PORTAL HYPERTENSION IN OSLER-WEBER-RENDU SYNDROME: A CASE REPORT

2021 ◽  
pp. 128-129
Author(s):  
Himanshu Dhiman ◽  
Akhil Katna
Keyword(s):  
Portal Hypertension ◽  
Liver Disease ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Clinical Manifestations ◽  
Biliary Disease ◽  
Vascular Disorder ◽  
Ultrasound Computed Tomography ◽  
Predominant Type ◽  
High Output

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 patient with Osler-Weber-Rendu syndrome with portal hypertension without evidence of liver cirrhosis.

scholarly journals The Management of Extrahepatic Portal Vein Aneurysms: Observe or Treat?

HPB Surgery ◽  
1996 ◽  
Vol 10 (2) ◽  
pp. 113-116 ◽  
Author(s):  
Philip D. Feliciano ◽  
Joseph J. Cullen ◽  
John D. Corson
Keyword(s):  
Portal Hypertension ◽  
Liver Disease ◽  
Portal Vein ◽  
Process Management ◽  
Asymptomatic Patient ◽  
Underlying Liver Disease ◽  
Surgical Exploration ◽  
Portal Vein Aneurysm

A case of a 70 year old man who was found to have an extrahepatic portal vein aneurysm during an evaluation for hematuria is reported. Extrahepatic portal vein aneurysms are rare with only twenty cases reported in the literature. Typically, patients present with hemorrhage requiring surgical exploration or the aneurysm is discovered during evaluation of another abdominal process. Management includes careful follow-up in the asymptomatic patient without underlying liver disease or portal hypertension.

Liver Disease in Pregnancy

2015 ◽  
Author(s):  
Matthew S Chang ◽  
Anna E. Rutherford
Keyword(s):  
Portal Hypertension ◽  
Liver Disease ◽  
Viral Hepatitis ◽  
Chronic Liver Disease ◽  
Hyperemesis Gravidarum ◽  
Treatment Options ◽  
Chronic Liver ◽  
Biliary Disease ◽  
Elevated Liver Enzymes ◽  
In Pregnancy

Liver disease in pregnancy can be unique to pregnancy or coincidental to pregnancy, or pregnancy can occur in women with chronic liver disease, cirrhosis, or portal hypertension. The gestational age of the pregnancy can help determine the diagnosis. Liver disease occurring during pregnancy has the potential to be life threatening to mother and fetus, and any elevation in liver tests should be investigated. Disorders unique to pregnancy include hyperemesis gravidarum; intrahepatic cholestasis of pregnancy; preeclampsia/eclampsia; hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome; and acute fatty liver of pregnancy. Liver diseases coincidental to pregnancy include acute viral hepatitis, gallstones/biliary disease, and thrombosis such as Budd-Chiari syndrome/hepatic vein thrombosis. Pregnant women may have chronic liver disease or cirrhosis from all of the same etiologies as nonpregnant women, most commonly chronic viral hepatitis. Treatment options may differ during pregnancy because of the potential risks to the fetus. Women who have cirrhosis or portal hypertension or who are post liver transplantation should be managed in conjunction with a hepatologist.  This review contains 4 figures, 6 tables and 40 references Key words: acute liver failure, delivery, trimester  

scholarly journals The Role of Liver Imaging in Hereditary Hemorrhagic Telangiectasia

2020 ◽  
Vol 9 (11) ◽  
pp. 3750
Author(s):  
Joelle Harwin ◽  
Mark D. Sugi ◽  
Steven W. Hetts ◽  
Miles B. Conrad ◽  
Michael A. Ohliger
Keyword(s):  
Portal Hypertension ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Vascular Malformations ◽  
Antiangiogenic Therapy ◽  
Liver Imaging ◽  
Ultrasound Computed Tomography ◽  
Quantify Blood Flow ◽  
Radiologic Imaging ◽  
Magnetic Resonance Imaging Mri ◽  
Related Liver Disease

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.

scholarly journals Distinct Forms of Pulmonary Hypertension Complicate Hereditary Hemorrhagic Telangiectasia

2015 ◽  
Vol 14 (3) ◽  
pp. 145-149 ◽  
Author(s):  
John T. Battaile ◽  
Cristhiaan D. Ochoa
Keyword(s):  
Pulmonary Hypertension ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Vascular Malformations ◽  
Clinical Care ◽  
Clinical Manifestations ◽  
Left Heart ◽  
High Output Heart Failure ◽  
Pulmonary Arterial ◽  
Heritable Pulmonary Arterial Hypertension ◽  
High Output

Pulmonary hypertension (PH) associated with hereditary hemorrhagic telangiectasia (HHT) occurs most commonly as a consequence of high-output heart failure related to excessive shunting through visceral vascular malformations. A smaller number of HHT patients develop a form of heritable pulmonary arterial hypertension, characterized by an elevated pulmonary vascular resistance with normal left heart filling pressures. In this review, we will discuss the clinical manifestations of HHT in general, the pathophysiology behind the different forms of PH associated with HHT, and the clinical care of the HHT patient with PH.

