Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Chapter 2 The clinical problem. Morfology, ethiology, imaging 2.1 Morfology of abdominal aortic aneurysms An aneurysm is an abnormal localized sac or an irreversible dilation caused by weakness (decreased elastin) of the arterial wall. Aneurysms are classified as either fusiform, round shape with a diameter that can reach even 20 cm (fig. 2.1), or saccular, tube-like shape (fig. 2.2); the former is a much more common type than the latter. In saccular aneurysms, one side of the artery wall has a large strain they vary between 5 to 20 cm in diameter and are the result of processes which affect only part of the circumference of the vessel wall. Their lumen is connected with the lumen of the affected vessel through an opening of varying size. As a result, flow through the aneurysm is turbulent and thrombosis is common. A subtype of saccular aneurysm is the Berry aneurysm, these are localized dilatation of an intracranial artery. In fusiform aneurysms, the entire circumference of the artery is strained; they are shaped like a spindle ("fusus" means spindle in Latin) with widening all around the circumference of the aorta. 8 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Fig 2.1 Fusiform aneurysm. The drawing shows the typical spindle-like shape, naturally every different fusiform aneurysm is characterized by having his own size and shape, however the blood flow is always coaxial with the main part of the aneurysm. Fig. 2.2 Saccular aneurysm. It is possible to note that saccular aneurysm is a spherical dilatation which project from one aspect of the affected vessel. This work has been focused only on fusiform aneurysms and from now on I will refer to fusiform abdominal aneurysms simply as abdominal aneurysms. It is important to realize that an aneurysm is not just an extremely wide aortic diameter but also reflects changes in the aorta wall, not present in normal aortas, that make it susceptible to rupture. The abdominal aorta is the most common site for an aneurysm to develop, therefore it is important to localize exactly the piece at issue. The abdominal aorta begins at the aortic hiatus of the diaphragm, in front of the lower border of the body of the last thoracic vertebra, and, descending in front of the vertebral column, ends on the body of the fourth lumbar vertebra, commonly a little to the left of the middle line, by dividing into the two common iliac arteries. It diminishes rapidly in size, in consequence of the many large branches which it gives off. As it lies upon the bodies of the vertebræ, the curve which it describes, is convex forward, the summit of the convexity corresponding to the third lumbar vertebra (fig. 2.3). 9 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Fig. 2.3 The diameter of the human abdominal aorta in non-aneurysmatic subjects varies according to gender. In studies of subjects older than 50 years the mean ulrasound diameter of the infrarenal abdominal aorta ranges from 12 to 19 mm in women and from 14 to 21 mm in men [J.L. Cronenwett et al., 1985]. AAA is most common in Caucasians, with men affected four times more than women, they are most prevalent between the ages of sixty and seventy and first-degree relatives of persons with AAA develop dilation at earlier ages (fig. 2.4). 10 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Fig. 2.4 Age and sex distribution for open reconstructed aneurysms (n = 462) [H. J. C. M. Pleumeekers, 1995] Some studies take this variance of the normal aorta diameter into account and several cut-off points are used to evaluate the presence of an aneurysm. An aneurysm is considered to be present for instance if: the diameter of the abdominal aorta is larger than the diameter of the aorta at the renal bifurcation or there is an increase of 5 mm of the abdominal aorta compared with the suprarenal segment of the aorta or there is a localized dilatation with at least a 50% increase in diameter compared to the aorta diameter at the bifurcation of the renal artery etc. If an AAA is untreated its natural course is to expand and rupture and this leads to hemorrhage in the space surrounding the aneurysm, considering that the vessel at issue is the aorta (the main artery of the body), it is easy to understand the importance of this disease. 