Surgical management of pancreatic neoplasms in children: a single-institution experience over 15 years.

Introduction: Pancreatic neoplasms are rare among children and very few studies have reported on surgical outcomes for pediatric pancreatic neoplasms. Therefore, we aimed to describe patient and tumor characteristics and report on the surgical outcomes of pediatric pancreatic neoplasm. Methods: In this retrospective single-center study, we reviewed and analyzed the data of patients who underwent surgery for pediatric pancreatic neoplasms at Severance Children's Hospital between January 2007 and December 2022. Clinical data including demographics, surgical procedures, and postoperative and long-term outcomes were evaluated. Results: A total of 28 patients underwent surgical treatment for pancreatic neoplasms with a median age of 11.7 years (range: 0.4-17.8). The most common histological diagnosis among benign tumors was solid pseudopapillary neoplasm (SPN), which occurred in 20 patients (71.4%). This was followed by a mucinous cyst, nesidioblastosis, pseudocyst, duplication cyst, and benign cyst, each occurring in one patient (3.5%). Regarding malignant tumors, pancreatoblastoma, solid pseudopapillary carcinoma, and malignant pheochromocytoma were noted in one patient each (3.5%). Tumor locations included the head in 4 patients (14.2%), the body in 7 (25%), and the tail in 16 (57.1%), and was diffuse in 1 (3.5%). The most common surgical resection range was distal pancreatectomy, found in 22 patients (78.5%), followed by pylorus-preserving pancreaticoduodenectomy, found in 2 (7.2%); duodenum-preserving pancreatic resection, central pancreatectomy, tumor enucleation, and near-total pancreatectomy were performed in one patient each (3.5%). Overall, 4 patients developed grade B or C postoperative pancreatic fistulas, and 1 experienced postoperative mortality due to uncontrollable bleeding. The mean follow-up period was 6.1 years (range: 1-15.6 years), during which no significant impact on growth after surgery was detected. Among the 20 patients with SPN, tumor rupture occurred in 4 (20%), among whom 2 experienced tumor recurrences. Conclusions: Histological diagnosis of benign tumors was predominant in this case series and various extents of surgical resection were performed. Surgical treatment for pediatric pancreatic neoplasms appears to be safe and effective. However, considering the long-term prognosis of these patients, it is essential to determine the appropriate extent of surgical resection based on the location of the tumor.

Optimal timing of surgery for prenatally diagnosed choledochal cysts.

Objective: Choledochal cysts are increasingly being diagnosed antenatally. The appropriate time of surgical treatment has the greatest impact on the prognosis of choledochal cyst treatment. The purpose of this study was to compare the clinical outcomes of prenatally diagnosed choledochal cysts in infants according to the surgical treatment timing. Methods: We retrospectively reviewed the medical records of infants who underwent surgery for choledochal cysts with antenatal diagnoses. We investigated each patient's demographic information, type of choledochal cyst, serum liver enzyme levels, and surgical outcomes according to the surgical intervention timing. Results: Between May 2006 and December 2020, 93 infants underwent surgery to treat choledochal cysts; among them, 68 had antenatally suspected choledochal cysts. Of the 68 patients, 21 developed symptoms directly after birth. While 38 patients remained asymptomatic, 9 developed symptoms before operation. To compare surgical outcomes, asymptomatic patients were divided into early (13 cases) and late (25 cases) operation groups based on an age benchmark of 30 days. The early surgical group experienced longer times to resume a full diet (6.0 ± 1.6 vs. 4.5 ± 0.7, p < 0.001) and longer postoperative hospital stays (11 ± 3.9 vs. 7.5 ± 0.8, p < 0.001). Surgical complications occurred in two patients in the early operation group. Minimally invasive surgery was performed in 12 patients in the late operation group. In both groups, postoperative liver function recovered at 6 months, with no significant difference. Conclusion: The results of this study showed longer hospital stays, increased diet durations, and postoperative complications in early surgery patients. However, liver function recovery was not different between the early and late operation groups. Thus, asymptomatic patients should be closely monitored, and we recommend that definitive surgical intervention be postponed until 4 months of age or until weight reaches 7 kg.

Pre- and Immediate Post-Kasai Portoenterostomy Shear Wave Elastography for Predicting Hepatic Fibrosis and Native Liver Outcomes in Patients With Biliary Atresia.

