Pulmonary arteriovenous malformations in Rendu-Osler-Weber syndrome

Rendu-Osler-Weber syndrome, also known as hereditary hemorrhagic telangiectasia, is an autosomal dominant hereditary disorder. It is characterized by presence of multiple arteriovenous malformations (AVMs) and telangiectasias. This article reports two cases of patients with Rendu-Osler-Weber syndrome who had pulmonary AVMs and underwent successful endovascular treatment. A brief review of the literature shows that up to 50% of patients with the syndrome have pulmonary AVMs and there is usually a positive family history in these patients. These pulmonary AVMs are multiple in 30% of cases and are associated with the most severe disease complications. Most patients are asymptomatic, even in the presence of AVMs with right-left shunts. When these shunts exceed 25% of the total blood volume, dyspnea, cyanosis, digital clubbing, and extracardiac murmurs may occur. Endovascular treatment is safe and offers control of complications from hereditary hemorrhagic telangiectasia and is currently the treatment of choice for these lesions.

Keywords:
embolization, therapeutic; arteriovenous fistula; telangiectasia, hereditary hemorrhagic; Rendu-Osler-Weber

Authorship

Cristiane Ferreira de Araújo-Gomes

conception and design

analysis and interpretation

data collection

writing the article

critical revision of the article

final approval of the article

overall responsibility

Primary physicians, Serviço de Cirurgia Vascular, Hospital Universitário Pedro Ernesto (HUPE), Universidade do Estado do Rio de Janeiro (UERJ).

Universidade do Estado do Rio de Janeiro - UERJ, Hospital Universitário Pedro Ernesto - HUPE, Rio de Janeiro, RJ, Brasil.Universidade do Estado do Rio de JaneiroBrasilRio de Janeiro, RJ, BrasilUniversidade do Estado do Rio de Janeiro - UERJ, Hospital Universitário Pedro Ernesto - HUPE, Rio de Janeiro, RJ, Brasil.

https://orcid.org/0000-0003-4329-5374

Carlos Eduardo Virgini-Magalhães

critical revision of the article

final approval of the article

Associate professor, Faculdade de Ciências Médicas (FCM), Universidade do Estado do Rio de Janeiro (UERJ); Head of Department, Serviço de Cirurgia Vascular, Hospital Universitário Pedro Ernesto (HUPE), Universidade do Estado do Rio de Janeiro (UERJ).

https://orcid.org/0000-0002-0422-2298

Leonardo Silveira de Castro

analysis and interpretation

data collection

final approval of the article

Primary physicians, Serviço de Cirurgia Vascular, Hospital Universitário Pedro Ernesto (HUPE), Universidade do Estado do Rio de Janeiro (UERJ).

https://orcid.org/0000-0002-9914-0868

Eduardo de Oliveira Rodrigues Neto

conception and design

data collection

final approval of the article

Primary physicians, Serviço de Cirurgia Vascular, Hospital Universitário Pedro Ernesto (HUPE), Universidade do Estado do Rio de Janeiro (UERJ).

https://orcid.org/0000-0002-9217-3480

Alex Antunes Bezerra

conception and design

writing the article

final approval of the article

Primary physicians, Serviço de Cirurgia Vascular, Hospital Universitário Pedro Ernesto (HUPE), Universidade do Estado do Rio de Janeiro (UERJ).

https://orcid.org/0009-0006-6013-2744

Monica Rochedo Mayall

analysis and interpretation

final approval of the article

Primary physicians, Serviço de Cirurgia Vascular, Hospital Universitário Pedro Ernesto (HUPE), Universidade do Estado do Rio de Janeiro (UERJ).

https://orcid.org/0000-0001-6011-6783

Cristina Ribeiro Riguetti-Pinto

data collection

final approval of the article

Assistant professor, Faculdade de Ciências Médicas (FCM), Universidade do Estado do Rio de Janeiro (UERJ).

https://orcid.org/0000-0001-6509-4018

Felipe Borges Fagundes

conception and design

data collection

final approval of the article

Primary physicians, Serviço de Cirurgia Vascular, Hospital Universitário Pedro Ernesto (HUPE), Universidade do Estado do Rio de Janeiro (UERJ).

https://orcid.org/0000-0003-4466-0513

Conflicts of interest: No conflicts of interest declared concerning the publication of this article.

Correspondence Carlos Eduardo Virgini-Magalhães Universidade do Estado do Rio de Janeiro - UERJ, Hospital Universitário Pedro Ernesto - HUPE, Serviço de Cirurgia Vascular, Secretaria da Cirurgia Vascular e Endovascular Boulevard Vinte e Oito de Setembro, 77, 4º andar - Vila Isabel CEP 20551-030 - Rio de Janeiro (RJ), Brasil Tel.: +55 (21) 99100-1597 / +55 (21) 2868-8643 E-mail: cevirgini@gmail.com

*
All authors have read and approved of the final version of the article submitted to J Vasc Bras.

