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.

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 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.

Purpose: Biliary atresia (BA) is a rare but progressive cholangiopathy and the leading cause of pediatric liver transplantation worldwide. The Korean Association of Pediatric Surgeons (KAPS) has conducted three national surveys (2001, 2011, and 2023) to assess long-term trends in the diagnosis, treatment, and outcomes of BA. This study provides a comparative analysis of the 2nd and 3rd national surveys, with reference to selected findings from the 1st survey. Methods: This study included 453 patients from the 3rd national survey (2011–2021) and 435 patients from the 2nd survey (2001–2010), all of whom underwent Kasai portoenterostomy. Data were collected via electronic case report forms from pediatric surgical centers nationwide. Comparisons were made regarding demographics, clinical features, diagnostic patterns, operative details, follow-up outcomes, and survival. Kaplan–Meier analysis was used to evaluate long-term survival. Results: The mean number of BA patients per year remained stable between surveys (43.5 in the 2nd, 41.18 in the 3rd), though centralization of care increased, with 61.5% of cases managed by two major institutions in the 3rd survey. The median age at surgery decreased, and the use of preoperative imaging (especially magnetic resonance cholangiopancreatography) increased. The 10-year native liver survival rate declined from 59.8% to 53.7%, while overall 10-year survival improved slightly (92.9% to 93.2%). Postoperative complications, such as cholangitis and liver failure, persisted but were better categorized. The 3rd survey also reported improved mortality (4.9%) and reduced follow-up loss (11.5%) compared to the 2nd survey. Conclusion While overall survival after Kasai operation has remained high and even improved, native liver survival has slightly declined. The findings reflect earlier diagnosis, more consistent diagnostic imaging, and increasing centralization of care. These trends underscore the importance of long-term nationwide data collection in guiding future strategies for BA management in Korea.

Purpose: This study aims to investigate and compare the incidence, demographic characteristics, clinical manifestations, preoperative diagnostic methods, anatomical classifications, associated anomalies, operative treatments, and postoperative outcomes of patients with intestinal atresia treated by the members of the Korean Association of Pediatric Surgeons (KAPS) through three nationwide surveys. Methods: KAPS conducted 3 national surveys in 1998, 2010, and 2024 to examine the patients diagnosed with intestinal atresia. In preparation for the survey, we developed a customized case registration form to obtain data on patient sex, birth weight, gestational age, clinical manifestations, preoperative diagnostic methods, anatomical types, associated anomalies, operative treatments, and postoperative outcomes. Authorized KAPS members completed the case registration form. Results: The first, second, and third national surveys included 218, 222, and 236 individuals diagnosed with intestinal atresia, respectively. The male-to-female ratios were 1.5:1, 1.1:1, and 1.1:1, respectively. The first, second, and third national surveys revealed that 34.3%, 43.3%, and 53.4% of patients were born before 37 weeks of gestation, respectively. Additionally, 28.7%, 32.0%, and 40.7% of patients had a birth weight under 2,500 g. In the third national survey, duodenoduodenostomy was the most common procedure, performed in 70 out of 82 patients diagnosed with duodenal atresia. Resection and anastomosis were the main surgical procedures conducted in 47 out of 54 cases of jejunal atresia and 74 out of 92 cases of ileal atresia. The mortality rates in the first, second, and third national surveys were 13.8%, 3.6%, and 1.3% respectively, with the lowest rate observed in the third national survey. Conclusion These national surveys offer valuable insights into the current state of intestinal atresia, including specific surgical interventions and postoperative outcomes in South Korea. For pediatric surgeons aiming to enhance their understanding of intestinal atresia and its treatment options, these surveys could be an indispensable resource and guide.

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.