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Villanueva B, Cañabate A, Torres-Iglesias R, Cerdà P, Gamundí E, Ordi Q, Alba E, Sanz-Astier LA, Iriarte A, Ribas J, Castellote J, Pintó X, Riera-Mestre A. Minimal encephalopathy in hereditary hemorrhagic telangiectasia patients with portosystemic vascular malformations. Orphanet J Rare Dis 2024; 19:484. [PMID: 39709450 DOI: 10.1186/s13023-024-03493-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2024] [Accepted: 11/29/2024] [Indexed: 12/23/2024] Open
Abstract
BACKGROUND Hereditary hemorrhagic telangiectasia (HHT) is characterized by telangiectasia and larger vascular malformations. Liver malformations are the most frequent visceral involvement including the presence of portosystemic malformations (PSM) that can cause hepatic encephalopathy. Minimal hepatic encephalopathy (mHE) is characterized by alterations of brain function in neuropsychological or neurophysiological tests and decreases quality of life. The evidence of mHE in HHT patients is scarce. The aim of this study is to assess the prevalence and health impact of mHE in patients with and without PSM. METHODS We performed a cross-sectional observational study in a cohort of patients from an HHT referral unit. Adult patients with definite HHT and PSM and age and sex matched HHT controls without PSM (1:1) were included. Baseline clinical, imaging and laboratory tests and different neuropsychological tests for the screening of mHE were compared between both groups. RESULTS Eighteen patients with PSM and 18 controls out of 430 HHT patients were included. Patients with PSM showed higher prevalence of attention disturbances (50% vs. 11.1%, p = 0.027), falls during last 12 months (22.2% vs. 5.6%, p = 0.338), sleep disorders (50% vs. 16.7%, p = 0.075) and a worst performance in s-ANT1 test (14 vs. 19.5 points score, p = 0.739) than HHT controls. CONCLUSIONS HHT patients with PSM showed higher attention difficulties than HHT controls, though both PSM and HHT controls showed findings of mHE. Specific neuropsychological tests for early detection of mHE should be considered in HHT patients.
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Affiliation(s)
- B Villanueva
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Internal Medicine Department, Hospital Universitari Bellvitge, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
| | - A Cañabate
- Internal Medicine Department, Hospital Universitari Son Espases, Mallorca, Spain
| | - R Torres-Iglesias
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Internal Medicine Department, Hospital Universitari Bellvitge, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
| | - P Cerdà
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Internal Medicine Department, Hospital Universitari Bellvitge, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
| | - E Gamundí
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
- Cytology and Hematology Laboratory, Anatomic Pathology Department, Hospital Universitari Bellvitge, Barcelona, Spain
| | - Q Ordi
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
- Angioradiology, Radiology Department, Hospital Universitari Bellvitge, Barcelona, Spain
| | - E Alba
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Angioradiology, Radiology Department, Hospital Universitari Bellvitge, Barcelona, Spain
| | - L A Sanz-Astier
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Internal Medicine Department, Hospital Universitari Bellvitge, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
| | - A Iriarte
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Internal Medicine Department, Hospital Universitari Bellvitge, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
| | - J Ribas
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
- Pneumology Department, Hospital Universitari Bellvitge, Barcelona, Spain
| | - J Castellote
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain
- Department of Digestive Diseases, Hospital Universitari Bellvitge, Barcelona, Spain
- Clinical Sciences Department, Faculty of Medicine and Health Sciences, Universitat de Barcelona, Barcelona, Spain
| | - X Pintó
- Internal Medicine Department, Hospital Universitari Bellvitge, Barcelona, Spain
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain
- Angioradiology, Radiology Department, Hospital Universitari Bellvitge, Barcelona, Spain
- Clinical Sciences Department, Faculty of Medicine and Health Sciences, Universitat de Barcelona, Barcelona, Spain
| | - A Riera-Mestre
- HHT Unit. Hospital Universitari Bellvitge, C/Feixa Llarga S/N. L'Hospitalet de Llobregat, 08907, Barcelona, Spain.
- Internal Medicine Department, Hospital Universitari Bellvitge, Barcelona, Spain.
- Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain.
- Clinical Sciences Department, Faculty of Medicine and Health Sciences, Universitat de Barcelona, Barcelona, Spain.
- Center for Biomedical Research in Obesity and Nutrition Physiopathology Network (CIBEROBN), Carlos III Health Institute, Madrid, Spain.
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Fogang D C, Martin G, Boehlen F, Savoldelli GL. Anaesthetic management for caesarean section in a patient with hereditary haemorrhagic telangiectasia and severe epistaxis during pregnancy. BMJ Case Rep 2023; 16:e255530. [PMID: 37788916 PMCID: PMC10551871 DOI: 10.1136/bcr-2023-255530] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 09/15/2023] [Indexed: 10/05/2023] Open
Abstract
A primigravida patient, with a history of hereditary haemorrhagic telangiectasia (HHT) manifesting as nasal angiodysplasia and hepatic arteriovenous malformations (AVM), presented for delivery planning and anaesthetic evaluation at 29 weeks of gestation. She was hospitalised several times during the second and third trimester for serious recurrent epistaxis, leading to severe anaemia. In total, she required the transfusion of 20 units of packed red blood cells during her pregnancy as well as surgical nasal haemostasis under general anaesthesia (GA). The patient was referred to our tertiary centre for delivery. In the context of recurrent severe epistaxis and high cardiac output (due to hepatic AVM) in the third trimester, a multidisciplinary decision was made to plan an elective caesarean section at 35 4/7 weeks combined with nasal packing under GA. This report discusses the implications of HHT, the multidisciplinary planning of the caesarean section, intraoperative anaesthetic management and patient follow-up.
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Affiliation(s)
- Claudie Fogang D
- Department of Anaesthesiology, Clinical Pharmacology, Intensive Care and Emergency Medicine, Geneva University Hospitals, Geneva, Switzerland
| | - Gallice Martin
- Department of Anaesthesiology, Clinical Pharmacology, Intensive Care and Emergency Medicine, Geneva University Hospitals, Geneva, Switzerland
| | - Françoise Boehlen
- Division of Angiology and Hemostasis, Department of Medicine, Geneva University Hospitals, Geneva, Switzerland
- University of Geneva, Faculty of Medicine, Geneva, Switzerland
| | - Georges L Savoldelli
- Department of Anaesthesiology, Clinical Pharmacology, Intensive Care and Emergency Medicine, Geneva University Hospitals, Geneva, Switzerland
- University of Geneva, Faculty of Medicine, Geneva, Switzerland
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Ravindranayagam N, Vaz K, Gow P. Not Just Another "Bloody" Case of Right Heart Failure. Gastroenterology 2023; 165:538-540. [PMID: 37178736 DOI: 10.1053/j.gastro.2023.05.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2022] [Revised: 04/30/2023] [Accepted: 05/05/2023] [Indexed: 05/15/2023]
Affiliation(s)
| | - Karl Vaz
- Victorian Liver Transplant Unit, Austin Health, Melbourne, Victoria, Australia
| | - Paul Gow
- Department of Gastroenterology, Austin Hospital, Melbourne, Victoria, Australia; Department of Medicine, The University of Melbourne, Melbourne, Victoria, Australia
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Ielasi L, Tonnini M, Piscaglia F, Serio I. Current guidelines for diagnosis and management of hepatic involvement in hereditary hemorrhagic teleangiectasia. World J Hepatol 2023; 15:675-687. [PMID: 37305373 PMCID: PMC10251273 DOI: 10.4254/wjh.v15.i5.675] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/07/2023] [Revised: 04/04/2023] [Accepted: 04/12/2023] [Indexed: 05/24/2023] Open
Abstract
Hereditary hemorrhagic teleangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is the most common cause of hepatic vascular malformations in adults. Different vascular shunts (arteriovenous, arterioportal or portovenous) lead to different clinical manifestations. Even though no hepatic-related symptoms are reported in the majority of cases, the severity of liver disease could lead to refractory medical conditions, in some cases requiring liver transplantation. The aim of this manuscript is to provide an updated overview of the current evidence regarding the diagnosis and treatment of HHT liver involvement and liver-related complications.
