Background: In the era of nucleoside analogs (NA), we investigated the safety of using hepatitis B surface antigen (HBsAg)-positive grafts in liver transplantation (LT) using nationwide KOTRY data. Methods: Among 4,265 adult LTs in the KOTRY registry (April 2014–January 2020), 20 (0.5%) used HBsAg(+) grafts. The S(+) group was compared with HBsAg-nega-tive groups, both HBcAb(+) (C[+]) and HBcAb(−) (SC[−]), using 1:1 propensity scorematching. Patient and graft survival were evaluated using Kaplan–Meier analysis.Cox regression was used to identify prognostic factors. Results: No significant differences were observed in patient or graft survival be-tween S(+) and C(+) or SC(−) groups. Key prognostic factors for patient survivalincluded age, HCC, MELD score, ascites, and encephalopathy. For graft survival, HCC, preoperative HCC treatment, MELD score, ascites, and encephalopathy were significant. HBV recurrence occurred in the S(+) group, but did not compromise outcomes. Conclusion In HBV-endemic regions, HBsAg(+) liver grafts can be safely used to expand the donor pool without compromising LT outcomes when combined with appropriate prophylaxis.

Purpose: Malignant hepatocellular neoplasm, not otherwise specified (HCN-NOS) is a provisional diagnostic entity, characterised by intermediate or a combination of hepatoblastoma and pediatric hepatocellular carcinoma (p-HCC) features. We compared the characteristics of HCN-NOS with hepatoblastoma and p-HCC. Materials and Methods: The records of 148 pediatric patients diagnosed with hepatocellular malignancy after resection were retrieved from the institutional database. Clinical parameters and histopathology slides were reviewed to re-establish each patient’s diagnosis. Molecular analyses were conducted in 37 patients. Results: Patients were profiled as 21 (14.2%) with HCN-NOS, 109 (73.6%) with hepatoblastoma, and 18 (12.2%) with p-HCC. The median age was 8.6 years in HCN-NOS, 1.2 years in hepatoblastoma, and 7.9 years in p-HCC. Background liver disease was frequently observed in p-HCC (11/18, 61%) but infrequent in HCN-NOS (4/21, 19%) and hepatoblastoma (4/109, 3.7%). HCN-NOS presented with a more advanced PRETEXT stage (p=0.012), metastasis (p < 0.001), and lymphovascular invasion (p < 0.001) than hepatoblastoma and p-HCC. Patients with HCN-NOS received longer cycles of preoperative chemotherapy; however, they reported a lower decrease in serum alpha-fetoprotein and tumor size than hepatoblastoma (p=0.043, p=0.004, and p=0.044, respectively). HCN-NOS was an independent poor prognostic factor for event-free survival (hazard ratio, 4.968; 95% confidence interval, 2.004 to 12.32; p < 0.001). Conclusion The possibility of HCN-NOS should be considered in pediatric patients with liver cancer, especially those ≥ 5 years old with no background liver disease. Because HCN-NOS exhibits poor chemoresponsiveness and unfavourable postoperative prognosis, liver transplantation should be strongly considered.

Purpose: The purpose of this study was to determine the characteristics of patients with failed initial Kasai portoenterostomy (KP) and to compare the long-term prognosis of redo-KP with that of liver transplantation (LT) in these patients. Methods: The medical records of patients with biliary atresia (BA) who failed initial KP from 2010 to 2021 at a single center were retrospectively analyzed. KP failure was defined as persistent jaundice (total bilirubin concentration, ≥2.0 mg/dL) after KP or the performance of LT. Results: During the study period, 32 patients experienced initial KP failure, with 10 undergoing redo-KP and 22 undergoing LT. Redo-KP was successful in a minority of patients with failed initial KP, but the complications, particularly cholangitis, were more frequent in the redo-KP group. The long-term prognosis of redo-KP compared to LT showed that while some patients benefited from native liver survival after redo-KP, LT remains the more definitive solution for sustained liver function and survival in patients with BA. Conclusion The only factor differing significantly between patients who underwent redo-KP and LT after failed initial KP was complications of cholangitis. Redo-KP was successful in 4 of 10 patients with failed initial KP, suggesting that redo-KP may be a treatment option in patients with BA and failed initial KP.

