Chir Vol. 31 - n. 10 - pp. 429-432

Ottobre 2010

 

 

Angiomegaly and arterial aneurysms

 

V. D’Andrea, V. Cantisani, A. Catania, A. Todini, F. Stio, F.M. Di Matteo,

C. Di Marco, R. Greco, M. Di Certo, E. Guaitoli, E. De Antoni

 

“Sapienza” University of Rome

Department of Surgical Sciences

(Chief: Prof. A. Redler)

School of Specialization

General Surgery III

(Chief: Prof. E. De Antoni)

 

 

Summary: Angiomegaly and arterial aneurysms.

 

V. D’Andrea, V. Cantisani, A. Catania, A. Todini, F. Stio, F.M. Di Matteo, C. Di Marco, R. Greco, M. Di Certo,

E. Guaitoli, E. De Antoni

 

Angiomegaly is characterized by an alteration in the elastic component of arterial and venous vessels determining their elongation and tortuousness. This involves an increased risk of thromboembolism and aneurysmal degeneration in affected subjects, even if they have been asymptomatic for a long time.

The aim of this study is to demonstrate the correlation between angiomegaly and aneurysmal disease. A total of 163 patients suffering from a peripheral arterial aneurysm were included, 74 of these with an iliac aneurysm, 41 with a femoral aneurysm and 48 suffering from popliteal aneurysm. All patients were examined by color Doppler ultrasonography (CDU) and angio-CT with contrast medium. Eighteen cases of arteriomegaly were diagnosed, and the prevalence in the examined population was 11%.

This study demonstrates the close association existing between aneurysms in peripheral arteries and arteriomegaly. Peripheral arterial aneurysms in association with arteriomegaly involve an increased risk of complications like thrombosis, embolism and rupture. The showed familiarity in the arteriomegaly incidence leads to predisposition of screening programs, using CDU, among relatives of patients affected by arteriomegaly and/or peripheral arterial aneurysms.

Riassunto: Angiomegalia e aneurismi arteriosi.

 

V. D’Andrea, V. Cantisani, A. Catania, A. Todini, F. Stio, F.M. Di Matteo, C. Di Marco, R. Greco, M. Di Certo,

E. Guaitoli, E. De Antoni

 

L’angiomegalia è caratterizzata da un'alterazione della componente elastica dei vasi arteriosi e venosi che determina allungamento e tortuosità di questi. Ciò comporta un aumentato rischio di tromboembolie e di degenerazione aneurismatica nei soggetti affetti, anche se asintomatici per un lungo periodo di tempo.

Il presente studio ha voluto dimostrare la correlazione tra angiomegalia e patologia aneurismatica. Sono stati inclusi 163 pazienti affetti da aneurisma arterioso periferico, di cui 74 da aneurisma iliaco, 41 da aneurisma femorale e 48 da aneurisma popliteo. Tutti i pazienti sono stati studiati con ecocolor-Doppler e con angio-TC con mezzo di contrasto. Sono stati diagnosticati 18 casi di arteriomegalia con una prevalenza nella popolazione in esame dell’11%.

Il presente studio dimostra la stretta associazione tra aneurismi delle arterie periferiche ed arteriomegalia. Gli aneurismi arteriosi periferici in associazione con l’arteriomegalia comportano un rischio più elevato di complicanze quali la trombosi, l’embolia e la rottura. La dimostrazione della familiarità nell’incidenza dell’arteriomegalia conduce alla predisposizione di programmi di screening mediante eco-color-Doppler dei familiari dei pazienti affetti da arteriomegalia e/o aneurismi arteriosi periferici.

 

Key Words: Arterial aneurysm - Angiomegaly - Arteriomegaly - Thrombosis - Embolism - Rupture.

            Aneurismi arteriosi - Angiomegalia - Arteriomegalia - Trombosi - Embolia - Rottura.

 

 

Introduction

 

Many authors of scientific publications treating about arterial aneurysms believe that the causes of degenerating aneurysms in peripheral arteries are the same ones of aortic aneurysms (1). On the contrary both clinical features and epidemiological studies about peripheral arterial aneurysms show that they are different pathologies.

