case_images: PMC9395736_01
case_images: Imaging of HHT involvement in the liver. (A) The MRI results suggested right hepatobiliary dilatation with stone formation (arrow); (B) The arterial phase of CT indicated diffuse telangiectasia and shunting in the liver; (C) Three-dimensional reconstruction showed hepatic telangiectasia and bile duct dilatation (arrow); (D) Three-dimensional reconstruction showed telangiectasia and intrahepatic bile duct stones (arrow).
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case_images: F1
case_images: Magnetic resonance imaging (MRI) showed that intrahepatic bile duct stones were concentrated in the right hemi-liver (Figure 1A). Moreover, hepatic arteriovenous malformations (AVMs) accompanied by bile duct necrosis were identified by computed tomography (CT) (Figure 1B). Finally, three-dimensional reconstruction vividly showed the dilatation of the intrahepatic bile ducts, bile duct calculi, and extensive telangiectasia (Figures 1C, D).
case_images: The operative procedure and key intraoperative pictures. (A) Surgical exploration revealed extensive telangiectasia in the liver; (B) Portal venous pressure measurement prior to the surgery; (C) The common hepatic artery and gastroduodenal artery were suspended and pre-occluded; (D), The common hepatic artery and gastroduodenal artery were ligated; (E) Right hemihepatectomy was performed successfully; (F) Right liver specimen and intrahepatic bile duct dilatation with stone formation.
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case_images: F2
case_images: On intraoperative exploration, the erythema caused by telangiectasia was diffuse throughout the liver, which was consistent with the imaging findings (Figure 2A). Therefore, several measures were taken to reduce surgical complications:1, portal pressure was monitored to ensure that the portal pressure was reduced while normal perfusion was maintained (Figure 2B); 2, the blood flow velocities of the hepatic artery, portal vein and hepatic vein were monitored by ultrasound to ensure nearly normal blood flow signal after ligation (Figures 2C,D; Supplementary Table S2); and 3, since all indicators met our expectations, right hemihepatectomy was performed as the last step (Figures 2E,F).
case_images: Curve of postoperative liver function-related indexes. Postoperative changes in albumin (A) total bilirubin (B) prothrombin time (C) and prothrombin activity (D).
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case_images: F3
case_images: As expected, even with her extensive preoperative preparation, there was a period of liver insufficiency after surgery (Figure 3).
case_images: CT image comparison of the hepatic arteriovenous shunt before and after operation. CT arterial phase showed a significant decrease in telangiectasia and the shunt (A, preoperative; B, postoperative). Enhanced CT in the portal phase also showed a significant decrease in the shunt (C, preoperative; D, postoperative).
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case_images: F4
case_images: The patient has been followed up regularly (every 6 months, including enhanced upper abdominal CT and liver function), and the treatment effect was satisfactory (pain disappeared, liver function returned, weight increased, liver volume was partially compensated for, and hepatic AVMs were relieved) (Figure 4).