OBJECTIVE: To evaluate the feasibility of ultrasound shear wave elastography (SWE) for predicting hepatic fibrosis and native liver outcomes in patients with biliary atresia. MATERIALS AND METHODS: This prospective study included 33 consecutive patients with biliary atresia (median age, 8 weeks [interquartile range, 6-10 weeks]; male:female ratio, 15:18) from Severance Children's Hospital between May 2019 and February 2022. Preoperative (within 1 week from surgery) and immediate postoperative (on postoperative days [PODs] 3, 5, and 7) ultrasonographic findings were obtained and analyzed, including the SWE of the liver and spleen. Hepatic fibrosis, according to the METAVIR score at the time of Kasai portoenterostomy and native liver outcomes during postsurgical follow-up, were compared and correlated with imaging and laboratory findings. Poor outcomes were defined as intractable cholangitis or liver transplantation. The diagnostic performance of SWE in predicting METAVIR F3-F4 and poor hepatic outcomes was analyzed using receiver operating characteristic (ROC) analyses. RESULTS: All patients were analyzed without exclusion. Perioperative advanced hepatic fibrosis (F3-F4) was associated with older age and higher preoperative direct bilirubin and SWE values in the liver and spleen. Preoperative liver SWE showed a ROC area of 0.806 and 63.6% (7/11) sensitivity and 86.4% (19/22) specificity at a cutoff of 17.5 kPa for diagnosing F3-F4. The poor outcome group included five patients with intractable cholangitis and three undergoing liver transplantation who showed high postoperative liver SWE values. Liver SWE on PODs 3-7 showed ROC areas of 0.783-0.891 for predicting poor outcomes, and a cutoff value of 10.3 kPa for SWE on POD 3 had 100% (8/8) sensitivity and 73.9% (17/23) specificity. CONCLUSION: Preoperative liver SWE can predict advanced hepatic fibrosis, and immediate postoperative liver SWE can predict poor native liver outcomes in patients with biliary atresia.

Long-term usage pattern and satisfaction survey of continent catheterizable channels.

INTRODUCTION: We investigated the long-term usage pattern and satisfaction of continent catheterizable channels (CCCs). METHODS: From 2005 to 2018, CCCs, including Mitrofanoff and antegrade continent enema (ACE) channels, were made in 67 patients (Mitrofanoff in 21 patients, ACE channels in 43 patients, and both in three patients) in our institution. An online survey was conducted for these patients in order to assess usage pattern, continent status, difficulty in usage, and patient satisfaction. RESULTS: Sixteen (66.7%) out of 24 patients with the Mitrofanoff channel and 39 (84.7%) out of 46 patients with the ACE channel completed the online survey. In the Mitrofanoff channel group, 10 (62.5%) patients had spina bifida, two (12.5%) had Hinman syndrome, one (6.3%) had posterior urethral valves, and three (18.8%) had urethral trauma or atresia. Additionally, the mean age of the patients at the time of surgery was 10.0 years, and the median follow-up duration was 10.9 years. All patients were using the Mitrofanoff channel to perform clean intermittent catheterization (CIC). Eleven patients (68.8%) had difficulty with catheterization, mostly at the stomal site. Most patients conducted CIC more than four times a day (13, 81.3%). Regarding urination status, seven patients (43.8%) responded that they were satisfied and nine (56.2%) responded they were neutral. In the ACE channel group, 35 patients (89.7%) had spina bifida, seven (17.9%) had cloacal anomalies, and 26 (66.7%) had anorectal malformations. The mean age of the patients at the time of surgery was 8.4 years, and the median follow-up period was 7.4 years. Two (5.1%) patients were no longer using their ACE channels, but 15 (38.5%) patients were still using their channels almost daily. Twenty-eight (71.8%) patients complained that performing enema was time-consuming, and seven (17.9%) patients reported pain when performing ACE and fecal incontinence. Most patients were satisfied with their defecation status (23, 59%), 15 (38.5%) were neutral, and one (2.6%) was dissatisfied. CONCLUSIONS: While most patients who had either Mitrofanoff or ACE channels were still using their channels effectively, approximately half of the patients with CCCs demonstrated neutral satisfaction with their current status; this shows a poor result compared to previous reports. Considering the results of our patient-based study, thorough explanations should be provided to patients who are candidates for Mitrofanoff and ACE procedures; additionally, the discomfort related to the procedures should be comprehensively assessed during follow-up consults.

