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Erschienen in: European Radiology 3/2018

10.10.2017 | Chest

Pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia: Correlations between computed tomography findings and cerebral complications

verfasst von: Johan Etievant, Salim Si-Mohamed, Nicolas Vinurel, Sophie Dupuis-Girod, Evelyne Decullier, Delphine Gamondes, Chahera Khouatra, Vincent Cottin, Didier Revel

Erschienen in: European Radiology | Ausgabe 3/2018

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Abstract

Objectives

Computed tomography (CT) is the modality of choice to characterise pulmonary arteriovenous malformations (PAVMs) in patients with hereditary haemorrhagic telangiectasia (HHT). Our objective was to determine if CT findings were associated with frequency of brain abscess and ischaemic stroke.

Methods

This retrospective study included patients with HHT-related PAVMs. CT results, i.e. PAVM presentation (unique, multiple, disseminated or diffuse), the number of PAVMs and the largest feeding artery size, were correlated to prevalence of ischaemic stroke and brain abscess. All CTs were reviewed in consensus by two radiologists.

Results

Of 170 patients, 73 patients had unique (42.9 %), 49 multiple (28.8 %), 36 disseminated (21.2 %) and 12 diffuse (7.1 %) PAVMs. Fifteen patients presented with brain abscess; 26 patients presented with ischaemic stroke. The number of PAVMs was significantly correlated with brain abscess (11.5 vs. 6.2, respectively; p=0.025). The mean diameter of the largest feeding artery was significantly correlated with ischaemic stroke frequency (4.9 vs. 3.2 mm, respectively; p=0.0098).

Conclusions

The number of PAVMs correlated significantly with risk of brain abscess, and a larger feeding artery significantly with more ischaemic strokes. These findings can lead to a better recognition and management of the PAVMs at risk of cerebral complications.

