Department of General Surgery, Capital Institute of Pediatrics, Beijing, People's Republic of China; Graduate School of Peking Union Medical College, Beijing, People's Republic of China.
Department of General Surgery, Capital Institute of Pediatrics, Beijing, People's Republic of China; Graduate School of Peking Union Medical College, Beijing, People's Republic of China.
J Vasc Surg Venous Lymphat Disord. 2020 Sep;8(5):756-761. doi: 10.1016/j.jvsv.2019.11.016. Epub 2020 Feb 1.
This study investigated the morphologic changes of portal cavernoma in children with extrahepatic portal venous obstruction and explored the relationship with prognosis.
From February 2008 to October 2017, there were 107 patients with cavernous transformation of the portal vein admitted to our hospital. Rex shunts were performed in 99 cases, Warren shunts in 7 cases, and laparoscopic splenic vessel ligation in 1 case. Intraoperative superior mesenteric venography was used to determine the structure of the portal venous system. According to the morphologic features of the portal vein shown by portal venography, groups were assigned as follows: patients with the cotton form of portal cavernoma; patients with visible collateral veins of portal cavernoma; patients with and without a visible left gastric vein; and patients with and without a clearly visible intrahepatic portal vein. The preoperative and postoperative portal pressure, preoperative incidence of esophageal varices, time at onset, incidence of postoperative rebleeding, preoperative and postoperative size of the spleen, and age at time of operation were compared between these groups.
The preoperative incidence of esophageal varices, time at onset, postoperative size of spleen, and age at time of operation were significantly lower in the group with the cotton form than in those with visible collateral veins (P < .05). There was a significant correlation between the visible left gastric vein and esophageal varices (P = .002). The time at onset, preoperative and postoperative size of the spleen, and age at time of operation were markedly lower in the group with a good visible intrahepatic portal vein than in those without a clearly visible intrahepatic portal vein (P < .05). The visible left gastric vein was notably associated with the performance of a gastroportal shunt (P = .000), and the group with a visible left gastric vein had a higher ratio of children undergoing a gastroportal shunt.
The cotton form, an early-stage manifestation of cavernous transformation of the portal vein, typically occurs in younger children with a shorter time to onset. Children with the cotton form of portal cavernoma typically have a better prognosis after Rex shunt.
本研究旨在探讨肝外门静脉高压症患儿门静脉海绵样变性的形态学变化，并探讨其与预后的关系。
2008 年 2 月至 2017 年 10 月，我院收治 107 例门静脉海绵样变性患者。99 例行雷氏分流术，7 例行 Warren 分流术，1 例行腹腔镜脾血管结扎术。术中肠系膜上静脉造影确定门静脉系统结构。根据门静脉造影显示的门静脉形态特征将患者分为以下几组：门静脉海绵样变性呈棉花状；门静脉海绵样变性可见侧支静脉；可见与不可见左胃静脉；可见与不可见肝内门静脉。比较各组患者术前、术后门静脉压力、术前食管静脉曲张发生率、发病时间、术后再出血发生率、术前、术后脾脏大小及手术年龄。
棉花状组食管静脉曲张发生率、发病时间、术后脾脏大小及手术年龄均明显低于可见侧支静脉组（P<.05）。可见左胃静脉与食管静脉曲张显著相关（P=0.002）。可见良好肝内门静脉组的发病时间、术前、术后脾脏大小及手术年龄均明显低于不可见肝内门静脉组（P<.05）。可见左胃静脉与行胃门分流术显著相关（P=0.000），可见左胃静脉组行胃门分流术的比例较高。
棉花状是门静脉海绵样变性的早期表现，多见于发病时间短、年龄较小的儿童。雷氏分流术治疗门静脉海绵样变性棉花状患儿预后较好。