Peripheral arterial aneurysms affects men almost exclusively (2, 3), they are often bilateral (4-7) and they involve thrombosis (8-11) or embolism (12) more than rupture (13-23). The peripheral arterial aneurysms in association with arteriomegaly involve an increased risk of complications like thrombosis, embolism and rupture (24). Patients affected by a peripheral arterial aneurysm have a synchronous peripheral aneurysm in 20-50% of cases (25, 26).

Arteriomegaly is a term used to describe the increase in dimension of arterial vessels, both in length and in diameter (27). This condition was initially described by Lèriche (1942) (28) as extended and ectasic arteries, and later (1971) the word arteriomegaly was coined by Lea Thomas (29). In 1983 Callum et al. (27) carried out a study about the diameter in the arteries of the lower extremities measuring their circumference in fixed points on dead bodies, their diameter and their length using arteriography. The authors studied a group of patients with dilated and extended arteries and they gave a definition for the arteriographic condition known as arteriomegaly. The arterial length was expressed as a percentage of fixed bone points to eliminate the effect due to the different patients’ statures. The fixed limit for the normality was, for the length and the diameter, the mean + 2 times the standard deviation (SD); as regards the length, the upper limit for common iliac arteries is 110% of the corresponded bone length, and it is 105% for aortic, femoral and popliteal regions. The authors found an incidence of about 6% for generalized or localized arteriomegaly (27).

To define arterial aneurysmal pathologies we give the criteria suggested by the Society for Vascular Surgery and the International Society for Cardiovascular Surgery: an aneurysm is classified as a permanent and localized dilatation of an artery, with an increase more than 50% of his diameter (30, 31). The arteriomegaly is defined as a diffused increase in the dimensions of the arterial vessels, involving many vascular segments (not focal) with an increase in diameter more than 50% compared to the respective healthy vascular segments (30).

About 36% of patients affected by arteriomegaly has first-degree relatives affected by the same pathology and arteriomegaly therefore is an important predictive factor for familiar aneurysms. A male relative of an affected patient has a 6,7% risk to develop a clinically meaningful aneurysm (30). In the study of Lawrence et al. (27) there were peripheral arterial aneurysms in 5% of male first-degree relatives, never in female relatives.

In one of our previous studies (32) we have demonstrated that arteriomegaly is the arterial form of a more complex pathology that we described as angiomegaly. In this research we have studied a population of patients affected by aneurysm in iliac, femoral and popliteal arteries and we have examined the prevalence of arteriomegaly in the same population, in order to demonstrate that angiomegaly, that includes arteriomegaly, is strictly related to the genesis of the peripheral aneurysm.

 

 

Patients and methods

 

In order to classify aneurysms we have used the criteria suggested by the Society for Vascular Surgery and by International Society for Cardiovascular Surgery: a permanent and localized dilatation of an artery, with an increase > than 50% in his diameter was defined as aneurysm (30, 31). The diagnosis of aneurysm and arteriomegaly in every patient is based on the clinical features, the radiographic, ultrasonographic and arteriographic examinations.

A total of 163 consecutive patients were involved in the present study, 74 of them were affected by an iliac aneurysm, 41 by a femoral aneurysm and 48 by a popliteal aneurysm. All the patients were studied with color-Doppler ultrasonography (CDU) (Fig. 1) and angio-CT with contrast medium, with multislide technique and three-dimensional image reconstruction.

 

 

Results

 

Eighteen cases of arteriomegaly were diagnosed with a 11% frequency in the examined population.

The arteriomegaly was associated in 2 patients with iliac artery aneurysm, in 6 patients with femoral artery aneurysm and in 10 patients with popliteal artery aneurysm.

 

 

Discussion

 

The present study shows the close association between peripheral arterial aneurysms and arteriomegaly. The prevalence of arteriomegaly in the etiopathogenesis of peripheral arterial aneurysm was 11% in our study, an unexpected percentage.

The demonstration of familiarity for the incidence of arteriomegaly leads to predisposition of screening programs, using CDU, among relatives of patients affected by arteriomegaly and/or peripheral arterial aneurysms.

For histopathological features, angiomegaly can be considered as a pathology connected mainly to an alteration of the elastic matrix, which can involve arteries (megadolichoarteries) and veins (megaveins). The differences existing in arterial walls of patients with arteriosclerosis and arteriomegaly, leads us to understand that the wearing out in the wall where is an angiomegaly must be compared to dystrophic alterations in elastic tissue, probably of congenital nature. The different etiopathogenesis of the two pathologies can be seen also in their different clinical features.