Changes in outcomes and operative trends with pediatric robot-assisted resection of choledochal cyst.

BACKGROUND: This study aimed to report our experience with a robot-assisted resection of choledochal cysts (CCs) in pediatric patients, especially focusing on changes in outcomes and operative trends. METHODS: We retrospectively reviewed medical records of all 158 patients under 18 years of age who underwent robot-assisted resection of CC in a single tertiary center between July 2008 and January 2021. Patients were divided into the first period (P1, July 2008-March 2016; N = 79) and second period (P2, April 2016-January 2021; N = 79) with equal number of participants. The patients of P2 were compared with those of P1 to assess clinical outcomes with operative details. Operative characteristics and postoperative prognosis were compared for each group. RESULTS: The mean operative time was 383.6 min for the P2 group and 462.6 min for the P1 group (p < 0.001). The mean estimated blood loss was 28 mL in the P2 group and 63 mL in the P1 group (p = 0.025). The rate of emergency department visit after the operation was lower in the P2 group (3.8% vs. 13.9%, respectively, p = 0.047). The two groups showed no significant differences in the rate of late postoperative complications and reoperations. CONCLUSION: With the increase in the center's experience, robot-assisted resection of CC can be safely adopted and feasible, especially for pediatric patients. LEVELS OF EVIDENCE: Treatment Study, Level III.

Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI.

PURPOSE: This study compared clinical and radiologic differences between cystic biliary atresia (cBA) and choledochal cyst (CC) type Ia/b. METHODS: Infants (≤12 months old) who were diagnosed with cBA or CC type Ia/b from 2005 to 2019 were retrospectively reviewed. Imaging features on preoperative ultrasonography (US) and magnetic resonance imaging (MRI) were compared between the cBA and CC groups. Logistic regression and area under the receiver operating characteristic curve (AUC) analyses were performed for the diagnosis of cBA. Changes in cyst size were also evaluated when prenatal US exams were available. RESULTS: Ten patients (5.5% of biliary atresia cases) with cBA (median age, 48 days) and 11 infants with CC type Ia/b (Ia:Ib=10:1; median age, 20 days) were included. Triangular cord thickness on US (cutoff, 4 mm) showed 100% sensitivity and 90.9% specificity (AUC, 0.964; 95% confidence interval [CI], 0.779 to 1.000) and cyst size on MRI (cutoff, 2.2 cm) had 70% sensitivity and 100% specificity (AUC, 0.900; 95% CI, 0.690 to 0.987) for diagnosing cBA. Gallbladder mucosal irregularity on US and an invisible distal common bile duct on MRI were only seen in the cBA group (10 of 10). Only the CC group showed prenatal cysts exceeding 1 cm with postnatal enlargement. CONCLUSION: Small cyst size (<1 cm) on prenatal US, triangular cord thickening (≥4 mm) and gallbladder mucosal irregularity on postnatal US, and small cyst size (≤2.2 cm) and an invisible distal common bile duct on MRI can discriminate cBA from CC type Ia/b in infancy.

Validating the BAVENO VI criteria to identify low risk biliary atresia patients without endoscopy for esophageal varix.

BACKGROUND AND AIMS: Portoenterostomy is the initial surgical treatment for biliary atresia (BA); however, no curative therapy exists for BA. Varix bleeding is a major complication of end-stage liver disease and must be determined in patients with BA, necessitating routine surveillance using esophagogastroduodenoscopy (EGD). We attempted to validate criteria to identify BA patients requiring EGD. METHODS: From January 2007 to December 2017, we selected BA patients who underwent Kasai surgery, transient elastography (TE), and EGD at Severance hospital. In total, 190 cases were included; laboratory tests and EGDs were carried out from 3 months before TE to 3 months after TE. RESULTS: Based on the cut-off value (<10) of the liver stiffness measurement (LSM), 35 (81.4%) patients with low-risk varix (LRV) and 8 (18.6%) with high-risk varix (HRV) were identified. Based on platelet counts (>150,000), 87 (77.68%) patients with LRV and 25 (22.32%) with HRV were identified. Based on this, the BAVENO VI criteria, which identify patients who can safely avoid screening EGD, missed 9/68 (13.24%) of HRV patients. The expanded BAVENO VI criteria missed 21/68 (30.88%) of HRV patients. However, the criteria using LSM <10 and platelet count >150,000 missed identifying only 4/68 (5.88%) HRV patients. CONCLUSIONS: The BAVENO criteria may be as useful in children with BA as in adults with liver cirrhosis. Regular laboratory tests, imaging studies, and EGD may avoid missing diagnoses of varices in BA patients. However, LSM<10 and platelet count>150,000 may provide more accurate criteria and help identify patients who does not need endoscopy.

