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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_3711_Библиотеки_им_академика_М_И_Перельмана

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27 Aortoenteric Fistula (Gastric, Small Intestine, Colonic, Biliary)
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the more chronic stula patient, oral nutrition may be resumed in these patients as they have been eating and toler­ating the passage of food prior to their admission. However, we do not feed the patient with the rst acute episode until they are stable and treatment initiated. Since using the above approach, the treatment of aortoenteric stula patients has shown a remarkable improvement in patient recovery and long-term survival results. Using this approach, each of our patients has survived the procedure to lead a quality life unless they had other simultaneous non-stulous health pro­cesses. These patients demonstrate the challenge of polymi­crobial infections in vascular surgery. Both gram-positive and gram-negative bacterial organisms and fungi may be present at the time of the stula culture. We favor the in situ or endograft approach except in the very unusual patient where an extra anatomic graft may be utilized as some advo­cate [19].
If one could predict which patients would develop a s­tula, different surgical techniques might be utilized. When repairing aortic aneurysms or performing aortoiliac grafts, one needs to avoid the possibility of entering the bowel during dissection. We have closed the abdomen and awak­ened the patient upon inadvertently entering the bowel in elective patients—to return another day to perform the vascular surgery—in hopes of avoiding graft infections. Further, vascular grafts under tension should be avoided to prevent erosion of the contiguous tissue—such as the ure­ter or bowel [20]. Also when closing, the abdomen, the synthetic graft should be protected from direct contact with the surface of the bowel, to prevent or reduce the pos­sible formation of pressure necrosis and a stula. Placing the synthetic aortic bifurcation graft limbs beneath the ure­ters and covering the graft with aneurysmal wall, perito­neum and omentum should reduce the chance of stula formation. When urgency dictates, we have utilized a transthoracic or a subdiaphragmatic approach for proximal control of the aorta as temporizing blood-sparing approaches in the critical patient. Each of these methods however may add other potential complications and thus are avoided when possible. We abandoned suture closure of the distal aortic stump as we developed the current tech­nique and have had no aortic or iliac vessel disruptions since utilizing this approach.
When closing the intestinal defect, we have utilized a two-layer absorbable suture closure when possible with all potentially ischemic, irregular, or possibly infected material resected or inverted into the lumen of the small bowel or colon. If appropriate, edges of the intestinal defect may be debrided along with any necrotic material. It has been inter­esting to nd that in some patients the stula is more mature and the surrounding tissue is not necrotic but more brotic in nature. Also depending on the stula type and the location of the stula in reference to the graft, there may be areas of the
aortic graft with no evidence of contamination or necrosis. The area around the stulous tract may be densely adherent and brotic with no liqueed pus. On occasion, in these situ­ations, when further dissection was felt to be highly risky, a short ring (1/2–3/4 inch) of previously placed prosthetic graft has been left in place along with prior sutures. We then suture the new prosthesis to the previous ring of the residual Dacron prosthesis.
In the instance of a primary infected aorta, we have resected the necrotic infected aorta and placed a prosthetic graft wrapped with omentum. These septic patients are criti­cally ill and, consequently, carry a high morbidity and mor­tality risk. Certainly, repeat infection of the graft in these patients is a consideration but, fortunately, we have not encountered this concern.
Clamping of the aorta and the iliac vessels for the dura­tion (up to 5.5hours) of the operation has uncommonly led to limb ischemia and amputation unless previous ischemia, occlusion, or clot were present. Blood and stula cultures along with cultures from about the graft have all been acquired to direct antibiotic treatment postoperatively. Secondary infections have not been a concern in our experi­ence. Placement of omentum between the repaired duode­num and aorta to avoid direct contact and decrease the possibility of repeat aortoenteric stula formation is a necessity.
We began using the endovascular stent (endograft) stabi­lization of the hemorrhaging stula patient in 2002 [12]. In the selected patient, we have felt this was not only appropri­ate but life-saving. Some surgeons have reported long-time use of this approach without subsequent resection of the ini­tiating or infected graft. We have taken the approach that the stula needs to be closed (bleeding controlled), and in the future the previous infected graft resected, the endograft removed and a new replacement graft inserted. Other options, utilizing the endovascular repair for control of the bleeding, have included insertion of an intra-aortic balloon catheter and attempts at embolization. We have not used these tech­niques nor have we placed postoperative sump drains about the graft. Necrotic material and pus have been found on these grafts at surgery, but no large abscess has been encountered by us. Our approach has been to use the endovascular or EVAR graft in the select patient as a short- or possible longer- term treatment. This may be for a few days or a few months, but not necessarily for the duration of one’s life unless the patient’s other medical conditions contradict fur­ther intervention.
