Original Paper Received: March 30, 2013 Accepted: April 16, 2013 Published online: November 5, 2013
Dig Surg 2013;30:410–414 DOI: 10.1159/000351446
Liver Resection in Patients with Hepatic Hereditary Hemorrhagic Telangiectasia Sébastien Gaujoux a, d Margot Bucau b Maxime Ronot c–e Valerie Paradis b, d Valerie Vilgrain c–e Jacques Belghiti a, d Departments of aHepato-Pancreato-Biliary Surgery and b Pathology, Pôle des Maladies de l’Appareil Digestif (PMAD), AP-HP, Beaujon Hospital, and c Department of Radiology, Beaujon University Hospitals Paris Nord Val de Seine, Beaujon Hospital (AP-HP), Clichy, d University Paris Diderot, Sorbonne Paris Cité, and e INSERM U773, Centre de recherche biomédicale Bichat-Beaujon, CRB3, Paris, France
Key Words Liver resection · Hereditary hemorrhagic telangiectasia · Arteriovenous malformations · Hepatic shunting
Abstract Background: Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Rendu-Weber disease, is a rare genetic disorder characterized by widespread telangiectasia and vascular malformations involving the liver in most of the cases. The consequences of this pathologically underlying parenchyma on liver resection have been poorly described. Methods: More than 2,000 liver resections were performed at our institution over a 14-year period, whereby 2 major hepatectomies for malignancy were performed on patients with HHT with liver involvement. In addition, a systematic search was performed in the PubMed database to identify all original articles on hepatectomy in patients with HHT. Results: The first patient underwent a left hepatectomy for cholangiocarcinoma with an uneventful postoperative course. The second patient underwent right hepatectomy and segment 3 resection for colorectal liver metastases. The postoperative course was marked by ascites without liver failure. For both patients, 90-day mortality was nil. Conclusion: In selected HHT patients with liver involvement, liver
© 2013 S. Karger AG, Basel 0253–4886/13/0306–0410$38.00/0 E-Mail [email protected] www.karger.com/dsu
resections, including major hepatectomies, can be safely performed. Specific attention should be paid to postoperative liver function and ascites. © 2013 S. Karger AG, Basel
Introduction
Hereditary hemorrhagic telangiectasia (HHT), or Osler-Rendu-Weber (ORW) disease, is a rare genetic disorder characterized by widespread telangiectasia and vascular malformations. The liver is involved in about 40–75% of the patients [1–3], this incidence possibly increasing with age and in females [4, 5]. These diffuse vascular malformations harbor different patterns from small telangiectasia to large arteriovenous malformations leading to intrahepatic shunting. Their consequences on liver resection have been poorly described. We herein describe 2 patients with HHT undergoing major hepatectomy for malignancy, with specific attention to the postoperative course and liver function.
S. Gaujoux and M. Bucau contributed equally to the work.
Prof. Jacques Belghiti, MD Department of Hepatobiliary and Pancreatic Surgery Hospital Beaujon, 100, Bd du Général Leclerc FR–92110 Clichy (France) E-Mail jacques.belghiti @ bjn.aphp.fr
Patients and Methods Data Collection From 1998 to 2012, over 2,000 liver resections were performed at our institution, 2 of which were on patients with liver vascular malformation from HHT. Demographic, operative, perioperative, radiographic, and pathologic data were obtained from our institutional database with additional retrospective medical record review. Literature Search Studies were identified by performing electronic searches in MEDLINE via PubMed, including studies from January 1990 to April 2012, and using the following search strategy for the MESH and non-MESH heading: (liver resection OR hepatectomy) AND (Osler-Rendu-Weber disease OR hereditary hemorrhagic telangiectasia). After identifying relevant titles, abstracts were read and eligible articles retrieved. A manual cross-reference search of the bibliography of all publications retrieved was performed for relevant references, and the ‘related article’ function in PubMed was also used to identify studies that may have been missed in the database search. Relevant original clinical studies in English or French of any level of evidence were included.
