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남궁정만,황신,박길천,권현희,Suhyeon Ha,Oh Seak Hee,Kim Kyung Mo 대한이식학회 2022 Korean Journal of Transplantation Vol.36 No.2
Biliary rhabdomyosarcoma is a rare tumor, but it is still the most common tumor of the biliary tract in children. We report a case of a 6-year-old boy with biliary embryonal rhabdomyosarcoma and liver metastasis, which were treated with neoadjuvant and adjuvant chemotherapy combined with living donor liver transplantation (LDLT). Ini- tial imaging studies showed a low-attenuation intraductal mass from the left hepatic duct to the intrapancreatic common bile duct with diffuse upstream dilatation of the intrahepatic duct and liver metastasis. Endoscopic biopsy revealed embryonal rhab- domyosarcoma. After tumor size reduction through neoadjuvant chemotherapy, LDLT was planned to remove the tumor completely. A left lateral section graft weighing 330 g was harvested from his 38-year-old mother and the graft-to-recipient weight ratio was 1.94%. Routine pediatric LDLT operation was performed with deep excavation of intra- pancreatic distal bile duct. The explant liver showed minimal residual embryonal rhab- domyosarcoma with no lymph node metastasis. The patient recovered uneventfully from LDLT operation. Scheduled adjuvant chemotherapy was performed for 6 months. The patient is doing well without any evidence of tumor recurrence for 26 months after LDLT. In conclusion, liver transplantation could be an effective treatment for unresect- able biliary rhabdomyosarcoma in children according to the location of tumor.
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문덕복,황신,정동환,안철수,박길천,하태용,송기원,윤영인,이성규 대한이식학회 2021 Korean Journal of Transplantation Vol.35 No.3
We have preferentially used the right gastroepiploic artery (RGEA) as an alternative for the recipient hepatic artery (HA) inflow during living donor liver transplantation (LDLT), but it was not always available. We herein present a case of adult LDLT with HA recon- struction using a greater saphenous vein (GSV) conduit because of the absence of the RGEA due to prior subtotal gastrectomy. A 55-year-old male patient diagnosed with hepatitis B virus-associated liver cirrhosis and secondary biliary cirrhosis underwent LDLT using a modified right liver graft. The upper abdominal cavity was heavily adhered due to prior abdominal surgeries, thus we had to sacrifice the common bile duct and the right HA completely. A 6-cm-long GSV segment was harvested from the left ankle and interposed between the recipient gastroduodenal artery and the graft HA. The patient recovered from LDLT and HA complications did not occur. However, 8 years after LDLT, chronic rejection occurred, thus repeated deceased donor liver transplantation was per- formed. This patient has been doing well for 2 years after retransplantation. In conclu- sion, we suggest that interposition of an autologous GSV conduit can be an alternative for establishing HA inflow in LDLT when other inflow source is not available.
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Use of minor donors for living donor liver transplantation and associated ethical issues
황신,송기원,정동환,하태용,박길천,안철수,문덕복,이성규 대한이식학회 2021 Korean Journal of Transplantation Vol.35 No.3
Background: Living liver donation by minors is regarded as justifiable only if minors pos- sess the capacity to consent to donation and the procedure is in their best interests. This study analyzed the incidence of and reasons for living donor liver transplantation (LDLT) by minor donors in Korea, and discussed ethical issues regarding liver donation by mi- nors. Methods: The databases of the Korean Network for Organ Sharing (KONOS) and Asan Medical Center (AMC) from 2010 to 2019 were retrospectively reviewed to determine the incidence of LDLT by minor donors. Results: From 2010 to 2019, 590 (4.1%) of 14,243 liver donors in the KONOS database and 276 (7.5%) of 3,401 liver donors in the AMC database were minors. The proportions of minor donors in the KONOS and AMC databases were highest in 2012, at 4.1% and 12.6%, respectively, and lowest in 2019, at 1.1% and 3.0%, respectively. Because most LDLT re- cipients had relatively low model for end-stage liver disease scores and hepatocellular carcinoma, they were unlikely candidates for deceased-donor liver transplantation and were highly likely to drop out of LDLT if they waited for 1–2 years. The donor-recipient relationship of minor donors in the AMC database was first-degree in 256 (92.8%) and second- or third-degree in 20 (7.2%). Conclusions: Liver donation by minors is limitedly acceptable only when the minor proves informed, well‐considered, and autonomous consent to the procedure and the procedure is in the minor's best interests. We suggest that minors be allowed to donate only to first-degree family members.
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Indications and outcomes of liver transplantation for post-Kasai biliary atresia in young adults
남궁정만,황신,안철수,문덕복,하태용,송기원,정동환,박길천,윤영인,김경모,이성규 대한이식학회 2021 Korean Journal of Transplantation Vol.35 No.3
Background: Some young adults have a long survival period with native liver after Kasai portoenterostomy (KPE) for biliary atresia (BA). However, a considerable proportion of these patients require liver transplantation (LT). This study aimed to analyze the indica- tions and outcomes of LT in young adults after a long survival period with native liver after KPE. Methods: We selected seven patients who were 18 years or older at the time of LT out of 116 BA patients who underwent primary LT from 2008 to 2019 at Asan Medical Center. Results: The mean ages at KPE and LT were 2.1±0.9 months and 22.0±5.1 years, respec- tively. Mean serum total bilirubin level and model for end-stage liver disease score at LT were 7.91±7.22 mg/dL and 15.3±6.0, respectively. The main reasons for LT were liver cirrhosis with portal hypertension-associated complications in five patients and intracta- ble cholangitis in two patients. There were five cases of living donor LT and two cases of deceased donor LT. All the seven patients are currently alive during the mean follow-up period of 74.7±40.9 months. One patient suffered from outflow graft vein obstruction re- quiring endovascular stenting. Another patient showed core antibody-positivity-induced de novo hepatitis B virus infection, which was well managed with antiviral therapy. Conclusions: Young adult patients with BA are a unique group of patients requiring spe- cialist care regarding transition from pediatric to adult services. The outcomes of LT in young adult BA patients were excellent. Therefore, LT should be considered in patients showing serious BA-associated complications.
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