggest that SLC6A20 may have roles in HSCR development and in the extent of aganglionic segment during enteric nervous system development.

Association Between Prenatal Sonographic Findings of Duodenal Obstruction and Adverse Outcomes.

OBJECTIVES: The purpose of this study was to evaluate the association of prenatal sonographic findings with adverse outcomes and the causes of duodenal obstruction. METHODS: A total of 59 cases of congenital duodenal obstruction were included in this study. The sonographic findings, including the degree of duodenal dilatation, polyhydramnios, and their change over gestation, were investigated. Adverse outcomes were defined as fetal death in utero, postnatal death, and gastrointestinal complications requiring readmission or reoperation during the follow-up period. The cause of duodenal obstruction was also assessed. RESULTS: Among the patients studied, 2 (3.4%) had fetal death in utero and 2 (3.5%) had postnatal death. Gastrointestinal complications requiring readmission or reoperation occurred in 10.9%. In the cases with or without adverse outcomes, no significant differences were observed in the prenatal sonographic findings: maximum duodenal dilatation, mean amniotic fluid index, and the changes in these parameters with advancing gestation. The cases with adverse outcomes were associated with a younger gestational age at delivery compared to the cases without adverse outcomes. Notably, the degree of duodenal dilatation and amniotic fluid volume were greater in duodenal atresia than in other causes of obstruction, including duodenal stenosis, a duodenal web, and an annular pancreas. In the study population, the overall postoperative survival rate was 98.2%. CONCLUSIONS: Prenatal sonographic findings of duodenal obstruction were not associated with adverse outcomes; however, they may be helpful for differentiating the cause of duodenal obstruction.

Sarcopenia with decreased total psoas muscle area in children with high-risk neuroblastoma.

BACKGROUND: We calculated psoas muscle area (PMA) z-scores in high-risk neuroblastoma patients undergoing treatment to examine the clinical significance of sarcopenia in this cohort. METHODS: We analyzed retrospective data from patients aged 0-18 who were diagnosed with abdominal neuroblastoma between 2005 and 2019 at Samsung Medical Center. Patients categorized as high-risk undergone induction chemotherapy, neuroblastoma excision, and tandem high-dose chemotherapy with autologous stem cell transplantation (HDCT/auto-SCT) were selected. L3-4 lumbar levels on axial CT images were identified and we measured the areas of the left and right psoas muscles to determine tPMA. Total PMA z-scores were calculated using an open online tool. RESULTS: There were 45 boys and 25 girls with a mean age of 3.86 years. CT images taken at initial diagnosis and after tandem HDCT/auto-SCT were selected to calculate tPMA z-scores. Mean elapsed time between the two measurements was 12.91 ± 1.73 months. Mean tPMA z-score significantly decreased from -0.21 ± 1.29 to -0.66 ± 0.97 (p = 0.022). Length of hospital stay was significantly longer in the group of patients whose tPMA z-scores decreased by more than .45 (177.62 ± 28.82 days vs. 165.75 ± 21.34 days, p = 0.049). Presence of sarcopenia at initial diagnosis was a significant risk factor for bacterial infection during neuroblastoma treatment. CONCLUSION: tPMA z-scores in high-risk neuroblastoma patients decreased significantly following a treatment regimen that included induction chemotherapy, tumor resection surgery, and HDCT/auto-SCT. A greater decrease in tPMA z-score was associated with longer hospital stay during treatment.

Long-term results of biliary atresia in the era of liver transplantation.

PURPOSE: The purpose of this study was to analyze the survival of patients with biliary atresia (BA) after Kasai operation and liver transplantation (LT) and to analyze the factors affecting survival. METHODS: Seventy-two patients diagnosed with BA were operated on between April 1995 and December 2009 and retrospectively analyzed. RESULTS: Out of the 72 patients, 59 received Kasai operation and 13 received LT without prior Kasai operation. Twenty-seven patients received LT after Kasai operation. Survival with native liver was 39 % at 10 years. With the application of LT, overall 10-year survival for patients with BA was 94.9 %. Among patients alive with native livers after Kasai operation, 14 patients (58.3 %) have at least one complication associated with biliary cirrhosis and portal hypertension. Age at which Kasai operation was performed (60 days) and postoperative normalization of bilirubin were independent risk factors for survival with the native liver, according to multivariate analysis (HR 2.90, p = 0.033 and HR 9.89, p = 0.002). CONCLUSIONS: Survival of BA patients has greatly increased in the era of LT. However, many patients surviving with native livers after Kasai operation continue to have signs of biliary cirrhosis and abnormal liver function.