Results
Case 2 A 64-year-old female with colorectal bilobar synchronous metastases was transferred to our unit after 11 cycles of Folfox chemotherapy (fig. 2a). HHT disease was diagnosed on familial history, associated with frequent nosebleed. Liver arteriovenous shunts were seen on preoperative imaging. She underwent right hepatectomy with segment 3 resection and pedicular lymphadenectomy. Operative time was 450 min, total blood loss was estimated to be 800 ml with a 120-min clamping duration, and intraoperative blood transfusion of 2 units. During selective arterial clamping, a portal flow decrease was the only observed hemodynamic modification. Liver function tests are described in figure 2b. Briefly, maximum bilirubin was 41 μmol/l on POD 9, minimum prothrombin ratio 71% on POD 2, and maximum ALAT and ASAT 186 and 631 IU/l on PODs 2 and 1, respectively. The postoperative course was marked by ascites, highest output of 1,450 ml/day on POD 5, treated by fluid restriction, diuretic, albumin and persistent drainage. Pathologic examination (fig. 2c, d) was consistent with multiple, often necrotic, colorectal metastases. The non-tumoral liver exhibited a heterogeneous pattern with hepatic nodular regenerative lesions, including characteristic hepatocytes foci and periportal vascular multiplication.
Case 1 A 44-year-old female with HHT disease and biopsyproven left intrahepatic cholangiocarcinoma was transferred to our unit (fig. 1a). HHT disease-related symptoms were daily nosebleeds, chronic anemia, pulmonary arteriovenous malformation responsible for stage II NYHA dyspnea (previously treated by embolization), and liver arteriovenous shunts seen on preoperative imaging. Left hepatectomy with lymphadenectomy was performed. Operative time was 240 min, total blood loss was 800 ml, with a 45-min clamping duration, and no need for intraoperative blood transfusion. There was no hemodynamic instability, need of catecholamines, or unusual hemodynamic change associated with pedicle clamping. Liver function tests are described in figure 1b. Briefly, maximum bilirubin was 46 μmol/l on postoperative day (POD) 3, minimum prothrombin ratio 69% on POD 2, and maximum ALAT and ASAT 596 and 687 IU/l on PODs 3 and 1, respectively. Maximum drain output was of 75 ml on POD 4. The postoperative course was only characterized by a non-symptomatic segment VII hepatic vein thrombosis, treated by anticoagulation. Pathologic examination (fig. 1c, d) found a 13-cm combined hepatocellular and cholangiocarcinoma associated with a heterogeneous hepatic parenchyma characterized by multiple arteriovenous shunts in portal areas, typical of ORW disease hepatic involvement.
Hepatic involvement in HHT disease is frequent and occurs in about 75% of patients [4]. It is characterized, with various intensities, by the presence of numerous intrahepatic abnormalities: arteriovenous, arterioportal, or portovenous shunts [2] as well as dilatation/aneurysm of the hepatic artery, telangiectasia, nodular regenerative hyperplasia or focal nodular hyperplasia whose relative risk is 100 compared with the general population [1]. ORW liver involvement can lead to high output cardiac failure and portal hypertension, cholangitis, ischemic hepatobiliary necrosis, or hepatic encephalopathy which could also lead to liver transplantation [2, 7–10].
Liver Resection and Hereditary Hemorrhagic Telangiectasia
Dig Surg 2013;30:410–414 DOI: 10.1159/000351446
Systematic Literature Search The search strategy revealed only one study, a case report [6], reporting a left lateral liver sectionectomy for hepatic-based arteriovenous malformations responsible for dyspnea and asthenia. This minor hepatectomy performed by laparotomy was associated with an uneventful postoperative course, discharge on POD 5, and symptom-free 4 years after surgery.
Discussion
411
Color version available online
a1
a2 800 700
Prothrombin ratio (%)
600
Total bilirubin (μmol/l) ASAT (IU)
500
ALAT (IU) Ascites (ml)
400 300 200 100
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Fig. 1. a Left intrahepatic cholangiocarcinoma in a 44-year-old female with HHT. a1 Preoperative enhanced abdominal CT scan, axial image on arterial phase obtained through the mid-part of the liver, showing a large and poorly enhanced tumor of the left liver; liver parenchyma is very heterogeneous due to small telangiectasias. a2 Oblique maximum intensity projection image (15-mm thick section) obtained in the early arterial phase showing a liver fed by a replaced right hepatic artery (dashed white arrow in a2) larger than the splenic artery. b Postoperative liver function test. c Macroscopic aspect of the lesion showing a 13-cm whitish tumor
with two satellite nodules. d Microscopic aspect of the lesion showing tumor of the liver consisting in nests of tumoral cells surrounded by desmoplastic stroma, corresponding to a mixed hepatocellular-cholangiocarcinoma (d1; HE, ×100); enlarged portal areas with dilated vessels, arteries, veins and lymphatics (d2; HE); non-tumoral liver with numbers of tortuous thick-walled veins between portal tracts and centrolobular vein (d3; HE, ×100), and non-tumoral liver showing nodules of regenerative hepatocytes surrounded by atrophic cell plates (d4; HE, ×100).