Periportal thickening on magnetic resonance imaging for hepatic fibrosis in infantile cholestasis.

BACKGROUND: Untreated neonatal cholestasis can progress to liver cirrhosis and end stage liver disease in infancy due to prolonged hepatocyte and biliary tree injury and may require liver transplantation. Therefore, non-invasive evaluation of hepatic fibrosis is important in infants with cholestasis. AIM: To investigate the usefulness of periportal thickening (PT) measured on liver magnetic resonance imaging (MRI) for the assessment of hepatic fibrosis in infants with cholestasis including biliary atresia (BA). METHODS: This retrospective study included infants less than 6 mo who underwent liver MRI and biopsy for the evaluation of infantile cholestasis. PT and spleen size were measured on MRI. Serologic assessment was based on aspartate transaminase to platelet ratio index (APRI). The grade of histopathologic fibrosis was assessed by the METAVIR grading system. Correlation and diagnostic performance of PT, normalized spleen size ratio (SR, using the upper normal size limit), and APRI for diagnosing hepatic fibrosis were obtained by receiver-operating characteristic (ROC) curve analysis. RESULTS: A total of 155 patients were included, 110 of which were diagnosed with BA. Mean age at the time of MRI was 57.6 ± 34.4 d. There were positive correlations between fibrosis grade and PT and SR, even after adjusting age (all, P < 0.001). For the diagnosis of significant fibrosis (METAVIR grade F2-F4), the area under the ROC curve was 0.899 (95%CI: 0.840-0.941) for PT (cutoff, 4.2 mm), which was higher than 0.741 (95%CI: 0.664-0.808) for SR and 0.712 (95%CI: 0.634-0.782) for APRI (both, P < 0.001). For the diagnosis of cirrhosis (F4), the area under the ROC curve was the highest with SR as 0.790 (95%CI: 0.718-0.852). CONCLUSION: Liver MRI findings of PT and SR are useful to assess clinically significant hepatic fibrosis (F2 and higher) in infants with cholestasis including BA.

Incidence and Risk Factors of Acute Kidney Injury after Kasai Operation for Biliary Atresia: A Retrospective Study.

Background: Biliary atresia is a progressive, inflammatory, and destructive pathology of the bile ducts. Patients who undergo surgery for correction of biliary atresia (Kasai operation) are at risk of acute kidney injury (AKI) because of their young age at the time of surgery, long operation time, and liver fibrosis or failure as complication of biliary atresia. Conversely, AKI is associated with poor outcomes after surgery. This study therefore aimed to evaluate the incidence, risk factors, and outcomes of AKI after Kasai operation. Methods: All consecutive patients who underwent Kasai operation between March 2006 and December 2015 in a single tertiary-care university hospital were enrolled. AKI was defined based on the Acute Kidney Injury Network criteria. Multivariate logistic regression models were used to assess risk factors for AKI. Results: One hundred sixty-six patients received Kasai operation during study period. Of these, AKI occurred in 37 of 166 patients (22.3%). In multivariate logistic regression analysis, age older than 30 days, higher preoperative estimated glomerular filtration rate, and preoperative contrast use within 7 days were associated with the development of AKI. Perioperative packed red blood cells transfusion was related to reduced occurrence of AKI. AKI was associated with longer ICU stay (OR = 1.015, p = 0.016). More patients with AKI were also found to receive additional surgery except liver transplantation within 1 year compared to those without AKI (10.8 % vs. 2.3 %, p = 0.045). Conclusions: Increased age is strongly associated with the development of AKI after Kasai operation. These findings indicate a rational basis for early corrective surgery for biliary atresia, early screening for AKI, and intervention to improve the results of Kasai operation.

Comparison of the outcomes of biliary atresia with cystic degeneration and isolated biliary atresia: A matched-pair analysis.