11 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ 2.2 Ethiology The exact cause of abdominal aortic aneurysms is unknown. AAAs were first thought to be of arteriosclerotic origin. Martin [P. Martin, 1978] was the first to study this question, suggesting that arteriosclerosis may not be the cause rather the consequence of aortic degeneration. Arteriosclerotic changes in the intimal layers of the abdominal aorta are thought to affect the diffusion of oxygen and nutrients to the outer layers of the abdominal aorta. This may lead to changes in the aortic wall structure, making the aorta susceptible to dilatation [D. Reed et al., 1992]. Sterpetti [A. V. Sterpetti et al., 1988] proposed the existence of two types of abdominal aneurysms. The first type is associated with arteriosclerotic occlusive disease and the other is not. In their study of 526 patients undergoing aneurysmal resection, 25% of aneurysms were believed to be non-arteriosclerotic. There were significantly more ruptures in this group, compared to the arteriosclerotic group, moreover there were a generalized weakness of the aorta wall in non-arteriosclerotic group. In the Rotterdam study they founded that a history of inguinal hernia surgery at a relative young age was related to the presence of AAAs indicates that congenital weakening of the collagen structure may predispose to aneurysm formation. In this study the majority (approximately 90%) of abdominal aortic aneurysms, however, can be attributed to risk factors associated to arteriosclerosis [H.J.C.M. Pleumeekers, 1995]. The finding that men with a first degree relative with an AAA experience a 10 fold increased risk of developing an abdominal aneurysm, provides a strong argument for a genetic component [H.J.C.M. Pleumeekers, 1995]. Another interesting theory is the trace metal theory. It is based on the observation that in blotchy mouse aneurysm formation is related to an X-linked chromosome defect leading to an abnormality of the cupper metabolism [D.H. Rowe et al., 1974]. Copper is thought to play a role in the cross linkages of collagen and elastin, which forms the extracellular matrix of the aortic wall. A deficiency of the copper metalloenzyme lysyl oxidase could result in a deficiency of 12 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ collagen and elastin and weaken the aortic wall, thus making it prone to aneurysm formation. However, risks associated with AAA include atherosclerosis, hypertension and smoking. Other risks include trauma to the arterial wall and infection of the artery wall. Alterations in the delivery of oxygen and nutrients to the artery wall may also play a role in the pathophysiology, because the infrarenal aorta lacks medial vasa vasorum (literally vessels of the vessels, they carry oxygen and nutrients to the cells of the blood vessel wall, fig.2.5) even though the aortic wall is nearly as thick as the proximal segments that have medial vasa vasorum. Fig. 2.5a High power view (300x) shows the vasa vasorum, which supply blood for theFig. wall4c of this large vein. Fig. 2.5b Vasa vasorum openings show continuity of endothelial lining (SEM 2000x). Most AAAs occur below the level of the renal artery and involve the bifurcation of the aorta as well as the proximal ends of the iliac arteries. Stasis of blood can lead to thrombus formation along arterial wall. Peripheral emboli can develop causing arterial insufficiency. A person with an abdominal aortic aneurysm often becomes aware of a pulsing sensation in the abdomen. The aneurysm may cause pain, typically a deep, penetrating pain mainly in the back. The pain can be severe and is usually steady, although changing position may relieve it. The anatomic structure of abdominal aneurysms seems to be of importance for the risk of rupture. Longer, fusiform aneurysms have a poorer prognosis than saccular ones [K. Ouriel et al., 1989]. Aortic 13 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ blebs, consisting of protrusions in the aortic wall and filled with thrombus en debris, are an indication of impending rupture [G.C. Hunter et al., 1989]. Also the risk of rupture seems to be higher when there is no evidence of peripheral vascular disease. The first sign of a rupture is usually excruciating pain in the lower abdomen and back and tenderness over the aneurysm. With severe internal bleeding, a person may rapidly go into shock. A ruptured abdominal aneurysm is often fatal. Once an aneurysm forms it often increase in size and consequently the chance of rupture also increase. Aneurysm rupture can lead to haemorrhage and death. Aneurysms in the segment of the aorta that passes through the abdomen (AAA) tend to run in families. Many times, these aneurysms occur in people with high blood pressure. The mechanic role of high blood pressure in the formation of aneurysms may seem obvious. Studies in this issue, however, have produced conflicting results. Allen [P. I. M. Allen et al., 1987] found 5.3% prevalence of abdominal aortic aneurysms among 168 hypertensive men and women, however this report did not include a