Sanada Y, Mizuta K, Kawano Y, Egami S, Hayashida M, Wakiya T, Mori M, Hishikawa S, Morishima K, Fujiwara T, Sakuma Y, Hyodo M, Yasuda Y, Kobayashi E, Kawarasaki H
Department of Transplant Surgery, Jichi Medical University, 3311-1, Yakushiji, Shimotsuke City, Tochigi, 329-0498, Japan.
Transplant Proc. 2009 Dec;41(10):4214-9. doi: 10.1016/j.transproceed.2009.08.080.
The congenital absence of the portal vein (CAPV) is a rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. Liver transplantation (OLT) may be indicated for patients with symptomatic CAPV refractory to medical treatment, especially due to hyperammonemia, portosystemic encephalopathy, hepatopulmonary syndrome, or hepatic tumors. Because portal hypertension and collateral circulation do not occur with CAPV, significant splanchnic congestion may occur when the portocaval shunt is totally clamped during portal vein (PV) reconstruction in OLT. This phenomenon results in severe bowel edema and hemodynamic instability, which negatively impact the patient's condition and postoperative recovery. We have successfully reconstructed the PV in living donor liver transplantation (LDLT) using a venous interposition graft, which was anastomosed end-to-side to the portocaval shunt by a partial side-clamp, using a patent round ligament of the liver, which was anastomosed end-to-end to the graft PV with preservation of both the portal and caval blood flows. Owing to the differences in anatomy among patients, at LDLT for CAPV liver transplant surgeons should seek to preserve both portal and caval blood flows.
先天性门静脉缺如（CAPV）是一种罕见的静脉畸形，其中肠系膜静脉血直接引流至体循环。对于药物治疗无效的有症状CAPV患者，尤其是因高氨血症、门体性脑病、肝肺综合征或肝肿瘤的患者，可能需要进行肝移植（OLT）。由于CAPV不会发生门静脉高压和侧支循环，在OLT的门静脉（PV）重建过程中，当门静脉-腔静脉分流完全夹闭时，可能会出现明显的内脏充血。这种现象会导致严重的肠水肿和血流动力学不稳定，对患者的病情和术后恢复产生负面影响。我们在活体肝移植（LDLT）中使用静脉间置移植物成功重建了PV，该移植物通过部分侧钳与门静脉-腔静脉分流进行端侧吻合，利用肝脏的一条开放圆韧带，将其与移植的PV端端吻合，同时保留门静脉和腔静脉血流。由于患者之间存在解剖学差异，在CAPV的LDLT中，肝移植外科医生应设法保留门静脉和腔静脉血流。
