Successful Surgical Ligation Under Intraoperative Portal Vein Pressure Monitoring of a Large Portosystemic Shunt Presenting as an Intrapulmonary Shunt: Report of a Case
Successful Surgical Ligation Under Intraoperative Portal Vein Pressure Monitoring of a Large Portosystemic Shunt Presenting as an Intrapulmonary Shunt: Report of a Case
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DOI：
10.1007/s00595-004-2851-0
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发表时间：
2004-12
期刊：
影响因子：
2.5
通讯作者：
H. Yagi;Y. Takada;Y. Fujimoto;Y. Ogura;K. Kozaki;M. Ueda;Koichi Tanaka
中科院分区：
文献类型：
--
作者：
H. Yagi;Y. Takada;Y. Fujimoto;Y. Ogura;K. Kozaki;M. Ueda;Koichi Tanaka
我们报告一例罕见的静脉导管未闭合并肝内门脉分支萎陷及肺内分流的病例。在术中门静脉压力（PVP）监测下结扎大静脉导管可显著改善肝内门静脉血流。一名3岁男孩因高半乳糖血症在当地医院接受随访，发现有轻度唇紫绀、劳力性呼吸困难、杵状指和轻度肝功能障碍伴转氨酶和氨水平升高。心导管检查显示肺内分流率为33%。腹部超音波及电脑断层显示门静脉与下腔静脉之间有明显的交通。我们进行了剖腹手术，并在PVP监测下成功结扎了PDV。PVP直到术后7天取出导管才增加。患者的肝功能检查结果在2周内恢复正常。他的血清半乳糖水平在饮用牛奶后为0 mg/dl，他在室内空气中的SpO2和劳力性呼吸困难也得到改善。他在手术后18天出院，没有任何并发症。我们建议在PVP监测下结扎PDV可能是一种治疗选择，记住PDV与肝内门脉分支塌陷有关。
We report a rare case of patent ductus venosus (PDV) with collapsed intrahepatic portal branches and an intrapulmonary shunt. Excellent improvement of the intrahepatic portal vein flow was achieved by ligating the large ductus venosus under intraoperative portal vein pressure (PVP) monitoring. A 3-year-old boy being followed up for hypergalactosemia at a local hospital was found to have mild lip cyanosis, exertional dyspnea, clubbed fingers, and mild liver dysfunction with high levels of transaminase and ammonia. Cardiac catheterization indicated an intrapulmonary shunt with a ratio of 33%. Abdominal ultrasonography and computed tomography showed remarkable communication between the portal vein and the inferior vena cava. We performed laparotomy and successfully ligated the PDV under PVP monitoring. The PVP did not increase until the catheter was removed 7 days postoperatively. The patient’s liver function test results returned to normal within 2 weeks. His serum galactose level was 0 mg/dl after drinking milk, and his SpO2in room air and exertional dyspnea also improved. He was discharged 18 days after his operation, without any complications. We propose that ligation of a PDV under PVP monitoring could be a treatment of choice, bearing in mind that PDV is associated with collapsed intrahepatic portal branches.