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

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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.


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(A) Preoperative CT scan shows a giant right HAA measuring 72 mm in diameter, with a dilated tortuous hepatic vasculature, in addition to VMs scattered predominantly in the left lobe. (B) Postoperative CT scan shows disappearance of the aneurysms and adequate hepatic blood flow with a patent distal anastomosis (arrow) as well as the absence of hepatic atrophy and necrosis. CT = computed tomography, HAA = hepatic artery aneurysm, VMs = vascular malformations.
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Figure 1: (A) Preoperative CT scan shows a giant right HAA measuring 72 mm in diameter, with a dilated tortuous hepatic vasculature, in addition to VMs scattered predominantly in the left lobe. (B) Postoperative CT scan shows disappearance of the aneurysms and adequate hepatic blood flow with a patent distal anastomosis (arrow) as well as the absence of hepatic atrophy and necrosis. CT = computed tomography, HAA = hepatic artery aneurysm, VMs = vascular malformations.

Mentions: A 62-year-old female with known HHT was referred to our facility to seek further treatment including possible liver transplantation for a giant HAA. She denied any symptoms except recurrent epistaxis. The patient had been diagnosed with HHT at the age of 56, when presenting with recurrent epistaxis and hematochezia. Definite diagnosis of HHT had been made based on 3 of the 4 Curaçao criteria (the presence of hepatic VMs revealed by abdominal ultrasound imaging, in addition to recurrent epistaxis and skin telangiectasia on her fingers). Because her hepatic VMs were asymptomatic, she had had no previous medical, radiological, or surgical management of her disease. On physical examination, multiple telangiectases were noted on her fingers. Laboratory examination was significant for elevated levels of alkaline phosphatase, 968 g/dL and gamma-glutamyl transpeptidase, 481 U/L. She was classified as Child-Pugh class A (total bilirubin, 0.8 mg/dL, and international normalized ratio, 1.0). Computed tomography (CT) scan revealed 2 right HAAs (72 × 63 mm and 29 × 28 mm) and a common HAA (28 × 27 mm). The larger right HAA was located in the liver bed and just proximal to the bifurcation of the anterior and posterior segmental branches of the right hepatic artery. The smaller right HAA gave off the A6 (the artery feeding S6), and the common HAA gave off the gastroduodenal artery (GDA). In addition, continuously dilated and highly tortuous arteries running from the celiac trunk to the right and left hepatic arteries were noted (Fig. 1A). The biliary duct was dilated in the right lobe, seemingly due to compression of the larger right HAA. Patchy lesions with early enhancement were scattered predominantly in the left lobe, and the middle and left hepatic veins were also enhanced in the early phase. These findings suggested the presence of arteriovenous shunts in the left lobe. Additionally, the left lobe was significantly enlarged, measuring 1105 mL in volume on the basis of CT liver volumetry, whereas the right lobe measured 726 mL.


Open arterial reconstruction of multiple hepatic artery aneurysms in a patient with hereditary hemorrhagic telangiectasia
(A) Preoperative CT scan shows a giant right HAA measuring 72 mm in diameter, with a dilated tortuous hepatic vasculature, in addition to VMs scattered predominantly in the left lobe. (B) Postoperative CT scan shows disappearance of the aneurysms and adequate hepatic blood flow with a patent distal anastomosis (arrow) as well as the absence of hepatic atrophy and necrosis. CT = computed tomography, HAA = hepatic artery aneurysm, VMs = vascular malformations.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: (A) Preoperative CT scan shows a giant right HAA measuring 72 mm in diameter, with a dilated tortuous hepatic vasculature, in addition to VMs scattered predominantly in the left lobe. (B) Postoperative CT scan shows disappearance of the aneurysms and adequate hepatic blood flow with a patent distal anastomosis (arrow) as well as the absence of hepatic atrophy and necrosis. CT = computed tomography, HAA = hepatic artery aneurysm, VMs = vascular malformations.
Mentions: A 62-year-old female with known HHT was referred to our facility to seek further treatment including possible liver transplantation for a giant HAA. She denied any symptoms except recurrent epistaxis. The patient had been diagnosed with HHT at the age of 56, when presenting with recurrent epistaxis and hematochezia. Definite diagnosis of HHT had been made based on 3 of the 4 Curaçao criteria (the presence of hepatic VMs revealed by abdominal ultrasound imaging, in addition to recurrent epistaxis and skin telangiectasia on her fingers). Because her hepatic VMs were asymptomatic, she had had no previous medical, radiological, or surgical management of her disease. On physical examination, multiple telangiectases were noted on her fingers. Laboratory examination was significant for elevated levels of alkaline phosphatase, 968 g/dL and gamma-glutamyl transpeptidase, 481 U/L. She was classified as Child-Pugh class A (total bilirubin, 0.8 mg/dL, and international normalized ratio, 1.0). Computed tomography (CT) scan revealed 2 right HAAs (72 × 63 mm and 29 × 28 mm) and a common HAA (28 × 27 mm). The larger right HAA was located in the liver bed and just proximal to the bifurcation of the anterior and posterior segmental branches of the right hepatic artery. The smaller right HAA gave off the A6 (the artery feeding S6), and the common HAA gave off the gastroduodenal artery (GDA). In addition, continuously dilated and highly tortuous arteries running from the celiac trunk to the right and left hepatic arteries were noted (Fig. 1A). The biliary duct was dilated in the right lobe, seemingly due to compression of the larger right HAA. Patchy lesions with early enhancement were scattered predominantly in the left lobe, and the middle and left hepatic veins were also enhanced in the early phase. These findings suggested the presence of arteriovenous shunts in the left lobe. Additionally, the left lobe was significantly enlarged, measuring 1105 mL in volume on the basis of CT liver volumetry, whereas the right lobe measured 726 mL.

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