The liver surgery postoperative course is in part related to the underlying parenchyma, with an increased mortality in patients with cirrhosis, cholestasis, steatosis [11–13] or chemotherapy-associated steatohepatitis [14]. Consequently, hepatectomy in patients with HHT disease could be considered at risk. Our experience has shown that major hepatectomy can be safely performed in selected patients. However, it is likely that both liver
parenchyma and vascularization modification could be associated with an increased risk of bleeding and postoperative ascites, as seen after right hepatectomy in patient 2. Moreover, this patient presented with typical nodular regenerative hyperplasia lesions, which can be both due to HHT disease and/or oxaliplatin-based neoadjuvant chemotherapy. In retrospect, in this setting, the use of an angiogenesis inhibitor, namely bevacizumab (Avastin®;
412
Dig Surg 2013;30:410–414 DOI: 10.1159/000351446
Gaujoux /Bucau /Ronot /Paradis /Vilgrain / Belghiti
Color version available online
a1
a2
1,600
Prothrombin ratio (%)
1,400
Total bilirubin (μmol/l) ASAT (IU)
1,200
ALAT (IU)
1,000
Ascites (ml)
800 600 400 200
b
0
1
d1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
d2
c
d3
liver showing the large and poorly enhanced tumor of the right atrophic liver appearing heterogeneous on the portal phase (white arrows). Liver parenchyma shows a characteristic aspect and is very heterogeneous on the arterial phase due to small telangiectasias (arrowheads). b Postoperative liver function test. c Macro-
scopic aspect of the lesion showing multiple, often necrotic ill-limited tumors and heterogeneous aspect of non-tumoral liver. d Microscopic aspect of the lesion in non-tumoral liver showing dilated anastomosing sinusoidal channels, with various dilated vessels in the center (d1; HE, ×100); enlarged portal tract showing several dilated vessels including large caliber arteries (d2; HE, ×200), and reticulin staining showing regenerative nodular hyperplasia (d3; ×50).
Genetech Roche, Welwyn Garden City, UK), could have been beneficial on both tumoral and normal liver as previously reported in patients with HHT disease. Indeed, some patients with high-output heart failure have improved significantly after treatment, both clinically and on hemodynamic parameters, with sometimes dramatic
regression of hepatic vascular malformations [15, 16]. Our experience confirms and extends the result of a previous published minor hepatectomy (lateral liver sectionectomy) for hepatic-based arteriovenous malformations with an uneventful postoperative course [6]. It is interesting to note that in very selected cases, as the one
Liver Resection and Hereditary Hemorrhagic Telangiectasia
Dig Surg 2013;30:410–414 DOI: 10.1159/000351446
Fig. 2. a Right hepatic colorectal metastases in a 64-year-old female with HHT. Preoperative enhanced (arterial (a1) and portal phase (a2)) abdominal CT scan obtained through the mid-part of the
413
previously discussed, hepatectomy could be used in order to selectively remove a symptomatic hepatic-based arteriovenous malformation when alternative treatment appears disproportionate or impossible. In conclusion, in selected cases, liver resection in patients with ORW disease appears safe and effective, but possibly associated with an increased postoperative liver dysfunction. In this setting, the use of preoperative bev-
acizumab treatment surgery could be an interesting option in order to normalize underlying liver hemodynamics.
Disclosure Statement The authors have no conflicts of interest to disclose.
