Journal of Surgery Current Trends & Innovations Category: Clinical Type: Editorial
Marfan Syndrome and Dsyphagia Lusoria: The Hustle
- Nicola Marziliano1*, Alessandro Medoro2, Stefano Folzani3, Claudio Reverberi4, Mariano Intrieri2
- 1 Laboratory Of Clinical Pathology Asl Taranto, San Marco Hospital, Grottaglie, Italy
- 2 Department Of Medicine And Health Sciences V Tiberio, University Of Molise, Campobasso, Italy
- 3 Department Of Radiology, Polimabulatorio Città Di Collecchio, Collecchio, Italy
- 4 Department Of Cardiology And Genetics, Poliambulatorio Gemini, Parma, Italy
*Corresponding Author:Nicola Marziliano
Laboratory Of Clinical Pathology Asl Taranto, San Marco Hospital, Grottaglie, Italy
Received Date: Aug 27, 2019 Accepted Date: Aug 27, 2019 Published Date: Sep 03, 2019
Aberrant Right Subclavian Artery (ARSA) or Arteria Lusoria (AL) is the most common congenital aortic arch anomaly occurring in less than 1-2% of the general population. A right ASA typically occurs in a normal (left) aortic arch and arises as the fourth supra-aortic vessel from the distal arch). Due to this left-sided origin, the artery crosses the posterior mediastinum to reach the right arm. Similarly, a left ASA arises as the last branch from a right aortic arch, crossing the mediastinum to reach the left arm. Clinically, the aberrant, usually retro-oesophageal course of the artery, may be associated with dysphagia or respiratory symptoms (dyspnoea), although the majority of the affected adults is asymptomatic . Although pharingeal symptoms are not generally considered a main feature of Marfan syndrome, many patients have a degree of underlying pharyngeal pathologies. Dysphagia Lusoria (DL) is a rare condition that might overlap with MFS due to vascular abnormalities.
Thus the combination of a rare genetic condition such as MFS together with DL represents per se a extremely rare clinical condition. The authors of the article entitled “Hybrid Endovascular Repair for Dysphagia Lusoria in a Patient with Marfan Syndrome” addressed this coincident finding by means of extremely informative imaging approach (contrast CTA scanning) that guided a carotid artery transposition and a ValiantTM (Medtronic) device positioning in order to cover the origin of the aberrant subclavian artery .
This is the strength of the present paper: The association of MFS with DL elegantly treated with the ValiantTM device.
It might seems a that this paper is intended for a niche audience due to the combinations of MFS and DL; however such as papers should serve for future cases presentations and, if still needed, support the importance of a proper imaging pre-study when approaching rare inherited/genetic conditions with surgery.
- Meester JAN, Verstraeten A, Schepers D, Alaerts M, Van Laer L, et al. (2017) Differences in manifestations of Marfan syndrome, Ehlers-Danlos syndrome, and Loeys-Dietz syndrome. Ann Cardiothorac Surg 6: 582-594.
- Mccallen AM, Schaff B (1956) Aneurysm of an anomalous right subclavian artery. Radiology 66: 561-563.
- Stone WM, Ricotta JJ, Fowl RJ, Garg N, Bower TC, et al. (2011) Contemporary management of aberrant right subclavian arteries. Ann Vasc Surg 25: 508-514.
Citation:Marziliano N, Medoro A, Folzani S, Reverberi C, Intrieri M (2019) Marfan Syndrome and Dsyphagia Lusoria: The Hustle. J Surg Curr Trend Innov 3: 018.
Copyright: © 2019 Nicola Marziliano, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.