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Affiliation(s)
- Luca Ielasi
- Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy
- Department of Internal Medicine, Ospedale per gli Infermi di Faenza, Faenza 48018, Italy
| | - Matteo Tonnini
- Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy
- Division of Internal Medicine, Hepatobiliary and Immunoallergic Diseases, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna 40138, Italy
| | - Fabio Piscaglia
- Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy
- Division of Internal Medicine, Hepatobiliary and Immunoallergic Diseases, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna 40138, Italy
| | - Ilaria Serio
- Division of Internal Medicine, Hepatobiliary and Immunoallergic Diseases, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna 40138, Italy
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Schmid CD, Olsavszky V, Reinhart M, Weyer V, Trogisch FA, Sticht C, Winkler M, Kürschner SW, Hoffmann J, Ola R, Staniczek T, Heineke J, Straub BK, Mittler J, Schledzewski K, ten Dijke P, Richter K, Dooley S, Géraud C, Goerdt S, Koch P. ALK1 controls hepatic vessel formation, angiodiversity, and angiocrine functions in hereditary hemorrhagic telangiectasia of the liver. Hepatology 2023; 77:1211-1227. [PMID: 35776660 PMCID: PMC10026949 DOI: 10.1002/hep.32641] [Citation(s) in RCA: 14] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/12/2021] [Revised: 06/26/2022] [Accepted: 06/27/2022] [Indexed: 12/21/2022]
Abstract
BACKGROUND AND AIMS In hereditary hemorrhagic telangiectasia (HHT), severe liver vascular malformations are associated with mutations in the Activin A Receptor-Like Type 1 ( ACVRL1 ) gene encoding ALK1, the receptor for bone morphogenetic protein (BMP) 9/BMP10, which regulates blood vessel development. Here, we established an HHT mouse model with exclusive liver involvement and adequate life expectancy to investigate ALK1 signaling in liver vessel formation and metabolic function. APPROACH AND RESULTS Liver sinusoidal endothelial cell (LSEC)-selective Cre deleter line, Stab2-iCreF3 , was crossed with Acvrl1 -floxed mice to generate LSEC-specific Acvrl1 -deficient mice ( Alk1HEC-KO ). Alk1HEC-KO mice revealed hepatic vascular malformations and increased posthepatic flow, causing right ventricular volume overload. Transcriptomic analyses demonstrated induction of proangiogenic/tip cell gene sets and arterialization of hepatic vessels at the expense of LSEC and central venous identities. Loss of LSEC angiokines Wnt2 , Wnt9b , and R-spondin-3 ( Rspo3 ) led to disruption of metabolic liver zonation in Alk1HEC-KO mice and in liver specimens of patients with HHT. Furthermore, prion-like protein doppel ( Prnd ) and placental growth factor ( Pgf ) were upregulated in Alk1HEC-KO hepatic endothelial cells, representing candidates driving the organ-specific pathogenesis of HHT. In LSEC in vitro , stimulation or inhibition of ALK1 signaling counter-regulated Inhibitors of DNA binding (ID)1-3, known Alk1 transcriptional targets. Stimulation of ALK1 signaling and inhibition of ID1-3 function confirmed regulation of Wnt2 and Rspo3 by the BMP9/ALK1/ID axis. CONCLUSIONS Hepatic endothelial ALK1 signaling protects from development of vascular malformations preserving organ-specific endothelial differentiation and angiocrine signaling. The long-term surviving Alk1HEC-KO HHT model offers opportunities to develop targeted therapies for this severe disease.
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Affiliation(s)
- Christian David Schmid
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Victor Olsavszky
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Manuel Reinhart
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Vanessa Weyer
- Department of Neuroradiology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- Department of Radiation Oncology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Felix A. Trogisch
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- Department of Cardiovascular Physiology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- DZHK (German Center for Cardiovascular Research), partner site Heidelberg/Mannheim, Mannheim, Germany
| | - Carsten Sticht
- Core Facility Platform Mannheim, NGS Core Facility, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Manuel Winkler
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Sina W. Kürschner
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Johannes Hoffmann
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Roxana Ola
- Department of Cardiovascular Pharmacology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Theresa Staniczek
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Joerg Heineke
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- Department of Cardiovascular Physiology, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- DZHK (German Center for Cardiovascular Research), partner site Heidelberg/Mannheim, Mannheim, Germany
| | - Beate K. Straub
- Institute of Pathology, University Medical Center of the Johannes Gutenberg‐University Mainz, Mainz, Germany
| | - Jens Mittler
- Department of General, Visceral, and Transplant Surgery, University Medical Center of the Johannes Gutenberg‐University Mainz, Mainz, Germany
| | - Kai Schledzewski
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- European Center for Angioscience, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Peter ten Dijke
- Oncode Institute, Department of Cell and Chemical Biology, Leiden University Medical Center, Leiden, The Netherlands
| | - Karsten Richter
- Division of Molecular Genetics, German Cancer Research Center (DKFZ), Heidelberg, Germany
| | - Steven Dooley
- Department of Medicine II, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Cyrill Géraud
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
- Section of Clinical and Molecular Dermatology, Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Sergij Goerdt
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Philipp‐Sebastian Koch
- Department of Dermatology, Venereology and Allergology, University Medical Center and Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
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Perioperative Complications and Long-Term Follow-Up of Liver Transplantation in Hemorrhagic Hereditary Telangiectasia: Report of Three Cases and Systematic Review. J Clin Med 2022; 11:jcm11195624. [PMID: 36233492 PMCID: PMC9573297 DOI: 10.3390/jcm11195624] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/03/2022] [Revised: 09/11/2022] [Accepted: 09/19/2022] [Indexed: 11/17/2022] Open
Abstract
The aim was to describe three patients with hemorrhagic hereditary telangiectasia (HHT) requiring liver transplantation (LT) and to perform a systematic review focusing on surgical complications and long-term follow-up. Unrestricted searches of the Medline and Embase databases were performed through February 2022. Forty-five studies were selected including 80 patients plus the three new reported patients, 68 (81.9%) were female and mean age was 50 (27–72) years. Main indications for LT were high-output cardiac failure (n = 40; 48.2%), ischemic cholangitis (n = 19; 22.9%), and a combination of both conditions (n = 13;15.6%). Mean cold ischemic time and red blood cell units transfused during LT were 554 (300–941) minutes and 11.4 (0–88) units, respectively. Complications within 30 days were described in 28 (33.7%) patients, mainly bleeding complications in 13 patients, hepatic artery (HA) thrombosis in four and hepatic vein thrombosis in one. Mean follow-up was 76.4 (1–288) months, and during it, four new patients developed thrombotic complications in HA, HA aneurysm, celiac artery, and the portal–splenic–mesenteric vein. HHT relapse in the transplant allograft was detected in 13 (17.1%) patients after 1–19 years (including two fatal recurrences). Overall mortality was 12%. In conclusion, previous assessment of HA anatomy and hyperdynamic circulatory state could reduce LT complications. The risk of relapse in the hepatic graft supports a multidisciplinary follow-up for HHT patients with LT.
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Donato P, Facas J, Alves FC. Hepatic Vascular Disorders: From Diagnosis to Interventional Radiology. Semin Ultrasound CT MR 2022; 43:466-475. [DOI: 10.1053/j.sult.2022.06.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
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Cusumano LR, Tesoriero JA, Wilsen CB, Sayre J, Quirk M, McWilliams JP. Predictors of heart failure symptoms in hereditary hemorrhagic telangiectasia patients with hepatic arteriovenous malformations. Orphanet J Rare Dis 2021; 16:478. [PMID: 34794458 PMCID: PMC8600745 DOI: 10.1186/s13023-021-02109-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/18/2021] [Accepted: 11/06/2021] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Hepatic arteriovenous malformations (AVMs) in hereditary hemorrhagic telangiectasia (HHT) patients are most commonly hepatic artery to hepatic venous shunts which can result in high-output heart failure. This condition can be debilitating and is a leading cause of liver transplantation in HHT patients. However, it is not known what characteristics can discriminate between asymptomatic patients and those who will develop heart failure symptoms. RESULTS 176 patients with HHT were evaluated with computed tomography angiography (CTA) between April 2004 and February 2019 at our HHT Center of Excellence. 63/176 (35.8%) patients were found to have hepatic AVMs on CTA. 18 of these patients were excluded because of the presence of another condition which could confound evaluation of heart failure symptoms. In the remaining 45 patients included in our cohort, 25/45 (55.6%) patients were classified as asymptomatic and 20/45 (44.4%) were classified as symptomatic, and these groups were compared. In symptomatic patients, mean common hepatic artery (CHA) diameter was significantly higher (11.1 versus 8.4 mm) and mean hemoglobin levels were significantly lower (10.7 vs 12.6 g/dL). A stepwise multiple logistic regression analysis demonstrated that both CHA diameter and hemoglobin level were independent predictors of heart failure symptoms with ORs of 2.554 (95% CI 1.372-4.754) and 0.489 (95% CI 0.299-0.799), respectively. The receiver operator characteristic (ROC) curve of our analysis demonstrated an AUC of 0.906 (95% CI 0.816-0.996), sensitivity 80.0% (95% CI 55.7-93.4%), and specificity 75.0% (95% CI 52.9-89.4%). CONCLUSIONS CTA is an effective and easily reproducible method to evaluate hepatic involvement of HHT. Utilizing CTA, clinical, and laboratory data we determined CHA diameter and hemoglobin level were independent predictors of heart failure symptoms.
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Affiliation(s)
- Lucas R Cusumano
- Division of Interventional Radiology, Department of Radiology, David Geffen School of Medicine at UCLA, 757 Westwood Plaza, 2nd Floor, Room 2125, Los Angeles, CA, 90095, USA
| | - Joseph A Tesoriero
- Division of Interventional Radiology, Department of Radiology, David Geffen School of Medicine at UCLA, 757 Westwood Plaza, 2nd Floor, Room 2125, Los Angeles, CA, 90095, USA
| | - Craig B Wilsen
- Division of Interventional Radiology, Department of Radiology, David Geffen School of Medicine at UCLA, 757 Westwood Plaza, 2nd Floor, Room 2125, Los Angeles, CA, 90095, USA
| | - James Sayre
- Department of Biostatistics, Fielding School of Public Health, University of California, Los Angeles, CA, USA
| | - Matthew Quirk
- Division of Interventional Radiology, Department of Radiology, David Geffen School of Medicine at UCLA, 757 Westwood Plaza, 2nd Floor, Room 2125, Los Angeles, CA, 90095, USA
| | - Justin P McWilliams
- Division of Interventional Radiology, Department of Radiology, David Geffen School of Medicine at UCLA, 757 Westwood Plaza, 2nd Floor, Room 2125, Los Angeles, CA, 90095, USA.
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Ramanathan S, Raghu V, Virmani V, Sheikh A, Al Heidous M, Tirumani S. Unveiling the unreal: Comprehensive imaging review of hepatic pseudolesions. Clin Imaging 2021; 80:439-453. [PMID: 34560516 DOI: 10.1016/j.clinimag.2021.09.008] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2021] [Revised: 08/06/2021] [Accepted: 09/06/2021] [Indexed: 12/25/2022]
Abstract
Hepatic pseudolesions are defined as non-neoplastic focal abnormalities of the liver which can mimic or conceal true liver lesions. It is particularly common in liver due to its unique dual blood supply and the existence of multilevel anastomosis between them. Because of the recent advances in CT and MRI technology, they are being increasingly encountered in daily practice. Broadly they can be categorised in to (1) Focal parenchymal abnormalities like focal fatty change, focal fat sparing, focal confluent fibrosis, segmental hypertrophy and regenerative nodules, (2) Perfusion abnormalities which include transient hepatic parenchymal enhancement in portal vein obstruction, third inflow, intrahepatic shunts, hepatic arterial occlusion and hepatic venous obstruction, (3) Imaging pitfalls like parenchymal compression, unenhanced vessels and pseudolipoma. It is essential for the radiologists to be familiar with the typical and atypical imaging features of pseudolesions to avoid mistaking them for sinister pathologies and also to avoid overlooking underlying hidden pathologies.
