British Journal of Radiology (2004) 77, 620-622
© 2004 British Institute of Radiology
doi: 10.1259/bjr/49154570
Transpleural ultrasound diagnosis of a pulmonary arteriovenous malformation
A M Groves, MRCP, FRCR
1
T C See, FRCS, FRCR
1
D S Appleton, MA, FRCR
1
T Win, MRCP
2 and
N J Screaton, MRCP, FRCR
1
1 Department of Radiology, Addenbrooke's Hospital, Cambridge University Teaching Hospitals Trust, Hills Road, Cambridge CB2 2QQ and 2 Department of Medicine, Papworth Hospital, Papworth Everard, Cambridge CB3 8RE, UK
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Abstract
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Pulmonary arteriovenous malformations (PAVMs) are rare. We discuss a case of an 81-year-old female who attended hospital with a haemothorax. Ultrasound not only demonstrated an echogenic effusion in the right pleural space, but also identified an associated tubular structure. Doppler was applied to this structure, which exhibited pulsatile flow. This raised the possibility of a PAVM, which was subsequently confirmed on CT and angiography. Although, PAVM is a rare cause of haemothorax, the diagnosis should still be considered and transpleural ultrasound can detect these malformations non-invasively by the bedside.
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Introduction
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Abnormal connections between the pulmonary arteries and veins, "pulmonary arteriovenous malformations" (PAVMs), may occur congenitally, of which about 90% are associated with hereditary haemorrhagic telangectasia (HHT) [1]. They can be acquired secondary to trauma, cirrhosis, infection and metastases [2]. In the thorax the main complications are hypoxaemia often exacerbated in the erect position ("orthodeoxia"), and life threatening haemoptysis. There are also sporadic case reports of spontaneous haemothorax [3], especially in pregnancy [4]. In one series, during a 6 year follow up of 159 patients who were referred for transcatheter vaso-occlusion for PAVM, haemothorax was only a feature in 5 subjects [5]. We describe a patient who presented with a spontaneous haemothorax secondary to PAVM, the diagnosis of which was suspected initially on transpleural Doppler ultrasound.
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Case report
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An 81-year-old woman presented with vague right sided chest and abdominal pain, and a productive cough with blood stained sputum. Examination showed intermittent tachycardia, labile blood pressure, reduced air entry at the right lung base and mild right sided abdominal tenderness. There were no visible signs of telangiectasia. She was found to be progressively anaemic. Chest X-ray showed a right pleural effusion. Ultrasound was performed for the purpose of possible pleural aspiration. This demonstrated loculated fluid in the right pleural space, with multiple internal echoes. In addition, a tubular structure was identified (Figure 1a
) and interrogation with spectral Doppler exhibited an arterial trace (Figure 1b), interpreted as likely to represent a PAVM. Pleural aspiration revealed blood. CT (Figure 2) confirmed the ultrasound appearances, and demonstrated a homogeneously enhancing lesion peripherally in the middle lobe with an exophytic configuration. This had a single feeding vessel arising from the middle lobe pulmonary artery and a single draining vein characteristic of a PAVM.
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Figure 1. Transverse ultrasound image of the right chest demonstrating: (a) a low echogenicity tubular structure, containing a high echogenic focus in its periphery, presumed to represent vessel with calcification in its wall. An echogenic pleural effusion is present in (b) spectral Doppler trace from the vessel, confirming arterial blood flow.
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Figure 2. Coronal maximal intensity projection (MIP) 4-detector multislice CT image demonstrating an enhancing lesion (arrow) arising from the middle lobe and clearly identifying the feeding and draining vessels.
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Urgent pulmonary arteriography from a femoral approach demonstrated the PAVM in the right middle lobe artery with a single feeding artery (Figure 3). Coil embolisation was performed to occlude the PAVM (Figure 4). The patient was stabilized and the haemothorax slowly resolved. Repeat CT showed the PAVM was occluded and the patient was discharged 2 weeks later without recurrence.
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Figure 3. Selective digitally subtracted right pulmonary artery angiogram (predominantly arterial phase) demonstrating the right middle lobe arteriovenous malformation.
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Discussion
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Mortality from PAVM is claimed to be 11% arising from complications, which include cerebral vascular accident, intracranial abscess, as well as from the pulmonary manifestations [6]. For this reason it is important to make a definitive diagnosis when the condition is suspected. Bubble contrast enhanced echocardiography has been utilized as a screening tool for the detection of PAVM [7]. The method detects the presence of a pulmonary right to left shunt and hence does not directly visualize the PAVM. Although transpleural ultrasound was successfully employed in a small series of pateints to diagnose PAVMs [8], its use has not been widely reported.
Many other investigations have been used such as CT, nuclear medicine techniques and angiography [2]. Injection of Technetium 99m labelled macroaggregates has been used to screen for PAVMs [9]. However like echo, it is an indirect method, relying on the detection of a right to left shunt. As our case demonstrates, CT is believed to be the most useful modality in the routine diagnosis of PAVM [10]. It can detect the site and number of PAVMs, as well as elegantly demonstrating vascular architecture, which is essential prior to therapy. With respect to the latter, it is important to assess the number of vessels connecting into the malformation as well as the orientation of the feeding arteries. In order to maximize the architectural detail, it has been recommended that both axial and three-dimensional reconstructions be performed and analysed concomitantly, although both sets of data can be obtained from a single acquisition [10]. Angiography is still useful in planning treatment, particularly for demonstrating feeding vessel anatomy [1]. Transpleural ultrasound is commonly employed to visualize and assist drainage of pleural effusion. Although, PAVM is a rare cause of haemothorax, the diagnosis should still be considered. This case demonstrates that transpleural ultrasound can detect these malformations non-invasively by the bedside.
Received for publication May 8, 2003.
Revision received September 15, 2003.
Accepted for publication November 12, 2003.
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References
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Abstract
Introduction