BACKGROUND: Patients with biliary atresia (BA) with extrahepatic cystic degeneration (BACD) have a unique pathophysiology; however, clinical outcomes and progression of perinatal degeneration are not well-defined. We aimed to investigate the differences in clinical characteristics and outcomes between BACD and isolated BA (IBA). METHODS: We performed a retrospective analysis of patients with BA who underwent Kasai portoenterostomy (KPE) from August 1997 to January 2018 and compared the clinical features and outcomes between BACD (n = 21) and IBA (n = 237). Matched-pair analysis for age and sex was performed between BACD and IBA groups to reduce confounding. RESULTS: Before matched-pair analysis, we found that BACD patients were younger at KPE (45 vs. 64 days, p = 0.008), showed lower total bilirubin at the 3-month follow-up (0.5 vs. 1.4 mg/dL, p = 0.002), and higher 5-year native liver survival rate (95.2% vs. 61.4%, p = 0.006) than IBA patients. After matching, the BACD group showed significantly lower total bilirubin levels at the 3-month follow-up (0.5 vs. 1.5 mg/dL, p = 0.036) and higher 5-year native liver survival rate (95.2% vs. 57.5%, p = 0.006) than the IBA group. CONCLUSION: BACD demonstrated higher bilirubin clearance and native liver survival rates than IBA. LEVELS OF EVIDENCE: Treatment Study, Level III.

Hepatic subcapsular or capsular flow in biliary atresia: is it useful imaging feature after the Kasai operation?

OBJECTIVES: To evaluate the implications of hepatic subcapsular and capsular flows using ultrasonography (US) in children after Kasai operation. METHODS: Children who underwent liver US including color Doppler US and microvascular imaging (MVI) from May 2017 to October 2017 were retrospectively included. Children who underwent the Kasai operation for biliary atresia were included in the Kasai group and children with normal liver were included in the control group. Using US results, the number of intrahepatic biliary cysts and the maximum diameter of the spleen were evaluated in the Kasai group. Liver stiffness values were included when patients in the Kasai group had transient elastography (TE) or shear wave elastography (SWE) results. Hepatic subcapsular and capsular flows on color Doppler US and MVI were compared between the two groups using the following scores: 0, no flow reaching the hepatic capsule; 1, any flow reaching the hepatic capsule; and 2, contiguous hepatic capsular flow. The logistic regression test was used to identify associations between age, intrahepatic biliary cysts, spleen size, SWV, TE results, and flow scores measured on Doppler US and MVI in the Kasai group using the odds ratio (OR) and 95% confidence interval (CI). RESULT: A total of 65 children (mean 7.6 ± 5.3 years), 44 in the Kasai group and 21 in the control group, were included. In the control group, one child had score 1 on MVI and others had score 0 on both Doppler US and MVI. Among the Kasai group, 28 children (63.6%) had score 1, while others had score 0 using Doppler US. Using MVI, 24 children (54.5%) had score 2, 18 children had score 1, and one child had score 0. In the Kasai group, increased liver stiffness on TE was the only factor significantly associated with the presence of subcapsular flow on color Doppler US (OR 1.225, 95% CI 1.020-1.470) and increased spleen size was the only factor significantly associated with increased flow scores on MVI (OR 1.397, 95% CI 1.002-2.724). CONCLUSION: Detection of hepatic subcapsular, capsular flows on US would be meaningful for children after receiving the Kasai operation. KEY POINTS: • Hepatic subcapsular or capsular flows can be useful not only for the diagnosis but also for the postoperative follow-up in patients with biliary atresia. • Increased liver stiffness and splenomegaly after the Kasai operation were associated with presence of subcapsular or capsular flow on ultrasonography. • Evaluation of hepatic subcapsular and capsular flows could be needed to assess disease progression after receiving the Kasai operation.

Predicting gastroesophageal varices through spleen magnetic resonance elastography in pediatric liver fibrosis.