scholarly journals Serum keratin 19 (CYFRA21-1) links ductular reaction with portal hypertension and outcome of various advanced liver diseases

BMC Medicine ◽  
2020 ◽  
Vol 18 (1) ◽  
Author(s):  
Karim Hamesch ◽  
Nurdan Guldiken ◽  
Mahmoud Aly ◽  
Norbert Hüser ◽  
Daniel Hartmann ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Liver Disease ◽  
Chronic Liver Disease ◽  
Alcoholic Cirrhosis ◽  
Chronic Liver ◽  
Decompensated Cirrhosis ◽  
Ductular Reaction ◽  
Risk Of Death

Abstract Background Keratins (Ks) represent tissue-specific proteins. K18 is produced in hepatocytes while K19, the most widely used ductular reaction (DR) marker, is found in cholangiocytes and hepatic progenitor cells. K18-based serum fragments are commonly used liver disease predictors, while K19-based serum fragments detected through CYFRA21-1 are established tumor but not liver disease markers yet. Since DR reflects the severity of the underlying liver disease, we systematically evaluated the usefulness of CYFRA21-1 in different liver disease severities and etiologies. Methods Hepatic expression of ductular keratins (K7/K19/K23) was analyzed in 57 patients with chronic liver disease (cohort i). Serum CYFRA21-1 levels were measured in 333 Austrians with advanced chronic liver disease (ACLD) of various etiologies undergoing hepatic venous pressure gradient (HVPG) measurement (cohort ii), 231 French patients with alcoholic cirrhosis (cohort iii), and 280 hospitalized Germans with decompensated cirrhosis of various etiologies (cohort iv). Results (i) Hepatic K19 levels were comparable among F0–F3 fibrosis stages, but increased in cirrhosis. Hepatic K19 mRNA strongly correlated with the levels of other DR-specific keratins. (ii) In ACLD, increased serum CYFRA21-1 associated with the presence of clinically significant portal hypertension (CSPH; HVPG ≥ 10 mmHg) (OR = 5.87 [2.95–11.68]) and mortality (HR = 3.02 [1.78–5.13]; median follow-up 22 months). (iii) In alcoholic cirrhosis, elevated serum CYFRA21-1 indicated increased risk of death/liver transplantation (HR = 2.59 [1.64–4.09]) and of HCC (HR = 1.74 [1.02–2.96]) over the long term (median follow-up 73 months). (iv) In decompensated cirrhosis, higher serum CYFRA21-1 predicted 90-day mortality (HR = 2.97 [1.92–4.60]) with a moderate accuracy (AUROC 0.64), independently from established prognostic scores. Conclusions Hepatic K19 mRNA and serum CYFRA21-1 levels rise in cirrhosis. Increased CYFRA21-1 levels associate with the presence of CSPH and reliably indicate mortality in the short and long term independently of conventional liver biochemistry markers or scoring systems. Hence, the widely available serum CYFRA21-1 constitutes a novel, DR-related marker with prognostic implications in patients with different settings of advanced liver disease.

Hepatic Disorders

2017 ◽  
Author(s):  
Andrew Goldsmith
Keyword(s):  
Portal Hypertension ◽  
Liver Disease ◽  
Bacterial Infections ◽  
Wide Spectrum ◽  
Clinical Manifestations ◽  
Hepatic Abscess ◽  
High Morbidity ◽  
Prevention Measures ◽  
Complex Disorders ◽  
Bacterial Peritonitis

Hepatic disorders are characterized by a variety of etiologies that can present to the emergency department (ED) as acute, chronic, or acute on chronic liver disease. Unfortunately, a large number of these complex disorders can progress to cirrhosis, a progressive and severe clinical condition associated with high morbidity and mortality. Primary prevention, measures include vaccine prophylaxis and abstaining from alcohol. Unfortunately, liver disease can lead to a wide spectrum of clinical manifestations that are in need of urgent and/or emergent therapy mainly attributable to hepatic insufficiency and portal hypertension. Major complications of portal hypertension include ascites, gastrointestinal variceal bleeding, hepatic encephalopathy, renal failure, and bacterial infections. This review covers the epidemiology, pathophysiology, diagnosis, treatment, and disposition of patients who present to the ED with liver disease. This review contains 3 figures, 4 tables, and 59 references. Key words: abdominal pain, ascites, cirrhosis, encephalopathy, hepatic abscess, hepatic liver transplant, hepatitis, hepatorenal syndrome, spontaneous bacterial peritonitis

Lower Risk of Intracranial Arteriovenous Malformation Hemorrhage in Patients With Hereditary Hemorrhagic Telangiectasia