Purpose: The purpose of this study was to determine the characteristics of patients with failed initial Kasai portoenterostomy (KP) and to compare the long-term prognosis of redo-KP with that of liver transplantation (LT) in these patients. Methods: The medical records of patients with biliary atresia (BA) who failed initial KP from 2010 to 2021 at a single center were retrospectively analyzed. KP failure was defined as persistent jaundice (total bilirubin concentration, ≥2.0 mg/dL) after KP or the performance of LT. Results: During the study period, 32 patients experienced initial KP failure, with 10 undergoing redo-KP and 22 undergoing LT. Redo-KP was successful in a minority of patients with failed initial KP, but the complications, particularly cholangitis, were more frequent in the redo-KP group. The long-term prognosis of redo-KP compared to LT showed that while some patients benefited from native liver survival after redo-KP, LT remains the more definitive solution for sustained liver function and survival in patients with BA. Conclusion The only factor differing significantly between patients who underwent redo-KP and LT after failed initial KP was complications of cholangitis. Redo-KP was successful in 4 of 10 patients with failed initial KP, suggesting that redo-KP may be a treatment option in patients with BA and failed initial KP.

Purpose: The purpose of this study was to determine the characteristics of patients with failed initial Kasai portoenterostomy (KP) and to compare the long-term prognosis of redo-KP with that of liver transplantation (LT) in these patients. Methods: The medical records of patients with biliary atresia (BA) who failed initial KP from 2010 to 2021 at a single center were retrospectively analyzed. KP failure was defined as persistent jaundice (total bilirubin concentration, ≥2.0 mg/dL) after KP or the performance of LT. Results: During the study period, 32 patients experienced initial KP failure, with 10 undergoing redo-KP and 22 undergoing LT. Redo-KP was successful in a minority of patients with failed initial KP, but the complications, particularly cholangitis, were more frequent in the redo-KP group. The long-term prognosis of redo-KP compared to LT showed that while some patients benefited from native liver survival after redo-KP, LT remains the more definitive solution for sustained liver function and survival in patients with BA. Conclusion The only factor differing significantly between patients who underwent redo-KP and LT after failed initial KP was complications of cholangitis. Redo-KP was successful in 4 of 10 patients with failed initial KP, suggesting that redo-KP may be a treatment option in patients with BA and failed initial KP.

Replacement of the retrohepatic inferior vena cava (IVC) after concurrent resection of IVC and tumor-bearing liver is regarded as a feasible living donor liver transplantation (LDLT) technique to cope with tumors around the IVC. This method can make the resection extent of LDLT comparable to that of deceased-donor liver transplantation. We present one pediatric LDLT case with IVC replacement using an enlarged iliac vein conduit to treat advanced hepatoblastoma. The patient was a 33-monthold and 12 kg-weighing girl suffering from large multiple hepatoblastomas invading the retrohepatic IVC. At 2-month waiting after deciding LDLT, we obtained a coldstored iliac vein graft and LDLT was performed with the father’s left lateral section graft. A 1.3 cm-wide and 10 cm-long iliac vein was transformed to be a 2 cm-wide and 5 cm-long vein graft through a double-barrel unification venoplasty. The left lateral section graft was implanted along the standard procedure of LDLT. The patient recovered uneventfully and is undergoing scheduled adjuvant chemotherapy. IVC replacement with vein homograft is a feasible option for LDLT in pediatric patients with advanced liver malignancy.

Purpose: The International Society for the Study of Vascular Anomalies (ISSVA) classification is crucial in diagnosing vascular anomalies (VAs), surpassing the International Classification of Diseases 10th Revision. This study aims to reevaluate diagnoses using ISSVA criteria and explore diagnostic patterns. Methods: Analyzing 138 pediatric VA patients diagnosed via magnetic resonance imaging from 2018 to 2023 at Asan Medical Center, we reviewed clinical, imaging, pathology, and genetic data. Diagnoses were revised per 2018 ISSVA criteria, assessing discrepancies. Results: Among 133 VA cases, 125 were malformations and eight were tumors, mostly in the head and neck. Clinical and imaging diagnoses disagreed in 51 cases. Some initially complex malformations were simplified. Lymphatic malformation cases shifted to VAs and vascular tumors were identified post-initial diagnosis. Conclusion Accurate diagnosis of VAs is essential for prognosis, treatment planning, and predicting outcomes. However, 14.2% of patients showed discordance between clinical diagnoses and imaging findings. Capillary malformations were often overlooked in imaging but became evident with relevant clinical findings. Adopting a multidisciplinary approach and a unified diagnosis based on ISSVA classification is crucial for clearly defining VAs.