The patient with primary aortoenteric stula presents a different concern because of the friability of the aorta and the cause of their original sepsis. Thus, the risk for addi­tional complications seems to be much greater in these indi­viduals, including mortality. Postoperative intensive care monitoring, stabilization, and treatment will include blood
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counts, temperature checks, blood cultures, vital signs, and frequent physical exams in these individuals.
Prognosis
The lesions herein described present the patient and the treating team a difcult and high-risk situation. Left untreated, the mortality is almost 100%. When treated, the complication rate is great, but the possibility of survival and success are present [17]. In our experience, by using the newer techniques of endografting, proper vascular grafting and placement techniques, appropriate antibiotics, omental wraps and patient-centered nursing care, these patients have a reasonable chance for survival [21]. But reports of post-EVAR aneurysm therapy have shown that a
0.8% of the patients (32 of 3932) developed an aortoenteric stula within 18.5 months of aneurysm treatment, espe­cially when EVAR was utilized for pseudo- aneurysm or emergency therapy [22]. Whether the spiral saphenous vein reconstruction of the infected aorta by Heyligers and Vriens will prove superior to the endograft will require further evaluation [23]. Similarly, other research, such as litho­plasty, will require time for analysis [24]. All of the studies will be observed for the restrictive value in blood transfu­sion and, in morbidity, mortality, and cost [25].
Unfortunately, many of these patients have multiple simultaneous disease processes which require concomitant treatment. Amputation of an extremity, renal failure, pro­longed intubation, bowel concerns, and long-term respirator care are all considerations. With the current aggressive mul­tidisciplinary therapy programs, the mortality rate has been reduced, and long-term survival may be available to a large percentage of these patients [26, 27]. Infections, a challenge of vascular surgery, and other potential complications con­tinue to be monitored [26].
Along with medical therapy, the expense of treatment of these patients continues to rise, unfortunately placing an additional burden on the patient, the family, and the treating medical team. These stulae are an uncommon but life- threatening process requiring diagnosis and treat­ment on an urgent basis—many within minutes or hours. The associated age and medical condition of these patients have a great impact on their potential outcome. Another possible stula might be an aortoappendiceal communica­tion. We have never seen one of these, but literature review has demonstrated a few articles regarding aortoappendi­ceal stula formation (A-A-F). Rectal bleeding seems to be a major sign of the A-A-F.As a result, colonoscopic examination has been used in the diagnosis of such a s­tula [28]. These stulas may also arise from the chronic, infected, and contained rupture of an abdominal aortic aneurysm [29].
Bronchobiliary and gastrobiliary stula are uncommon but may occur in the young or the adult patient [30]. We have not seen a patient with an aortobiliary stula, but arte­riobiliary stulas do occur on the rare occasion [26]. Kawakami etal. have reported their experience, utilizing a fully covered self-expandable metallic stent placed endo­scopically to treat an arteriobiliary stula [30]. Fedakar etal. published in 2011 an aortobiliary stula in their report of 93 patients with an abdominal aortic aneurysm [27]. We have not encountered another possible stula, an aortoap­pendiceal communication, but we have found references in the literature. Prosthetic aortic graft intervention may also lead to multiple aortic stulas in the same patient and includes the aortoenteric (e.g., duodenal) and aorto-caval stula. With the new and unusual approaches, including the transvenous approach to the aorta and cardiac lesions (e.g., valvular lesions), it is assumed more such stulas will develop.
Anesthetic Consideration
It goes without saying that a competent anesthesiologist and OR team are very important to the provision of successful major vascular surgery, especially when that surgery involves the aorta and aortic complications. This capability require­ment involves both the open aortic procedures and the closed or endovascular procedures. Various techniques have been espoused for the multiple requirements to accomplish stabi­lization and treatment of patients with aortic lesions. The elective correction of aortic lesions may be approached much differently than the emergency patient in shock or receiving cardiopulmonary resuscitation.
Thus, in our experience, the patient in critical condition must have respiratory and cardiac control as the primary goal during the resuscitative efforts. Endotracheal intubation along with venous access (usually two or more) to maintain oxygenation, to improve or obtain a blood pressure, blood infusion, cardiac monitoring and attempts to stabilize the patients are primary. Additional assist considerations such as an A-line, central venous access, antibiotics, and TEE may all be considered when the situation is better controlled.
