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(a) Pandigital uniform clubbing with cyanosis. (b) Chest X-ray showing homogenous radio-dense lesion in the left lung field suggestive of pulmonary arteriovenous (AV) malformation. (c) Computed tomography (CT) pulmonary angiogram showing pulmonary AV malformation. (d) 3D reconstruction of CT pulmonary angiogram showing the feeders of the malformation.
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Percutaneous device closure is an established method to treat pulmonary arteriovenous malformation (PAVM). This report describes the case of a 23-year-old man with hereditary hemorrhagic telangiectasia (HHT) presenting with dyspnea and hypoxia. The patient was found to have a giant left-sided PAVM. The patient underwent percutaneous closure of PAVM...
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... The patient had symptoms of recurrent spontaneous epistaxis until three years before presentation. The patient's mother and elder brother also had symptoms of recurrent spontaneous epistaxes. On examination, the patient was deeply cyanosed with an arterial oxygen saturation of 82% while breathing room air. He had pandigital grade III clubbing (Fig. 1a). Telangiectatic lesions were noted in the oral cavity. His right arm blood pressure was 110/ 76 mmHg. The precordial examination was unremarkable. On further evaluation, his chest X-ray revealed a well defined homogenous opacity in the middle of his left lung field suggestive of pulmonary arteriovenous malformation (Fig. 1b). The ...
Context 2
... grade III clubbing (Fig. 1a). Telangiectatic lesions were noted in the oral cavity. His right arm blood pressure was 110/ 76 mmHg. The precordial examination was unremarkable. On further evaluation, his chest X-ray revealed a well defined homogenous opacity in the middle of his left lung field suggestive of pulmonary arteriovenous malformation (Fig. 1b). The patient was clinically diagnosed to have HHT based on Curaçao criteria. Contrast echocardiography was performed which showed almost immediate opacification of left-sided chambers. A 64-slice computed tomography of the thorax confirmed the presence of PAVM consisting of a conglomeration of multiple, tortuous, dilated, and intensely ...
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... criteria. Contrast echocardiography was performed which showed almost immediate opacification of left-sided chambers. A 64-slice computed tomography of the thorax confirmed the presence of PAVM consisting of a conglomeration of multiple, tortuous, dilated, and intensely enhancing vascular channels in inferior lingular segment of left upper lobe (Fig. 1c). The lesion measured Percutaneous device closure is an established method to treat pulmonary arteriovenous malformation (PAVM). This report describes the case of a 23-year-old man with hereditary hemorrhagic telangiectasia (HHT) presenting with dyspnea and hypoxia. The patient was found to have a giant left-sided PAVM. The patient ...
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... mm  49 mm  47 mm. Feeding arteries originated from the superior and inferior lingular segmental branches which measured 13 and 10.5 mm respectively (Fig. 1d). The two major feeders coursing into the lesion measured 8 and 7 mm respectively. The two draining veins (14 and 7 mm) emptied into the superior pulmonary vein. Contrast enhanced computed tomography of the abdomen revealed heterogenous enhancement of liver parenchy- ma suggestive of multiple telangiectasias and also evidence of a ...
Context 5
... The patient had symptoms of recurrent spontaneous epistaxis until three years before presentation. The patient's mother and elder brother also had symptoms of recurrent spontaneous epistaxes. On examination, the patient was deeply cyanosed with an arterial oxygen saturation of 82% while breathing room air. He had pandigital grade III clubbing (Fig. 1a). Telangiectatic lesions were noted in the oral cavity. His right arm blood pressure was 110/ 76 mmHg. The precordial examination was unremarkable. On further evaluation, his chest X-ray revealed a well defined homogenous opacity in the middle of his left lung field suggestive of pulmonary arteriovenous malformation (Fig. 1b). The ...
Context 6
... grade III clubbing (Fig. 1a). Telangiectatic lesions were noted in the oral cavity. His right arm blood pressure was 110/ 76 mmHg. The precordial examination was unremarkable. On further evaluation, his chest X-ray revealed a well defined homogenous opacity in the middle of his left lung field suggestive of pulmonary arteriovenous malformation (Fig. 1b). The patient was clinically diagnosed to have HHT based on Curaçao criteria. Contrast echocardiography was performed which showed almost immediate opacification of left-sided chambers. A 64-slice computed tomography of the thorax confirmed the presence of PAVM consisting of a conglomeration of multiple, tortuous, dilated, and intensely ...
Context 7
... criteria. Contrast echocardiography was performed which showed almost immediate opacification of left-sided chambers. A 64-slice computed tomography of the thorax confirmed the presence of PAVM consisting of a conglomeration of multiple, tortuous, dilated, and intensely enhancing vascular channels in inferior lingular segment of left upper lobe (Fig. 1c). The lesion measured Percutaneous device closure is an established method to treat pulmonary arteriovenous malformation (PAVM). This report describes the case of a 23-year-old man with hereditary hemorrhagic telangiectasia (HHT) presenting with dyspnea and hypoxia. The patient was found to have a giant left-sided PAVM. The patient ...
Context 8
... mm  49 mm  47 mm. Feeding arteries originated from the superior and inferior lingular segmental branches which measured 13 and 10.5 mm respectively (Fig. 1d). The two major feeders coursing into the lesion measured 8 and 7 mm respectively. The two draining veins (14 and 7 mm) emptied into the superior pulmonary vein. Contrast enhanced computed tomography of the abdomen revealed heterogenous enhancement of liver parenchyma suggestive of multiple telangiectasias and also evidence of a small ...
