Ann Thorac Surg 2009;87:946-948. doi:10.1016/j.athoracsur.2008.07.034
© 2009 The Society of Thoracic Surgeons
Case Reports
Giant Ductal Aneurysm in an Asymptomatic 4-Year-Old Girl
Ritu Sachdeva, MDa,*,
Clinton Smith, MDd,
Bruce S. Greenberg, MDb,
Robert D.B. Jaquiss, MDc
a Division of Pediatric Cardiology, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, Arksansas
b Department of Radiology, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, Arksansas
c Division of Pediatric Cardiothoracic Surgery, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, Arksansas
d Division of Pediatric Cardiology, University of Mississippi, Jackson, Mississippi
Accepted for publication July 11, 2008.
* Address correspondence to Dr Sachdeva, 1900 Maryland, Slot 512-3, Little Rock, AR 72202 (Email: sachdevaritu{at}uams.edu).
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Abstract
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Ductal aneurysm is a rare but potentially fatal condition. We report the case of an asymptomatic 4-year-old girl who was noted to have a murmur and with further evaluation by echocardiography and computed tomographic angiography was diagnosed with a giant ductal aneurysm. The aneurysm occupied a large space in the mediastinum, compressed the pulmonary arteries, and was in close proximity to the sternum. At surgery, the aneurysm was successfully resected after careful separation from adjacent structures. Because the anterolateral wall of the ascending aorta was noted to be extraordinarily thinned-out, it was replaced with a patch.
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Introduction
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Ductal aneurysm is a rare lesion that is usually diagnosed in infancy. Although some of these aneurysms spontaneously regress, others cause complications such as compression of adjacent structures (ie, bronchi, pulmonary arteries, esophagus), thromboembolism, infection, and rupture [1–3]. Rarely, acute collapse of a lung or recurrent laryngeal nerve palsy may also occur [4, 5]. We describe successful resection of a giant ductal aneurysm that was incidentally diagnosed in a 4-year-old girl.
A murmur was noted in a previously healthy 4-year-old girl on routine examination. She was the full-term product of an uncomplicated pregnancy, had not been hospitalized since birth, and had no family history of premature death in children or young adults. An echocardiogram showed a large aneurysm arising from the undersurface of the aortic arch, with normal intracardiac anatomy. A computed tomographic angiogram showed a giant ductal aneurysm (5.4 cm x 5.7 cm x 4.2 cm) causing mild compression of the right pulmonary artery. Possibility of connective tissue disorders including Ehler-Danlos, Marfan's, and Loeys-Dietz syndrome was raised, but was considered unlikely by a genetics consultant. The patient was referred for treatment to a pediatric cardiac surgical center. On examination, the patient had a grade 3/6 continuous murmur with a visible and palpable impulse in the second and third left interspaces in the parasternal region. There were no obvious physical features of connective tissue disorder. Because there had been a 6-month interval since the original computed tomographic scan, the study was repeated, demonstrating an increase in size of the aneurysm to 6.1 cm x 5.9 cm x 4.2 cm (Fig 1). The aneurysm wrapped around the ascending aorta, displacing the left pulmonary artery and mildly compressing the right pulmonary artery (Fig 2). It was also noted to be in very close proximity (3 mm) to the posterior sternal table. The ascending aorta measured 1.6 cm and the descending aorta measured 1.3 cm. Intraoperatively the ascending aorta wrapped by the aneurysm was discovered to be remarkably thinned out, but this finding was not appreciated on the computed tomographic scan.
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Fig 1. Axial image of computed tomographic angiogram showing the giant ductal aneurysm (DA), with a broad origin from the aortic arch (AO).
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Fig 2. A three-dimensional reconstructed image from computed tomographic angiogram showing the giant ductal aneurysm (DA) extending anteriorly and wrapping around the ascending aorta (AAO).