From the point of view of the diagnosis, the demonstration of vessel dilatation, extension and tortuousness is fundamental and, in addition to other traditional analysis (angiography, Doppler, angio-CT), the duplex scanner and the color-Doppler are the most appropriated means and the most useful to underline eventual vascular pathologies in asymptomatic patients.

Surgical treatment must be considered when there is an acute symptomatology due to thromboembolism and aneurysms with a risk of rupture. Surgical treatments aims to rebuild extended segments hit from the pathology.  Multiple surgical treatments are often requested, depending on the aneurysms localization and general conditions of the patient, in order to reduce surgical trauma because these patients often have an elevated cardiological risk. For this reason both the kind of surgical treatment and the indication to it can be different for different patients.

In our study we have demonstrated that there are deeply differences between arteriomegaly and arteriosclerosis. In arteriosclerosis myocytes penetrate into the internal elastic membrane and the cytoplasm of myocytes in the tunica media shows clear signs of pathological process (lipid inclusion, vacuoles, elevated numbers of mitochondria), tunica media contains many inclusions of lipids and cholesterol with “fats cells”. In endothelial cells a high grade of pinocytic activities was seen. Among myocytes of the tunica media it’s possible to see an important accumulation of collagen microfibrils that replace the degenerated myocytes. In arteriomegaly, instead, the increase in the number of collagen microfibrils near the myocytes is less frequent (Figs. 2 and 3). Arteriomegaly therefore must not be confused with arteriosclerosis, as it is often happened in the past.

In a previous study we have demonstrated that angiomegaly is caused, pathogenetically, by a specific alteration in elastic fibre documented by the electronic microscope (angiomegaly) and that, contrary to what people believed in the past, it’s not an anatomic variant but a proper pathology, that can evolve in complications (rupture of associated aneurysms, thrombosis and embolism) within the competence of emergency surgery. Moreover we have demonstrated that the incidence of this pathology is much greater than in the past and the reason why it’s considered rare is that it’s not often recognised and it is mistaken for an arteriosclerosis with ectasia.

 

 

Conclusion

 

The association between arteriomegaly and peripheral arterial aneurysms is frequent. On 163 patients affected by a peripheral arterial aneurysm (iliac, femoral and popliteal), 18 had an associated arteriomegaly (11%). So a close relation between the arteriomegaly and the onset of peripheral arterial aneurysms was demonstrated.

 

 

References

 

1.         Flanigan DP. Aneurysm of the peripheral arteries incidence. In: Moore W, editor. Vascular surgery: a comprehensive review. Phildelphia: WB Saunders 1993; 424-434.

2.         Harbuzariu C, Duncan AA, Bower TC, et al. Profunda femoris artery aneurysms: association with aneurysmal disease and limb ischemia. J Vasc Surg 2008; 47:31-35.

3.         Posner SR, Wilensky J, Dimick J, Henke PK. A true aneurysm of the profunda femoris artery: a case report and review of the English language literature. Ann Vasc Surg 2004; 18:740-746.

4.         Thompson MM, Bell PR. ABC of arterial and venous disease.Arterial aneurysms. BMJ 2000; 320:1193-1196.

5.         Galland RB. Popliteal aneurysms: from John Hunter to the 21st century. Ann R Coll Surg Engl 2007; 89:466-471.

6.         Pappas G, Janes JM, Bernatz PE, Schirger A. Femoral aneurysms. Review of surgical management. JAMA 1964; 190:489-493.

7.         Dent TL, Lindenauer SM, Ernst CB, Fry WJ. Multiple arteriosclerotic arterial aneurysms. Arch Surg 1972; 105:338-344.

8.         Pulli R, Dorigo W, Troisi N, et al. Surgical management of popliteal artery aneurysms: which factors affect outcomes? J Vasc Surg 2006; 43:481-487.

9.         Harder Y, Notter H, Nussbaumer P, et al. Popliteal aneurysm: diagnostic workup and results of surgical treatment. World J Surg 2003; 27:788-792.

10.       Raut CP, Cambria RP, LaMuraglia GM, et al. Surgical management of popliteal artery embolism at the turn of the millennium. Ann Vasc Surg 2004; 18:79-85.