control group and were of limited size. In two other studies comprising a control group, the prevalence of AAA in non-hypertensive was similar to that in hypertensive patients [J. Collin et al., 1988; R.A.P. Scott, 1991]. O’ Kelly [J. O’Kelly et al., 1989] demonstrated a significantly higher risk of abdominal aneurysms in patients with a systolic blood pressure of 180 mmHg or over compared with subjects with a lower systolic blood pressure (a 4.1% prevalence versus 1.2%). No increased risk in patients with diastolic hypertension was found. Such aneurysms often become larger than 76mm and may rupture. There is a 20% chance of rupture within a year, for an aneurysm that is larger than 6 cm. Ruptured aneurysms occur in approximately 5 out of 10,000 people that reveals an aneurysm. The study of Pleumeekers [1995] shows a pronounced increase from and hospital discharge rates for aneurysms of the abdominal aorta in The Netherlands during the last two decades (’72 and ’92). This increase remained after adjustment for age and was more prominent in men than in women. 14 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Several factors have to be considered when interpreting an upward trend in data obtained from routine statistics. 1. A first explanation could be an increase in detection rate. The widespread use of ultrasound in hospitals since the mid seventies will have lead to the detection of many aneurysms previously unknown (In 1989 the board of radiologist in The Netherlands recommended that during all sonographic examinations of the abdomen an attempt should be made to visualize the abdominal aorta). 2. Changes in coding practice could be a second explanation for the observed increase. The introduction of a new, promising diagnostic method and the improvement in surgical techniques will have increased the medical awareness for AAAs. This might result in improved case finding and more deaths attributed to aneurysms and can explain part of the observed increase in mortality from AAAs. However, results from autopsy studies still suggest that many aneurysms remain undetected during life [M.J. Mc Farlane, 1991]. Apart from the, undisputed, improvement in diagnostic capabilities and perhaps a change in coding practice, the rise in mortality and morbidity from AAAs could reflect a true increase in incidence. First of all, the increase was not the same in men and women. Both for mortality and for the number of hospital admissions men showed a stronger increase than women. If the increase was solely caused by an improved detection rate one would expect similar effects in both sexes. Secondly, there is a marked increase in the number of ruptured aneurysms where ultrasonographic detection does not play a major role. 2.3 Diagnostic and imaging techniques Given the high rate of morbidity and mortality associated with abdominal aortic aneurysms, accurate diagnosis and preoperative evaluation are essential for improved patient outcomes. In general physical examination is considered to be inadequate to detect asymptomatic aneurysm because only very large aneurysm can be palpated, it 15 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ is estimated that only 30% to 50% of all abdominal aneurysms can be detected through abdominal palpation [F.A. Lederle et al., 1988], few is know about the value of auscultation. A plain X-ray of the abdomen may give an adequate estimate of the diameter of the aorta but calcification of the aorta wall is needed to make measurement of the aortic diameter possible and these calcification are present in about 75% of the patients with an AAA. The aim of imaging is to accurately delineate the proximal and the distal landing sites in the infarenal aorta and common iliac arteries. Visualization of the attachment sites is vitally important to guarantee the success of the exclusion procedure and to prevent endoleaks. Negative or adverse anatomical or morphological features must be detected in order to size or select the stent correctly. Imaging obtained before, during and after stent placement by pressure injector is of cardinal importance to ensure procedural success of endovascular repair of AAA, especially to detect endoleak formation. It is an integral part of the technique of endoluminal repair of AAA and high-quality imaging is essential. Ultrasonography is the standard method of screening and monitoring AAAs that have not ruptured. In the past, aortography was commonly used for preoperative planning in the repair of AAAs. More recently, computer tomography (CT) has largely replaced older, more invasive methods. Recent advances in CT imaging technology, such as helical CT and CT angiography, offer significant advantages