Clinical Outcome of Ultrasound-Detected Perforated Necrotizing Enterocolitis without Radiographic Pneumoperitoneum in Very Preterm Infants.

OBJECTIVES: To investigate the clinical outcomes of ultrasound (US)-detected perforated necrotizing enterocolitis (NEC) without radiographic pneumoperitoneum in very preterm infants. METHODS: In this single-center retrospective study, very preterm infants who underwent a laparotomy for perforated NEC during their neonatal intensive care unit stay were classified into two groups according to the absence or presence of pneumoperitoneum on radiographs (the case versus the control groups). The primary outcome was death before discharge, and the secondary outcomes included major morbidities and body weight at 36 weeks postmenstrual age (PMA). RESULTS: Of the 57 infants with perforated NEC, 12 (21%) had no pneumoperitoneum on the radiographs and were diagnosed with perforated NEC on the US. In the multivariable analyses, the primary outcome of death before discharge was significantly lower in infants with perforated NEC without radiographic pneumoperitoneum than in those with perforated NEC and radiographic pneumoperitoneum (8% [1/12] vs. 44% [20/45]; adjusted odds ratio [OR], 0.02; 95% confidence interval [CI], 0.00-0.61; p = 0.025). The secondary outcomes (short bowel syndrome, total parenteral nutrition dependence for 3 months or more, the length of their hospital stay, a bowel stricture requiring surgery, sepsis after the laparotomy, acute kidney injury after the laparotomy, and body weight at 36 weeks PMA) did not differ significantly between the two groups. CONCLUSIONS: Very preterm infants with US-detected perforated NEC without radiographic pneumoperitoneum had a lower risk of death before discharge than those with perforated NEC and radiographic pneumoperitoneum. Bowel USs may have a potential role in surgical decision-making in infants with advanced NEC.

Posteromedial diaphragmatic hernia following pediatric liver transplantation.

Diaphragmatic hernia is a rare complication following pediatric LT. Here, four children who developed right-sided posteromedial diaphragmatic hernias after LT are reported. The primary disease was biliary atresia in two patients, hemangioendothelioma in one, and angiosarcoma in one patient. All of the patients underwent living-donor LT using a left lateral graft. The patients presented with abdominal and/or pulmonary signs and symptoms. The diaphragmatic hernias were diagnosed at 28 days to seven months post-transplant by standard radiographs or chest CT. The defects were located at the posteromedial aspect of the diaphragm and were closed by primary closure. After diaphragm repair, the post-operative course was unremarkable and there were no recurrences. Thermal or mechanical injuries to the bare area, especially in cases of excessive adhesion between the liver and diaphragm after Kasai operation, were the possible causes of the posteromedial diaphragmatic hernia after pediatric LT.

The relationship between biliary amylase and the clinical features of choledochal cysts in pediatric patients.

BACKGROUND: Although the clinical features of choledochal cysts (CC) in different age groups have been widely studied, the causes of differences in clinical features are unknown. To determine the relationship between biliary amylase and the clinical features of CC in pediatric patients, clinical outcomes were compared in two groups with different amylase levels. METHODS: From May 1995 to August 2010, 80 patients under 18 years old who underwent choledochal cyst excision and hepaticojejunostomy and measurements of biliary amylase levels were allocated to a low-amylase-level group (amylase level < 200 U/L, n = 26) and a high-amylase-level group (amylase level > 200 U/L, n = 54). Their medical records were retrospectively reviewed. RESULTS: The median age was 4 months (range = 17 days-169 months) in the low group and 48 months (range = 22 days-147 months) in the high group (p = 0.008). In the low group, jaundice was the most common symptom, while abdominal pain was the main symptom in the high group. In histological findings, bile duct proliferation and cholestasis predominated in the low group and portal inflammation predominated in the high group. Radiological findings and preoperative laboratory data were also significantly different between the groups. Postoperative complications occurred in the high group only. There was no mortality in either group. CONCLUSION: This study shows a relationship between biliary amylase level and clinical manifestations in pediatric patients with CC, implying that there are different underlying pathophysiologies with anomalous pancreaticobiliary ductal union (APBDU).