11.       Mahmood A, Salaman R, Sintler M, et al. Surgery of popliteal artery aneurysms: a 12-year experience. J Vasc Surg 2003; 37:586-593.

12.       Bonamigo TP, Frankini AD. Aneurisma da artéria poplítea. Circ Vasc Ang 1987; 3:22-25.

13.       Holden A, Merrilees S, Mitchell N, Hill A. Magnetic resonance imaging of popliteal artery pathologies. Eur J Radiol 2008; 67:159-168.

14.       Kudo FA, Nishibe T, Miyazaki K, et al. A rare case of atherosclerotic popliteal artery aneurysm in a young adult. J Cardiovascular Surg (Torino) 2002; 43:515–517.

15.       Claridge M, Hobbs S, Quick C, et al. Screening for popliteal aneurysms should not be a routine part of a community-based aneurysm screening program. Vasc Health Risk Manag 2006; 2:189-191.

16.       Graham AR, Lord SRA, Bellemore M,Tracy GD. Popliteal aneurysm. Aust N Z J Surg 1983; 53:99-103.

17.       Barroy JP, Barthel J, Locufier JL, et al. Atherosclerotic popliteal aneurysm: report of one ruptured popliteal aneurysm-survey and analysis of the literature. J Cardiovasc Surg (Torino) 1986; 27:42-45.

18.       Wolf YG, Kobzantsev Z, Zelmanovich L. Size of normal and aneurysmal popliteal arteries: duplex ultrasound study. J Vasc Surg 2006; 43:488-492.

19.       Kallakuri S, Ascher E, Hingorani A, et al. Effect of duplex arteriography in the management of acute limb-threatening ischemia from thrombosed popliteal aneurysms. Angiology 2008 (Epub ahead of print).

20.       Vermillion BD, Kimmins SA, Pace WG, Evans WE. A review of one hundred forty-seven popliteal aneurysms with long term follow-up. Surgery 1981; 90:1009-1014.

21.       Lichtenfels E, Frankini AD, Bonamigo TP, et al. Popliteal artery aneurysm surgery: the role of emergency setting. Vasc Endovasc Surg 2008; 42:159-164.

22.       Oliveira RSM, Ferreira DA, Terra JA Jr, et al. Ruptura de aneurisma da artéria poplitea: relato de caso e revisão da literatura dos últimos 50 anos. J Vasc Bras 2005; 4:105-110.

23.       Hamish M, Lockwood A, Cosgrove C, et al. Management of popliteal artery aneurysms. ANZ J Surg 2006; 76:912-915.

24.       Hands LJ, Collin J. Infra-inguinal aneurysms: outcome for patient and limb. Br J Surg 1991; 78:996-998.

25.       Cole CW, Thijssen AM, Barber GG, et al. Popliteal aneurysms: an index of generalized vascular disease. Can J Surg 1989; 32:65-68.

26.       Takolander RJ, Bergqvist D, Bergentz SE, et al. Aneurysms of the popliteal artery. Acta Chir Scand 1984; 150:135-140.

27.       Callum KG, Lea Thomas M, Browse NL. A definition of arteriomegaly and the size of arteries supplying the lower limbs. Br J Surg 1983; 70:524-529.

28.       Leriche R. Dilatation pathologique des alteres en dehors des arteres aneurysmes vie tissulaire des arteres. Presse Med 1942; 50:641-642.

29.       Thomas ML. Arteriomegaly. Br J Surg 1971; 58: 690-694.

30.       Lawrence PE, Wallis C, Dobrin PB, et al. Peripheral aneurysms and arteriomegaly: is there a familial pattern? J Vasc Surg 1998; 28:599-605.

31.       Johnston KW, Rutherford RB, Tilson MD, et al. Suggested standards for reporting on arterial aneurysms. Subcommittee on Reporting Standards for Arterial Aneurysms, Ad Hoc Committee on Reporting Standards, Society for Vascular Surgery and North American Chapter, International Society for Cardiovascular Surgery. J Vasc Surg 1991; 13:452-458.

32.       D’Andrea V, Malinovsky L, Cavallotti C, et al. Angiomegaly. J Cardiovasc Surg (Torino) 1997; 38:447-455.