Nagao Yoshihiro, Toshida Katsuya, Morinaga Akinari, Tomiyama Takahiro, Kosai Yukiko, Shimagaki Tomonari, Tomino Takahiro, Wang Huanlin, Kurihara Takeshi, Toshima Takeo, Morita Kazutoyo, Itoh Shinji, Harada Noboru, Yoshizumi Tomoharu
Department of Advanced Medical Initiatives, Kyushu University Hospital, Fukuoka, Japan.
Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-Ku, Fukuoka, 812-8582, Japan.
Surg Case Rep. 2022 Oct 6;8(1):190. doi: 10.1186/s40792-022-01533-3.
Congenital portosystemic shunt is an infrequent abnormal connection between the portal vascular system and the systemic circulation. Portosystemic shunts are common findings in patients with cirrhosis, causing gastroesophageal varices, hepatic encephalopathy, and others. However, there is no consensus or literature describing how to manage asymptomatic patients with portosystemic shunts and normal liver.
The patient was a 39-year-old female who underwent donor right hepatectomy for living donor liver transplantation. The patient was healthy by nature, however, developed hepatic encephalopathy after the surgery due to a development of portosystemic shunt. Portosystemic shunt stole portal blood flow, and imaging modalities revealed narrowing of the portal trunk, representing prolonged depletion of portal blood flow. Balloon-occluded retrograde transvenous obliteration (B-RTO) was performed for occlusion of the portosystemic shunt. B-RTO increased portal blood flow, and hepatic encephalopathy with hyperammonemia was successfully resolved without the outbreak of any other symptom of portal hypertension.
A congenital portosystemic shunt itself is not a contraindication for donor hepatectomy, but perioperative endovascular shunts occlusion or intraoperative ligature of these shunts should be considered.
先天性门体分流是门静脉系统与体循环之间罕见的异常连接。门体分流在肝硬化患者中很常见，可导致胃食管静脉曲张、肝性脑病等。然而，对于如何处理无症状的门体分流且肝脏正常的患者，尚无共识或相关文献报道。
该患者为一名39岁女性，接受了活体供肝肝移植的供体右半肝切除术。患者平素健康，但术后因发生门体分流而出现肝性脑病。门体分流使门静脉血流减少，影像学检查显示门静脉主干变窄，提示门静脉血流长期减少。采用球囊闭塞逆行静脉栓塞术（B-RTO）闭塞门体分流。B-RTO增加了门静脉血流，成功解决了伴有高氨血症的肝性脑病，且未出现门静脉高压的任何其他症状。
先天性门体分流本身并非供体肝切除术的禁忌证，但应考虑围手术期血管内分流闭塞或术中结扎这些分流。