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Affiliation(s)
- Subramaniyan Ramanathan
- Department of Clinical imaging, Al-Wakra Hospital, Hamad Medical Corporation, PO Box: 82228, Doha, Qatar; Department of Radiology, Weil Cornell Medical College, Doha, Qatar.
| | - Vineetha Raghu
- Department of Radiology, Columbia Asia Referral Hospital, Yeshwanthpur, India
| | - Vivek Virmani
- Department of Radiology, Dr. Everett Chalmers Hospital, Fredericton, Canada
| | - Adnan Sheikh
- Department of Emergency and Trauma Radiology, University of British Columbia, Vancouver, Canada
| | - Mahmoud Al Heidous
- Department of Clinical imaging, Al-Wakra Hospital, Hamad Medical Corporation, PO Box: 82228, Doha, Qatar; Department of Radiology, Weil Cornell Medical College, Doha, Qatar
| | - SreeHarsha Tirumani
- Department of Radiology, University Hospitals Cleveland Medical Centre, Case Western Reserve University, Cleveland, OH, USA
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Faughnan ME, Mager JJ, Hetts SW, Palda VA, Lang-Robertson K, Buscarini E, Deslandres E, Kasthuri RS, Lausman A, Poetker D, Ratjen F, Chesnutt MS, Clancy M, Whitehead KJ, Al-Samkari H, Chakinala M, Conrad M, Cortes D, Crocione C, Darling J, de Gussem E, Derksen C, Dupuis-Girod S, Foy P, Geisthoff U, Gossage JR, Hammill A, Heimdal K, Henderson K, Iyer VN, Kjeldsen AD, Komiyama M, Korenblatt K, McDonald J, McMahon J, McWilliams J, Meek ME, Mei-Zahav M, Olitsky S, Palmer S, Pantalone R, Piccirillo JF, Plahn B, Porteous MEM, Post MC, Radovanovic I, Rochon PJ, Rodriguez-Lopez J, Sabba C, Serra M, Shovlin C, Sprecher D, White AJ, Winship I, Zarrabeitia R. Second International Guidelines for the Diagnosis and Management of Hereditary Hemorrhagic Telangiectasia. Ann Intern Med 2020; 173:989-1001. [PMID: 32894695 DOI: 10.7326/m20-1443] [Citation(s) in RCA: 265] [Impact Index Per Article: 53.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/18/2023] Open
Abstract
DESCRIPTION Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease with an estimated prevalence of 1 in 5000 that is characterized by the presence of vascular malformations (VMs). These result in chronic bleeding, acute hemorrhage, and complications from shunting through VMs. The goal of the Second International HHT Guidelines process was to develop evidence-based consensus guidelines for the management and prevention of HHT-related symptoms and complications. METHODS The guidelines were developed using the AGREE II (Appraisal of Guidelines for Research and Evaluation II) framework and GRADE (Grading of Recommendations Assessment, Development and Evaluation) methodology. The guidelines expert panel included expert physicians (clinical and genetic) in HHT from 15 countries, guidelines methodologists, health care workers, health care administrators, patient advocacy representatives, and persons with HHT. During the preconference process, the expert panel generated clinically relevant questions in 6 priority topic areas. A systematic literature search was done in June 2019, and articles meeting a priori criteria were included to generate evidence tables, which were used as the basis for recommendation development. The expert panel subsequently convened during a guidelines conference to conduct a structured consensus process, during which recommendations reaching at least 80% consensus were discussed and approved. RECOMMENDATIONS The expert panel generated and approved 6 new recommendations for each of the following 6 priority topic areas: epistaxis, gastrointestinal bleeding, anemia and iron deficiency, liver VMs, pediatric care, and pregnancy and delivery (36 total). The recommendations highlight new evidence in existing topics from the first International HHT Guidelines and provide guidance in 3 new areas: anemia, pediatrics, and pregnancy and delivery. These recommendations should facilitate implementation of key components of HHT care into clinical practice.
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Affiliation(s)
- Marie E Faughnan
- St. Michael's Hospital, Li Ka Shing Knowledge Institute, and University of Toronto, Toronto, Ontario, Canada (M.E.F.)
| | | | - Steven W Hetts
- University of California, San Francisco, San Francisco, California (S.W.H., M.C.)
| | | | | | | | - Erik Deslandres
- Centre Hospitalier de l'Université de Montréal, Hôtel-Dieu, Montreal, Quebec, Canada (E.D.)
| | - Raj S Kasthuri
- University of North Carolina at Chapel Hill, Chapel Hill, North Carolina (R.S.K., J.D.)
| | - Andrea Lausman
- St. Michael's Hospital and University of Toronto, Toronto, Ontario, Canada (A.L., R.P.)
| | - David Poetker
- Froedtert and Medical College of Wisconsin, Milwaukee, Wisconsin (D.P., P.F.)
| | - Felix Ratjen
- The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada (F.R.)
| | - Mark S Chesnutt
- VA Portland Health Care System and Oregon Health & Science University, Portland, Oregon (M.S.C.)
| | | | - Kevin J Whitehead
- University of Utah Medical Center, Salt Lake City, Utah (K.J.W., J.M.)
| | - Hanny Al-Samkari
- Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts (H.A.)
| | - Murali Chakinala
- Washington University School of Medicine, St. Louis, Missouri (M.C., K.K., J.F.P., A.J.W.)
| | - Miles Conrad
- University of California, San Francisco, San Francisco, California (S.W.H., M.C.)
| | - Daniel Cortes
- St. Michael's Hospital and Unity Health Toronto, Toronto, Canada (D.C.)
| | | | - Jama Darling
- University of North Carolina at Chapel Hill, Chapel Hill, North Carolina (R.S.K., J.D.)
| | | | | | | | - Patrick Foy
- Froedtert and Medical College of Wisconsin, Milwaukee, Wisconsin (D.P., P.F.)
| | - Urban Geisthoff
- University Hospital of Marburg and Phillips University Marburg, Marburg, Germany (U.G.)
| | | | - Adrienne Hammill
- Cincinnati Children's Hospital and University of Cincinnati, Cincinnati, Ohio (A.H.)
| | - Ketil Heimdal
- Oslo University Hospital, Rikshospitalet, Oslo, Norway (K.H.)
| | | | | | | | | | - Kevin Korenblatt
- Washington University School of Medicine, St. Louis, Missouri (M.C., K.K., J.F.P., A.J.W.)
| | - Jamie McDonald
- University of Utah Medical Center, Salt Lake City, Utah (K.J.W., J.M.)
| | | | - Justin McWilliams
- University of California, Los Angeles, Los Angeles, California (J.M.)
| | - Mary E Meek
- University of Arkansas for Medical Sciences, Little Rock, Arkansas (M.E.M.)
| | - Meir Mei-Zahav
- Schneider Children's Medical Center of Israel and Sackler School of Medicine of Tel Aviv University, Tel Aviv, Israel (M.M.)
| | | | | | - Rose Pantalone
- St. Michael's Hospital and University of Toronto, Toronto, Ontario, Canada (A.L., R.P.)
| | - Jay F Piccirillo
- Washington University School of Medicine, St. Louis, Missouri (M.C., K.K., J.F.P., A.J.W.)
| | | | | | - Marco C Post
- St. Antonius Hospital, Nieuwegein, and University Medical Center Utrecht, Utrecht, the Netherlands (M.C.P.)
| | - Ivan Radovanovic
- Toronto Western Hospital and University of Toronto, Toronto, Ontario, Canada (I.R.)
| | - Paul J Rochon
- University of Colorado Hospital, Aurora, Colorado (P.J.R.)
| | | | | | - Marcelo Serra
- Hospital Italiano de Buenos Aires, Buenos Aires, Argentina (M.S.)
| | | | | | - Andrew J White
- Washington University School of Medicine, St. Louis, Missouri (M.C., K.K., J.F.P., A.J.W.)
| | - Ingrid Winship
- Royal Melbourne Hospital and University of Melbourne, Melbourne, Victoria, Australia (I.W.)
| | - Roberto Zarrabeitia
- Hospital Sierrallana (Servicio Cántabro de Salud), Torrelavega, Spain (R.Z.)
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11
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Harwin J, Sugi MD, Hetts SW, Conrad MB, Ohliger MA. The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia. J Clin Med 2020; 9:jcm9113750. [PMID: 33233377 PMCID: PMC7700186 DOI: 10.3390/jcm9113750] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2020] [Revised: 11/03/2020] [Accepted: 11/04/2020] [Indexed: 01/01/2023] Open
Abstract
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by spontaneous epistaxis, telangiectasia, and visceral vascular malformations. Hepatic vascular malformations are common, though a minority are symptomatic. Symptoms are dependent on the severity and exact type of shunting caused by the hepatic malformation: Arteriosystemic shunting leads to manifestations of high output cardiac failure, and arterioportal shunting leads to portal hypertension. Radiologic imaging, including ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), is an important tool for assessing liver involvement. Doppler ultrasonography is the first-line screening modality for HHT-related liver disease, and it has a standardized scale. Imaging can determine whether shunting is principally to the hepatic vein or the portal vein, which can be a key determinant of patients’ symptoms. Liver-related complications can be detected, including manifestations of portal hypertension, focal liver masses as well as ischemic cholangiopathy. Ultrasound and MRI also have the ability to quantify blood flow through the liver, which in the future may be used to determine prognosis and direct antiangiogenic therapy.