References 1 Ianora AAS, Memeo M, Sabba C, Cirulli A, Rotondo A, Angelelli G: Hereditary hemorrhagic telangiectasia: multi-detector row helical CT assessment of hepatic involvement. Radiology 2004;230:250–259. 2 Faughnan ME, Palda VA, Garcia-Tsao G, Geisthoff UW, McDonald J, Proctor DD, Spears J, Brown DH, Buscarini E, Chesnutt MS, Cottin V, Ganguly A, Gossage JR, Guttmacher AE, Hyland RH, Kennedy SJ, Korzenik J, Mager JJ, Ozanne AP, Piccirillo JF, Picus D, Plauchu H, Porteous MEM, Pyeritz RE, Ross DA, Sabba C, Swanson K, Terry P, Wallace MC, Westermann CJJ, White RI, Young LH, Zarrabeitia R: International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet 2011;48:73–87. 3 Buscarini E, Danesino C, Olivieri C, Lupinacci G, De Grazia F, Reduzzi L, Blotta P, Gazzaniga P, Pagella F, Grosso M, Pongiglione G, Buscarini L, Plauchu H, Zambelli A: Doppler ultrasonographic grading of hepatic vascular malformations in hereditary hemorrhagic telangiectasia – results of extensive screening. Ultraschall Med 2004;25:348–355. 4 Buscarini E, Plauchu H, Garcia Tsao G, White RI Jr, Sabbà C, Miller F, Saurin JC, Pelage JP, Lesca G, Marion MJ, Perna A, Faughnan ME: Liver involvement in hereditary hemorrhagic telangiectasia: consensus recommendations. Liver Int 2006;26:1040–1046. 5 Lesca G, Olivieri C, Burnichon N, Pagella F, Carette MF, Gilbert-Dussardier B, Goizet C, Roume J, Rabilloud M, Saurin JC, Cottin V, Honnorat J, Coulet F, Giraud S, Calender A, Danesino C, Buscarini E, Plauchu H: Genotype-phenotype correlations in hereditary hemorrhagic telangiectasia: data from the French-Italian HHT network. Genet Med 2007;9:14–22.
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6 Orlando G, Sabbà C, De Liguori Carino N, Scelzo C, Tariciotti L, Bonanni L, D’Angelo M, Baiocchi L, Lerut J, Tisone G: Non-transplant surgical approach to liver-based hereditary haemorrhagic telangiectasia: a first report. Liver Int 2008;28:574–577. 7 Garcia-Tsao G, Korzenik JR, Young L, Henderson KJ, Jain D, Byrd B, Pollak JS, White RI Jr: Liver disease in patients with hereditary hemorrhagic telangiectasia. N Engl J Med 2000;343:931–936. 8 Lerut J, Orlando G, Adam R, Sabb C, Pfitzmann R, Klempnauer J, Belghiti J, Pirenne J, Thevenot T, Hillert C, Brown CM, Gonze D, Karam V, Boillot O: Liver transplantation for hereditary hemorrhagic telangiectasia. Ann Surg 2006;244:854–864. 9 Dupuis-Girod S, Chesnais AL, Ginon I, Dumortier J, Saurin JC, Finet G, Decullier E, Marion D, Plauchu H, Boillot O: Long-term outcome of patients with hereditary hemorrhagic telangiectasia and severe hepatic involvement after orthotopic liver transplantation: a single-center study. Liver Transpl 2010;16:340–347. 10 Buscarini E, Leandro G, Conte D, Danesino C, Daina E, Manfredi G, Lupinacci G, Brambilla G, Menozzi F, De Grazia F, Gazzaniga P, Inama G, Bonardi R, Blotta P, Forner P, Olivieri C, Perna A, Grosso M, Pongiglione G, Boccardi E, Pagella F, Rossi G, Zambelli A: Natural history and outcome of hepatic vascular malformations in a large cohort of patients with hereditary hemorrhagic teleangiectasia. Dig Dis Sci 2011;56:2166–2178.
11 Hammond JS, Guha IN, Beckingham IJ, Lobo DN: Prediction, prevention and management of postresection liver failure. Br J Surg 2011; 98:1188–1200. 12 Midorikawa Y, Kubota K, Takayama T, Toyoda H, Ijichi M, Torzilli G, Mori M, Makuuchi M: A comparative study of postoperative complications after hepatectomy in patients with and without chronic liver disease. Surgery 1999;126:484–491. 13 Kamiyama T, Nakanishi K, Yokoo H, Kamachi H, Tahara M, Yamashita K, Taniguchi M, Shimamura T, Matsushita M, Todo T: Perioperative management of hepatic resection toward zero mortality and morbidity: analysis of 793 consecutive cases in a single institution. J Am Coll Surg 2010;211:443–449. 14 Lehmann K, Rickenbacher A, Weber A, Pestalozzi BC, Clavien PA: Chemotherapy before liver resection of colorectal metastases: friend or foe? Ann Surg 2012;255:237–247. 15 Dupuis-Girod S, Ginon I, Saurin JC, Marion D, Guillot E, Decullier E, Roux A, Carette MF, Gilbert-Dussardier B, Hatron PY, Lacombe P, Lorcerie B, Rivière S, Corre R, Giraud S, Bailly S, Paintaud G, Ternant D, Valette PJ, Plauchu H, Faure F: Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output. JAMA 2012;307;948–955. 16 Mitchell A, Adams LA, MacQuillan G, Tibballs J, van den Driesen R, Delriviere L: Bevacizumab reverses need for liver transplantation in hereditary hemorrhagic telangiectasia. Liver Transpl 2008;14:210–213.
