Management and pitfall for patients with portal obstruction after pediatric living donor liver transplantation
The subject of the present study is six children with portal obstruction occurring after living donor liver transplant (LDLT). Disease that required LDLT was biliary atresia in all cases. Five out of six children were transplanted within one year after birth. Only one case showed a clinical symptom...
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| Published in | Japanese Journal of Portal Hypertension Vol. 16; no. 1; pp. 31 - 35 |
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| Main Authors | , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
The Japan Society for Portal Hypertension
2010
日本門脈圧亢進症学会 |
| Subjects | |
| Online Access | Get full text |
| ISSN | 1344-8447 2186-6376 |
| DOI | 10.11423/jsph.16.31 |
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| Abstract | The subject of the present study is six children with portal obstruction occurring after living donor liver transplant (LDLT). Disease that required LDLT was biliary atresia in all cases. Five out of six children were transplanted within one year after birth. Only one case showed a clinical symptom (refractory ascites) before diagnosis was made. Although radiological intervention was tried, it failed in all cases. Three cases developed gastrointestinal hemorrhage. Two of them were treated with splenorenal shunt therapy and recovered. One patient who had additional portopulmonary hypertension received re-transplantation, but she died of right cardiac heart failure during surgery. Other three cases including a child who had symptoms during the pre-diagnostic period were well without any clinical symptoms and liver function abnormalities. In conclusion, children with portal obstruction after LDLT were difficult to treat. Therefore, this complication should be detected with serial examination such as Doppler ultrasound before it occurs. |
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| AbstractList | The subject of the present study is six children with portal obstruction occurring after living donor liver transplant (LDLT). Disease that required LDLT was biliary atresia in all cases. Five out of six children were transplanted within one year after birth. Only one case showed a clinical symptom (refractory ascites) before diagnosis was made. Although radiological intervention was tried, it failed in all cases. Three cases developed gastrointestinal hemorrhage. Two of them were treated with splenorenal shunt therapy and recovered. One patient who had additional portopulmonary hypertension received re-transplantation, but she died of right cardiac heart failure during surgery. Other three cases including a child who had symptoms during the pre-diagnostic period were well without any clinical symptoms and liver function abnormalities. In conclusion, children with portal obstruction after LDLT were difficult to treat. Therefore, this complication should be detected with serial examination such as Doppler ultrasound before it occurs.
当科でフォローしている小児生体肝移植術後門脈閉塞症6例を対象にその現状と問題点について検討した．現疾患は全例胆道閉鎖症で移植時年齢は6例中5例が乳児期であった．診断されたのは移植後平均1年3カ月で，診断前臨床症状としては難治性腹水がみられた1例のみであった．治療はバルーン拡張術が試みられたが全例で不成功に終わった．その後3例で消化管出血を認め，2例で脾腎シャント手術を行い改善がみられたが，1例はportopulmonary hypertension(PPHTN)を合併しており，再移植を行ったが右心不全のため失った．難治性腹水の1例は利尿剤と経過観察で改善し，他の2例は観察期間8年と12年の現在，臨床症状を認めていない．本症は発症前において臨床症状に乏しく，一旦発症すれば難治性で，消化管出血，肺血管合併症をきたすと致死的となるため，定期的に頻回のドップラー超音波検査など早期発見・早期治療が肝要と考えられた． The subject of the present study is six children with portal obstruction occurring after living donor liver transplant (LDLT). Disease that required LDLT was biliary atresia in all cases. Five out of six children were transplanted within one year after birth. Only one case showed a clinical symptom (refractory ascites) before diagnosis was made. Although radiological intervention was tried, it failed in all cases. Three cases developed gastrointestinal hemorrhage. Two of them were treated with splenorenal shunt therapy and recovered. One patient who had additional portopulmonary hypertension received re-transplantation, but she died of right cardiac heart failure during surgery. Other three cases including a child who had symptoms during the pre-diagnostic period were well without any clinical symptoms and liver function abnormalities. In conclusion, children with portal obstruction after LDLT were difficult to treat. Therefore, this complication should be detected with serial examination such as Doppler ultrasound before it occurs. |