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A 76-year-old woman with hereditary hemorrhagic telangiectasia presented to the emergency department with chest pain. Workup revealed multiple bilateral pulmonary arteriovenous malformations (PAVMs) with large aneurysmal venous outflow. A collaborative approach between interventional radiology and thoracic surgery was used in the treatment of these...
CASE PRESENTATION Among other causes, arteriovenous malformations (AVMs) can be congenital and associated with Rendu-Osler-Weber disease. It is a conglomerate of anomalous vessels of variable size and number characterized by the lack of an intermediate normal capillary network between the arterial and the venous circuits. Its prevalence is of appro...
Background
Embolization is the standard of care for treatment of pulmonary arteriovenous malformations (PAVMs). Persistence of PAVMs after embolization occurs for undefined reasons but may include inflammation related to smoking in dysregulated angiogenesis.
Purpose
To determine whether patients with hereditary hemorrhagic telangiectasia (HHT) who...
Background:
Computed tomography (CT) is considered the imaging modality of choice to diagnose pulmonary arteriovenous malformations PAVMs. The drawback of this technique is that it requires ionizing radiation. Magnetic resonance (MR) imaging does not have the limitation, but little is known about the performance of MR compared to CT for the detect...
Objectives
This study evaluated the diagnostic value of transesophageal contrast echocardiography (TECE) with the agitated saline test for diagnosing pulmonary arteriovenous malformations (PAVMs) in comparison with chest CT or pulmonary angiography.
Background
Although transthoracic contrast echocardiography (TTCE) is the recommended screening tes...
Citations
... Embolisation is technically difficult for such cases because it requires occlusion of multiple supply arteries. 1 Recently, embolisation of the sac, in addition to the pulm onary artery, has been suggested to reduce artery reopening. 2 We followed this approach in this case; therefore, more coils and time were required. ...
... In recent years, Amplatzer ® devices have been the preferred device for occlusion of this type and, in general, for vascular malformations 5,6 . In the review of national and international publications, we did not find another case in which this number of malformations has been occluded percutaneously in the same patient and especially in the same lung, in some of the reported cases they were single lesions, and in those with multiple pulmonary arteriovenous fistulas, the location and/or size was not feasible for closure [7][8][9] . ...
... In recent years, Amplatzer® devices have been the preferred device for occlusion of this type and, in general, for vascular malformations 5,6 . In the review of national and international publications, we did not find another case in which this number of malformations has been occluded percutaneously in the same patient and especially in the same lung, in some of the reported cases they were single lesions, and in those with multiple pulmonary arteriovenous fistulas, the location and/or size was not feasible for closure [7][8][9] . ...
Hereditary hemorrhagic telangiectasia (HHT) or Osler Weber Rendu Syndrome (OWRS) is a multiorgan disorder, of autosomal dominant inheritance, resulting in fibrovascular dysplasia with multiple arteriovenous malformations in different organs1. Pulmonary arteriovenous malformations are the most common pulmonary findings found in patients with HHT2,3. They can be asymptomatic or may present various symptoms such as cyanosis, dyspnea, heart failure, hemoptysis, and paradoxical embolism4.
... However, due to the presence of a resistant blood-brain barrier and abundant cerebral circulation, the probability of these abscesses following pulmonary AVM is about 5% (11), which was also observed in our patient, that can be due to the spread, reanalyzed and new lesion with a high volume of the shunt inside the lungs (12). ...
... Safety and efficacy of endovascular methods in patients with AVMs has been proven, but due to high preva- lence of complications and the risk of recurrence of longterm follow-up lesions, it is recommended to undergo high resolution CT scans every three to five years for lung lesions (11). Furthermore, the use of anticoagulants have been reported to prevent brain damage, nevertheless whether these drugs are safe or not should be further investigated (15). ...
Introduction: Arteriovenous malformations (AVM) are rare chronic congenital diseases whose nature are not yet fully understood. Two different types of these disorders are pulmonary and brain malformations, which are seen sporadically or in combination with other syndromes. Symptoms of these diseases appear at different ages and present differently in patients, but in general, the combination of these two disorders is rarely seen. There are several treatments based on the type of lesion and symptoms and other accompanying conditions, one of which is interventional procedure. Case Presentation: In this article, a rare case of pulmonary and brain malformation has been reported, which has progressed even after treatment and has been closed interventionally. Conclusions: Patients with pulmonary AVMs need careful examination for other congenital disorders. Furthermore, due to the unknown nature of the disease and its progression, the treatment of these lesions is completely personal and varies according to each patient.
Pulmonary arteriovenous malformations (PAVMs) are rare vascular lesions characterised by abnormal connections between the pulmonary artery and vein bypassing the pulmonary capillary bed and causing right-to-left shunt. Paradoxical embolism is known to occur in these cases, leading to inoculation of septic focus in the systemic circulation. We report a case of multiple PAVMs who presented clinically with seizures and altered sensorium. On radiological work-up, multiple brain abscesses and large PAVMs were evident. The patient was successfully treated by endovascular embolisation of the PAVMs using a vascular plug and multiple coils. The patient showed complete clinical recovery and resolution of brain abscesses on follow-up.