BACKGROUND: A recent retrospective study confirmed that hepatic stiffness and splenic stiffness measured with magnetic resonance elastography (MRE) are strongly associated with the presence of esophageal varices. In addition, strong correlations have been reported between splenic stiffness values measured with MRE and hepatic venous pressure gradients in animal models. However, most studies have been conducted on adult populations, and previous pediatric MRE studies have only demonstrated the feasibility of MRE in pediatric populations, while the actual clinical application of spleen MRE has been limited. AIM: To assess the utility of splenic stiffness measurements by MRE to predict gastroesophageal varices in children. METHODS: We retrospectively reviewed abdominal MRE images taken on a 3T system in pediatric patients. Patients who had undergone Kasai operations for biliary atresia were selected for the Kasai group, and patients with normal livers and spleens were selected for the control group. Two-dimensional spin-echo echo-planar MRE acquisition centered on the liver, with a pneumatic driver at 60 Hz and a low amplitude, was performed to obtain hepatic and splenic stiffness values. Laboratory results for aspartate aminotransferase to platelet ratio index (APRI) were evaluated within six months of MRE, and the normalized spleen size ratio was determined with the upper normal size limit. All Kasai group patients underwent gastroesophageal endoscopy during routine follow-up. The Mann-Whitney U test, Kendall's tau b correlation and diagnostic performance analysis using the area under the curve (AUC) were performed for statistical analysis. RESULTS: The median spleen MRE value was 5.5 kPa in the control group (n = 9, age 9-18 years, range 4.7-6.4 kPa) and 8.6 kPa in the Kasai group (n = 22, age 4-18 years, range 5.0-17.8 kPa). In the Kasai group, the APRI, spleen size ratio and spleen MRE values were higher in patients with portal hypertension (n = 11) than in patients without (n = 11) (all P < 0.001) and in patients with gastroesophageal varices (n = 6) than in patients without (n = 16) (all P < 0.05), even though their liver MRE values were not different. The APRI (τ = 0.477, P = 0.007), spleen size ratio (τ = 0.401, P = 0.024) and spleen MRE values (τ = 0.426, P = 0.016) also correlated with varices grades. The AUC in predicting gastroesophageal varices was 0.844 at a cut-off of 0.65 (100% sensitivity and 75% specificity) for the APRI, and 0.844 at a cut-off of 9.9 kPa (83.3% sensitivity and 81.3% specificity) for spleen MRE values. CONCLUSION: At a cut-off of 9.9 kPa, spleen MRE values predicted gastroesophageal varices as well as the APRI and spleen size ratio in biliary atresia patients after the Kasai operation. However, liver MRE values were not useful for this purpose.

A periodic comparison of the survival and prognostic factors of biliary atresia after Kasai portoenterostomy: a single-center study in Korea.

PURPOSE: We explored the chronologic changes in prognostic factors and clinical outcomes of Kasai portoenterostomy (KPE) for biliary atresia (BA). METHODS: Patients undergoing KPE between 1997 and 2016 were analyzed retrospectively. Ninety-two consecutive patients who underwent KPE from 1997 to 2006 (Era 1) were compared with 150 patients who underwent KPE from 2007 to 2016 (Era 2) for clinical outcomes and prognostic factors. RESULTS: The jaundice clearance rate increased by 8.8% (66.7% vs. 75.5% for Eras 1 and 2, respectively, p = 0.180), and the 5-year native liver survival (NLS) rate improved slightly (62.5% vs. 64.0% for Eras 1 and 2, respectively, p = 0.617) in Era 2. The hazard ratio for age at KPE (≥ 90 days) with regard to 5-year NLS and the odds ratio for age at KPE (< 90 days) with regard to jaundice clearance were both lower in Era 2 than in Era 1 (1.95 vs. 2.25 and 2.67 vs. 5.21, respectively). CONCLUSION: The clinical outcomes improved over a period in a single surgeon's practice. We demonstrated that the impact of age at the time of KPE on operative outcomes became less significant over time with the increase in the single surgeon's experience and improvement in medical treatment for BA.

Mitochondrial Mutations in Cholestatic Liver Disease with Biliary Atresia.