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At surgery, the sternum was carefully divided because of the proximity of the aneurysm, which fortunately was not adherent. The extent of the aneurysm was such that there was not enough normal aorta to permit direct cannulation for bypass; therefore a 4-mm thin-walled polytetrafluoroethylene graft was anastomosed to the innominate artery to use for arterial inflow. The patient was then heparinized and bi-caval venous cannulation was accomplished. Only after the patient was connected to the bypass circuit was preliminary dissection of the aneurysm undertaken (Fig 3). Cardiopulmonary bypass was initiated, and the patient was cooled to 22°C. During cooling additional dissection was accomplished, separating the aneurysm from the pulmonary arteries. The ligamentum arteriosum connecting this aneurysm to the pulmonary artery was then suture ligated and divided. The aneurysm was separated from the ascending aorta the wall, which was noted to be remarkably thin, as though it was eroded by the aneurysm. An antegrade cardioplegia catheter was placed very proximally on the ascending aorta in an area with normal wall thickness. A cross clamp was placed just distal to the cardioplegia site and cardioplegia was administered. Next, the proximal descending aorta was clamped, the left carotid and subclavian arteries were occluded with vascular tapes, and a clamp was placed on the base of the innominate artery with simultaneous reduction in bypass flow. With the aortic arch thus isolated, the aneurysm was resected completely from the undersurface of the arch. The left lateral and anterior wall of the ascending aorta, which had abutted the aneurysm, was also resected as it appeared paper thin. The resultant large defect in the ascending aorta and aortic arch was then repaired with a large patch fashioned from a tubular Dacron graft (Hemashield [Boston Scientific, Natick, MA]). The postoperative course was uneventful, and the patient was discharged home on day 3 after surgery. Examination of the pathologic specimen from the ductal aneurysm revealed atherosclerotic plaques (Fig 4). The thinned out aortic wall specimen showed reactive intimal proliferation.
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Fig 4. Specimen of the resected giant ductal aneurysm showing atherosclerotic plaques over the intimal surface.
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Comment
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Our case demonstrates that an unrecognized ductal aneurysm can potentially become a giant mediastinal mass and cause compression of adjacent structures. This ductal aneurysm was constantly subjected to systemic pressures due to its direct communication with the aorta and was significantly at risk of rupturing. Thinning of the wall of the ascending aorta was an unexpected finding in this case; we believe that this has not been previously reported. Lund and colleagues [1] reviewed 144 cases of ductal aneurysm present in the literature [1]. Of these, 106 appeared spontaneously and 38 followed surgical treatment of patent ductus arteriosus. Serious complications associated with spontaneous ductal aneurysm were rupture, erosion, infection, and thromboembolism. The complication rate was 31% in infants less than 2 months and increased to 66% in children between 2 months and 15 years and 47% in adults. The rate of complications after postoperative ductal aneurysms was even higher, with death in 91% of cases related to infection or rupture. The operative mortality was higher in infants less than 2 months of age and those with postoperative ductal aneurysm. Based on their review, Lund and colleagues [1] recommended prompt surgical treatment of all spontaneous ductal aneurysms in patients older than 2 months and all those with postoperative aneurysms. In a multicenter experience of 24 cases of ductal aneurysms, 9 were detected antenatally and 15 postnatally [2]. All but 2 patients in this series were diagnosed prior to 2 months of age. One fourth of these patients had chromosomal anomalies or genetic syndromes. As in this case, the size and extent of a giant ductal aneurysm may require significant alterations in operative strategy, particularly with regard to the conduct of bypass, such as the use of regional low-flow cerebral perfusion in this case.
In conclusion, giant ductal aneurysm, a potentially lethal condition may present with subtle clinical signs in children and can be associated with significant thinning of the ascending aorta. Surgical resection of such aneurysms should be performed to avoid potentially fatal complications.
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References
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- Lund JT, Jensen MB, Hjelms E. Aneurysm of the ductus arteriosus. A review of the literature and the surgical implications. Eur J Cardiothorac Surg 1991;5:566-570.[Abstract/Free Full Text]
- Dyamenahalli U, Smallhorn JF, Geva T, et al. Isolated ductus arteriosus aneurysm in the fetus and infant: a multi-institutional experience J Am Coll Cardiol 2000;36:262-269.[Abstract/Free Full Text]
- Acherman RJ, Siassi B, Wells W, et al. Aneurysm of the ductus arteriosus: a congenital lesion Am J Perinatol 1998;15:653-659.[Medline]
- Taskar VS, John PJ, Mahashur AA. Ductal aneurysm presenting as an acute lung collapse J Assoc Physicians India 1992;40:475-476.[Medline]
- Day JR, Walesby RK. A spontaneous ductal aneurysm presenting with left recurrent laryngeal nerve palsy Ann Thorac Surg 2001;72:608-609.[Abstract/Free Full Text]
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Abstract
Introduction