Gaujoux /Bucau /Ronot /Paradis /Vilgrain / Belghiti
Copyright: S. Karger AG, Basel 2014. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
Dig Surg 2013;30:410–414 DOI: 10.1159/000351446
Liver Resection in Patients with Hepatic Hereditary Hemorrhagic Telangiectasia Sébastien Gaujoux a, d Margot Bucau b Maxime Ronot c–e Valerie Paradis b, d Valerie Vilgrain c–e Jacques Belghiti a, d Departments of aHepato-Pancreato-Biliary Surgery and b Pathology, Pôle des Maladies de l’Appareil Digestif (PMAD), AP-HP, Beaujon Hospital, and c Department of Radiology, Beaujon University Hospitals Paris Nord Val de Seine, Beaujon Hospital (AP-HP), Clichy, d University Paris Diderot, Sorbonne Paris Cité, and e INSERM U773, Centre de recherche biomédicale Bichat-Beaujon, CRB3, Paris, France
Key Words Liver resection · Hereditary hemorrhagic telangiectasia · Arteriovenous malformations · Hepatic shunting
Abstract Background: Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Rendu-Weber disease, is a rare genetic disorder characterized by widespread telangiectasia and vascular malformations involving the liver in most of the cases. The consequences of this pathologically underlying parenchyma on liver resection have been poorly described. Methods: More than 2,000 liver resections were performed at our institution over a 14-year period, whereby 2 major hepatectomies for malignancy were performed on patients with HHT with liver involvement. In addition, a systematic search was performed in the PubMed database to identify all original articles on hepatectomy in patients with HHT. Results: The first patient underwent a left hepatectomy for cholangiocarcinoma with an uneventful postoperative course. The second patient underwent right hepatectomy and segment 3 resection for colorectal liver metastases. The postoperative course was marked by ascites without liver failure. For both patients, 90-day mortality was nil. Conclusion: In selected HHT patients with liver involvement, liver
© 2013 S. Karger AG, Basel 0253–4886/13/0306–0410$38.00/0 E-Mail [email protected] www.karger.com/dsu
resections, including major hepatectomies, can be safely performed. Specific attention should be paid to postoperative liver function and ascites. © 2013 S. Karger AG, Basel
Introduction
Hereditary hemorrhagic telangiectasia (HHT), or Osler-Rendu-Weber (ORW) disease, is a rare genetic disorder characterized by widespread telangiectasia and vascular malformations. The liver is involved in about 40–75% of the patients [1–3], this incidence possibly increasing with age and in females [4, 5]. These diffuse vascular malformations harbor different patterns from small telangiectasia to large arteriovenous malformations leading to intrahepatic shunting. Their consequences on liver resection have been poorly described. We herein describe 2 patients with HHT undergoing major hepatectomy for malignancy, with specific attention to the postoperative course and liver function.
S. Gaujoux and M. Bucau contributed equally to the work.
Prof. Jacques Belghiti, MD Department of Hepatobiliary and Pancreatic Surgery Hospital Beaujon, 100, Bd du Général Leclerc FR–92110 Clichy (France) E-Mail jacques.belghiti @ bjn.aphp.fr
Patients and Methods Data Collection From 1998 to 2012, over 2,000 liver resections were performed at our institution, 2 of which were on patients with liver vascular malformation from HHT. Demographic, operative, perioperative, radiographic, and pathologic data were obtained from our institutional database with additional retrospective medical record review. Literature Search Studies were identified by performing electronic searches in MEDLINE via PubMed, including studies from January 1990 to April 2012, and using the following search strategy for the MESH and non-MESH heading: (liver resection OR hepatectomy) AND (Osler-Rendu-Weber disease OR hereditary hemorrhagic telangiectasia). After identifying relevant titles, abstracts were read and eligible articles retrieved. A manual cross-reference search of the bibliography of all publications retrieved was performed for relevant references, and the ‘related article’ function in PubMed was also used to identify studies that may have been missed in the database search. Relevant original clinical studies in English or French of any level of evidence were included.