Biliary atresia (BA) results in severe bile blockage and is caused by the absence of extrahepatic ducts. Even after successful hepatic portoenterostomy, a considerable number of patients are likely to show progressive deterioration in liver function. Recent studies show that mutations in protein-coding mitochondrial DNA (mtDNA) genes and/or mitochondrial genes in nuclear DNA (nDNA) are associated with hepatocellular dysfunction. This observation led us to investigate whether hepatic dysfunctions in BA is genetically associated with mtDNA mutations. We sequenced the mtDNA protein-coding genes in 14 liver specimens from 14 patients with BA and 5 liver specimens from 5 patients with choledochal cyst using next-generation sequencing. We found 34 common non-synonymous variations in mtDNA protein-coding genes in all patients examined. A systematic 3D structural analysis revealed the presence of several single nucleotide polymorphism-like mutations in critical regions of complexes I to V, that are involved in subunit assembly, proton-pumping activity, and/or supercomplex formation. The parameters of chronic hepatic injury and liver dysfunction in BA patients were also significantly correlated with the extent of hepatic failure, suggesting that the mtDNA mutations may aggravate hepatopathy. Therefore, mitochondrial mutations may underlie the pathological mechanisms associated with BA.

Correlation between gamma-glutamyl transpeptidase activity and outcomes after Kasai portoenterostomy for biliary atresia.

BACKGROUND/PURPOSE: The role of serum gamma-glutamyl transpeptidase (GGT) levels in predicting clinical outcomes after Kasai portoenterostomy (KPE) is unknown. This study analyzed whether postoperative GGT along with the aspartate aminotransferase-to-platelet ratio index (APRi) predicted prognosis of biliary atresia (BA). METHODS: Data were retrospectively reviewed for 169 BA patients categorized into jaundice-free (JF) (total bilirubin <2.0 mg/dL ≤6 months post-KPE) and persistent jaundice (PJ) groups (total bilirubin ≥2.0 mg/dL ≤6 months post-KPE). Serum biochemical markers, including GGT levels, were measured monthly after KPE, and mean GGT levels and APRi were compared between groups. Factors predicting native liver survival (NLS) were determined using a Cox regression analysis. RESULTS: GGT concentrations >550 IU/L at month 5 (hazard ratio: 1.74, P < 0.05), an APRi >0.605 at month 4 (hazard ratio: 3.78, P = 0.001), and being jaundice-free at 6 months (hazard ratio: 5.49, P < 0.001) were independent risk factors for decreased NLS. CONCLUSIONS: Serum GGT concentrations >550 IU/L at month 5 and an APRi >0.605 at month 4 post-KPE were associated with significantly lower NLS rates. Among JF patients, those with GGT concentrations >550 IU/L at month 5 and APRi >0.605 at month 4 showed poorer outcomes. TYPE OF STUDY: Retrospective comparative study LEVEL OF EVIDENCE: Level III.

Laparoscopic Surgical Glue Injection Hernioplasty: A Single-Institution Experience.

PURPOSE: The aim of this study was to provide clinical evidence supporting the safety and effectiveness of laparoscopic surgical glue injection hernioplasty (LSGIH) for indirect inguinal hernia repair in female patients. METHODS: We conducted a prospective study of LSGIH beginning in January 2013. N-butyl-2-cyanoacrylate was used as the surgical glue for closure of the hernia sac. Twenty-eight female patients with a mean age of 51.1 ± 35.3 months (24 days to 10 years) received LSGIH. The total number of LSGIH operations was 42, including bilateral inguinal hernioplasties. RESULTS: Postoperatively, all the patients were discharged from the hospital within 24 hours without incident. The mean operation time for LSGIH was 41.0 ± 9.7 minutes (30-66 minutes). The mean follow-up period was 23.2 ± 8.3 months (14.6-38.2 months) up to now. There were no postoperative complications or recurrences during the follow-up period. CONCLUSIONS: Our prospective study supports LSGIH as a simple, safe, and useful technique for pediatric inguinal hernioplasty. This method required only one working port and one camera port. However, further clinical prospective trials, including male patients, should be performed to confirm the long-term safety and effectiveness of LSGIH.

Choledochal cyst rupture with an intrahepatic pseudocyst mimicking hepatic mesenchymal hamartoma in an infant.

Spontaneous choledochal cyst rupture and consequent biliary peritonitis are rare in children. Here, we report a unique case of intrahepatic bile duct rupture of choledochal cyst type IV that resulted in loculated biliary peritonitis with an intrahepatic pseudocyst mimicking a hepatic mesenchymal hamartoma in a 3-month-old boy. Magnetic resonance cholangiopancreatography can help differentiate these conditions. Regardless of the location, pseudocysts resulting from biliary peritonitis should be considered in cases of choledochal cysts with adjacent fluid collection or cystic lesions.