SCIMAGO INSTITUTIONS RANKINGS

Figures | Tables

Figures (11)
Tables (1)

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Figure 1
Angiotomography of the chest (case 1) with hypervascularized nodular lesions characteristic of arteriovenous malformations.

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Figure 2
Selective arteriography identifying voluminous pulmonary arteriovenous malformations (case 1).

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Figure 3
Embolization of arteriovenous malformations in case 1. (A) Selective catheterization of the arteriovenous malformation in the lower right lobe; and (B) result immediately after embolization with six pushable coils.

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Figure 4
Embolization of arteriovenous malformations in case 1. (A) Selective catheterization of peripheral arteriovenous malformation; and (B) deployment of pushable coil.

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Figure 5
Embolization of arteriovenous malformations in case 1. (A) Selective catheterization of voluminous arteriovenous malformation; and (B) deployment of Amplatzer^® plug. Detail (arrow) of deployment of the last ring of the plug to occlude the smaller caliber arteriovenous malformation.

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Figure 6
Physical examination of the patient (case 2) - normal, except for a small telangiectasia in the oral cavity.

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Figure 7
Angiotomography identified multiple nodular hypervascularized images in the hepatic parenchyma compatible with arteriovenous malformations (case 2).

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Figure 8
Treatment sequence of the voluminous arteriovenous malformation (case 2). (A) Selective catheterization of arteriovenous malformation in the lower right lobe; and (B) deployment of the Amplatzer^® plug. Control arteriography (C) still shows flow through the plug mesh, which is to be expected immediately after deployment.

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Figure 9
Embolization of arteriovenous malformations in case 2. Selective catheterization of arteriovenous malformation in the lower right lobe (A) and control angiography after embolization with four pushable coils (B).

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Figure 10
Embolization of arteriovenous malformations in case 2. Selective catheterization of the arteriovenous malformation in the upper right lobe (A) and control angiography after embolization with two pushable coils (B).

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Figure 11
Reintervention in case 2. Selective catheterization and identification of a de novo arteriovenous malformation adjacent to the occluder plug deployed in the first procedure (A) and control angiography after embolization with ten pushable coils (B).

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Table 1
Curaçao criteria.

Figure 1 Angiotomography of the chest (case 1) with hypervascularized nodular lesions characteristic of arteriovenous malformations.

Figure 2 Selective arteriography identifying voluminous pulmonary arteriovenous malformations (case 1).

Figure 3 Embolization of arteriovenous malformations in case 1. (A) Selective catheterization of the arteriovenous malformation in the lower right lobe; and (B) result immediately after embolization with six pushable coils.

Figure 4 Embolization of arteriovenous malformations in case 1. (A) Selective catheterization of peripheral arteriovenous malformation; and (B) deployment of pushable coil.

Figure 5 Embolization of arteriovenous malformations in case 1. (A) Selective catheterization of voluminous arteriovenous malformation; and (B) deployment of Amplatzer^® plug. Detail (arrow) of deployment of the last ring of the plug to occlude the smaller caliber arteriovenous malformation.

Figure 6 Physical examination of the patient (case 2) - normal, except for a small telangiectasia in the oral cavity.

Figure 7 Angiotomography identified multiple nodular hypervascularized images in the hepatic parenchyma compatible with arteriovenous malformations (case 2).

Figure 8 Treatment sequence of the voluminous arteriovenous malformation (case 2). (A) Selective catheterization of arteriovenous malformation in the lower right lobe; and (B) deployment of the Amplatzer^® plug. Control arteriography (C) still shows flow through the plug mesh, which is to be expected immediately after deployment.

Figure 9 Embolization of arteriovenous malformations in case 2. Selective catheterization of arteriovenous malformation in the lower right lobe (A) and control angiography after embolization with four pushable coils (B).

Figure 10 Embolization of arteriovenous malformations in case 2. Selective catheterization of the arteriovenous malformation in the upper right lobe (A) and control angiography after embolization with two pushable coils (B).

Figure 11 Reintervention in case 2. Selective catheterization and identification of a de novo arteriovenous malformation adjacent to the occluder plug deployed in the first procedure (A) and control angiography after embolization with ten pushable coils (B).

Table 1 Curaçao criteria.

1. Multiple telangiectasias at typical sites (face, lips, hands, and oral cavity)

2. Recurrent nosebleeds

3. AVMs with visceral involvement (pulmonary, hepatic, cerebral, gastrointestinal, or spinal)

4. Family history (first degree relative with ROWS)

Note: The diagnosis of ROWS is definitive if three criteria are present; possible or suspected if two criteria are positive; and improbable if only one criterion is identified. AVMs = arteriovenous malformations; ROWS = Rendu-Osler-Weber syndrome.

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[1] Conflicts of interest: No conflicts of interest declared concerning the publication of this article.