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Affiliation(s)
- Joelle Harwin
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA; (J.H.); (M.D.S.); (S.W.H.); (M.B.C.)
| | - Mark D. Sugi
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA; (J.H.); (M.D.S.); (S.W.H.); (M.B.C.)
- Department of Radiology, Zuckerberg San Francisco General Hospital, San Francisco, CA 94110, USA
| | - Steven W. Hetts
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA; (J.H.); (M.D.S.); (S.W.H.); (M.B.C.)
- Department of Radiology, Zuckerberg San Francisco General Hospital, San Francisco, CA 94110, USA
| | - Miles B. Conrad
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA; (J.H.); (M.D.S.); (S.W.H.); (M.B.C.)
- Department of Radiology, Zuckerberg San Francisco General Hospital, San Francisco, CA 94110, USA
| | - Michael A. Ohliger
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA 94143, USA; (J.H.); (M.D.S.); (S.W.H.); (M.B.C.)
- Department of Radiology, Zuckerberg San Francisco General Hospital, San Francisco, CA 94110, USA
- Correspondence:
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12
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Ammonia Predicts Hepatic Involvement and Pulmonary Hypertension in Patients With Hereditary Hemorrhagic Telangiectasia. Clin Transl Gastroenterol 2020; 11:e00118. [PMID: 31977452 PMCID: PMC7056052 DOI: 10.14309/ctg.0000000000000118] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
INTRODUCTION: Hepatic involvement in hereditary hemorrhagic telangiectasia (HHT) is common and can be associated with severe clinical consequences, including portal hypertension, cardiac failure, and encephalopathy. However, there are no reliable clinical predictors of hepatic involvement and its associated complications, limiting appropriate identification of these patients. In this work, we define the utility of serum ammonia and liver biochemical tests (LFTs) in predicting hepatic HHT involvement and its complications. METHODS: We performed a retrospective study examining a well-characterized cohort of patients with HHT. Clinical characteristics, laboratory tests, liver imaging, transthoracic echocardiography assessment of right ventricular systolic pressure (RVSP), and history of other HHT-related outcomes were assessed. Patients were followed for the development of encephalopathy. RESULTS: Of 45 patients with definite HHT, 18 (40%) had elevated ammonia levels. An elevated ammonia associated with the presence of hepatic arteriovenous malformations (AVMs) on imaging (P < 0.03) and when combined with elevated liver tests increased the sensitivity for hepatic AVMs by 18% (55% for LFTs vs 73% for LFTs plus ammonia). Furthermore, an elevated serum ammonia in patients with HHT associated with an elevated RVSP (>35 mm Hg), providing an 80% sensitivity and 71% specificity for predicting the presence of pulmonary hypertension. In contrast, there was no association with an elevated serum ammonia and encephalopathy over a total of 859 months of follow-up. DISCUSSION: Elevated ammonia in a cohort of patients with HHT was associated with the presence of hepatic AVMs and elevated RVSP, but no other complications of HHT, including encephalopathy.
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13
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Vernuccio F, Porrello G, Cannella R, Vernuccio L, Midiri M, Giannitrapani L, Soresi M, Brancatelli G. Benign and malignant mimickers of infiltrative hepatocellular carcinoma: tips and tricks for differential diagnosis on CT and MRI. Clin Imaging 2020; 70:33-45. [PMID: 33120287 DOI: 10.1016/j.clinimag.2020.10.011] [Citation(s) in RCA: 17] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/01/2020] [Revised: 09/23/2020] [Accepted: 10/07/2020] [Indexed: 12/13/2022]
Abstract
Hepatocellular carcinoma (HCC) may have an infiltrative appearance in about 8-20% of cases. Infiltrative HCC can be a challenging diagnosis and it is associated with the worst overall survival among HCC patients. Infiltrative HCC is characterized by the spread of multiple minute nodules throughout the liver, without a dominant one, ultimately resulting into macrovascular invasion. On CT and MRI, infiltrative HCC appears as an ill-defined, large mass, with variable degree of enhancement, and satellite neoplastic nodules in up to 52% of patients. On MRI, it may show restriction on diffusion weighted imaging, hyperintensity on T2- and hypointensity on T1-weighted images, and, if hepatobiliary agent is used, hypointensity on hepatobiliary phase. Infiltrative HCC must be differentiated from other liver diseases, such as focal confluent fibrosis, steatosis, amyloidosis, vascular disorders of the liver, cholangiocarcinoma, and diffuse metastatic disease. In cirrhotic patients, the identification of vascular tumor invasion of the portal vein and its differentiation from bland thrombosis is of utmost importance for patient management. On contrast enhanced CT and MRI, portal vein tumor thrombosis appears as an enhancing thrombus within the portal vein, close to the main tumor and results into vein enlargement. The aim of this pictorial review is to show CT and MRI features that allow the diagnosis of infiltrative HCC and portal vein tumor thrombosis. A particular point of interest includes the tips and tricks for differential diagnosis with potential mimickers of infiltrative HCC.
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Affiliation(s)
- Federica Vernuccio
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy; University Paris Diderot, Sorbonne Paris Cité, Paris, France; I.R.C.C.S. Centro Neurolesi Bonino Pulejo, Contrada Casazza, SS113, 98124 Messina, Italy; Department of Biomedicine, Neuroscience and Advanced Diagnostics (BIND), University Hospital of Palermo, Via del Vespro 129, 90127 Palermo, Italy.
| | - Giorgia Porrello
- Department of Biomedicine, Neuroscience and Advanced Diagnostics (BIND), University Hospital of Palermo, Via del Vespro 129, 90127 Palermo, Italy
| | - Roberto Cannella
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy; Department of Biomedicine, Neuroscience and Advanced Diagnostics (BIND), University Hospital of Palermo, Via del Vespro 129, 90127 Palermo, Italy
| | - Laura Vernuccio
- U.O.C. Geriatria e Lungodegenza, AOUP University Hospital Palermo, CDCD Geriatria, Palermo, Italy
| | - Massimo Midiri
- Department of Biomedicine, Neuroscience and Advanced Diagnostics (BIND), University Hospital of Palermo, Via del Vespro 129, 90127 Palermo, Italy
| | - Lydia Giannitrapani
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy
| | - Maurizio Soresi
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy
| | - Giuseppe Brancatelli
- Department of Biomedicine, Neuroscience and Advanced Diagnostics (BIND), University Hospital of Palermo, Via del Vespro 129, 90127 Palermo, Italy
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14
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Naganuma H, Ishida H, Kuroda H, Suzuki Y, Ogawa M. Hereditary hemorrhagic telangiectasia: how to efficiently detect hepatic abnormalities using ultrasonography. J Med Ultrason (2001) 2020; 47:421-433. [PMID: 32390074 DOI: 10.1007/s10396-020-01022-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/03/2020] [Accepted: 03/30/2020] [Indexed: 12/12/2022]
Abstract
INTRODUCTION Hereditary hemorrhagic telangiectasia (HHT) is a multiorgan genetic angiodysplastic affection characterized by visceral vascular malformations. It affects mainly the brains, lungs, gastrointestinal tract, and nasal mucosa. Unlike those organs, hepatic involvement, although very frequently occurring, is insufficiently recognized, mainly because of the complex vascular structure of this organ. Thus, treating HHT patients requires a solid understanding of these hepatic anomalies. It is especially important for any general clinicians to be able to recognize clinical findings in HHT, which leads to a high suspicion of HHT and have an index of suspicion for liver abnormalities of HHT. For this purpose, keen awareness of clinical as well as hepatic sonographic (US) findings is paramount. AIM The aim of this review is to summarize previously reported findings on the hepatic US through a thorough analysis of related articles, and to (a) determine the role of US in the diagnosis of hepatic involvement in HHT patients and (b) propose the most simple and easy way to detect HHT-related abnormalities during routine US examinations. CONCLUSION Hepatic US serves to diagnose the detailed complex hepatic changes typical of HHT, and contributes to increased diagnostic confidence of hepatic changes in HHT patients, with the most simple way not to overlook HHT-related abnormalities being to find hepatic artery dilatation.
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Affiliation(s)
- Hiroko Naganuma
- Department of Gastroenterology, Yokote Municipal Hospital, 5-31 Negishi-cho, Yokote, Akita, 013-8602, Japan.
| | - Hideaki Ishida
- Center of Diagnostic Ultrasound, Akita Red Cross Hospital, Akita, Japan
| | - Hidekatsu Kuroda
- Division of Hepatology, Department of Internal Medicine, Iwate Medical University, Iwate, Japan
| | - Yasuaki Suzuki
- Department of Gastroenterology, Nayoro City General Hospital, Hokkaido, Japan
| | - Masahiro Ogawa
- Department of Gastroenterology and Hepatology, Nihon University Hospital, Tokyo, Japan
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15
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Abstract
Disorders of the mesenteric, portal, and hepatic veins and mesenteric and hepatic arteries have important clinical consequences and may lead to acute liver failure, chronic liver disease, noncirrhotic portal hypertension, cirrhosis, and hepatocellular carcinoma. Although literature in the field of vascular liver disorders is scant, these disorders are common in clinical practice, and general practitioners, gastroenterologists, and hepatologists may benefit from expert guidance and recommendations for management of these conditions. These guidelines represent the official practice recommendations of the American College of Gastroenterology. Key concept statements based on author expert opinion and review of literature and specific recommendations based on PICO/GRADE analysis have been developed to aid in the management of vascular liver disorders. These recommendations and guidelines should be tailored to individual patients and circumstances in routine clinical practice.