Key Points

Chest CT helps clinicians to facilitate appropriate PAVM management strategies.
Pulmonary arteriovenous malformation CT findings are correlated with risk of cerebral complications.
Risk of brain abscess is significantly correlated with number of PAVMs.
Risk of ischaemic stroke is significantly correlated with large feeding artery PAVMs.
Prevalence of observed of brain abscess and ischaemic stroke is 26 %.
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Literatur
1.
Zurück zum Zitat Guttmacher AE, Marchuk DA, White RI (1995) Hereditary hemorrhagic telangiectasia. N Engl J Med. 333:918–924CrossRefPubMed Guttmacher AE, Marchuk DA, White RI (1995) Hereditary hemorrhagic telangiectasia. N Engl J Med. 333:918–924CrossRefPubMed
2.
Zurück zum Zitat Cottin V, Chinet T, Lavolé A, Corre R, Marchand E, Reynaud-Gaubert M et al (2007) Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients. Medicine (Baltimore). 86:1–17CrossRefPubMed Cottin V, Chinet T, Lavolé A, Corre R, Marchand E, Reynaud-Gaubert M et al (2007) Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients. Medicine (Baltimore). 86:1–17CrossRefPubMed
3.
Zurück zum Zitat Burke CM, Safai C, Nelson DP, Raffin TA (1986) Pulmonary arteriovenous malformations: a critical update. Am Rev Respir Dis. 134:334–339PubMed Burke CM, Safai C, Nelson DP, Raffin TA (1986) Pulmonary arteriovenous malformations: a critical update. Am Rev Respir Dis. 134:334–339PubMed
4.
Zurück zum Zitat Gossage JR, Kanj G (1998) Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med. 158:643–661CrossRefPubMed Gossage JR, Kanj G (1998) Pulmonary arteriovenous malformations. A state of the art review. Am J Respir Crit Care Med. 158:643–661CrossRefPubMed
5.
Zurück zum Zitat Lacombe P, Lacout A, Marcy P-Y, Binsse S, Sellier J, Bensalah M et al (2013) Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. Diagn Interv Imaging. 94:835–848CrossRefPubMed Lacombe P, Lacout A, Marcy P-Y, Binsse S, Sellier J, Bensalah M et al (2013) Diagnosis and treatment of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: An overview. Diagn Interv Imaging. 94:835–848CrossRefPubMed
6.
Zurück zum Zitat Cottin V, Dupuis-Girod S, Lesca G, Cordier J-F (2007) Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). Respir Int Rev Thorac Dis. 74:361–378 Cottin V, Dupuis-Girod S, Lesca G, Cordier J-F (2007) Pulmonary vascular manifestations of hereditary hemorrhagic telangiectasia (rendu-osler disease). Respir Int Rev Thorac Dis. 74:361–378
7.
Zurück zum Zitat Trerotola SO, Pyeritz RE (2010) PAVM embolization: an update. AJR Am J Roentgenol. 195:837–845CrossRefPubMed Trerotola SO, Pyeritz RE (2010) PAVM embolization: an update. AJR Am J Roentgenol. 195:837–845CrossRefPubMed
8.
Zurück zum Zitat McAllister KA, Grogg KM, Johnson DW, Gallione CJ, Baldwin MA, Jackson CE et al (1994) Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nat Genet. 8:345–351CrossRefPubMed McAllister KA, Grogg KM, Johnson DW, Gallione CJ, Baldwin MA, Jackson CE et al (1994) Endoglin, a TGF-beta binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nat Genet. 8:345–351CrossRefPubMed
9.
Zurück zum Zitat Johnson DW, Berg JN, Baldwin MA, Gallione CJ, Marondel I, Yoon SJ et al (1996) Mutations in the activin receptor-like kinase 1 gene in hereditary haemorrhagic telangiectasia type 2. Nat Genet. 13:189–195CrossRefPubMed Johnson DW, Berg JN, Baldwin MA, Gallione CJ, Marondel I, Yoon SJ et al (1996) Mutations in the activin receptor-like kinase 1 gene in hereditary haemorrhagic telangiectasia type 2. Nat Genet. 13:189–195CrossRefPubMed
10.
Zurück zum Zitat Iyer NK, Burke CA, Leach BH (2010) Parambil JG. SMAD4 mutation and the combined syndrome of juvenile polyposis syndrome and hereditary haemorrhagic telangiectasia. Thorax. 65:745–746CrossRefPubMed Iyer NK, Burke CA, Leach BH (2010) Parambil JG. SMAD4 mutation and the combined syndrome of juvenile polyposis syndrome and hereditary haemorrhagic telangiectasia. Thorax. 65:745–746CrossRefPubMed
11.
Zurück zum Zitat Gallione CJ, Repetto GM, Legius E, Rustgi AK, Schelley SL, Tejpar S et al (2004) A combined syndrome of juvenile polyposis and hereditary haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4). Lancet Lond Engl. 363:852–859CrossRef Gallione CJ, Repetto GM, Legius E, Rustgi AK, Schelley SL, Tejpar S et al (2004) A combined syndrome of juvenile polyposis and hereditary haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4). Lancet Lond Engl. 363:852–859CrossRef
