Limits...
Open arterial reconstruction of multiple hepatic artery aneurysms in a patient with hereditary hemorrhagic telangiectasia

View Article: PubMed Central - PubMed

ABSTRACT

Background:: Hereditary hemorrhagic telangiectasia (HHT) is characterized by mucocutaneous telangiectasia and visceral vascular malformations (VMs). Liver involvement with VMs may lead to high-output cardiac failure, portal hypertension, and biliary disease. There is no curative treatment for the disease, and liver transplantation is indicated for life-threatening complications. Herein, we report a case of multiple hepatic artery aneurysms (HAAs) in a patient with HHT in which open arterial reconstruction was performed. There have only been a few case reports on HAA occurring with HHT. Thus, this case provides important information for the management of HHT-associated HAAs.

Case summary:: A 62-year-old female with known HHT was referred to our facility to seek further treatment for a giant HAA. She denied any symptoms except recurrent epistaxis. A computed tomography (CT) scan revealed a right HAA with a diameter of 72 mm, in addition to 2 other minor HAAs. The CT scan also revealed the VMs that were scattered in the liver, and a continuously dilated and tortuous artery existing from the celiac trunk to the right and left hepatic arteries. We performed open arterial reconstruction of the HAAs. Her postoperative course was uneventful.

Conclusions:: When treating HAAs, there are a variety of options. However, hepatic VMs might affect HHT patients in various ways postprocedurally. Ligation and embolization of the hepatic artery may lead to complications, such as massive hepatic necrosis. Hepatectomy should be avoided if possible, because a postoperative hyperperfusive state in the remaining liver can cause adverse events. We believe that arterial reconstruction of HHT-associated HAAs might reduce the risk of postprocedural complications with minimal hemodynamic changes in the liver, thus obviating the need for hepatectomy or liver transplantation.

No MeSH data available.


Related in: MedlinePlus

Pre- and postoperative schemas of the hepatic artery anatomy are shown above the angiography. The 3 HAAs are labeled with asterisks. Vessels that were resected are depicted with red lines. Bold lines in the preoperative schema indicate the sites where the hepatic artery was transected. (A) Preoperative angiography shows 2 right HAAs and a common HAA with a dilated tortuous hepatic vasculature, in addition to VMs located in the left lobe. (B) Completion angiography reveals the reconstructed hepatic arteries via direct suturing. A6 = the artery feeding S6, GDA = gastroduodenal artery, HAA = hepatic artery aneurysm, LHA = left hepatic artery, RHA = right hepatic artery, VMs = vascular malformations.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC5120944&req=5

Figure 2: Pre- and postoperative schemas of the hepatic artery anatomy are shown above the angiography. The 3 HAAs are labeled with asterisks. Vessels that were resected are depicted with red lines. Bold lines in the preoperative schema indicate the sites where the hepatic artery was transected. (A) Preoperative angiography shows 2 right HAAs and a common HAA with a dilated tortuous hepatic vasculature, in addition to VMs located in the left lobe. (B) Completion angiography reveals the reconstructed hepatic arteries via direct suturing. A6 = the artery feeding S6, GDA = gastroduodenal artery, HAA = hepatic artery aneurysm, LHA = left hepatic artery, RHA = right hepatic artery, VMs = vascular malformations.

Mentions: During the operation, an initial angiogram was obtained (Fig. 2A). Following laparotomy, the larger right HAA was found in the liver bed, compressing the right robe. By exposing the hepatic artery system, the other 2 HAAs were also identified. Then, the A6 off the smaller right HAA was ligated. After intravenous heparin was given, the right hepatic artery was clamped and the larger right HAA was then opened without distal control. Because the larger right HAA was deeply embedded within liver parenchyma, the outflow was identified from inside the aneurysm following the aneurysmotomy and a 2F Fogarty balloon catheter was placed into the outflow in order to control the back bleeding (Fig. 3A). The right hepatic artery just proximal to the smaller right HAA was transected and anastomosed directly to the distal orifice inside the larger right HAA in an end-to-end fashion with running 5-0 polypropylene, using the inclusion technique (Fig. 3B). Resection of the common HAA was done, after the GDA was ligated. Then, an end-to-end anastomosis of the proximal and distal edges of the common hepatic artery was performed. Finally, a completion angiogram showed the absence of the 3 aneurysms and that the hepatic blood flow was maintained without anastomotic stenosis or hepatic artery kinking (Fig. 2B).


