Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection

Authors

  • R. K. Zhuraev
  • P. V. Kuzyk

DOI:

https://doi.org/10.14739/2310-1237.2012.2.16745

Keywords:

Marfan syndrome, aortic dissection, pathomorphology, prognosis, diagnosis

Abstract

The article presents the pathologic changes in the aortic wall in patients with MFS (three men at the age of 25, 27, 35 years) operated on the aneurysm with dissection of the ascending aorta. Morphological changes in the middle of the shell of the aorta in patients with MFS were characterized by severe restructuring with profound irreversible alterative changes in all of its components. The pathological process captured media of the aorta throughout its entire length, not just in the areas of rupture and separation. The main pathomorphological signs of MFS were the focal accumulation of mucoid substances, ribbon-like nuclear-free zones, degenerative changes in smooth muscle cells, the formation of cystic media degeneration cavities, changes in elastic fibers - fragmentation, hyperelastosis, multiplication, thinning and straightening, areas of significant elastolysis. Multiple CMD was detected in all patients with MFS, the changes revealed the same type, but at different stages of the process.

References

Зербіно Д.Д. Синдром Марфана: історичний ракурс і сучасний погляд на етіологію, патогенез, діагностику, клініку та лікування / Зербіно Д.Д., Ольхова О.В., Жураєв Р.К. // Український медичний часопис. – 2010. – №6. – С. 97–100.

Жураєв Р.К. Розриви і розшарування стінки аорти у хворих на синдром Марфана / Жураєв Р.К. // Мистецтво лікування. – 2011. – №7. – С. 124–128.

Кузик Ю.І. Розшаровуючі аневризми аорти: клініко-морфологічні особливості / Кузик Ю.І. // Буковинський медичний вісник. – 2001. – №4. – С. 73–76.

Ситар Л.Л. Расслаивающая аневризма аорты / Ситар Л.Л., Слета А.А. // Medicus Amicus. – 2003. – №5–6. – С. 1–4.

Albornoz G. Familial thoracic aortic aneurysms and dissections – Incidence, modes of inheritance, and phenotypic patterns/ Albornoz G., Coady M.A., Roberts M., et al. // Ann. Thorac. Surg. – 2006. – №82(4). – Р. 1400–1405.

Carta L. Discrete contributions of elastic fiber components to arterial development and mechanical compliance / Carta L., Wagenseil J.E., Knutsen R.H., et al. // Arterioscler. Thromb. Vasc. Biol. – 2009. – №29 (12). – Р. 2083–2089.

Chung A.W. Loss of elastic fiber integrity and reduction of vascular smooth muscle contraction resulting from the upregulated activities of matrix metalloproteinase-2 and -9 in the thoracic aortic aneurysm in Marfan syndrome / Chung A.W., Au Yeung K., Sandor G.G., et.al. // Circ Res. – 2007. – №101 (5). – Р. 512–522.

Gao Guo Induction of Macrophage Chemotaxis by Aortic Extracts from Patients with Marfan Syndrome Is Related to Elastin Binding Protein / Gao Guo, Petra Gehle, Sandra Doelken, Jose. Luis Martin-Ventura, Yskert von Kodolitsch,Roland Hetzer, Peter N. Robinson // PLoS ONE. – 2011. – №6 (5). – Р. 1–7.

John A. Elefteriades Thoracic Aortic Aneurysm: Reading the Enemy’s Playbook / John A. Elefteriades. (2008) // Yale journal of biology and medicine. – 2008. – №81. – Р. 175–186.

Klintschar M. Marfan syndrome: clinical consequences resulting from a medicolegal autopsy of a case of sudden death due to aortic rupture / Klintschar M., Bilkenroth U., Arslan-Kirchner M. et al. // Int. J. Legal Med. – 2009. – №123. – Р. 55–58.

Lindeman J.H. Distinct defects in collagen microarchitecture underlie vessel-wall failure in advanced abdominal aneurysms and aneurysms in Marfan syndrome / Lindeman J.H., Ashcroft B.A., Beenakker J.W., et.al. // Proc. Natl. Acad. Sci. USA. – 2010. – №107 (2). – Р. 862–865.

Panagiotis Hountis Acute aortic dissection in a young patient without Marfan fibrillinopathy: a case report / Panagiotis Hountis, Panagiotis Dedeilias, Konstadinos Bolos // Cases Journal. – 2002. – №2. – Р. 70–76.

Pratt B. Arterial elastic fiber structure. Function and potential roles in acute aortic dissection / Pratt B, Curci J. // J. Cardiovasc. Surg. – 2010. – №51 (5). – Р. 647–656.

Syyong H.T. Dysfunction of endothelial and smooth muscle cells in small arteries of a mouse model of Marfan syndrome / Syyong H.T., Chung A.W.Y., Yang H.H.C., van Breemen C. // Br. J. Pharmacol. – 2009. – №158(6). – Р. 1597–1608.

Tang P.C. Hyperplastic cellular remodeling of the media in ascending thoracic aortic aneurysms / Tang P.C., Coady M.A., Lovoulos C. et al. // Circulation. – 2005. – №112 (8). – Р. 1082–1084.

Downloads

How to Cite

1.
Zhuraev RK, Kuzyk PV. Marfan syndrome: pathologic features of aneurysm with dissection and aortic dissection. Pathologia [Internet]. 2013Aug.21 [cited 2024Jul.17];(2). Available from: http://pat.zsmu.edu.ua/article/view/16745

Issue

No. 2 (2012)

Section

Original research

License

Authors who publish with this journal agree to the following terms:

    1. Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
Лицензия Creative Commons
    1. Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.

  1. Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (SeeThe Effect of Open Access).