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16
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Tortora A, Riccioni ME, Gaetani E, Ojetti V, Holleran G, Gasbarrini A. Rendu-Osler-Weber disease: a gastroenterologist's perspective. Orphanet J Rare Dis 2019; 14:130. [PMID: 31174568 PMCID: PMC6555961 DOI: 10.1186/s13023-019-1107-4] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2019] [Accepted: 05/27/2019] [Indexed: 12/17/2022] Open
Abstract
Hereditary hemorrhagic teleangectasia (HHT, or Rendu-Osler-Weber disease) is a rare inherited syndrome, characterized by arterio-venous malformations (AVMs or Telangiectasia). The most important and common manifestation is nose bleeds (epistaxis). The telangiectasias (small AVMs) are most evident on the lips, tongue, buccal mucosa, face, chest, and fingers, however; large arterio-venous malformations can also occur in the lungs, liver, pancreas, or brain. Telangiectasias in the upper gastrointestinal tract are known to occur, however data regarding possible small-bowel involvement is limited due to technical difficulties in visualizing the entire gastrointestinal tract. The occurrence of AVMs in the stomach and small bowel can result in chronic bleeding and anaemia. Less frequently, this may occur due to bleeding from oesophageal varices, as patients with HHT can develop hepatic parenchymal AVMs or vascular shunts which cause hepatic cirrhosis and portal hypertension. Gastroenterologists have a crucial role in the management of these patients, however difficulties remain in the detection and management of complications of HHT in the gastrointestinal tract.
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Affiliation(s)
- Annalisa Tortora
- Fondazione Policlinico Universitario A Gemelli IRCCS, Roma, Italy. .,Università Cattolica del Sacro Cuore, Roma, Italy.
| | - Maria Elena Riccioni
- Fondazione Policlinico Universitario A Gemelli IRCCS, Roma, Italy.,Università Cattolica del Sacro Cuore, Roma, Italy
| | - Eleonora Gaetani
- Fondazione Policlinico Universitario A Gemelli IRCCS, Roma, Italy.,Università Cattolica del Sacro Cuore, Roma, Italy
| | - Veronica Ojetti
- Fondazione Policlinico Universitario A Gemelli IRCCS, Roma, Italy.,Università Cattolica del Sacro Cuore, Roma, Italy
| | - Grainne Holleran
- Department of Clinical Medicine, Trinity College Dublin, Dublin 2, Ireland
| | - Antonio Gasbarrini
- Fondazione Policlinico Universitario A Gemelli IRCCS, Roma, Italy.,Università Cattolica del Sacro Cuore, Roma, Italy
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17
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Rodrigues RA, Rodrigues RA, Freitas VC, Araujo ALED, Parente DB. Radiological findings in the liver of a patient with Rendu-Osler-Weber syndrome. Radiol Bras 2019; 52:200-201. [PMID: 31210698 PMCID: PMC6561370 DOI: 10.1590/0100-3984.2017.0158] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Affiliation(s)
| | | | | | | | - Daniella Braz Parente
- Universidade Federal do Rio de Janeiro, Brazil; Instituto D'Or de Pesquisa e Ensino, Brazil
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18
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Specchi S, Rossi F, Weisse C, Morabito S, Petrovitch NP, Drees R, Thierry F, Ricciardi M, Penchome R, Armenise A, Pey P, Paek M, Panopoulos I, Nicoli S, Schwarz T. Canine and feline abdominal arterioportal communications can be classified based on branching patterns in computed tomographic angiography. Vet Radiol Ultrasound 2018; 59:687-696. [DOI: 10.1111/vru.12685] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2018] [Revised: 07/04/2018] [Accepted: 07/12/2018] [Indexed: 11/26/2022] Open
Affiliation(s)
- Swan Specchi
- Diagnostic Imaging; Istituto Veterinario di Novara; Granozzo con Monticello Novara 28060 Italy
| | | | - Chick Weisse
- Animal Medical Center; New York New York 10065 USA
| | - Simona Morabito
- Diagnostic Imaging; Istituto Veterinario di Novara; Granozzo con Monticello Novara 28060 Italy
| | | | - Randi Drees
- The Royal Veterinary College; Hatfield AL97TA UK
| | - Florence Thierry
- Royal (Dick) School of Veterinary Studies & Roslin Institute; University of Edinburgh; Roslin EH25 9RG UK
| | - Mario Ricciardi
- Ospedale Veterinario; Pingry via delle Medaglie d'Oro 5; Bari 70126 Italy
| | - Rampaipat Penchome
- Diagnostic Imaging Unit; Faculty of Veterinary Science; Small Animal Teaching Hospital; Chulalongkorn University; Bangkok Thailand
| | | | - Pascaline Pey
- École Nationale Vétérinaire d'Alfort; Maisons-Alfort 94799 France
| | - Matthew Paek
- Synergy Veterinary Imaging Partners, Radiology; Maryland 21029 USA
| | - Ioannis Panopoulos
- Diagnostic Imaging; Istituto Veterinario di Novara; Granozzo con Monticello Novara 28060 Italy
- Alphavet Veterinary Clinic; Athens 14564 Greece
| | - Stefano Nicoli
- Diagnostic Imaging; Istituto Veterinario di Novara; Granozzo con Monticello Novara 28060 Italy
- Clinica Veterinaria Roma Sud; Roma 00173 Italy
| | - Tobias Schwarz
- Royal (Dick) School of Veterinary Studies & Roslin Institute; University of Edinburgh; Roslin EH25 9RG UK
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Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report. Radiol Case Rep 2018; 13:51-54. [PMID: 29552242 PMCID: PMC5851303 DOI: 10.1016/j.radcr.2017.10.011] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2017] [Revised: 10/07/2017] [Accepted: 10/08/2017] [Indexed: 01/10/2023] Open
Abstract
Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients with hereditary hemorrhagic telangiectasia), although symptomatic liver disease is quite infrequent. In symptomatic cases, clinical manifestations relate most commonly to the predominant type of vascular shunting present (arteriovenous, arterioportal, or portovenous). Clinically, liver disease can manifest as a high-output cardiac failure, portal hypertension, or biliary disease. Imaging plays an important role in diagnosis, characterization, and follow-up of liver involvement, with ultrasound, computed tomography, magnetic resonance imaging, and angiography being useful in this context. We present a case of congestive cirrhosis with florid liver findings in a patient with Osler-Weber-Rendu syndrome. Imaging findings that clinched the diagnosis are reviewed. A brief literature review is also provided.
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Martín-Llahí M, Albillos A, Bañares R, Berzigotti A, García-Criado MÁ, Genescà J, Hernández-Gea V, Llop-Herrera E, Masnou-Ridaura H, Mateo J, Navascués CA, Puente Á, Romero-Gutiérrez M, Simón-Talero M, Téllez L, Turon F, Villanueva C, Zarrabeitia R, García-Pagán JC. Enfermedades vasculares del hígado. Guías Clínicas de la Sociedad Catalana de Digestología y de la Asociación Española para el Estudio del Hígado. GASTROENTEROLOGIA Y HEPATOLOGIA 2017; 40:538-580. [PMID: 28610817 DOI: 10.1016/j.gastrohep.2017.03.011] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/13/2017] [Accepted: 03/29/2017] [Indexed: 12/11/2022]
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21
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Typical and atypical benign liver lesions: A review. Clin Imaging 2017; 44:79-91. [PMID: 28486156 DOI: 10.1016/j.clinimag.2017.05.002] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/01/2017] [Revised: 04/10/2017] [Accepted: 05/01/2017] [Indexed: 02/06/2023]
Abstract
Focal liver lesions are routinely encountered by clinical radiologists and represent a wide spectrum of pathology. Majority of these lesions are likely to be benign in nature, especially in the absence of chronic liver disease or primary cancer. A radiologist must be aware of common and uncommon imaging features of benign lesions across the various imaging modalities. This review discusses pathognomonic imaging features of common benign focal liver lesions seen on ultrasound, computed tomography and magnetic resonance, and adds to existing knowledge with the recent updates to have emerged in this area.
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22
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Elsayes KM, Shaaban AM, Rothan SM, Javadi S, Madrazo BL, Castillo RP, Casillas VJ, Menias CO. A Comprehensive Approach to Hepatic Vascular Disease. Radiographics 2017; 37:813-836. [DOI: 10.1148/rg.2017160161] [Citation(s) in RCA: 48] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Affiliation(s)
- Khaled M. Elsayes
- From the Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030 (K.M.E., S.J.); Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, Utah (A.M.S.); Department of Diagnostic and Interventional Imaging, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, Tex (S.M.R.); Department of Diagnostic Radiology, University of Miami Health System, Miami, Fla (B.L.M., R.P.C., V.J.C.)
| | - Akram M. Shaaban
- From the Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030 (K.M.E., S.J.); Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, Utah (A.M.S.); Department of Diagnostic and Interventional Imaging, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, Tex (S.M.R.); Department of Diagnostic Radiology, University of Miami Health System, Miami, Fla (B.L.M., R.P.C., V.J.C.)
| | - Sarah M. Rothan
- From the Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030 (K.M.E., S.J.); Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, Utah (A.M.S.); Department of Diagnostic and Interventional Imaging, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, Tex (S.M.R.); Department of Diagnostic Radiology, University of Miami Health System, Miami, Fla (B.L.M., R.P.C., V.J.C.)
| | - Sanaz Javadi
- From the Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030 (K.M.E., S.J.); Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, Utah (A.M.S.); Department of Diagnostic and Interventional Imaging, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, Tex (S.M.R.); Department of Diagnostic Radiology, University of Miami Health System, Miami, Fla (B.L.M., R.P.C., V.J.C.)
| | - Beatrice L. Madrazo
- From the Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030 (K.M.E., S.J.); Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, Utah (A.M.S.); Department of Diagnostic and Interventional Imaging, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, Tex (S.M.R.); Department of Diagnostic Radiology, University of Miami Health System, Miami, Fla (B.L.M., R.P.C., V.J.C.)
| | - Rosa P. Castillo
- From the Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030 (K.M.E., S.J.); Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, Utah (A.M.S.); Department of Diagnostic and Interventional Imaging, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, Tex (S.M.R.); Department of Diagnostic Radiology, University of Miami Health System, Miami, Fla (B.L.M., R.P.C., V.J.C.)
| | - Victor J. Casillas
- From the Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030 (K.M.E., S.J.); Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, Utah (A.M.S.); Department of Diagnostic and Interventional Imaging, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, Tex (S.M.R.); Department of Diagnostic Radiology, University of Miami Health System, Miami, Fla (B.L.M., R.P.C., V.J.C.)
| | - Christine O. Menias
- From the Department of Diagnostic Radiology, University of Texas MD Anderson Cancer Center, 1400 Pressler St, Houston, TX 77030 (K.M.E., S.J.); Department of Radiology and Imaging Sciences, University of Utah, Salt Lake City, Utah (A.M.S.); Department of Diagnostic and Interventional Imaging, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, Tex (S.M.R.); Department of Diagnostic Radiology, University of Miami Health System, Miami, Fla (B.L.M., R.P.C., V.J.C.)
