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Abstract:

Introduction: congenital portosystemic venous shunts (CPVS) are rare vascular abnormalities that occur secondary to abnormal development or involution of fetal vasculature. They allow intestinal blood to enter the systemic circulation, bypassing the liver, which in the long term leads to various symptoms and complications. Today, thanks to advanced imaging techniques, the number of reported cases of CPVS is increasing, although for the most part these are single clinical cases or reports summarizing small series of cases. The overall incidence of CPVS is estimated at 1:30 000 births and 1:50 000 for those persisting beyond early childhood.

Material and methods: article consists of 44 foreign literature sources, that  highlight pathogenesis, classification, clinical picture, diagnosis and treatment of CPVS.

Conclusion: early diagnosis and correction of this anomaly using any (endovascular or surgical) occlusion regresses symptoms and prevents long-term complications. At present, given the rarity of this pathology, there is no large statistical analysis and no standards, developed for the management of this category of patients. However, further collection of material, an emphasis on the pathophysiology and anatomy of these lesions, will help to provide more effective care for patients with congenital portosystemic venous shunts.

  

 

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