Neurosurgery ◽  
2015 ◽  
Vol 78 (5) ◽  
pp. 684-693 ◽  
Author(s):  
Wuyang Yang ◽  
Ann Liu ◽  
Alice L. Hung ◽  
Maria Braileanu ◽  
Joanna Y. Wang ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Clinical Manifestations ◽  
Intracranial Arteriovenous Malformation ◽  
Hemorrhagic Risk ◽  
History Of ◽  
Ruptured Avm ◽  
Martin Grade ◽  
Lost To Follow Up

Abstract BACKGROUND: Patients diagnosed with hereditary hemorrhagic telangiectasia (HHT) are at risk of developing intracranial arteriovenous malformations (AVM). However, the clinical manifestations and natural history of HHT-related AVMs remain unclear due to the rarity of these lesions. OBJECTIVE: To clarify the clinical characteristics and hemorrhagic risk in HHT-related AVMs. METHODS: We performed a retrospective review of all patients diagnosed with both HHT and intracranial AVMs who were evaluated at our institution from 1990 to 2013. Patients with missing data or lost to follow-up were excluded. Baseline characteristics and subsequent hemorrhagic risk were evaluated. RESULTS: In an AVM database of 531 patients with 542 AVMs, a total of 12 HHT patients (2.3%) with 23 AVMs were found. Mean age at diagnosis was 36.5 years, with 41.7% male. Compared to patients with sporadic AVMs, patients with HHT were less likely to present with ruptured AVM (P = .04), headaches (P = .02), and seizures (P = .02), and presented with better modified Rankin scores (P < .01). HHT-related AVMs were smaller in size (P < .01), of lower Spetzler-Martin grade (P = .01), and had less temporal lobe involvement (P = .02) compared to sporadic AVMs. Six HHT patients (50.0%) were found with multiple intracranial AVMs. One hemorrhage was found during an observation period of 149.6 patient-years and 297.5 lesion-years, translating to 1.3% per patient per year or 0.7% per AVM per year. CONCLUSION: HHT-related AVMs are smaller in size with lower Spetzler-Martin grade and less temporal lobe involvement than sporadic AVMs. Patients with HHT frequently present with multiple intracranial AVMs. Conservative management is generally recommended due to lesion multiplicity and relatively low hemorrhagic risk.

scholarly journals Quando hipertensão arterial persistente no adolescente tem uma origem endócrina rara: relato de dois casos e revisão da literatura

2017 ◽  
Vol 27 (3) ◽  
pp. 26960
Author(s):  
Clara Gomes ◽  
Gabriela Laranjo ◽  
Elisabete Santos ◽  
Cristina Faria
Keyword(s):  
Blood Pressure ◽  
Clinical Presentation ◽  
Pediatric Population ◽  
Clinical Manifestations ◽  
Pathological Examination ◽  
Left Adrenal Gland ◽  
Ultrasound Computed Tomography ◽  
Lower Morbidity

*** When persistent hypertension  in adolescents has a rare endocrine etiology: report of two cases and literature review ***AIMS: To report two clinical cases of pheochromocytoma, a rare tumor in the pediatric population, that affects the adrenal medulla, is malignant in up to 47% of cases,  and whose clinical manifestations result from hypersecretion of catecholamines.CASE DESCRIPTION: Two cases of pheochromocytoma in adolescents are described, both with persistent hypertension and one with headache, sweating, and tachycardia. Renal and adrenal ultrasound, computed tomography angiography, and measurements of catecholamines and their metabolites revealed pheochromocytoma in the left adrenal gland. Adrenalectomy was performed after pretreatment with α-blockers. Pathological examination confirmed the diagnosis. Blood pressure returned to normal in both patients after treatment.CONCLUSIONS: Pheochromocytoma has a varied and nonspecific clinical presentation, but this condition should always be considered in the differential diagnosis of high blood pressure. Early diagnosis may imply a less aggressive treatment, lower morbidity, and a lesser impact on patients’ quality of life. The high likelihood of recurrence of pheochromocytoma, including as metastatic disease, requires follow-up visits for several years after its initial clinical presentation, even in the case of an apparent cure.

scholarly journals Synthetic dural graft septoplasty in epistaxis from hereditary hemorrhagic telangiectasia

2013 ◽  
pp. 189-191
Author(s):  
Claudia Patricia Guerra ◽  
Wilfred Burckhardt B
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Autosomal Dominant ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Skin Grafts ◽  
Vascular Disorder ◽  
Dural Graft ◽  
Bleeding Episodes

It is an autosomal dominant vascular disorder, which has a variety of clinical manifestations, with epistaxis being one of the most common. Many treatment options exist for epistaxis, but with no consensus on which is the method of choice. We describe the case of a patient with hereditary hemorrhagic telangiectasia (HHT) secondary epistaxis with septoplasty managed with synthetic hard graft, which improved intensity and frequency of bleeding episodes. This technique is a variant of the septodermoplasty described by several authors, but the use of synthetic dura can help in obtaining better results and avoid taking skin grafts from other sites different from the surgical site.

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