over traditional CT. These methods allow for more rapid scans and can produce three-dimensional images of the AAA and important adjacent vascular structures. Use of endovascular stent grafts has increased recently and is less invasive for the repair of AAAs in selected cases. Aortography and CT angiography can precisely determine the size and surrounding anatomy of the AAA to identify appropriate candidates for the use of endovascular stent grafts. Helical CT and CT angiography represent an exciting future in the preoperative evaluation of AAAs. However, this technology is not the standard of care because of the lack of widespread availability, the cost associated with obtaining new equipment, and the lack of universal protocols necessary for acquisition and reconstruction of these images [Am. Fam. Physician, 2002]. 16 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ The choice of imaging modality depends on the clinical presentation of the patient (Table 1). Screening and serial monitoring are performed most efficiently with ultrasonography. Aortography is invasive and it underestimates the aneurysm diameter when a thrombus is present. Ultrasonography and computer tomography (CT) of the abdomen have an accuracy varying from 97% to 100%. Of these two, ultrasonography is relatively less expensive and easier to perform, which makes it the method of choice for screening symptomatic patients with stable vital signs are usually evaluated with CT in an emergency situation, whereas those who are hemodynamically unstable typically go directly to surgery. Two drawbacks are the problem of contrast-induced: renal insufficiency in patients with renal impairment, and parallax errors which may contribute to sizing and interpolation errors. In table 1 a general overview of the imaging techniques is shown. 17 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Imaging modality Ultrasonography Aortography Advantages Disadvantages Lower cost Sub optimal in obese patients Widely available Sub optimal in patients with increased bowel gas Non-invasive Increased interobserver variation Visualize reno-vascular disease Invasive Identifies anomalous vessels Higher cost Aids placement of endovascular stent grafts Increased patient morbidity Underestimates aneurysm size Exposure to iodinated contrast MRI Non-invasive Higher cost Lack of ionizing radiation Motion artefact Contraindications with metal clips and pacemakers Patient claustrophobia Availability of scanner and software CT Non-invasive Use of ionizing radiation Highly predictive of aneurysm size Higher cost compared with ultrasonography Localize proximal extent of aneurysm Limited information regarding arterial anatomy Identify other abdominal pathology Procedure of choice for suspected rupture Helical CT and CTA Non-invasive Higher cost Faster scanning time Lack of availability of scanner and software Use in conjunction with endovascular stent grafts Use of ionizing radiation Table 1. Comparison of diagnostics methods. MRI = magnetic resonance imaging; CT = computer tomography; CTA = computer tomographic angiography. 18 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Ultrasonography Ultrasonography is the examination of choice for screening and monitoring the rate of AAA enlargement. Figures 2.6 and 2.7 are longitudinal and transverse sonograms, respectively, of an AAA. Fig. 2.6 Longitudinal view of the abdominal aorta demonstrating a focal area of enlargement (arrows) consistent with an abdominal aortic Fig 2.7 Transverse sonogram of the abdominal aorta demonstrating an AAA (arrows) with a small amount of mural thrombus (arrowhead). aneurysm. Ultrasonography has a sensitivity of nearly 100% in the diagnosis of AAAs [P. Vowden et al., 1989]. This procedure is largely preferred for screening and monitoring because of its relatively low cost, availability, and noninvasive nature. Ultrasonography is accurate to within 0.3 cm in estimating aneurysm diameter on serial scans and has little interobserver variation [K. Myers and T. Devine, 1997]. However, the procedure does have limitations. Images tend to be suboptimal in patients who are obese or who have excessive bowel gas. Ultrasonography cannot reliably identify the presence of periaortic disease, or the proximal and distal extent of the aneurysm. It also cannot determine the patency of visceral vessels, the relationship of the aneurysm with respect to renal vessels, or the presence of iliac aneurysms. As a result, ultrasonography alone cannot fully provide the necessary information required in the preoperative evaluation of a patient undergoing elective aneurysm repair. 