PELD score and age as a prognostic index of biliary atresia patients undergoing Kasai portoenterostomy.

PURPOSE: This study was designed to evaluate the validity of the pediatric end-stage liver disease (PELD) score as a prognostic index of native liver survival in biliary atresia before Kasai portoenterostomy. METHODS: Medical records of 63 patients who underwent Kasai portoenterostomy at our hospital were reviewed retrospectively. Rate of jaundice clearance and cumulative survival with native liver were calculated. Patients who were alive and did not require transplantation until the last follow up were included in the native-liver survival group. And patients who underwent any event, defined as death without transplantation, transplantation or listing for transplantation, were included in the native-liver failure group. Cox analysis was done. RESULTS: 10-year survival rate with native liver was 41.9%. PELD scores of ≥ 15 and an age at operation of ≥ 60 days were found to be significant risk factors of failure(P = 0.007, OR = 2.851 and P = 0.010, OR = 3.222, respectively). Furthermore, patients with both risk factors showed significantly higher risk of failure (P = 0.001, OR = 8.809). CONCLUSION: Since failure rate is higher for patients with high PELD score and older age, these factors should be helpful in estimating the prognosis. And with high-risk patients, cautious monitoring and consultation should be made whether the liver fails and requires transplantation.

Clinical Accuracy of Non-Contact Forehead Infrared Thermometer Measurement in Children: An Observational Study.

We evaluated the clinical reliability and utility of temperature measurements using no-contact forehead infrared thermometers (NCFITs) by comparing their temperature measurements with those obtained using infrared tympanic thermometers (IRTTs) in children. In this observational, prospective, and cross-sectional study, we enrolled 255 children (aged 1 month to 18 years) from the pediatric surgery ward at a tertiary medical center in Korea. The mean age of the children was 9.05 ± 5.39 years, and 54.9% were boys. The incidence rate of fever, defined as an IRTT reading of ≥38.0 °C, was 15.7%. The ICC coefficient for the assessment of agreement between temperatures recorded by the NCFIT and IRTT was 0.87, and the κ-coefficient was 0.83. The bias and 95% limits of agreement were 0.15 °C (−0.43 to 0.73). For an accurate diagnosis of fever (≥38 °C), the false-negative rate was much lower, but the false-positive rate was higher, especially in 6-year-old children. Therefore, NCFITs can be used to screen children for fever. However, a secondary check is required using another thermometer when the child's temperature is >38 °C. NCFITs are proposed for screening but not for measuring the temperature. For the latter, an accurate and reliable thermometer shall be used.

Fluoroscopic criteria for on-site evaluation of failed intussusception reduction during air enema technique.

Background: There is no reliable fluoroscopic criteria for failed intussusception reduction during air enema technique. Methods: This retrospective case-control study included 373 episodes of ileocolic intussusceptions who had undergone air enema under fluoroscopy. All procedures were initially classified by conventional fluoroscopic criteria: presumptive successful procedures (PSP) vs. presumptive failed procedures (PFP). PFP were divided into true failure, false failure, and undetermined groups. The configuration and size of the residual mass were evaluated on fluoroscopic images. Statistical analyses included Mann-Whitney U-test, Fisher's exact test, receiver operating characteristic (ROC) analysis, logistic regression analyses, and Kruskal-Wallis rank sum test with a post hoc Tukey test. Results: PSP was 264 episodes (71%) and PFP was 109 episodes (29%). The true failure was 40 (37%) and false failure was 48 (44%). The true failure group commonly showed a larger size and round configuration for the residual mass than false failure (P<0.001). Multivariable analysis revealed configuration (P=0.004) and transverse diameter (P=0.007) as significant parameters that differentiated true and false failure. The optimal cut-off value of the transverse diameter of the residual mass was 2.3 cm. The sensitivity and specificity of conventional fluoroscopic criteria for failed reduction was 100% and 85%, respectively. The combination of new fluoroscopic findings and conventional criteria increased the specificity to 100%. Conclusions: Fluoroscopic finding of round-shape and larger size residual mass combined with conventional criteria may be useful for differentiating false failure from truly failed enema reduction in children with intussusception.