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Robaina Cabrera DM, Verde González MP, Tarazona Chocano B, Amado Fernández C, Zarrabeitia Puente R. Telangiectasia hemorrágica hereditaria: enfermedad de Rendu-Osler-Weber. ACTA ACUST UNITED AC 2016. [DOI: 10.1016/j.fmc.2016.01.011] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
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Song W, Zhao D, Li H, Ding J, He N, Chen Y. Liver Findings in Patients with Hereditary Hemorrhagic Telangiectasia. IRANIAN JOURNAL OF RADIOLOGY 2016; 13:e31116. [PMID: 27046775 PMCID: PMC5116567 DOI: 10.5812/iranjradiol.31116] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/29/2015] [Revised: 08/13/2015] [Accepted: 09/07/2015] [Indexed: 12/11/2022]
Abstract
Background Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a rare autosomal dominant genetic vascular dysplasia. Objectives The aim is to characterize the liver involvement of HHT by multi-detector helical computed tomography. Patients and Methods Through the use of multi-detector helical computed tomography, scan data derived from 12 cases of HHT were retrospectively studied, and an abdominal scan was performed on all of the patients. Results (i) Three types of shunting were found in the livers, including arteriovenous (hepatic artery to hepatic vein) in 6 cases, arterioportal (hepatic artery to portal vein) in 2 cases, and portal venous (portal vein to hepatic vein) in 4 cases, (ii) Biliary diseases were detected in 8 cases, including 3 cases of bilomas accompanied by increases of alkaline phosphatase (mean 271 units, ranging from 152 to 479 units) and arteriovenous shunts, and the other 5 cases revealed slight biliary dilation with no increase of alkaline phosphates, but were instead accompanied by arteriovenous shunts (3 cases) and portal venous shunts (2 cases). (iii) Vascular anatomic variants were observed in 4 cases, including 3 with accompanying arteriovenous shunts and 1 with accompanying arterioportal shunting. Conclusion The involved liver with HHT typically shows vascular shunting and biliary diseases. Also, arteriovenous shunts may be vulnerable to biliary diseases.
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Affiliation(s)
- Wenyan Song
- Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China
- Corresponding authors: Wenyan Song, Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China. Tel: +86-13611096669, E-mail: ; Dawei Zhao, Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China, E-mail:
| | - Dawei Zhao
- Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China
- Corresponding authors: Wenyan Song, Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China. Tel: +86-13611096669, E-mail: ; Dawei Zhao, Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China, E-mail:
| | - Hongjun Li
- Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China
| | - Jinli Ding
- Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China
| | - Ning He
- Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing, China
| | - Yu Chen
- Beijing Artificial Liver Treatment and Training Center, Beijing YouAn Hospital, Capital Medical University, Beijing, China
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25
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Cavel A, Bleuzen A, Bertrand P, Patat F, Cottier JP. Comparison between Doppler ultrasonography and multiphase multidetector-row computed tomography in the detection of liver involvement in Rendu-Osler disease: An analysis of 62 patients. Diagn Interv Imaging 2016; 97:451-9. [DOI: 10.1016/j.diii.2014.01.023] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/27/2013] [Revised: 01/07/2014] [Accepted: 01/16/2014] [Indexed: 12/14/2022]
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26
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Kim TK, Lee E, Jang HJ. Imaging findings of mimickers of hepatocellular carcinoma. Clin Mol Hepatol 2015; 21:326-43. [PMID: 26770920 PMCID: PMC4712159 DOI: 10.3350/cmh.2015.21.4.326] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/13/2015] [Accepted: 08/15/2015] [Indexed: 12/27/2022] Open
Abstract
Radiological imaging plays a crucial role in the diagnosis of hepatocellular carcinoma (HCC) as the noninvasive diagnosis of HCC in high-risk patients by typical imaging findings alone is widely adopted in major practice guidelines for HCC. While imaging techniques have markedly improved in detecting small liver lesions, they often detect incidental benign liver lesions and non-hepatocellular malignancy that can be misdiagnosed as HCC. The most common mimicker of HCC in cirrhotic liver is nontumorous arterioportal shunts that are seen as focal hypervascular liver lesions on dynamic contrast-enhanced cross-sectional imaging. Rapidly enhancing hemangiomas can be easily misdiagnosed as HCC especially on MR imaging with liver-specific contrast agent. Focal inflammatory liver lesions mimic HCC by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging. It is important to recognize the suggestive imaging findings for intrahepatic cholangiocarcinoma (CC) as the management of CC is largely different from that of HCC. There are other benign mimickers of HCC such as angiomyolipomas and focal nodular hyperplasia-like nodules. Recognition of their typical imaging findings can reduce false-positive HCC diagnosis.
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Affiliation(s)
- Tae Kyoung Kim
- Department of Medical Imaging, Toronto General Hospital, University of Toronto, Toronto, ON, Canada
| | - Eunchae Lee
- Department of Radiology, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Hyun-Jung Jang
- Department of Medical Imaging, Toronto General Hospital, University of Toronto, Toronto, ON, Canada
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Affiliation(s)
- Rafel Tappouni
- Department of Radiology, Wake Forest University School of Medicine, Winston-Salem, NC.
| | - Michelle D Sakala
- Department of Radiology, Wake Forest University School of Medicine, Winston-Salem, NC
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29
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Hepatic involvement of hereditary hemorrhagic telangiectasia: evaluation with ultrasound. Ultrasound Q 2014; 30:221-4. [PMID: 25148498 DOI: 10.1097/ruq.0000000000000107] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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30
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MDCT of acute conditions affecting the mesenteric vasculature. Clin Radiol 2014; 69:765-72. [PMID: 24824975 DOI: 10.1016/j.crad.2013.12.020] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/17/2013] [Revised: 12/03/2013] [Accepted: 12/18/2013] [Indexed: 12/25/2022]
Abstract
Acute conditions affecting the mesenteric vessels can cause abdominal pain and result in significant morbidity and mortality if not diagnosed and treated quickly. As bowel viability depends on patency of the mesenteric vessels, prompt diagnosis is essential. Helical multidetector computed tomography (MDCT) provides a rapid, widely available, non-invasive method to promptly evaluate the mesenteric arteries, veins, and abdominopelvic viscera. Given the value of MDCT in diagnosing vascular disease, it is important the radiologist understand technical and imaging findings of mesenteric vascular injury. Therefore, successful diagnosis hinges on familiarity with MDCT angiography and the extended capabilities of volume rendering and multiplanar reformation. In this review, we illustrate and describe key MDCT findings of congenital, inflammatory, traumatic, infectious, and thromboembolic conditions affecting the mesenteric vasculature in adult patients.
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Virmani V, Ramanathan S, Virmani V, Kielar A, Sheikh A, Ryan J. Non-neoplastic hepatic vascular diseases: Spectrum of CT and MRI appearances. Clin Radiol 2014; 69:538-48. [DOI: 10.1016/j.crad.2013.12.017] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/24/2013] [Revised: 12/19/2013] [Accepted: 12/24/2013] [Indexed: 12/19/2022]
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32
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Roque Ramos L, Coelho ML. Hepatic haemangiomatosis: multinodular liver in an asymptomatic elderly man. BMJ Case Rep 2014; 2014:bcr-2013-202505. [PMID: 24623542 DOI: 10.1136/bcr-2013-202505] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023] Open
Abstract
A 66-year-old man, overweight and a heavy drinker, was sent to our clinic to investigate multiple liver nodules detected on ultrasound. The patient had no symptoms, and physical examination was unremarkable. Laboratory evaluation disclosed an isolated two-fold increase of γ-glutamyltransferase. The MRI revealed multiple millimetric hypervascular nodules suggestive of liver haemangiomatosis, though malignancy could not be ruled out. Liver biopsy was consistent with hepatic haemangiomatosis. We discuss the differential diagnosis and therapeutical approach of a patient with hepatic haemangiomatosis involving the entire liver without associated symptoms or liver dysfunction.
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Affiliation(s)
- Lídia Roque Ramos
- Department of Gastrenterology, Hospital Garcia de Orta, Almada, Portugal
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33
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Vlachou PA, Colak E, Koculym A, Kirpalani A, Kim TK, Hirschfield GM, Faughnan ME. Improvement of ischemic cholangiopathy in three patients with hereditary hemorrhagic telangiectasia following treatment with bevacizumab. J Hepatol 2013; 59:186-9. [PMID: 23439260 DOI: 10.1016/j.jhep.2013.02.006] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2012] [Revised: 01/23/2013] [Accepted: 02/11/2013] [Indexed: 12/29/2022]
Abstract
The ischemic biliary phenotype of hereditary hemorrhagic telangiectasia (HHT) is rare but distinct, with progressive biliary tree ischemia usually resulting in an irreversible secondary sclerosing cholangiopathy. When clinically severe, liver transplant is often indicated. We report three patients with marked HHT associated biliary disease, in whom prolonged anti-vascular endothelial growth factor therapy (bevacizumab) notably reversed imaging evidence of biliary disease and clinically obviated need for liver transplantation during the first year of follow-up.