Results
Case 2 A 64-year-old female with colorectal bilobar synchronous metastases was transferred to our unit after 11 cycles of Folfox chemotherapy (fig. 2a). HHT disease was diagnosed on familial history, associated with frequent nosebleed. Liver arteriovenous shunts were seen on preoperative imaging. She underwent right hepatectomy with segment 3 resection and pedicular lymphadenectomy. Operative time was 450 min, total blood loss was estimated to be 800 ml with a 120-min clamping duration, and intraoperative blood transfusion of 2 units. During selective arterial clamping, a portal flow decrease was the only observed hemodynamic modification. Liver function tests are described in figure 2b. Briefly, maximum bilirubin was 41 μmol/l on POD 9, minimum prothrombin ratio 71% on POD 2, and maximum ALAT and ASAT 186 and 631 IU/l on PODs 2 and 1, respectively. The postoperative course was marked by ascites, highest output of 1,450 ml/day on POD 5, treated by fluid restriction, diuretic, albumin and persistent drainage. Pathologic examination (fig. 2c, d) was consistent with multiple, often necrotic, colorectal metastases. The non-tumoral liver exhibited a heterogeneous pattern with hepatic nodular regenerative lesions, including characteristic hepatocytes foci and periportal vascular multiplication.
Case 1 A 44-year-old female with HHT disease and biopsyproven left intrahepatic cholangiocarcinoma was transferred to our unit (fig. 1a). HHT disease-related symptoms were daily nosebleeds, chronic anemia, pulmonary arteriovenous malformation responsible for stage II NYHA dyspnea (previously treated by embolization), and liver arteriovenous shunts seen on preoperative imaging. Left hepatectomy with lymphadenectomy was performed. Operative time was 240 min, total blood loss was 800 ml, with a 45-min clamping duration, and no need for intraoperative blood transfusion. There was no hemodynamic instability, need of catecholamines, or unusual hemodynamic change associated with pedicle clamping. Liver function tests are described in figure 1b. Briefly, maximum bilirubin was 46 μmol/l on postoperative day (POD) 3, minimum prothrombin ratio 69% on POD 2, and maximum ALAT and ASAT 596 and 687 IU/l on PODs 3 and 1, respectively. Maximum drain output was of 75 ml on POD 4. The postoperative course was only characterized by a non-symptomatic segment VII hepatic vein thrombosis, treated by anticoagulation. Pathologic examination (fig. 1c, d) found a 13-cm combined hepatocellular and cholangiocarcinoma associated with a heterogeneous hepatic parenchyma characterized by multiple arteriovenous shunts in portal areas, typical of ORW disease hepatic involvement.
Hepatic involvement in HHT disease is frequent and occurs in about 75% of patients [4]. It is characterized, with various intensities, by the presence of numerous intrahepatic abnormalities: arteriovenous, arterioportal, or portovenous shunts [2] as well as dilatation/aneurysm of the hepatic artery, telangiectasia, nodular regenerative hyperplasia or focal nodular hyperplasia whose relative risk is 100 compared with the general population [1]. ORW liver involvement can lead to high output cardiac failure and portal hypertension, cholangitis, ischemic hepatobiliary necrosis, or hepatic encephalopathy which could also lead to liver transplantation [2, 7–10].
Liver Resection and Hereditary Hemorrhagic Telangiectasia
Dig Surg 2013;30:410–414 DOI: 10.1159/000351446
Systematic Literature Search The search strategy revealed only one study, a case report [6], reporting a left lateral liver sectionectomy for hepatic-based arteriovenous malformations responsible for dyspnea and asthenia. This minor hepatectomy performed by laparotomy was associated with an uneventful postoperative course, discharge on POD 5, and symptom-free 4 years after surgery.
