Figure 1
Axial and reformatted CT images
Clinical History
Cigarette smoker who presented with a hoarse voice. Direct laryngoscopy demonstrated a left vocal cord palsy.
Imaging Findings
The patient, cigarette smoker, presented with a hoarse voice to our regional teaching hospital ENT clinic. At the time of attendance, direct laryngoscopy was performed demonstrating a paralysed left vocal cord, but otherwise normal appearances of the larynx and pharynx.
A chest radiograph was acquired which showed a left mediastinal mass. The patient was admitted for further investigation.
Computed tomography of the thorax confirmed the presence of a mediastinal mass, which was vascular in origin, aneurysmal in nature and arising from the inferior aspect of the aortic arch, the lesion measured 6cm in trans-axial dimension and contained considerable mural thrombus. This was a patent ductus arteriosus (PDA) aneurysm. The diagnosis of Ortners syndrome was established. Thorough examination of the patient's case notes revealed no history of childhood PDA. Selected axial CT images after rapid infusion of contrast along with reformatted images demonstrate the abnormality.
A chest radiograph was acquired which showed a left mediastinal mass. The patient was admitted for further investigation.
Computed tomography of the thorax confirmed the presence of a mediastinal mass, which was vascular in origin, aneurysmal in nature and arising from the inferior aspect of the aortic arch, the lesion measured 6cm in trans-axial dimension and contained considerable mural thrombus. This was a patent ductus arteriosus (PDA) aneurysm. The diagnosis of Ortners syndrome was established. Thorough examination of the patient's case notes revealed no history of childhood PDA. Selected axial CT images after rapid infusion of contrast along with reformatted images demonstrate the abnormality.
Discussion
The ducus arteriosus is a necessary fetal circulatory pathway allowing the carriage of oxygenated blood from the pulmonary artery to the aorta. Shortly after birth this closes, probably as a result of the drop in resistance in the pulmonary circulation, and the mechanism is thought to be due to prostaglandin inhibition. Continuing patency of the ductus arteriosus accounts for 5% of congenital heart disease.
Aneurysm formation in the PDA has been reported before in both adults and children. It is a rare but potentially fatal condition. The pathogenesis appears unclear. It is most commonly associated with missed childhood PDA. However, it has been reported in vasculitic processes ie. Kawasaki's disease, connective tissue disorders such as Marfan's syndrome or following recanalisation of the PDA after aneurysmal dilatation in aortic co-arctation.
Symptoms are often discrete, but the most usual presentation is dyspnoea due to heart failure. Left recurrent nerve palsy as a result of cardiovascular pathology is termed Ortner's syndrome. Laryngeal palsy as a presenting feature of PDA aneurysm is extremely rare. In rupture this condition is commonly fatal.
Investigation involves chest radiography and CT scanning, colour doppler echocardiography and aortography.
While there is controversy surrounding whether all silent PDAs require closure, most authors agree that the treatment of the symptomatic aneurysmal ductus arteriosus is mandatory, due to the risk of rupture and endarteritis. Treatment is often operative ligation of the patent ductus arteriosus, usually performed in a specialist cardiothoracic centre through a left thoracotomy, the risks including left recurrent laryngeal nerve damage and extensive haemorrhage. A move towards endovascular closure in the radiology department has been reported as far less invasive and associated with considerably shortened hospital stay. The approach includes access via common femoral artery puncture and cannulation of the PDA with deployment of a suitably sized Raskind occlusive device or coils. The risks include cerebrovascular accident, mal-deployment, and failure to occlude.
Aneurysm formation in the PDA has been reported before in both adults and children. It is a rare but potentially fatal condition. The pathogenesis appears unclear. It is most commonly associated with missed childhood PDA. However, it has been reported in vasculitic processes ie. Kawasaki's disease, connective tissue disorders such as Marfan's syndrome or following recanalisation of the PDA after aneurysmal dilatation in aortic co-arctation.
Symptoms are often discrete, but the most usual presentation is dyspnoea due to heart failure. Left recurrent nerve palsy as a result of cardiovascular pathology is termed Ortner's syndrome. Laryngeal palsy as a presenting feature of PDA aneurysm is extremely rare. In rupture this condition is commonly fatal.
Investigation involves chest radiography and CT scanning, colour doppler echocardiography and aortography.
While there is controversy surrounding whether all silent PDAs require closure, most authors agree that the treatment of the symptomatic aneurysmal ductus arteriosus is mandatory, due to the risk of rupture and endarteritis. Treatment is often operative ligation of the patent ductus arteriosus, usually performed in a specialist cardiothoracic centre through a left thoracotomy, the risks including left recurrent laryngeal nerve damage and extensive haemorrhage. A move towards endovascular closure in the radiology department has been reported as far less invasive and associated with considerably shortened hospital stay. The approach includes access via common femoral artery puncture and cannulation of the PDA with deployment of a suitably sized Raskind occlusive device or coils. The risks include cerebrovascular accident, mal-deployment, and failure to occlude.
Differential Diagnosis List
Aneurysm of the Patent Ductus Arteriosus - Ortner's syndrome
Final Diagnosis
Aneurysm of the Patent Ductus Arteriosus - Ortner's syndrome
References
[1]
Teneja K et al.
Ductus arteriosus aneurysm in the adult.
Clin radiol 1997 Mar; 52(3): 231-4. (PMID: 9091260)
[2]
Peivandi AA et al.
Aneurysm of the ductus arteriosus.
Herz 2000 Nov; 25(7): 703-6. (PMID: 11141680)
[3]
Botta AM et al.
Silent Patent Ductus Arteriosus.
Arq Bras Cardiol 2002 Sept; 79(3): 302-7. (PMID: 12386732)
[4]
Thilen U. et al.
Does the risk of endarteritis justify routine patent ductus arteriosus closure?
Eur Heart J; 18: 503-6. (PMID: 9076389)
[5]
Lund JT et al.
Aneurysm of the ductus arteriosus.
Eur J Cardiothoracic Surg 1991;5:566-70. (PMID: 1772665)
Case information
| URL: | https://eurorad.org/case/2684 |
| DOI: | 10.1594/EURORAD/CASE.2684 |
| ISSN: | 1563-4086 |