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Affiliation(s)
- Paraskevi A Vlachou
- Department of Diagnostic Imaging, St. Michael's Hospital, 30 Bond Street, Toronto, ON, Canada M5B 1W8
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Sunnapwar A, Katre R, Prasad SR, Chintapalli K, Philips S. Spectrum of Multidetector Computed Tomography/Magnetic Resonance Imaging Findings in Intrahepatic Vascular Shunts: Classification, Characterization, and Management. Curr Probl Diagn Radiol 2012; 41:189-97. [DOI: 10.1067/j.cpradiol.2011.11.003] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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35
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Candelli M, Pompili M, Suppressa P, Lenato GM, Bosco G, Rapaccini GL, Gasbarrini A, Scardapane A, Sabbà C. Liver involvement in hereditary hemorrhagic telangiectasia: can breath test unmask impaired hepatic first-pass effect? Intern Emerg Med 2012; 7:323-9. [PMID: 21305365 DOI: 10.1007/s11739-011-0531-9] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/29/2010] [Accepted: 01/22/2011] [Indexed: 02/05/2023]
Abstract
Hepatic arteriovenous malformations (HAVMs) in hereditary hemorrhagic telangiectasia (HHT) have long been considered to have scarce clinical significance in most cases. Nevertheless, data are lacking regarding the influence of HAVMs on the liver first-pass effect on drugs in HHT patients. To gain insight into the effect of HAVMs on hepatic drug clearance by means of two specific (13)C-labeled probes, namely the (13)C-methacetin and (13)C-aminopyrine, 46 HHT patients and 44-matched healthy controls were enrolled. The liver first-pass effect was studied by the (13)C-based breath test using methacetin and aminopyrine. The methacetin breath test showed statistically significant reduced metabolism rates (p < 0.0001) in HHT when compared with controls, both in patients with and without CT-detectable HAVMs, and when expressed both as cumulative (13)C-percentage dose per hour and as (13)C-percentage peak after 15 min. In contrast, no significant difference was found between HHT and controls regarding aminopyrin metabolism rates. In HHT, (13)C%-methacetin breath test values are significantly lower than those found in normal subjects, probably due to the effect of hepatic shunts. A reduced perfusion and an impaired hepatic metabolism might affect hepatic drug clearance in HHT. Therefore, an appropriate dosage adjustments should be considered when high-hepatic-metabolism drugs are administered to HHT patients.
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Affiliation(s)
- Marcello Candelli
- Department of Emergency Medicine, Catholic University of Sacred Heart, Rome, Italy
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36
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Imaging of acute conditions affecting the hepatic vasculature. Emerg Radiol 2012; 19:329-39. [PMID: 22415594 DOI: 10.1007/s10140-012-1036-1] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2012] [Accepted: 03/01/2012] [Indexed: 12/31/2022]
Abstract
Liver imaging primarily consists of evaluating the parenchyma and biliary system. However, the liver has a rich, complex vascularity which can also be affected by numerous disease processes. By considering disease processes that primarily affect the hepatic veins, portal veins, and hepatic arteries, an anatomy-based approach of hepatic vascular diseases can be applied to image interpretation to allow rapid diagnosis and prompt initiation of treatment. Computed tomography, magnetic resonance imaging, and ultrasound are all effectively used to evaluate the liver and can play complimentary roles. In this article, the key imaging findings of acute conditions affecting the hepatic veins (passive congestion, acute thrombosis/Budd-Chiari, stenosis), portal veins (thrombosis, phlebitis, stenosis), hepatic arteries (laceration, pseudoaneurysm, thrombosis), and arteriovenous structures (hereditary hemorrhagic telangiectasis, arteriovenous fistula) will be reviewed.
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37
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Hepatic nodular regenerative lesions in patients with hereditary haemorrhagic telangiectasia: computed tomography and magnetic resonance findings. Radiol Med 2012; 118:1-13. [DOI: 10.1007/s11547-012-0789-3] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/29/2011] [Accepted: 05/25/2011] [Indexed: 12/30/2022]
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Hereditary hemorrhagic telangiectasia: an overview of diagnosis, management, and pathogenesis. Genet Med 2011; 13:607-16. [PMID: 21546842 DOI: 10.1097/gim.0b013e3182136d32] [Citation(s) in RCA: 243] [Impact Index Per Article: 17.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome) is a disorder of development of the vasculature characterized by telangiectases and arteriovenous malformations in specific locations. It is one of most common monogenic disorders, but affected individuals are frequently not diagnosed. The most common features of the disorder, nosebleeds, and telangiectases on the lips, hands, and oral mucosa are often quite subtle. Optimal management requires an understanding of the specific presentations of these vascular malformations, especially their locations and timing during life. Telangiectases in the nasal and gastrointestinal mucosa and brain arteriovenous malformations generally present with hemorrhage. However, complications of arteriovenous malformations in the lungs and liver are generally the consequence of blood shunting through these abnormal blood vessels, which lack a capillary bed and thus result in a direct artery-to-vein connection. Mutations in at least five genes are thought to result in hereditary hemorrhagic telangiectasia, but mutations in two genes (ENG and ACVRL1/ALK1) cause approximately 85% of cases. The frequency of arteriovenous malformations in particular organs and the occurrence of certain rare symptoms are dependent on the gene involved. Molecular genetic testing is used to establish the genetic subtype of hereditary hemorrhagic telangiectasia in a clinically affected individual and family, and for early diagnosis to allow for appropriate screening and preventive treatment.
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Barral M, Sirol M, Placé V, Hamzi L, Borsik M, Gayat E, Boudiaf M, Soyer P. Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients. Eur Radiol 2011; 22:161-70. [PMID: 21861188 DOI: 10.1007/s00330-011-2243-y] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2011] [Accepted: 08/05/2011] [Indexed: 01/11/2023]
Abstract
PURPOSE To analyse quantitatively and qualitatively asymptomatic hepatic and pancreatic involvement in hereditary haemorrhagic telangiectasia (HHT) using 64-section helical CT. MATERIALS AND METHODS The 64-section helical CT examinations of 19 patients with HHT (8 men, 11 women; mean age, 58.6 years) were quantitatively and qualitatively analysed and compared to those of 19 control subjects who were matched for age and sex. Comparisons were made using univariate analysis. RESULTS Dilated and tortuous intrahepatic arterial branches was the most discriminating independent variable (P < 0.0001) and had the highest specificity (100%; 19/19; 95%CI: 82%-100%) and accuracy (97%; 37/38; 95%CI: 86%-100%) for the diagnosis of HHT. Heterogeneous enhancement of hepatic parenchyma, intrahepatic telangiectases, hepatic artery to hepatic vein shunting, hepatic artery enlargement (i.e. diameter > 6.5 mm) and portal vein enlargement (i.e. diameter > 13 mm) were other variables that strongly correlated with the presence of HHT. Intrapancreatic telangiectases and arteriovenous malformations were found in 42% and 16% of patients with HHT, respectively. CONCLUSION Liver and pancreatic involvement in asymptomatic HHT patients is associated with myriad suggestive findings on 64-section helical CT. It can be anticipated that familiarity with these findings would result in more confident diagnosis of HHT.
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Affiliation(s)
- Matthias Barral
- Department of Abdominal Imaging, Hôpital Lariboisière-APHP, 2 rue Ambroise Paré, 75475, Paris cedex 10, France.
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40
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Scardapane A, Stabile Ianora A, Sabbà C, Moschetta M, Suppressa P, Castorani L, Angelelli G. Dynamic 4D MR angiography versus multislice CT angiography in the evaluation of vascular hepatic involvement in hereditary haemorrhagic telangiectasia. Radiol Med 2011; 117:29-45. [PMID: 21643641 DOI: 10.1007/s11547-011-0688-3] [Citation(s) in RCA: 17] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/19/2010] [Accepted: 07/12/2010] [Indexed: 01/17/2023]
Abstract
PURPOSE Hereditary haemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is a rare autosomal dominant disorder characterised by mucocutaneous or visceral vascular abnormalities that may be widely distributed throughout the cardiovascular system. The purpose of this study was to compare multislice computed tomography angiography (MSCTA) and 4D dynamic contrast-enhanced magnetic resonance angiography (D-MRA) for evaluating vascular hepatic involvement in patients with HHT. MATERIALS AND METHODS Fifty-two consecutive HHT patients underwent MSCTA and D-MRA examinations for systematic analysis of vascular visceral involvement. The images from the two techniques were reviewed independently by two expert radiologists to identify the following vascular abnormalities: telangiectases or large vascular masses; perfusion disorders [transient hepatic attenuation differences (THADs)]; hepatic arteriovenous malformations (HAVMs). Data, as well as diameters of the common hepatic artery and portal vein, were compared with Cohen's kappa statistic, Student's t test and receiver operating characteristic (ROC) curve analysis, as appropriate. RESULTS Both MSCTA and D-MRA detected one or more of the following hepatic vascular abnormalities in 36/52 cases (telangiectases in 29/52, THADs in 23/52 and HAVMs in 25/52[CE1]). A good concordance was found between the two techniques when determining the type of hepatic shunt (κ=0.9). No statistically significant differences were found when comparing mean common hepatic artery and portal vein diameters (p=0.09 and 0.22, respectively) and their accuracy in predicting HAVMs. CONCLUSIONS D-MRA has the same diagnostic accuracy as MSCTA and has the advantage of being less invasive due to the absence of ionising radiation.
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Affiliation(s)
- A Scardapane
- Section of Radiology, Di.M.I.M.P., HHT Interdepartmental Centre, University Hospital, Policlinico of Bari, Piazza Giulio Cesare 11, 70124, Bari, Italy.
