| Background and ObjectiveCavernous transformation of portal veinus(CTPV)is a rare disease of portal vein system in children,accounting for about 40% of children portal hypertension.Splenomegaly and hypersplenism are the most common clinical manifestations of this disease.Most of the children were accompanied by esophageal and gastric varices,25%~40% children had upper gastrointestinal bleeding which was difficult to control.Upper gastrointestinal bleeding and severe hypersplenism were the indications of surgical treatment.Unlike the common intrahepatic portal hypertension in adults,most of the children had no progressive liver injury and normal liver function,but the risk of upper gastrointestinal bleeding was much higher than that of adults.At present,there is no consensus on the treatment of cavernous transformation of portal vein at home and abroad.Generally speaking,the first is to prevent upper gastrointestinal hemorrhage,and the second is to treat and reduce the risk of repeated and repeated bleeding.However,there is no better treatment for the formed vascular malformations.Drugs,interventions,and surgical procedures can be used to prevent or treat bleeding.Surgical procedures include devascularization,shunt,combined surgery and liver transplantation,of which shunt is the most commonly used method.This study analyzed and reviewed the history,physical signs,auxiliary examination,treatment process and follow-up of 40 cases of children,summarized and analyzed the experience of diagnosis and treatment and discussed the choice of treatment plan.Materials and methods MaterialsA total of 40 children with cavernous transformation of portal vein admitted in our hospital from January,2012 to January,2017 were selected,including 21 males and 19 females,aged from 1 to 16 years(mean(5.4±3.4)years).The first symptom of 30 cases was upper gastrointestinal hemorrhage: hematemesis and hematochezia.Initial symptoms of 2 cases had double lower extremity purpura,and 5 cases were admitted to hospital with splenomegaly and were diagnosed by further examination.The first symptom of 1 case was thrombocytopenia.The other 2 cases had no obvious symptoms of splenomegaly and hypersplenism.The age of the initial symptom was 1 to 16 years,with an average of(4.4±3.1)years.The neonatal septicemia,umbilical cord infection and the history of blood exchange through umbilical vein intubation were not followed up.MethodsThe clinical data of 40 children with cavernous transformation of portal vein from January 2012 to January 2017 in the Department of Pediatric surgery,the first affiliated Hospital of Zhengzhou University were collected and analyzed retrospectively,including medical history,physical examination,laboratory examination(blood routine examination).Liver and kidney function,electrolytes,hemagglutination and infectious disease screening,imaging examination(liver,pancreas,spleen and kidney ultrasound,portal vein ultrasound,enhanced CT on the upper abdomen,portal vein angiography)、the disease characteristics,surgical methods,postoperative gastrointestinal hemorrhage,hepatic encephalopathy and so on.The children were followed up by telephone or outpatient to the death or December 2017.The main contents of the follow-up were the survival of the children,whether the esophagogastric fundus vein bleeding again after operation,the times of repeated bleeding after operation and the treatment after bleeding.The data were analyzed by SPSS21.0,Fisher exact probability method and chi-square test were used to test the difference of different indexes(P < 0.05).Result1.14 children received conservative treatment: 2 cases received endoscopic therapy,1 cases received interventional therapy,1 cases died of hemorrhagic shock,and the rest received conservative treatment.After treatment,7 cases suffered from rebleeding within 2 years after discharge,but no bleeding occurred after conservative treatment in our hospital.2.26 cases received surgical treatment.15 cases received devascularization:2 cases underwent Rex shunt because of multiple bleeding within a short period time after operation;1 case of intraoperative hemorrhage 4 times received endoscopic variceal ligation;4 cases suffered from postoperative bleeding once,but no further bleeding occurred after conservative treatment;2 cases of postoperative bleeding 3 times after drug conservative treatment in our hospital had no further bleeding.10 cases received shunt operation: 6 cases underwent shunt operation,among them,1 case had no rebleeding after operation because of hypersplenism and bleeding,4 cases had no rebleeding after Warrn operation,and there was no rebleeding after operation.There was no bleeding after combined operation.No hepatic encephalopathy occurred in all the children after discharge.3.In the conservative group,the difference of white blood cells(WBC),platelet value(PLT)and hemoglobin value(HGB)before and after treatment was significant(P < 0.05),but there was no significant difference of alanine aminotransferase(ALT)、total bilirubin level(TBIL)and prothrombin time(PT)between before and after treatment(P > 0.05)。In the disconnection group,the difference of platelet value(PLT)and hemoglobin value(HGB)before and after treatment was significant(P < 0.05),but the white blood cells(WBC)、alanine aminotransferase(ALT)、total bilirubin level(TBIL)and prothrombin time(PT)compared before and after treatment were indicating that there was no significant difference(P > 0.05).In the shunt group,the difference of platelet value(PLT)、hemoglobin value(HGB)、portal venous pressure and Spleen thickness before and after treatment was significant(P < 0.05),but there was no significant difference of the white blood cells(WBC)、alanine aminotransferase(ALT)、total bilirubin level(TBIL)and prothrombin time(PT)between before and after treatment(P > 0.05)4.The results of t test showed that there was a significant difference in the number of postoperative bleeding between the disconnection group and the shunt group(P < 0.05).At the same time,the time of operation and the amount of intraoperative bleeding were compared by using rank sum test(P > 0.05),indicating that the difference was not statistically significant.The difference of leukocyte,platelet and hemoglobin between the two methods was statistically significant(P < 0.05),but there was no significant difference in platelet(P < 0.05).Conclusion 1.Rex shunt was the first choice in the surgical treatment of CTPV,and the distal splenorenal shunt could not be performed in children with Rex.2.Splenomegaly and hypersplenism are feasible for devascularization. |
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