Retrospective assessment of the validity of robotic surgery in comparison to open surgery for pediatric choledochal cyst.

PURPOSE: We evaluated the validity of robotic surgery (RS) for pediatric choledochal cyst (CC) in comparison to open surgery (OS). MATERIALS AND METHODS: From January 2009 to April 2013, clinical data from 79 consecutive pediatric patients with CC, who underwent RS (n=36) or OS (n=43) performed by a single pediatric surgeon, were analyzed retrospectively. RESULTS: In the RS group, the age of the patients was significantly older, compared to the OS group. Operation and anesthesia times were significantly longer in the RS group than the OS group. Fluid input rates to maintain the same urine output were significantly smaller in the RS group than the OS group. The American Society of Anesthesiologists (ASA) physical status, length of postoperative hospital stay, and the incidence of surgical complications did not differ significantly between the two groups. CONCLUSION: Although early complications could not be avoided during the development of robotic surgical techniques, RS for pediatric CC showed results comparable to those for OS. We believe that RS may be a valid and alternative surgery for pediatric CC. After further development of robotic surgical systems and advancement of surgical techniques therewith, future prospective studies may reveal more positive results.

Magnesium as an adjuvant for caudal analgesia in children.

BACKGROUND: There is a need for an adjuvant agent of caudal block that prolongs its duration and improves the analgesic efficacy to fasten functional recovery. Magnesium is an N-methyl-D-aspartate receptor antagonist that functions as an analgesic. This study was aimed to evaluate whether magnesium as an adjuvant for caudal block in children can improve postoperative analgesia and functional recovery. METHODS: Eighty children, 2-6 years of age, undergoing inguinal herniorrhaphy, were included in this prospective, randomized, double-blinded study. For caudal block, Group R received ropivacaine 1.5 mg·ml(-1), 1 ml·kg(-1) and Group RM received the same dose of ropivacaine mixed with 50 mg of magnesium. The Parents' Postoperative Pain Measure (PPPM) score, analgesic consumption, functional recovery, and adverse effects were evaluated at 6, 24, 48, and 72 h after surgery, as well as daily thereafter until the child showed full functional recovery. RESULTS: The PPPM score after hospital discharge was significantly lower for Group RM than for Group R at all times (P < 0.05). Children in Group RM required less fentanyl for rescue analgesia in the recovery area (16.2% vs 39.5%, P = 0.034) and less oral analgesics after discharge (20.5% vs 52.6%, P = 0.007). The time to return of normal functional activity was shorter in Group RM (P < 0.05). The incidence of adverse effects did not differ between groups. CONCLUSIONS: As an adjuvant for caudal analgesia, 50 mg magnesium provided superior quality of analgesia and faster return of normal functional activity than local anesthetic alone in children.

Relationship between Anomalous Pancreaticobiliary Ductal Union and Pathologic Inflammation of Bile Duct in Choledochal Cyst.

PURPOSE: Choledochal cyst is a cystic dilatation of common bile duct. Although the etiology is presently uncertain, anomalous pancreaticobiliary ductal union (APBDU) is thought to be a major etiology of choledochal cyst. In this study, we analyzed the clinical and anatomical characteristics and pathologies of patients diagnosed with choledochal cyst in a single institute for 25 years. METHODS: A total of 113 patients, diagnosed with choledochal cyst and who received an operation in Severance Children's Hospital from January 1988 to May 2013, were included. Medical records were reviewed, including clinical and demographic data, surgical procedures. Abdominal ultrasonography, magnetic resonance cholangiopancreatography, and intraoperative cholangiography were used as diagnostic tools for evaluation and classification of choledochal cyst and the presence of anomalous pancreaticobiliary ductal union. Todani's classification, and relationship between APBDU and surgical pathology. RESULTS: Among 113 patients, 77 patients (68.1%) presented symptoms such as hepatitis, pancreatitis and/or cholecystitis. Eighty three patients (73.5%) had APBDU, and 94 patients (83.2%) showed inflammatory pathologic changes. APBDU, pathologic inflammation, and serological abnormalities such as hepatitis or pancreatitis showed a statistically significant correlation to one another. CONCLUSION: APBDU is thought to be one of the etiologic factors of choledochal cyst. It is related to the inflammatory changes in bile duct that can lead to the cystic dilatation.