12.
Zurück zum Zitat Dupuis-Girod S, Giraud S, Decullier E, Lesca G, Cottin V, Faure F et al (2007) Hemorrhagic hereditary telangiectasia (Rendu-Osler disease) and infectious diseases: an underestimated association. Clin Infect Dis Off Publ Infect Dis Soc Am. 44:841–845CrossRef Dupuis-Girod S, Giraud S, Decullier E, Lesca G, Cottin V, Faure F et al (2007) Hemorrhagic hereditary telangiectasia (Rendu-Osler disease) and infectious diseases: an underestimated association. Clin Infect Dis Off Publ Infect Dis Soc Am. 44:841–845CrossRef
13.
Zurück zum Zitat Shovlin CL, Jackson JE, Bamford KB, Jenkins IH, Benjamin AR, Ramadan H et al (2008) Primary determinants of ischaemic stroke/brain abscess risks are independent of severity of pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia. Thorax. 63:259–266CrossRefPubMed Shovlin CL, Jackson JE, Bamford KB, Jenkins IH, Benjamin AR, Ramadan H et al (2008) Primary determinants of ischaemic stroke/brain abscess risks are independent of severity of pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia. Thorax. 63:259–266CrossRefPubMed
14.
Zurück zum Zitat Faughnan ME, Lui YW, Wirth JA, Pugash RA, Redelmeier DA, Hyland RH et al (2000) Diffuse pulmonary arteriovenous malformations: characteristics and prognosis. Chest. 117:31–38CrossRefPubMed Faughnan ME, Lui YW, Wirth JA, Pugash RA, Redelmeier DA, Hyland RH et al (2000) Diffuse pulmonary arteriovenous malformations: characteristics and prognosis. Chest. 117:31–38CrossRefPubMed
15.
Zurück zum Zitat Faughnan ME, Palda VA, Garcia-Tsao G, Geisthoff UW, McDonald J, Proctor DD et al (2011) International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet. 48:73–87CrossRefPubMed Faughnan ME, Palda VA, Garcia-Tsao G, Geisthoff UW, McDonald J, Proctor DD et al (2011) International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet. 48:73–87CrossRefPubMed
16.
Zurück zum Zitat Carette M-F, Nedelcu C, Tassart M, Grange J-D, Wislez M, Khalil A (2009) Imaging of hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol. 32:745–757CrossRefPubMed Carette M-F, Nedelcu C, Tassart M, Grange J-D, Wislez M, Khalil A (2009) Imaging of hereditary hemorrhagic telangiectasia. Cardiovasc Intervent Radiol. 32:745–757CrossRefPubMed
17.
Zurück zum Zitat Maher CO, Piepgras DG, Brown RD, Friedman JA, Pollock BE (2001) Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia. Stroke. 32:877–882CrossRefPubMed Maher CO, Piepgras DG, Brown RD, Friedman JA, Pollock BE (2001) Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia. Stroke. 32:877–882CrossRefPubMed
18.
Zurück zum Zitat Lee DW, White RI, Egglin TK, Pollak JS, Fayad PB, Wirth JA et al (1997) Embolotherapy of large pulmonary arteriovenous malformations: long-term results. Ann Thorac Surg. 64:930–939 discussion 939-940CrossRefPubMed Lee DW, White RI, Egglin TK, Pollak JS, Fayad PB, Wirth JA et al (1997) Embolotherapy of large pulmonary arteriovenous malformations: long-term results. Ann Thorac Surg. 64:930–939 discussion 939-940CrossRefPubMed
19.
Zurück zum Zitat Letourneau-Guillon L, Faughnan ME, Soulez G, Giroux M-F, Oliva VL, Boucher L-M et al (2010) Embolization of pulmonary arteriovenous malformations with amplatzer vascular plugs: safety and midterm effectiveness. J Vasc Interv Radiol JVIR. 21:649–656CrossRefPubMed Letourneau-Guillon L, Faughnan ME, Soulez G, Giroux M-F, Oliva VL, Boucher L-M et al (2010) Embolization of pulmonary arteriovenous malformations with amplatzer vascular plugs: safety and midterm effectiveness. J Vasc Interv Radiol JVIR. 21:649–656CrossRefPubMed
20.
Zurück zum Zitat White RI, Lynch-Nyhan A, Terry P, Buescher PC, Farmlett EJ, Charnas L et al (1988) Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 169:663–669CrossRefPubMed White RI, Lynch-Nyhan A, Terry P, Buescher PC, Farmlett EJ, Charnas L et al (1988) Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 169:663–669CrossRefPubMed
21.
Zurück zum Zitat White RI, Pollak JS, Wirth JA (1996) Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy. J Vasc Interv Radiol JVIR. 7:787–804CrossRefPubMed White RI, Pollak JS, Wirth JA (1996) Pulmonary arteriovenous malformations: diagnosis and transcatheter embolotherapy. J Vasc Interv Radiol JVIR. 7:787–804CrossRefPubMed
22.
