The value of screening for multiple arterio-venous malformations in hereditary hemorrhagic telangiectasia: a diagnostic study

Occult visceral arterio-venous malformations (AVMs) may be a constant threat to patients suffering from hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome (M. ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that c...

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Veröffentlicht in:European archives of oto-rhino-laryngology 2004-10, Vol.261 (9), p.509-516
Hauptverfasser: FOLZ, Benedikt J, WOLLSTEIN, Ana Cerra, ALFKE, Heiko, DÜNNE, Anja A, LIPPERT, Burkard M, GÖRG, Konrad, WAGNER, Hans-Joachim, BIEN, Siegfried, WERNER, Jochen A
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container_title European archives of oto-rhino-laryngology
container_volume 261
creator FOLZ, Benedikt J
WOLLSTEIN, Ana Cerra
ALFKE, Heiko
DÜNNE, Anja A
LIPPERT, Burkard M
GÖRG, Konrad
WAGNER, Hans-Joachim
BIEN, Siegfried
WERNER, Jochen A
description Occult visceral arterio-venous malformations (AVMs) may be a constant threat to patients suffering from hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome (M. ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that can alert physicians at an early stage of the disease. The purpose of this study was to investigate whether occult, visceral arterio-venous malformations could be detected by screening imaging studies in patients suffering from HHT. In a comprehensive diagnostic study, Rendu-Osler-Weber patients were examined for potential visceral arterio-venous malformations by physical examination and non-invasive imaging techniques. The Department of Otolaryngology of the Philipps University of Marburg is a major referral center and coordinated the screening procedures. Thirty-five individuals with the presumed diagnosis of HHT gave informed consent to the screening investigations. Eighteen of 35 individuals were found to suffer from visceral vascular malformations; most of the AVMs were diagnosed in the lung, but also the liver, spleen, brain and eyes were affected. Six patients could be treated preventively by arterial embolization for AVMs of the lung, liver and brain. Comprehensive screening for occult AVMs in HHT patients seems to be justified to avert potential complications in this group of patients.
doi_str_mv 10.1007/s00405-003-0719-3
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ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that can alert physicians at an early stage of the disease. The purpose of this study was to investigate whether occult, visceral arterio-venous malformations could be detected by screening imaging studies in patients suffering from HHT. In a comprehensive diagnostic study, Rendu-Osler-Weber patients were examined for potential visceral arterio-venous malformations by physical examination and non-invasive imaging techniques. The Department of Otolaryngology of the Philipps University of Marburg is a major referral center and coordinated the screening procedures. Thirty-five individuals with the presumed diagnosis of HHT gave informed consent to the screening investigations. Eighteen of 35 individuals were found to suffer from visceral vascular malformations; most of the AVMs were diagnosed in the lung, but also the liver, spleen, brain and eyes were affected. Six patients could be treated preventively by arterial embolization for AVMs of the lung, liver and brain. Comprehensive screening for occult AVMs in HHT patients seems to be justified to avert potential complications in this group of patients.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Age Distribution</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Arteriovenous Malformations - diagnosis</subject><subject>Arteriovenous Malformations - epidemiology</subject><subject>Biological and medical sciences</subject><subject>Blood and lymphatic vessels</subject><subject>Cardiology. Vascular system</subject><subject>Cerebral Angiography - methods</subject><subject>Cohort Studies</subject><subject>Comorbidity</subject><subject>Diseases of the peripheral vessels. Diseases of the vena cava. 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ROW). HHT patients predominantly become symptomatic through chronic, recurrent epistaxis, a symptom that can alert physicians at an early stage of the disease. The purpose of this study was to investigate whether occult, visceral arterio-venous malformations could be detected by screening imaging studies in patients suffering from HHT. In a comprehensive diagnostic study, Rendu-Osler-Weber patients were examined for potential visceral arterio-venous malformations by physical examination and non-invasive imaging techniques. The Department of Otolaryngology of the Philipps University of Marburg is a major referral center and coordinated the screening procedures. Thirty-five individuals with the presumed diagnosis of HHT gave informed consent to the screening investigations. Eighteen of 35 individuals were found to suffer from visceral vascular malformations; most of the AVMs were diagnosed in the lung, but also the liver, spleen, brain and eyes were affected. Six patients could be treated preventively by arterial embolization for AVMs of the lung, liver and brain. Comprehensive screening for occult AVMs in HHT patients seems to be justified to avert potential complications in this group of patients.</abstract><cop>Berlin</cop><pub>Springer</pub><pmid>14685882</pmid><doi>10.1007/s00405-003-0719-3</doi><tpages>8</tpages></addata></record>
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subjects Adolescent
Adult
Age Distribution
Aged
Aged, 80 and over
Arteriovenous Malformations - diagnosis
Arteriovenous Malformations - epidemiology
Biological and medical sciences
Blood and lymphatic vessels
Cardiology. Vascular system
Cerebral Angiography - methods
Cohort Studies
Comorbidity
Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous
Female
Humans
Incidence
Magnetic Resonance Imaging
Male
Mass Screening - methods
Medical sciences
Middle Aged
Non tumoral diseases
Otorhinolaryngology. Stomatology
Prognosis
Risk Assessment
Sensitivity and Specificity
Severity of Illness Index
Sex Distribution
Survival Rate
Telangiectasia, Hereditary Hemorrhagic - diagnosis
Telangiectasia, Hereditary Hemorrhagic - epidemiology
Tomography, X-Ray Computed
Ultrasonography, Doppler, Color
Upper respiratory tract, upper alimentary tract, paranasal sinuses, salivary glands: diseases, semeiology
title The value of screening for multiple arterio-venous malformations in hereditary hemorrhagic telangiectasia: a diagnostic study
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