Surgical Ligation of Portosystemic Shunt to Resolve Severe Hematuria and Hemafecia Caused by Type II Abernethy Malformation Chao Jiang, Wei Ye, Changwei Liu, Weiwei Wu, and Yongjun Li, Beijing, China
The purpose of this study was to report the use of venous pressure measurement during surgery for Abernethy malformation (AF). This is a case report of a 19-year-old man who suffered from hematuria and hemafecia for 3 months with worsening symptoms a week before being sent to the emergency room. He was diagnosed with type II AF based on portal phlebography. We performed an open surgery; measured portal vein, inferior mesenteric vein (IMV), and inferior vena cava pressure; and decided to completely suture the IMV. Anticoagulation therapy was used during follow-up, and CTV showed increased portal vein diameter at 12 months after the procedure. For type II AF, measuring extrahepatic portal venous pressure changes before and after shunt blockage during surgery can help determine whether it is feasible to block the shunt, and anticoagulation therapy can improve patient prognosis.
Congenital extrahepatic portosystemic shunt, also named Abernethy malformation (AF), was first reported by Dr. Abernethy in 1793.1 Subsequently, depending on whether the intrahepatic portal vein is absent or dysplasia is present, Morgan and Superina2 categorized AFs into 2 types. The type I AF is more common in women and is defined as the complete absence of the intrahepatic portal vein, resulting in the extrahepatic portal vein and inferior vena cava (IVC) forming an end-to-side shunt. Because of the complete absence of the intrahepatic portal vein, ligation of the portosystemic shunt is useless for treating this condition as it would result in an everlasting insufficient blood supply to the liver and eventually lead to severe cirrhosis and hepatic cancer.3 Liver transplantation is usually used to treat these deformities.4 The type II AF is defined as hypoplasia of multiple intrahepatic portal veins, with the
Department of Vascular surgery, Peking Union Medical College Hospital, Beijing, China. Correspondence to: Wei Ye, MD, Department of Vascular surgery, Peking Union Medical College Hospital, No. 53 Dongdan North Street, Dongcheng District, Beijing, China; E-mail: [email protected] Ann Vasc Surg 2015; 29: 1020.e11–1020.e16 http://dx.doi.org/10.1016/j.avsg.2015.01.023 Ó 2015 Elsevier Inc. All rights reserved. Manuscript received: November 7, 2014; manuscript accepted: January 20, 2015; published online: March 11, 2015.
extrahepatic portal vein and IVC forming a side-toside shunt. Therefore, partial blood supply to the liver is preserved. Surgical ligation of the shunt to increase the blood supply to the liver is possible in these cases; however, the long-term prognosis for these patients is still unclear.
CASE REPORT A 19-year-old male suffered from hematuria and hemafecia for 3 months with symptoms worsening a week before being sent to the emergency room. Laboratory tests showed severe anemia with a blood hemoglobin (Hgb) of 32 g/L. The white blood cell (WBC) count and liver and renal function values were all within the normal range, platelet (Plt) count 50 109/L. A coagulopathy test showed an elongated activated partial thromboplastin time (APTT) of 45 sec and an elongated prothrombin time of 19 sec and an Fbg of 0.8 mg/L and a D-dimer of 5.2 mg/L (normal 32 mm Hg. When it is
The purpose of this study was to report the use of venous pressure measurement during surgery for Abernethy malformation (AF). This is a case report of a 19-year-old man who suffered from hematuria and hemafecia for 3 months with worsening symptoms a week before being sent to the emergency room. He was diagnosed with type II AF based on portal phlebography. We performed an open surgery; measured portal vein, inferior mesenteric vein (IMV), and inferior vena cava pressure; and decided to completely suture the IMV. Anticoagulation therapy was used during follow-up, and CTV showed increased portal vein diameter at 12 months after the procedure. For type II AF, measuring extrahepatic portal venous pressure changes before and after shunt blockage during surgery can help determine whether it is feasible to block the shunt, and anticoagulation therapy can improve patient prognosis.
Congenital extrahepatic portosystemic shunt, also named Abernethy malformation (AF), was first reported by Dr. Abernethy in 1793.1 Subsequently, depending on whether the intrahepatic portal vein is absent or dysplasia is present, Morgan and Superina2 categorized AFs into 2 types. The type I AF is more common in women and is defined as the complete absence of the intrahepatic portal vein, resulting in the extrahepatic portal vein and inferior vena cava (IVC) forming an end-to-side shunt. Because of the complete absence of the intrahepatic portal vein, ligation of the portosystemic shunt is useless for treating this condition as it would result in an everlasting insufficient blood supply to the liver and eventually lead to severe cirrhosis and hepatic cancer.3 Liver transplantation is usually used to treat these deformities.4 The type II AF is defined as hypoplasia of multiple intrahepatic portal veins, with the
Department of Vascular surgery, Peking Union Medical College Hospital, Beijing, China. Correspondence to: Wei Ye, MD, Department of Vascular surgery, Peking Union Medical College Hospital, No. 53 Dongdan North Street, Dongcheng District, Beijing, China; E-mail: [email protected] Ann Vasc Surg 2015; 29: 1020.e11–1020.e16 http://dx.doi.org/10.1016/j.avsg.2015.01.023 Ó 2015 Elsevier Inc. All rights reserved. Manuscript received: November 7, 2014; manuscript accepted: January 20, 2015; published online: March 11, 2015.
extrahepatic portal vein and IVC forming a side-toside shunt. Therefore, partial blood supply to the liver is preserved. Surgical ligation of the shunt to increase the blood supply to the liver is possible in these cases; however, the long-term prognosis for these patients is still unclear.
CASE REPORT A 19-year-old male suffered from hematuria and hemafecia for 3 months with symptoms worsening a week before being sent to the emergency room. Laboratory tests showed severe anemia with a blood hemoglobin (Hgb) of 32 g/L. The white blood cell (WBC) count and liver and renal function values were all within the normal range, platelet (Plt) count 50 109/L. A coagulopathy test showed an elongated activated partial thromboplastin time (APTT) of 45 sec and an elongated prothrombin time of 19 sec and an Fbg of 0.8 mg/L and a D-dimer of 5.2 mg/L (normal 32 mm Hg. When it is
