BOB Ped Surg 2023 - Rustam Yuldashev, WOFAPS - Presentation

Hello, dear all. Thank you for kind invitation. My name is Rustam Mildashef. I am pediatric surgeon from Tashkent, Uzbekistan. Our research study dedicated to angiographic patterns of extrahepatic portal Venus system in children with prehepatic portal hypertension and its etiological and clinical relevance. Majority of children with extrahepatic portal hypertension have isolated portal vein obstruction. And the portal vein is replaced by multiple Venus collaterals, so-called portal cavernoma. And in these children, the portal Venus system can be variably occluded. Children often present with splenomegaly and thrombocyopenia, esophageal and gastric varices and GI bleeding. In 1869, Balfour and Stewart first described the cavernous transformation of the portal vein. More than 150 years have passed since then, and we have today we have a number of etiological factors that cause this disease. However, even today, the majority of patients, the majority of patients, the etiology of this disease remains unknown. The patterns of portal mesenteric obstruction may provide clue to its etiology and clinical manifestation. And we aimed to study the relationship between the angiographic pattern and its etiology and clinical manifestation. The inclusion criteria for this study was children under 80 years of age, normal liver function tests, presence of portal cavernoma and splenomegaly on ultrasound, normal values of liver stiffness according to lastography and no prior surgical or endoscopic interventions. The data we studied was age at presentation, gender, etiological factor presenting symptoms, upper GI endoscopic symptoms and patterns of extrahepatic portal Venus obstruction according to CT angiography. 155 children with portal vein obstruction included in our study. Median age at the time of evaluation was nine years. The majority of children with extrahepatic portal had pathological condition in neonatal period and ophalitis, musculo skeletal septic conditions, prolonged jaundice and prematurity were noted with almost equal frequency. Among known etiological factors, umbilical vein cathozation were most frequent. And however, in 48% of cases, children with sport vein obstruction in our observation have no identifiable etiological factors. geography, we defined five imaging patterns and named them type 1 to type five conventionally. Most frequent types was type 1 and type five type two pattern, where the occlusion of the portal vein in type one was at the level of the bifurcation of the portal vein, and at the level of the main portal vein trunk in type two. Both these types had a patent superior mesenteric vein and splenic veins. Least common pattern was type three pattern where the portal vein occlusion was extended to the splenic vein. And type four pattern was characterized by by portal vein occlusion and superior mesenteric vein occlusion with patent splenic vein. The worst from the surgical point of view pattern imaging pattern was uh widespread thrombosis of the portal vein. Type five pattern. When comparing known etiological factors and patterns of portal vein obstruction, it was found that children who had type one and type two, had higher incidence of umbilical vein catheterization neonatal period. Whereas children with widespread thrombosis of the portal vein system, type five pattern, had higher incidence of musculo skeletal septic conditions in neonatal period. According to data, children with type one and type three patterns admit to hospital significantly earlier than children in other groups. Nevertheless, the correlation of age and the types of portal vein obstruction was weak. According to upper GI endoscopy, children with type three and type five patterns had the highest incidence of GI bleeding episodes. Although these children had a higher incidence of gastric varices, whereas children with mesenteric vein thrombosis, it has type four pattern, never had uh portal hypertension of gastropathy and they had a lower incidence of gastric varices. Also these children had a lower rate of uh cherry red spots on. Additionally, according to upper GI endoscopy, children with type four pattern had significantly lower grades of esophageal varices, especially compared to type one and type two patterns. Thrombocyto penia was common clinical feature of type three pattern. And it was observed in all cases. Nevertheless, significant difference in platelet concentration were found only among children with type four and type five patterns. In conclusion, common angiographic pattern of extrahepatic portal vein obstruction in children is type one. Obstruction at the level of the bifurcation of the portal vein. Umbilical vein catheterization was more associated with type one and type two patterns, whereas children with widespread thrombosis had the highest incidence of septic condition neonatal period. Children's splenic vein thrombosis and widespread thrombosis of the portal vein had at the most severe clinical manifestation of symptoms. and children with mesenteric vein thrombosis, type four pattern is characterized by lower risk of bleeding from esophageal varices and less incidence of gastric varices on GI endoscopy. And thank you for your attention.