| Author | Lee, Kwan-Jong Oya, Yuuki Okajima, Hideaki Inomata, Yukihiro |
| Author_FL | 李 光鐘 岡島 英明 大矢 雄希 猪股 裕紀洋 |
| Author_FL_xml | – sequence: 1 fullname: 岡島 英明 – sequence: 2 fullname: 大矢 雄希 – sequence: 3 fullname: 李 光鐘 – sequence: 4 fullname: 猪股 裕紀洋 |
| Author_xml | – sequence: 1 fullname: Lee, Kwan-Jong organization: Department of Pediatric Surgery/Transplantation, Kumamoto University Hospital – sequence: 1 fullname: Oya, Yuuki organization: Department of Pediatric Surgery/Transplantation, Kumamoto University Hospital – sequence: 1 fullname: Inomata, Yukihiro organization: Department of Pediatric Surgery/Transplantation, Kumamoto University Hospital – sequence: 1 fullname: Okajima, Hideaki organization: Department of Pediatric Surgery/Transplantation, Kumamoto University Hospital |
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| References | 4) Millis JM, Seaman DS, Piper JB, et al: Portal vein thrombosis and stenosis in pediatric liver transplantation. Transplantation 1996; 62: 748-754 5) Funaki B, Rosenblum JD, Leef JA, et al: Portal vein stenosis in children with segmental liver tansplants: treatment with percutaneous transhepatic venoplasty. AJR Am J Roentgenol 1995; 165: 161-165 6) Funaki B, Rosenblum JD, Leef JA, et al: Percutaneous treatment of portal venous stenosis in children and adolescents with segmental hepatic transplants: long-term results. Radiology 2000; 215: 147-151 1) Broniszczak D, Szymczak M, Kamin´ski A, et al: Vascular complication after pediatric liver transplantation from the living donor. Transplant Proc 2006; 38: 1456-1458 2) Ueda M, Egawa H, Ogawa K, et al: Portal vein complica-tions in the long-term course after pediatric living donor liver transplantation. Transplant Proc 2005; 37: 1138-1140 3) Buell JF, Funaki B, Cronin DC, et al: Long-term venous complications after full-size and segmental pediatric liver transplantation. Ann Surg 2002; 236: 658-666 7) Shirouzu Y, Kasahara M, Morioka D, et al: Vascular reconstruction and complication in living donor liver transplantation in infants weighting less than 6 kilograms: The Kyoto experience. Liver Transpl 2005; 12: 1224-1232 8) Kyoden Y, Tamura S, Sugawara Y, et al: Portal vein complications after adult-to-adult living donor liver transplantation. Transplant Int 2008; 21: 1136-1144 9) Uemoto S, InomataY, Egawa H, et al: Effects of hypoxemiaon early postoperative course of liver transplanta-tion in pediatric patients with intrapulmonary shunting. Transplantation 1997; 63: 407-414 |
| References_xml | – reference: 4) Millis JM, Seaman DS, Piper JB, et al: Portal vein thrombosis and stenosis in pediatric liver transplantation. Transplantation 1996; 62: 748-754 – reference: 9) Uemoto S, InomataY, Egawa H, et al: Effects of hypoxemiaon early postoperative course of liver transplanta-tion in pediatric patients with intrapulmonary shunting. Transplantation 1997; 63: 407-414 – reference: 6) Funaki B, Rosenblum JD, Leef JA, et al: Percutaneous treatment of portal venous stenosis in children and adolescents with segmental hepatic transplants: long-term results. Radiology 2000; 215: 147-151 – reference: 8) Kyoden Y, Tamura S, Sugawara Y, et al: Portal vein complications after adult-to-adult living donor liver transplantation. Transplant Int 2008; 21: 1136-1144 – reference: 1) Broniszczak D, Szymczak M, Kamin´ski A, et al: Vascular complication after pediatric liver transplantation from the living donor. Transplant Proc 2006; 38: 1456-1458 – reference: 7) Shirouzu Y, Kasahara M, Morioka D, et al: Vascular reconstruction and complication in living donor liver transplantation in infants weighting less than 6 kilograms: The Kyoto experience. Liver Transpl 2005; 12: 1224-1232 – reference: 2) Ueda M, Egawa H, Ogawa K, et al: Portal vein complica-tions in the long-term course after pediatric living donor liver transplantation. Transplant Proc 2005; 37: 1138-1140 – reference: 3) Buell JF, Funaki B, Cronin DC, et al: Long-term venous complications after full-size and segmental pediatric liver transplantation. Ann Surg 2002; 236: 658-666 – reference: 5) Funaki B, Rosenblum JD, Leef JA, et al: Portal vein stenosis in children with segmental liver tansplants: treatment with percutaneous transhepatic venoplasty. AJR Am J Roentgenol 1995; 165: 161-165 |
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| Title | Management and pitfall for patients with portal obstruction after pediatric living donor liver transplantation |
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