Ann Thorac Surg 2002;74:245-246
© 2002 The Society of Thoracic Surgeons
Case report
Repair of a complex aortic arch anomaly associated with cutaneous hemangioma
Gaetano Gargiulo, MD*a,
Carlo Pace Napoleone, MDa,
Alessandro Giardini, MDb,
Roberto Formigari, MDb,
Angelo Pierangeli, MDa
a Department of Cardiac Surgery, University of Bologna, Policlinico S. Orsola-Malpighi, Bologna, Italy
b Department of Pediatric Cardiology, University of Bologna, Policlinico S. Orsola-Malpighi, Bologna, Italy
Accepted for publication February 17, 2002.
* Address reprint requests to Dr Gargiulo, Department of Cardiac Surgery, University of Bologna, Policlinico S. Orsola-Malpighi, Via Massarenti, 9, 40138, Bologna, Italy
e-mail: gargiulo{at}orsola-malpighi.med.unibo.it
 |
Abstract
|
|---|
Aortic coarctation and cutaneous hemangioma is a rare association. We describe the case of a neonate with abnormal looping of the aortic arch associated with hemangioma of the head and neck who underwent complex surgical repair without cardiopulmonary bypass.
|
Introduction
|
|---|
The association of hemangiomas and aortic coarctation has been reported in literature [1–3]. We describe an infant with hemangiomas of the head, anomalous looping of the aortic arch, and coarctation who underwent reconstruction of the entire aortic arch and the thoracic descending aorta without cardiopulmonary bypass.
A 40-day-old male infant was referred to our institution for failure to thrive. Clinical examination showed a large hemangioma of the left side of the head. The femoral pulses were weak with a 50 mm Hg systolic pressure gradient between the right arm and the lower limb arteries. Chest roentgenogram was normal. Magnetic resonance imaging revealed normal brain structures.
The echocardiographic examination showed a complex anomaly of the aortic arch with normal intracardiac anatomy and prompted us to perform diagnostic cardiac catheterization. Angiography showed a looped left aortic arch with significant narrowing of the descending thoracic aorta (Fig 1).
The ascending aorta was normal andthe left carotid artery raised in close proximity to the innominate artery (the so-called "bovine trunk"). The origin of the left subclavian artery was stenotic and displaced distally from its usual location and without any evidence of a patent ductus arteriosus. The patient was referred to surgery.
View larger version (153K):
[in this window]
[in a new window]
|
Fig 1. Angiographic picture of the ascending aorta, transverse arch, and descending aorta. Note the unusual aspect of the transverse arch, the origin of the left subclavian artery, and the obstruction of the distal thoracic aorta (*).
|
|
Through a midline sternotomy the ascending aorta, the aortic arch, and the descending thoracic aorta were isolated without extracorporeal circulation. The remnant of the ductus located between the pulmonary bifurcation and the aortic arch below the left common carotid artery was ligated and divided. The tortuous and narrowed aorta (Fig 2)
was excised from the origin of the left carotid artery to the left subclavian artery. The subclavian artery was then opened longitudinally and the distal end was sutured to the proximal aortotomy (Fig 3).
The entire aorta was then repaired using a patch of autologous pericardium (Fig 4).
Overall aortic cross-clamping time was 26 minutes.
View larger version (21K):
[in this window]
[in a new window]
|
Fig 3. The left subcalvian artery is used to repair the roof of the neoaorta (A). A patch of autologous pericardium completes the plasty of the arch, which is anastomosed to the ascending aorta (B). The position of the clamp allows adequate brain perfusion through the innominate artery.
|
|
The histologic examination of the native aorta and ductus showed a concentric fibrous intimal hyperplasia and thickening. Diffuse fragmentation of the elastic fibers and necrosis with inflammatory involvement of the media were also evident.
|
Comment
|
|---|
This infant presented some of the features formerly recognized as the PHACE syndrome, ie, the association of hemangiomas and arterial abnormalities with coarctation of the aorta [2]. The aortic arch anatomy was very unusual in this patient, however, resembling the so-called "macaroni aorta" [4], which has never been described so far in the setting of the PHACE syndrome. As a complete repair through primary arch advancement was not considered feasible because of the long distance between the arch and the normal descending aorta, a complex surgical approach with a large patch of autologous pericardium was needed, leaving the subclavian artery as the roof of the neoaorta. The cardiopulmonary bypass was not necessary because the clamping time of less than 30 minutes should be considered safe for the spinal cord. On the other side, the position of the aortic clamp allowed the perfusion of the brain and with the aid of proper pharmacologic hemodynamic control avoided cardiac overload. We observed no neurologic complications from taking this approach.
Our experience strengthens the concept that major aortic arch anomalies should be ruled out in all cases presenting with cutaneous hemangiomas [5]. It is our opinion that complete repair of complex anomalies of the aorta may be achieved without the use of cardiopulmonary bypass and that follow-up studies on larger series are needed to validate the use of native tissues instead of prosthetic conduits in infants and small children.
|
References
|
|---|
- Schneeweiss A., Blieden L.C., Shem-Tov A., Motro M., Feigel A., Neufeld H.N. Coarctation of the aorta with congenital hemangioma of the face and neck and aneurysm or dilatation of a subclavian or innominate artery. A new syndrome?. Chest 1982;82:186-187.[Abstract/Free Full Text]
- Frieden U., Reese V., Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996;132:307-311.[Abstract/Free Full Text]
- Pascual-Castroviejo I., Viano J., Moreno J., et al. Hemangiomas of the head, neck, and chest with associated vascular and brain anomalies: a complex neurocutaneous syndrome. Am J Neuroradiol 1996;17:461-471.[Abstract]
- McDonald A.H., Gerlis L.M., Somerville J. Familial arteriopathy with associated pulmonary and systemic arterial stenoses. Br Heart J 1969;31:375-385.[Free Full Text]
- Wong C.H., Wright J.G., Silove E.D., Willetts R., Brawn W.J. A new syndrome of multiple hemangiomas, right dominant double aortic arch, and coarctation. J Thorac Cardiovasc Surg 2000;121:1207-1209.[Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
D. Metry, G. Heyer, C. Hess, M. Garzon, A. Haggstrom, P. Frommelt, D. Adams, D. Siegel, K. Hall, J. Powell, et al.
Consensus Statement on Diagnostic Criteria for PHACE Syndrome
Pediatrics,
November 1, 2009;
124(5):
1447 - 1456.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
R. E. Beygui, F. Esmailian, D. A. Rigberg, and H. Laks
Repair of the symptomatic aberrant aortic arch aneurysm without hypothermic circulatory arrest
Interactive CardioVascular and Thoracic Surgery,
December 1, 2004;
3(4):
569 - 572.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Bronzetti, A. Giardini, A. Patrizi, D. Prandstraller, A. Donti, R. Formigari, M. Bonvicini, and F. M. Picchio
Ipsilateral Hemangioma and Aortic Arch Anomalies in Posterior Fossa Malformations, Hemangiomas, Arterial Anomalies, Coarctation of the Aorta, and Cardiac Defects and Eye Abnormalities (PHACE) Anomaly: Report and Review
Pediatrics,
February 1, 2004;
113(2):
412 - 415.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