Zurück zum Zitat Mager JJ, Overtoom TTC, Blauw H, Lammers JWJ, Westermann CJJ (2004) Embolotherapy of pulmonary arteriovenous malformations: long-term results in 112 patients. J Vasc Interv Radiol JVIR. 15:451–456CrossRefPubMed Mager JJ, Overtoom TTC, Blauw H, Lammers JWJ, Westermann CJJ (2004) Embolotherapy of pulmonary arteriovenous malformations: long-term results in 112 patients. J Vasc Interv Radiol JVIR. 15:451–456CrossRefPubMed
23.
Zurück zum Zitat Pollak JS, Saluja S, Thabet A, Henderson KJ, Denbow N, White RI (2006) Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. J Vasc Interv Radiol JVIR. 17:35–44 quiz 45CrossRefPubMed Pollak JS, Saluja S, Thabet A, Henderson KJ, Denbow N, White RI (2006) Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations. J Vasc Interv Radiol JVIR. 17:35–44 quiz 45CrossRefPubMed
24.
Zurück zum Zitat Gupta S, Faughnan ME, Bayoumi AM (2009) Embolization for pulmonary arteriovenous malformation in hereditary hemorrhagic telangiectasia: a decision analysis. Chest. 136:849–858CrossRefPubMed Gupta S, Faughnan ME, Bayoumi AM (2009) Embolization for pulmonary arteriovenous malformation in hereditary hemorrhagic telangiectasia: a decision analysis. Chest. 136:849–858CrossRefPubMed
25.
Zurück zum Zitat Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ et al (2000) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet. 91:66–67CrossRefPubMed Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ et al (2000) Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet. 91:66–67CrossRefPubMed
26.
Zurück zum Zitat Remy J, Remy-Jardin M, Giraud F, Wattinne L (1994) Angioarchitecture of pulmonary arteriovenous malformations: clinical utility of three-dimensional helical CT. Radiology. 191:657–664CrossRefPubMed Remy J, Remy-Jardin M, Giraud F, Wattinne L (1994) Angioarchitecture of pulmonary arteriovenous malformations: clinical utility of three-dimensional helical CT. Radiology. 191:657–664CrossRefPubMed
27.
Zurück zum Zitat Gamondès D, Si-Mohamed S, Cottin V, Gonidec S, Boussel L, Douek P et al (2016) Vein Diameter on Unenhanced Multidetector CT Predicts Reperfusion of Pulmonary Arteriovenous Malformation after Embolotherapy. Eur Radiol. 26:2723–2729CrossRefPubMed Gamondès D, Si-Mohamed S, Cottin V, Gonidec S, Boussel L, Douek P et al (2016) Vein Diameter on Unenhanced Multidetector CT Predicts Reperfusion of Pulmonary Arteriovenous Malformation after Embolotherapy. Eur Radiol. 26:2723–2729CrossRefPubMed
28.
Zurück zum Zitat Pierucci P, Murphy J, Henderson KJ, Chyun DA, White RI (2008) New definition and natural history of patients with diffuse pulmonary arteriovenous malformations: twenty-seven-year experience. Chest. 133:653–661CrossRefPubMed Pierucci P, Murphy J, Henderson KJ, Chyun DA, White RI (2008) New definition and natural history of patients with diffuse pulmonary arteriovenous malformations: twenty-seven-year experience. Chest. 133:653–661CrossRefPubMed
29.
Zurück zum Zitat Lacombe P, Lagrange C, Beauchet A, El Hajjam M, Chinet T, Pelage J-P (2009) Diffuse pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: long-term results of embolization according to the extent of lung involvement. Chest. 135:1031–1037CrossRefPubMed Lacombe P, Lagrange C, Beauchet A, El Hajjam M, Chinet T, Pelage J-P (2009) Diffuse pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: long-term results of embolization according to the extent of lung involvement. Chest. 135:1031–1037CrossRefPubMed
30.
Zurück zum Zitat Moussouttas M, Fayad P, Rosenblatt M, Hashimoto M, Pollak J, Henderson K et al (2000) Pulmonary arteriovenous malformations: cerebral ischemia and neurologic manifestations. Neurology. 55:959–964CrossRefPubMed Moussouttas M, Fayad P, Rosenblatt M, Hashimoto M, Pollak J, Henderson K et al (2000) Pulmonary arteriovenous malformations: cerebral ischemia and neurologic manifestations. Neurology. 55:959–964CrossRefPubMed
Metadaten
Titel
Pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia: Correlations between computed tomography findings and cerebral complications
verfasst von
Johan Etievant
Salim Si-Mohamed
Nicolas Vinurel
Sophie Dupuis-Girod
Evelyne Decullier
Delphine Gamondes
Chahera Khouatra
Vincent Cottin
Didier Revel
Publikationsdatum
10.10.2017
Verlag
Springer Berlin Heidelberg
Erschienen in
European Radiology / Ausgabe 3/2018
Print ISSN: 0938-7994
Elektronische ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-017-5047-x

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