Open arterial reconstruction of multiple hepatic artery aneurysms in a patient with hereditary hemorrhagic telangiectasia
Pre- and postoperative schemas of the hepatic artery anatomy are shown above the angiography. The 3 HAAs are labeled with asterisks. Vessels that were resected are depicted with red lines. Bold lines in the preoperative schema indicate the sites where the hepatic artery was transected. (A) Preoperative angiography shows 2 right HAAs and a common HAA with a dilated tortuous hepatic vasculature, in addition to VMs located in the left lobe. (B) Completion angiography reveals the reconstructed hepatic arteries via direct suturing. A6 = the artery feeding S6, GDA = gastroduodenal artery, HAA = hepatic artery aneurysm, LHA = left hepatic artery, RHA = right hepatic artery, VMs = vascular malformations.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5120944&req=5

Figure 2: Pre- and postoperative schemas of the hepatic artery anatomy are shown above the angiography. The 3 HAAs are labeled with asterisks. Vessels that were resected are depicted with red lines. Bold lines in the preoperative schema indicate the sites where the hepatic artery was transected. (A) Preoperative angiography shows 2 right HAAs and a common HAA with a dilated tortuous hepatic vasculature, in addition to VMs located in the left lobe. (B) Completion angiography reveals the reconstructed hepatic arteries via direct suturing. A6 = the artery feeding S6, GDA = gastroduodenal artery, HAA = hepatic artery aneurysm, LHA = left hepatic artery, RHA = right hepatic artery, VMs = vascular malformations.
Mentions: During the operation, an initial angiogram was obtained (Fig. 2A). Following laparotomy, the larger right HAA was found in the liver bed, compressing the right robe. By exposing the hepatic artery system, the other 2 HAAs were also identified. Then, the A6 off the smaller right HAA was ligated. After intravenous heparin was given, the right hepatic artery was clamped and the larger right HAA was then opened without distal control. Because the larger right HAA was deeply embedded within liver parenchyma, the outflow was identified from inside the aneurysm following the aneurysmotomy and a 2F Fogarty balloon catheter was placed into the outflow in order to control the back bleeding (Fig. 3A). The right hepatic artery just proximal to the smaller right HAA was transected and anastomosed directly to the distal orifice inside the larger right HAA in an end-to-end fashion with running 5-0 polypropylene, using the inclusion technique (Fig. 3B). Resection of the common HAA was done, after the GDA was ligated. Then, an end-to-end anastomosis of the proximal and distal edges of the common hepatic artery was performed. Finally, a completion angiogram showed the absence of the 3 aneurysms and that the hepatic blood flow was maintained without anastomotic stenosis or hepatic artery kinking (Fig. 2B).

View Article: PubMed Central - PubMed

ABSTRACT

Background:: Hereditary hemorrhagic telangiectasia (HHT) is characterized by mucocutaneous telangiectasia and visceral vascular malformations (VMs). Liver involvement with VMs may lead to high-output cardiac failure, portal hypertension, and biliary disease. There is no curative treatment for the disease, and liver transplantation is indicated for life-threatening complications. Herein, we report a case of multiple hepatic artery aneurysms (HAAs) in a patient with HHT in which open arterial reconstruction was performed. There have only been a few case reports on HAA occurring with HHT. Thus, this case provides important information for the management of HHT-associated HAAs.

Case summary:: A 62-year-old female with known HHT was referred to our facility to seek further treatment for a giant HAA. She denied any symptoms except recurrent epistaxis. A computed tomography (CT) scan revealed a right HAA with a diameter of 72 mm, in addition to 2 other minor HAAs. The CT scan also revealed the VMs that were scattered in the liver, and a continuously dilated and tortuous artery existing from the celiac trunk to the right and left hepatic arteries. We performed open arterial reconstruction of the HAAs. Her postoperative course was uneventful.

Conclusions:: When treating HAAs, there are a variety of options. However, hepatic VMs might affect HHT patients in various ways postprocedurally. Ligation and embolization of the hepatic artery may lead to complications, such as massive hepatic necrosis. Hepatectomy should be avoided if possible, because a postoperative hyperperfusive state in the remaining liver can cause adverse events. We believe that arterial reconstruction of HHT-associated HAAs might reduce the risk of postprocedural complications with minimal hemodynamic changes in the liver, thus obviating the need for hepatectomy or liver transplantation.

No MeSH data available.


Related in: MedlinePlus