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

Yang, Wuyang; Liu, Ann; Hung, Alice L.; Braileanu, Maria; Wang, Joanna Y.; Caplan, Justin M.; Colby, Geoffrey P.; Coon, Alexander L.; Tamargo, Rafael J.; Ahn, Edward S.; Huang, Judy

doi:10.1227/neu.0000000000001103

Cited by 42 publications

(51 citation statements)

References 32 publications

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“…The authors found an overall bleeding rate of 1% per year with a rupture rate of 0.4% per year (95% CI 0.1% to 1.7%) for unruptured AVMs and 10% per year for ruptured AVMs (95% CI 3.3% to 31.2%). Lastly, in a study by Yang et al ,2 the authors found that AVMs in HHT patients had a more favorable natural history than sporadic AVMs. However, there was no stratification of natural history by AVM type.…”

Section: Discussionmentioning

confidence: 96%

Natural history of brain capillary vascular malformations in hereditary hemorrhagic telangiectasia patients

Brinjikji

Iyer

Lanzino

et al. 2016

J NeuroIntervent Surg

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Section: Discussionmentioning

confidence: 96%

Natural history of brain capillary vascular malformations in hereditary hemorrhagic telangiectasia patients

Brinjikji

Iyer

Lanzino

et al. 2016

J NeuroIntervent Surg

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“…Namely, according to previously published studies, incidence of the cerebral AVM varies between 2.3% and 7.7% 5,13 . Also, recent data from an AVM database demonstrated that out of 531 patients, 12 (2.3%) had the cerebral AVM due to underlying HHT 14 .…”

Section: Discussionmentioning

confidence: 99%

Clinical characteristics of hereditary hemorrhagic telangiectasia - case series and review of the literature

Popović¹,

Sokic-Milutinovic²,

Djuranović³

et al. 2019

VOJNOSANIT PREGL

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Introduction. Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder with estimated prevalence of one in 5,000 to 10,000. The disease has age-related penetrance and the HHT signs and symptoms occur and worsen with age. A diagnosis of HHT is based on the Curacao`s criteria. Case report. We report a case series of 6 patients diagnosed with HHT, 5 with definite and one with probable diagnosis according to the Curacao criteria. In 5 patients, the recurrent epistaxis occurred in adolescence as the first presentation while one patient presented with melena. The diagnosis was delayed in 5 patients and the presence of HHT was diagnosed during or after the fifth decade. In 4 patients, the overt gastrointestinal bleeding occurred in the later course of the disease. The asymptomatic pulmonary circulation arteriovenous malformations were detected in 2 patients. The cerebral arteriovenous malformations were not detected. Conclusion. Hereditary hemorrhagic telangiectasia is a rare disorder affecting multiple organs. It should be considered in the adolescents with recurrent epistaxis and in the differential diagnosis of anemia with signs of the gastrointestinal bleeding in order to shorten the delay in the diagnosis and subsequently improve the outcome of the disease.Uvod. Nasledna hemoragijska teleangiektazija (HHT) je redak autosomno-dominantni poremaćaj sa prevalencijom javljanja 1 na 5,000 do 10,000. Bolest je uzrasno zavisna i HHT simptomi i znaci se rano javljaju i pogoršavaju sa godinama. Dijagnoza se postavlja na osnovu Curaco kriterijuma. Prikaz bolesnika. Ovo je serijski prikaz šest bolesnika sa HHT, pet sa definitivnom i jednog sa verovatnom dijagnozom HHT na osnovu Curaco kriterijuma. Kod pet bolesnika ponavljane epistakse su se javile u adolescentnom dobu kao prva manifestacija bolesti, dok je prvi znak bolesti kod jednog bolesnika bila melena. Kod pet bolesnika dijagnoza HHT postavljena je tek u toku i nakon pete decenije života. Kod četiri bolesnika manifestno gastrointestinalno krvarenje se javilo u daljem toku bolesti. Asimptomatske arteriovenske malformacije plućne cirkulacije uočene su kod dva bolesnika. Cerebralne arteriovenske malformacije nisu uočene ni kod jednog bolesnika. Zaključak. Nasledna hemoragijska teleangiektazija je retka bolest koja zahvata više organa. Na ovu bolest bi trebalo misliti kada adolescent imaju ponavljane epistakse i diferencijalno dijagnostički kod anemija sa znacima gastrointestinalnog krvarenja, a u cilju pravovremenog postavljanja dijagnoze bolesti i poboljšanja ishoda iste.

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“…As mentioned earlier, mainly three types of cerebral vascular lesions have been reported in HHT (capillary malformation [CM], “nidus‐type” [type III] AVMs, and direct high‐flow types I and II AVF) . HHT‐related AVMs are smaller in size with lower Spetzler‐Martin grade and with less temporal lobe involvement than sporadic AVMs …”

Section: High Flow Malformations: Arteriovenous Shuntsmentioning

confidence: 99%

Pediatric Congenital Cerebrovascular Anomalies

Goyal

Mangla

Gupta

et al. 2018

Journal of Neuroimaging

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Congenital cerebrovascular anomalies in the pediatric age group are myriad with diverse etiologies. The purpose of this paper is to provide an imaging overview of congenital vascular malformations and vascular tumors, as these conditions are varied and the characteristic vascular abnormality may even suggest the underlying systemic condition in helping to guide further management. For example, the identification of an arterial anomaly such as agenesis/hypoplasia/duplication may warrant further evaluation for an associated syndrome. Recognition of other pertinent findings, such as persistent carotid‐vertebrobasilar anastomoses for instance, would be particularly important for the planning of potential embolization/coiling of associated vascular malformation and/or aneurysm. Awareness of congenital dural sinus anomalies, including hypoplasia, aplasia, and or duplication, prevents the incorrect or overdiagnosis of sinus thrombosis. Moreover, multiple developmental venous anomalies in a patient may require further investigation for its familial association. In a similar context, there may be familial association with multiple cavernous malformations and children with central nervous system vascular malformations may have an association with other vascular conditions, eg, hereditary hemorrhagic telangiectasia, etc. An accurate description of vascular lesions is critical for understanding the natural history of the disease process and ultimately for guiding treatment. An important example of this includes Sturge‐Weber syndrome, which is often incorrectly viewed as an arteriovenous malformation, but actually a type of venous malformation. Another example which is important to recognize includes the differentiation between an infantile hemangioma and a vascular malformation, particularly as the course of the pathology and the treatment is so different between the two.

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

Cited by 42 publications

References 32 publications

Natural history of brain capillary vascular malformations in hereditary hemorrhagic telangiectasia patients

Natural history of brain capillary vascular malformations in hereditary hemorrhagic telangiectasia patients

Clinical characteristics of hereditary hemorrhagic telangiectasia - case series and review of the literature

Pediatric Congenital Cerebrovascular Anomalies

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