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41
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Abstract
The dual blood supply of the liver, originating from the portal vein and the hepatic artery, makes it relatively resistant to minor circulatory disturbances. However, hepatic manifestations of common cardiovascular disorders are frequently encountered in both the inpatient and outpatient setting. Beginning with the macro- and microcirculation of the liver, this article reviews the pathophysiology of hepatic blood flow and gives a detailed appraisal of ischemic hepatitis, congestive hepatopathy, and other less common hepatic conditions that arise when cardiovascular function is impaired.
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Affiliation(s)
- Ilan S Weisberg
- Division of Gastroenterology and Hepatology, Weill Cornell Medical Center, New York Presbyterian Hospital, 1305 York Avenue, 4th Floor, New York, NY 10021, USA
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42
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Kamaya A, Maturen KE, Tye GA, Liu YI, Parti NN, Desser TS. Hypervascular Liver Lesions. Semin Ultrasound CT MR 2009; 30:387-407. [DOI: 10.1053/j.sult.2009.06.001] [Citation(s) in RCA: 46] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
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Abstract
This guideline has been approved by the American Association for the Study of Liver Diseases (AASLD) and represents the position of the association.
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Affiliation(s)
- Laurie D DeLeve
- Division of Gastrointestinal and Liver Diseases and the Research Center for Liver Diseases, University of Southern California Keck School of Medicine, Los Angeles, CA, USA
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Fukunaga M, Fujiki R, Santa Y, Kato A, Yoshimura T. [Elderly hereditary hemorrhagic telangiectasia female with portosystemic encephalopathy]. Rinsho Shinkeigaku 2009; 49:271-274. [PMID: 19594105 DOI: 10.5692/clinicalneurol.49.271] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/28/2023]
Abstract
We present a 71-year-old woman with hereditary hemorrhagic telangiectasia (HHT) who at age 69, had undergone total gastrectomy because of repeated upper gastrointestinal bleeding. A day prior to admission she began to demonstrate abnormal behavior. Examination showed she was restless and had higher brain dysfunction. Triphasic waves were seen on EEG, and a high signal in the globus pallidus on T1-weighted MRI. Plasma NH3 level was increased after a meal. Abdominal CT scan showed vascular anomalies including a portohepatic vein shunt. She was diagnosed with portosystemic encephalopathy. After treatment with a low-protein diet, lactitol, and branched chain-amino acids, her clinical condition, plasma NH3 level after a meal, and EEG returned to normal. Because portosystemic shunt is rare in HHT, there have been few reports of portosystemic encephalopathy with this condition. However, with aging, the possibility of portosystemic encephalopathy increases because of age-related increases in portosystemic shunt volume.
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Affiliation(s)
- Mami Fukunaga
- Department of Neurology, Shimonoseki Kousei Hospital
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Torabi M, Hosseinzadeh K, Federle MP. CT of nonneoplastic hepatic vascular and perfusion disorders. Radiographics 2009; 28:1967-82. [PMID: 19001652 DOI: 10.1148/rg.287085067] [Citation(s) in RCA: 78] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
The unique dual blood supply of the liver (75% portal venous, 25% hepatic arterial) makes multiphase helical computed tomography (CT) a highly suitable technique for hepatic evaluation with imaging in two (arterial and portal venous) or more phases. Multiphase helical CT has become an important tool in the detection and characterization of hepatic tumors. In some situations, hemodynamic changes might mimic neoplastic or inflammatory lesions and evoke diagnostic uncertainty. To confidently identify hepatic conditions such as venous outflow obstruction (Budd-Chiari syndrome), arterioportal shunts, hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome), peliosis hepatis, passive congestion, and hepatic infarction, radiologists must be familiar with the disease-specific CT appearances and related clinical manifestations.
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Affiliation(s)
- Maha Torabi
- Department of Radiology, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA
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Sabbà C, Pompili M. Review article: the hepatic manifestations of hereditary haemorrhagic telangiectasia. Aliment Pharmacol Ther 2008; 28:523-33. [PMID: 18573106 DOI: 10.1111/j.1365-2036.2008.03775.x] [Citation(s) in RCA: 37] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/22/2022]
Abstract
BACKGROUND Liver involvement in hereditary haemorrhagic telangiectasia is mainly characterized by vascular malformations, such as telangiectasies and arteriovenous shunts, which are found in up to 80% of patients. AIM To analyse the current knowledge and controversies regarding the epidemiological, pathological, clinical, diagnostic and therapeutic aspects of liver involvement in hereditary haemorrhagic telangiectasia. Methods Systematic survey analysis of the indexed studies dealing with the above mentioned topics. RESULTS No more than 8% of patients with hepatic vascular abnormalities will have a symptomatic liver disease, mainly consisting in high-output heart failure, portal hypertension or biliary disease. CONCLUSIONS Colour Doppler ultrasonography is a non-invasive, highly accurate and relatively low-cost procedure for the screening of liver involvement in patients with hereditary haemorrhagic telangiectasia; computed tomography, magnetic resonance imaging and angiography can be reserved for the characterization of focal lesions and the study of severely ill patients in whom invasive therapeutic procedures are advisable. Patients with asymptomatic liver involvement should not receive any treatment, while the therapeutic options for symptomatic patients include treatment of the specific complication, invasive procedures for shunt reduction and liver transplantation. The newly developed antiangiogenetic therapies appear to be very promising, but still require further evaluation in clinical trials.
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Affiliation(s)
- C Sabbà
- Unit of Internal Medicine, Department of Internal Medicine and Public Health, Interdepartmental HHT Centre, University of Bari, Bari, Italy.
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Lin AE, Basson CT, Goldmuntz E, Magoulas PL, McDermott DA, McDonald-McGinn DM, McPherson E, Morris CA, Noonan J, Nowak C, Pierpont ME, Pyeritz RE, Rope AF, Zackai E, Pober BR. Adults with genetic syndromes and cardiovascular abnormalities: clinical history and management. Genet Med 2008; 10:469-94. [PMID: 18580689 PMCID: PMC2671242 DOI: 10.1097/gim.0b013e3181772111] [Citation(s) in RCA: 42] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/15/2023] Open
Abstract
Cardiovascular abnormalities, especially structural congenital heart defects, commonly occur in malformation syndromes and genetic disorders. Individuals with syndromes comprise a significant proportion of those affected with selected congenital heart defects such as complete atrioventricular canal, interrupted arch type B, supravalvar aortic stenosis, and pulmonary stenosis. As these individuals age, they contribute to the growing population of adults with special health care needs. Although most will require longterm cardiology follow-up, primary care providers, geneticists, and other specialists should be aware of (1) the type and frequency of cardiovascular abnormalities, (2) the range of clinical outcomes, and (3) guidelines for prospective management and treatment of potential complications. This article reviews fundamental genetic, cardiac, medical, and reproductive issues associated with common genetic syndromes that are frequently associated with a cardiovascular abnormality. New data are also provided about the cardiac status of adults with a 22q11.2 deletion and with Down syndrome.
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Affiliation(s)
- Angela E Lin
- Genetics Unit, Department of Pediatrics, MassGeneral Hospital for Children, Boston, Massachusetts 02114, USA.
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Siddiki H, Doherty MG, Fletcher JG, Stanson AW, Vrtiska TJ, Hough DM, Fidler JL, McCollough CH, Swanson KL. Abdominal findings in hereditary hemorrhagic telangiectasia: pictorial essay on 2D and 3D findings with isotropic multiphase CT. Radiographics 2008; 28:171-84. [PMID: 18203937 DOI: 10.1148/rg.281075037] [Citation(s) in RCA: 55] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/23/2023]
Abstract
The rapid evolution in multidetector computed tomographic (CT) technology has produced improvements in temporal and spatial resolution, leading to greater recognition of the spectrum of abdominal findings in hereditary hemorrhagic telangiectasia (HHT). In this multisystem vascular disorder, the abdominal findings are predominantly within the liver. Hepatic vascular lesions in HHT range from tiny telangiectases to transient perfusion abnormalities and large confluent vascular masses. Focal hepatic lesions are often associated with arteriovenous, arterioportal, or portovenous shunts. Pancreatic, splenic, and other vascular abnormalities are also observed because they are included in the field of view. By taking advantage of the increased z-axis spatial resolution and faster scanning times, and by using a bolus tracking technique, multiphase CT can be used to identify hepatic and extrahepatic lesions in HHT and to characterize the associated vascular shunts. Coronal maximum intensity projection images are particularly helpful in depiction of small hepatic vascular lesions.
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Affiliation(s)
- Hassan Siddiki
- Department of Radiology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA
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González-Casas R, Trapero-Marugán M, Moreno-Otero R. Enfermedad hepática en la telangiectasia hereditaria hemorrágica (enfermedad de Rendu-Osler-Weber). Med Clin (Barc) 2007; 129:629-31. [DOI: 10.1157/13111813] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/09/2023]
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Lin E, Stall L. Spectrum of biliary abnormalities in hepatic hereditary hemorrhagic telangiectasia: demonstration by multidetector computed tomography. Emerg Radiol 2007; 14:461-3. [PMID: 17453259 DOI: 10.1007/s10140-007-0609-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/21/2007] [Accepted: 03/21/2007] [Indexed: 12/14/2022]
Abstract
We present a case in which multidetector computed tomography demonstrated a specific pattern of biliary disease associated with hepatic hereditary hemorrhagic telangiectasia: multiple intrahepatic stenoses, focal cystic dilatations along the course of the intrahepatic ducts, and large biliary cysts in conjunction with a normal-appearing extrahepatic bile duct.
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Affiliation(s)
- Eugene Lin
- Department of Radiology, Virginia Mason Medical Center, 1100 Ninth Avenue, Seattle, WA, 98111, USA.
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