19 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Aortography Conventional aortography now has a limited role in the preoperative evaluation of AAAs. Aortography is often reserved for special situations, such as when patients have suspected renovascular stenosis, chronic mesenteric ischemia, occlusive iliac disease, juxtarenal or suprarenal AAAs, horseshoe kidneys (fig. 2.8), previous colectomy, and thoracoabdominal extension, and is also used in endovascular graft placement. Advantages of aortography include consistent visualization of the renal artery origins, renal and visceral artery stenoses, accessory renal arteries, and extension of an aneurysm into the iliac arteries [M. L. Errington et al., 1997]. It takes ca. 5 minutes to obtain images like the ones shown in fig. 2.9 and 2.10, but they are bi-dimensional. Fig. 2.8 Horseshoe kidneys have an abnormal axis. Draw a line between the uppermost calyx and the lowermost calyx. In normal kidneys this line will be closer to the vertebral column at the upper pole. In horseshoe kidneys, the line will be closer to the vertebral column at the lower pole. Horseshoe kidneys usually have multiple renal arteries. Fig 2.9 Early arterial phase of a posteroanterior abdominal aortogram showing a bilobed aneurysm (arrows) originating just below the renal arteries. Fig 2.10 Late arterial phase of the same posteroanterior abdominal aortogram, further defining the bilobed abdominal aortic aneurysm. 20 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ The use of aortography in the imaging of AAAs also has several drawbacks that prevent its more routine use. For instance, aortography tends to underestimate the size of the aneurysm because it only demonstrates the patent lumen, and typically there is a circumferential organized thrombus within most aneurysms. In some cases, an aneurysm is missed altogether. Other disadvantages of aortography include its invasive nature, cost, and risk of exposure to large amounts of iodinated contrast. MRI MRI is minimally invasive and, when combined with magnetic resonance angiography (MRA), can provide excellent details for the preoperative evaluation of AAAs. MRI with MRA has 100% sensitivity in detecting aneurysms, and successfully identifies the proximal and distal extent of the aneurysms, the number and origins of renal arteries, and the presence of inflammation [M. L. Errington et al., 1997] (fig.2.11). Renal artery stenoses greater than 50% can be detected with a sensitivity of 84 to 100% [J. R. Durham et al., 1993]. The advantages of MRI with MRA include lack of ionizing radiation and iodinated contrast medium exposure. Disadvantages include increased cost, patient claustrophobia during longer scanning times, and difficulties with patient compliance in lying still during the procedure to avoid motion artefact. The entire procedure time (since the patient is ready on the table) to obtain MRI images is ca. 20 minutes long, the longest part of the analysis is the data elaboration with the calculator. A limitation of this techniques is that contrast injection is needed, and so the trombotic tissue is not visible. During magnetic resonance (MRI) scan, a narrow table moves the patient through a tunnel-like structure which creates a magnetic field through which radio waves are sent, creating a 3-D image of the internal structure (fig. 2.12). 21 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Fig. 2.11 An MRI image of an AAA, with this kind of image it is possible to accomplish some geometrical considerations. Fig. 2.12 MRI set-up. 22 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ CT CT has a broad array of uses in the imaging of AAAs. It is used as a screening test when ultrasound images are sub optimal; as a diagnostic test when a haemodynamically stable, ruptured AAA is suspected; and in the preoperative workup for the repair of AAAs. CT is a superior diagnostic modality compared with ultrasonography because it offers the clinician valuable information about not only the aneurysm but also the surrounding anatomy (fig. 2.13). Unlike ultrasonography, there is no concern regarding reproducibility of results, interobserver technical error, or limitations caused by body size or the presence of bowel gas. CT technology is widely available, non-invasive, and can be performed quickly when needed to rule out a suspected AAA rupture (it takes no more than 20 minutes to obtain 2-D images, a bit more for 3-D images). While CT has numerous advantages, there are limitations to traditional CT; CT images contain limited information on arterial anatomy. Complex cases involving AAAs may require additional imaging modalities to obtain the necessary road map of nearby vessels for planning surgical repair. Fig 2.13 Computed tomographic scan of the abdominal aorta at the level of the kidneys (outline arrows) showing an aneurysm (arrows) with a small amount of mural thrombus along the posterior and lateral walls. The entire abdomen is also clearly visualized, providing useful information about the surrounding anatomy. 