Intractable Rare Dis Res. 2026;15(2):183-193. (DOI: 10.5582/irdr.2026.01006)

Surgical treatment and prognosis of type II congenital extrahepatic portosystemic shunts: A single-center experience of 31 cases

Hu Y, Gao S, Jiang H, Huo Y, Liu Y, Yang R, Liu W, Cao Y, Duan W, Tang H

Congenital extrahepatic portosystemic shunts (CEPS) are rare congenital vascular malformations characterized by an abnormal communication between the hepatic portal venous system and the systemic venous system. Type Ⅱ CEPS preserves partial portal venous blood flow and can usually be treated with conventional surgery rather than solely relying on liver transplantation. To determine the optimal surgical methods and complication management strategies for type Ⅱ CEPS patients, we retrospectively analyzed 31 predominantly adult patients with type Ⅱ CEPS, documenting their surgical approaches and the occurrence of postoperative complications. Five surgical approaches were employed: 11 patients underwent shunt occlusion with 5 cases of complications; 5 patients underwent splenic vessels ligation with 2 cases of complications; 5 patients underwent shunt occlusion combined with splenic artery ligation with 4 cases of complications; 8 patients underwent shunt occlusion combined with distal splenorenal shunt with 3 cases of complications; and 2 patients with lower extremity edema underwent inferior vena cava shunt bypass surgery, with no significant complications observed. In conclusion, surgery centering on the shunt occlusion demonstrates promising therapeutic value and remains the mainstay in the treatment of type II CEPS. Meanwhile, postoperative complications remain a concern, necessitating long-term monitoring and management.

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