Discussion
411
Color version available online
a1
a2 800 700
Prothrombin ratio (%)
600
Total bilirubin (μmol/l) ASAT (IU)
500
ALAT (IU) Ascites (ml)
400 300 200 100
b
0
1
2
3
d1
4
5
6
7
d2
8
c
9
d3
d4
Fig. 1. a Left intrahepatic cholangiocarcinoma in a 44-year-old female with HHT. a1 Preoperative enhanced abdominal CT scan, axial image on arterial phase obtained through the mid-part of the liver, showing a large and poorly enhanced tumor of the left liver; liver parenchyma is very heterogeneous due to small telangiectasias. a2 Oblique maximum intensity projection image (15-mm thick section) obtained in the early arterial phase showing a liver fed by a replaced right hepatic artery (dashed white arrow in a2) larger than the splenic artery. b Postoperative liver function test. c Macroscopic aspect of the lesion showing a 13-cm whitish tumor
with two satellite nodules. d Microscopic aspect of the lesion showing tumor of the liver consisting in nests of tumoral cells surrounded by desmoplastic stroma, corresponding to a mixed hepatocellular-cholangiocarcinoma (d1; HE, ×100); enlarged portal areas with dilated vessels, arteries, veins and lymphatics (d2; HE); non-tumoral liver with numbers of tortuous thick-walled veins between portal tracts and centrolobular vein (d3; HE, ×100), and non-tumoral liver showing nodules of regenerative hepatocytes surrounded by atrophic cell plates (d4; HE, ×100).
The liver surgery postoperative course is in part related to the underlying parenchyma, with an increased mortality in patients with cirrhosis, cholestasis, steatosis [11–13] or chemotherapy-associated steatohepatitis [14]. Consequently, hepatectomy in patients with HHT disease could be considered at risk. Our experience has shown that major hepatectomy can be safely performed in selected patients. However, it is likely that both liver
parenchyma and vascularization modification could be associated with an increased risk of bleeding and postoperative ascites, as seen after right hepatectomy in patient 2. Moreover, this patient presented with typical nodular regenerative hyperplasia lesions, which can be both due to HHT disease and/or oxaliplatin-based neoadjuvant chemotherapy. In retrospect, in this setting, the use of an angiogenesis inhibitor, namely bevacizumab (Avastin®;
412
Dig Surg 2013;30:410–414 DOI: 10.1159/000351446
Gaujoux /Bucau /Ronot /Paradis /Vilgrain / Belghiti
Color version available online
a1
a2
1,600
Prothrombin ratio (%)
1,400
Total bilirubin (μmol/l) ASAT (IU)
1,200
ALAT (IU)
1,000
Ascites (ml)
800 600 400 200
b
0
1
d1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
d2
c
d3
liver showing the large and poorly enhanced tumor of the right atrophic liver appearing heterogeneous on the portal phase (white arrows). Liver parenchyma shows a characteristic aspect and is very heterogeneous on the arterial phase due to small telangiectasias (arrowheads). b Postoperative liver function test. c Macro-
scopic aspect of the lesion showing multiple, often necrotic ill-limited tumors and heterogeneous aspect of non-tumoral liver. d Microscopic aspect of the lesion in non-tumoral liver showing dilated anastomosing sinusoidal channels, with various dilated vessels in the center (d1; HE, ×100); enlarged portal tract showing several dilated vessels including large caliber arteries (d2; HE, ×200), and reticulin staining showing regenerative nodular hyperplasia (d3; ×50).
Genetech Roche, Welwyn Garden City, UK), could have been beneficial on both tumoral and normal liver as previously reported in patients with HHT disease. Indeed, some patients with high-output heart failure have improved significantly after treatment, both clinically and on hemodynamic parameters, with sometimes dramatic
regression of hepatic vascular malformations [15, 16]. Our experience confirms and extends the result of a previous published minor hepatectomy (lateral liver sectionectomy) for hepatic-based arteriovenous malformations with an uneventful postoperative course [6]. It is interesting to note that in very selected cases, as the one
Liver Resection and Hereditary Hemorrhagic Telangiectasia
Dig Surg 2013;30:410–414 DOI: 10.1159/000351446
Fig. 2. a Right hepatic colorectal metastases in a 64-year-old female with HHT. Preoperative enhanced (arterial (a1) and portal phase (a2)) abdominal CT scan obtained through the mid-part of the
413
previously discussed, hepatectomy could be used in order to selectively remove a symptomatic hepatic-based arteriovenous malformation when alternative treatment appears disproportionate or impossible. In conclusion, in selected cases, liver resection in patients with ORW disease appears safe and effective, but possibly associated with an increased postoperative liver dysfunction. In this setting, the use of preoperative bev-
acizumab treatment surgery could be an interesting option in order to normalize underlying liver hemodynamics.
Disclosure Statement The authors have no conflicts of interest to disclose.
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Gaujoux /Bucau /Ronot /Paradis /Vilgrain / Belghiti
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