Liver transplantation is accepted as an effective therapeutic option for unresectable hepatoblastoma. We present a pediatric case of hepatoblastoma patient with portal vein (PV) tumor thrombosis (PVTT) that occluded the main PV completely despite aggressive neoadjuvant chemotherapy. The patient was a 4-year and 2-month-old 17-kg boy suffering from hepatoblastoma with PVTT, which was partially regressed by neoadjuvant chemotherapy. Viable PVTT remained after right trisectionectomy and repetition of systemic chemotherapy. To remove the tumor completely, we performed living donor liver transplantation (LDLT) using his father’s left liver graft.The native PV was completely occluded with PVTT and abundant pericholedochal collaterals developed. The native PV was meticulously dissected to fully expose the superior mesenteric vein-splenic vein confluence. This confluence portion was deeply clamped and opened to use as a viable PV inflow source. A cold-stored fresh external iliac vein homograft was anastomosed to the confluence portion as an end-to-end fashion and passed through the native retropancreatic PV tract after mechanical dilatation of the tract. Left liver graft implantation was performed using standard procedures of pediatric LDLT. The patient recovered uneventfully. He will undergo scheduled adjuvant chemotherapy. Customized design for PV reconstruction using interposition vein graft with viable PV inflow source can result in successful PV reconstruction with complete removal of PVTT.

Purpose: Hiatal hernia in children is a rare condition, and there is limited knowledge available about the disease itself. There is currently no agreement or consensus on the treatment of hiatal hernia in the pediatric population due to lack of evidence. In this study, we were to assess our experience with hiatal hernia, including the characteristics of our patients, surgical outcomes, and factors that influence the outcomes. Methods: We retrospectively reviewed the medical records of 49 patients below the age of 18 years who underwent hiatal hernia repair at the Asan Medical Center between 2006 and 2021. We analyzed and compared the general characteristics and surgical outcomes based on the presence of recurrence and coexisting congenital diseases. Results: Hiatal hernia progression was found to be associated with various medical conditions; however, no significant differences in patient characteristics or surgical outcomes between those with and without comorbidities were observed.There were no significant differences in patient characteristics or outcomes between the initial and redo operations. Fundoplication was performed in 19 patients (36.7%) during the initial operation and in 7 patients (87.5%) during repeat hiatal hernia repair. Conclusion The presence of an underlying disease didn’t influence the treatment and progression of hiatal hernia. Furthermore, there were no significant differences in the clinical course between patients with recurrent hiatal hernia and those experiencing it for the first time. Additionally, the impact of fundoplication on the recurrence of hiatal hernia in pediatric patients was found to be minimal.

Purpose: Liver grafts from donors with HBV infection contributed to expanding the donor pool under the hepatitis B immunoglobulin and antiviral agents (nucleos(t)ide analogues) in the HBV-endemic area. We report long-term outcomes of liver transplantations (LTs) using grafts from donors with active or chronic HBV infection. Methods: Overall, 2,260 LTs performed in 3 major hospitals in Seoul from January 2000 to April 2019 were assessed for inclusion. Twenty-six grafts (1.2%) were obtained from HBsAg (+), HBeAb (+), or HBcAb (+) donors, and recipient outcomes were retrospectively reviewed. Donor and recipient demographics and transplantation outcomes were analyzed. Results: Sixteen deceased donor LTs were performed using active HBsAg (+) grafts. Ten other LTs were sourced from 10 living donors. There was no significant difference in survival in patients who received deceased donor LTs compared with that in those who underwent LT with non–hepatitis virus-infected grafts. Fourteen patients who were followed up for >5 years were stable, and no difference in hepatocellular carcinoma recurrence rate was observed 5 years after transplantation between transplants from donors with and those without HBV. Conclusion Considering long-term outcomes, liver grafts from donors with active HBV replication can be safely used for LT.