Replacing the retrohepatic inferior vena cava (IVC) following concurrent resection of the IVC and tumor-involved liver is an emerging surgical technique in living donor liver transplantation (LDLT), particularly for managing hepatocellular carcinomas near the IVC. This approach makes the extent of hepatectomy in LDLT comparable to that of deceased donor liver transplantation. We report a pediatric LDLT case involving IVC and portal vein (PV) replacement with an iliac vein homograft to treat advanced hepatoblastomas. The patient was a 24-month-old girl weighing 9.1 kg, presenting with large, multiple hepatoblastomas infiltrating the retrohepatic IVC and PV. After a 2-week waiting period, a cold-stored iliac vein graft was obtained, and the transplantation was performed using a left lateral section graft from her father.A 4-cm iliac vein homograft was anastomosed between the suprahepatic and suprarenal IVC stumps. The liver graft was implanted following standard procedures, with the PV reconstructed via a telescope-shaped anastomosis using the vein graft interposition. The patient experienced an uncomplicated recovery and is currently receiving scheduled adjuvant chemotherapy. In conclusion, IVC and PV replacement with vein homografts represents a feasible and effective surgical strategy for pediatric LDLT patients with advanced liver malignancies involving vascular invasion.

Purpose: In the past decade, advancements in robotic surgery have significantly expanded its application into diverse fields, including urological, gastrointestinal, hepatobiliary, and gynecological procedures. However, comparative outcome data between robotic and laparoscopic cholecystectomy (LC) in pediatric patients are scarce. Therefore, this study aims to evaluate the clinical utility of robotic cholecystectomy (RC) by comparing its outcomes with those of LC. Methods: A retrospective study was conducted using patient records from a single institution involving individuals who underwent RC or LC. Patients who had undergone open cholecystectomy or previous open abdominal surgeries were excluded. Matching criteria included operative age, body mass index, and total bilirubin levels. Baseline and outcome variables were compared using appropriate statistical tests to assess significance. Results: Groups were well-matched for demographic variables. Regression-adjusted analysis showed no significant difference in operative time between RC and LC and hospital stay length (P > 0.05). Complication rates were higher in the RC group (25.0% vs. 3.4%, P = 0.040) and analgesic use was significantly higher in the RC group (adjusted odds ratio, ∞; P < 0.001), as all RC patients received postoperative analgesics. Conclusion The baseline characteristics between the 2 groups were well-matched. While most outcomes showed no statistically significant differences, the RC group had significantly higher postoperative analgesic use and complication rates. These findings highlight the need for careful patient selection and further studies to evaluate the safety profile of RC in pediatric patients.

In children with biliary atresia, recurrent episodes of cholangitis often lead to portal vein (PV) phlebosclerosis. We report a case of pediatric living donor liver transplantation (LDLT) for biliary atresia with PV occlusion and a large splenorenal shunt. A 3-year-5-month-old girl was diagnosed with syndromic biliary atresia and polysplenia. Because the patient’s condition progressively deteriorated with worsening jaundice, we opted to perform LDLT using a left lateral section graft from her father. The recipient’s native PV was severely atrophic and completely occluded, and the collateral veins around the hepatoduodenal ligament were too small to serve as a viable source of portal inflow.Consequently, renoportal anastomosis (RPA) was selected as an alternative approach.Iliac vein interposition was performed to provide a new portal inflow source, and anatomy-compliant PV reconstruction was performed. The patient recovered from transplant surgery, but PV conduit stenosis occurred with the development of ascites 2 months posttransplant. This RPA-related vascular complication was resolved through percutaneous balloon angioplasty of the PV conduit. The patient has been doing well for 6 months posttransplantation. This case demonstrates that RPA can represent a viable reconstructive option for PV inflow in liver transplantation for pediatric patients with phlebosclerotic PV and prominent splenorenal shunt.

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: Sirolimus has emerged as a safe and effective treatment for complicated lymphatic malformations (LMs). We aim to prove the effectiveness and safety of sirolimus as a therapeutic option for patients with complicated LMs. Methods: Fifty-eight patients with complicated LMs treated with sirolimus for at least 6 months at multicenter between July 2018 and January 2023 were enrolled. All patients were administered oral sirolimus starting at 0.8 mg/m 2 every 12 hours, with target serum concentration levels of 8–15 ng/mL. Evaluation for clinical symptoms and LMs volume on MRI were reviewed to assess treatment response and toxicities. Evaluation of disease response was divided into 3 values:complete response, partial response (significant, moderate, and modest), and progressive disease. Results: The median age at the initiation of sirolimus treatment was 6.0 years (range, 1 month–26.7 years). The median duration of treatment was 2.0 years (range, 6 months–4.4 years). The most common lesions were head and neck (25 of 58, 43.1%). Forty-six patients (79.3%) demonstrated a reduction in LMs volume on MRI or improvement of clinical symptoms including 2 complete responses. The young age group and the patients who underwent few prior therapies showed better responses. None of the patients had toxicities attributable to sirolimus with a Common Terminology Criteria for Adverse Events grade of ≥3. Conclusion Oral sirolimus treatment brought a successful outcome without severe adverse effects. It could be the firstline therapy, especially for the young age group of complicated LMs, and an additional option for refractory lesions that did not respond to conventional treatment.

Objective: This study aimed to develop and validate a comprehensive self-report questionnaire to assess emotional and behavioral problems and psychological trauma in maltreated children. Methods: The Mental Health Scale for Maltreated Children (MHS-MC) was constructed to encompass five major symptoms (depression, anxiety, inattention/hyperactivity/impulsivity, aggression/defiance, and psychological trauma) prevalent in maltreated children. Critical items and ego-resilience subscale were also devised to increase clinical utility. After informed consent, 205 children (maltreated children, n=157, 76.6%) were recruited nationwide, and they answered a package of self-report measures, including the MHS-MC. Reliability, construct validity, concurrent validity, and criterion-related validity were examined to explore the psychometric properties. Results: The reliability was good to excellent. Confirmatory factor analysis yielded a five-factorial solution for the symptom subscales supporting construct validity. In logistic regression, the total scores of the MHS-MC predicted membership in the maltreated group. Criterion-related validity was generally satisfactory in that all subscales of the MHS-MC showed significant correlations with relevant measures in the expected direction. Conclusion This is the first attempt to develop a comprehensive psychological scale based on nationwide data collected from maltreated Korean children. We hope that the continued standardization of this scale will contribute to evidence-based clinical and policy decisionmaking for maltreated children.

Purpose: Intrahepatic biliary cysts (IBCs) after Kasai portoenterostomy (KPE) are associated with intractable recurrent cholangitis. This study aimed to investigate the feasibility of its use as well as indication for surgical management of IBCs in pediatric patients. Methods: We retrospectively reviewed the medical records and imaging studies of patients who underwent KPE for biliary atresia from 2010 to 2020. Results: An imaging study identified IBCs in 28 of 129 patients who underwent KPE with biliary atresia (21.7%). Among them, 5 patients were subjected to surgical treatment for intractable cholangitis. The median time from KPE to the development of IBCs was 1.7 years. Four out of 5 patients had IBCs confined to the left lateral lobe, and in one patient, the IBCs were in the hepatic hilum. All 5 patients experienced more than one cholangitis. Although they received intravenous antibiotic treatment and percutaneous transhepatic cholangiodrainage as treatment, they were intractable. Three patients underwent hepatectomy, and 2 underwent cystojejunostomy. There was no recurrence of cholangitis during the median follow-up period of 2.9 years. Conclusion Surgical treatment for IBCs after KPE could be considered a safe and effective surgical procedure for children if appropriate indications are applied.