23 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Helical CT The development of helical CT and CT angiography will likely lead to wide use of these modalities in the preoperative evaluation of AAAs. Also, the use of endovascular stent grafts in the repair of AAAs requires a technologically advanced modality, such as helical CT and CT angiography, to provide the necessary detailed preoperative anatomic information. Helical CT is accomplished by rotation of the scanning device coupled with continuous table feed of the patient [G. D. Rubin et al., 1993]. When compared with conventional CT, helical CT has faster scanning time (30 to 60 seconds) and the ability to obtain all images in one breath hold, thereby eliminating the risk of respiratory artifacts. Dual-slice helical CT is a newer form of helical CT technology that allows the volume of data scanned within a given time to double, thus achieving even faster scanning times [S. D. Quanadli et al., 2000]. CT angiography is accomplished by combining all of the axial slices to produce a three-dimensional reconstructed image of the AAA. This image can be rotated into any plane that best demonstrates the relevant anatomy (Fig. 2.14). Dual-slice helical CT correlates well with surgical findings in measuring the proximal and distal extent of the aneurysm. CT and CT angiography are not only less invasive than conventional aortography but also allow for more rapid scanning times and evaluation of the rest of the abdomen. A complete evaluation of the abdomen is important in identifying relevant associated abnormalities, such as a horseshoe kidney, venous or arterial anomalies that would alter the surgical approach, or inflammatory/fibrotic changes within the aneurysm. Other details, including assessment for patency of the inferior mesenteric artery and possible involvement of the iliac arteries, are also reliably achieved with CT and CT angiography. Notably, even patients being considered for endovascular repair with a stent graft sometimes have preoperative evaluation with CT and CT angiography instead of aortography. 24 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ Fig. 2.14 Reconstructed computed tomographic angiogram in the frontal projection demonstrating the bilobed infrarenal aortic aneurysm (arrow heads). The renal arteries are well visualized (small arrows), revealing a severe stenosis of the proximal right renal artery (outline arrow) and the proximity of the aneurysm neck to the renal arteries. The relationship of the aneurysm to the iliac arteries and the aneurysmal dilatation of the right common iliac artery (outline arrow head) are also well displayed. 2.4 Pulsatile Wall Motion (PWM): a way to identify aneurysms Although rupture of the aneurysm can be accelerated by the metabolic disorder in the aneurysmal wall, aneurysm enlargement and rupture are not to be expected without pressurization of the aneurysm sac. Reduction of the aneurysmal diameter is probably the best evidence of successful exclusion. Unfortunately, this diameter decrease takes 6 to 12 months to become manifest. Therefore it is important to develop a monitoring technique that ca demonstrate as simply as possible both successful and unsuccessful exclusion of the aneurysm from the circulation as soon as possible after the operation. Imaging techniques are no sensitive enough to detect endoleaks. 25 Chapter 2 The clinical problem. Morfology, etiology, imaging ___________________________________________________________________________ The PWM (pulsatile wall motion) is the movement of the aneurysmal wall during the heart cycle; the reduction of PWM is smaller in presence of an endoleak or with continued expansion of the aneurysm. This means that the PWM may be predictive of the fate of the endovasculary treated aneurysm. However, no relation appears to exist between the level of PWM and the level of the mean pressure within the sac. A systemic level of pressure without pulsatility in the aneurysmal sac will not generate PWM. The presence of PWM is correlated with aneurysmal pulse pressure but not with the mean level of pressure inside the aneurysm. Therefore, PWM is related to a patent endoleak, but combination of a patent endoleak with the absence of PWM and thrombosed endoleak (as visualized by means of ultrasound scanning and Doppler scanning) with the presence of PWM also exist [G. W. H. Schurink et al., 2000]. 26