In the elective prior planned surgery in a patient with an aortic lesion, preoperative planned programs progress at a different speed and utilization. In these patients, when pos­sible, an anesthetic consultation before procedure should clarify some of the desired steps to be utilized. In these patients, preoperative antibiotics, blood typing and screen­ing, and the possible requirement for a central venous access are all determined. In the OR or special procedure room, the requirement for or not of an arterial line or TEE (transesoph­ageal echo) may be assessed along with the possibility of an epidural (including possible bleeding considerations).
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Postoperatively, the patient is usually taken to the recovery unit and then the intensive care unit as necessary. Nasogastric intubation may or may not be necessary.
Of note, more recently, an increase in deaths due to abdominal malignancy have been reported following aortic endograft procedures for abdominal aortic aneurysm ther­apy. These malignancy deaths are presumably due to the increased radiation dosage received during endograft implan­tation and the post procedure serial studies of the endovascu­lar implanted aortic aneurysm grafts.
Acknowledgments We appreciate the assistance of the librarian Julie Stielstra, Library Director of Northwestern at Central DuPage Hospital Knowledge Resource Library in Wineld, Illinois, and that of the chap­ter organizer and typist Lynn Murawski. This chapter is dedicated to Robert E.McCray, M.D., and Glen H.Asselmeier, M.D., co-founders of the Glen Ellyn Clinic LLC and the Department of Surgery (general, vascular, and cardiovascular surgery).
References
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Up-to-date. Wolters Kluwer. Mills JL Sr., Eidt JF, Collins KA.
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2. Chang JB, Chang RW, Graver LM. Lower extremity revascular-
ization for atherosclerotic occlusive disease (aortoenteric stula). Peripheral Arterial Disease. New York, NY: McGraw-Hill; 2009. (ch 37)747–786 (759–760).
3. Dieter RA Jr, Kuzycz GB, Dieter IIIRA.In: Dieter RS, Dieter Jr
RA, Dieter III RA, editors. Infrarenal abdominal aortic aneurysm: aortoenteric stula. Endovascular interventions: a case - based approach, vol. 31. NewYork, NY: Springer; 2014. p.367–72.
4. Raman SP, Kamaya A, Federle M, Fishman EK.Aortoenteric stu-
las: spectrum of CT ndings. Abdom Imaging. 2013;38:367–75.
5. Ranasinghe W, Loa J, Allaf N, Lewis K, Sebastian MG.Primary
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6. Mix JW, Pitta SR, Schwartz JP, Tuchek JM, Dieter RS, Freeman
MB. Abdominal aorta (aortoenteric stula). Peripheral Arterial Disease. NewYork, NY: McGraw-Hill; 2009. (Chapter 30) 569– 591 (586–587).
7. Hansen KS, Shiley RC.Aortoenteric stula in advanced heron cell
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8. Dieter RA Jr, Kuzycz GB, Dieter RA III, Dieter RS.Forty years
experience treating septic arteritis and vasculitis. Int J Angiol. 2009;18(1):33–6.
9. Al-Qahtani HH.Arterioenteric stula on a kidney graft site. A rare
cause of massive lower gastrointestinal bleeding. Saudi Med J. 2014;35(5):495–8.
10. Roylance A, Mosley J, Jameel M, Sylvan A, Walker V. Aorto -
enteric stula development secondary to mycotic abdominal aortic aneurysm following intravesical Bacillus Calmett -Guerin (BCG) treatment for transitional cell carcinoma of the bladder. Int J Surg Case Rep. 2013;4(1):88–90.
11. Ramanujam S, Shiels A, Zuckerman G, Prakash C. Unusual
presentations of aorto-enteric stula. Gastrointest Endosc. 2004;59(2):300–4.
12. Dieter RA Jr, Blum A, Pozen T, Kuzycz G.Endovascular repair of aorto-jejunal stula. Int Surg. 2002;87:83–6.i.
13. Tabach TJ, Kane PN, Madjarov JM, Halleman JH, Robischele F, Roush TS.Endovascular exclusion of mycotic aortic aneurysm. Tex Heart Inst. 2007;34:459–62.
14. Sakaguchi H, Marui A, Hirose K, Nomura T, Arai Y, Bir SC, Huang Y, Esaki J, Tabata Y, Ikeda T, Komeda M.Less invasive and highly effective method for preventing methicellen- resistant staphylocus aureus graft infection by local sustained release of vaxcomycin. J Thorac Cardiovasc Surg. 2008;135(1):25–31.
15. Truijers M, Carvers HA, Bredie SJ, Oyen WJ, Blankensteijn JD.In vivo imaging of abdominal aortic aneurysms: increased FDG uptake suggests inammation in the aneurysm wall. J Endovasc Ther. 2008;15(4):462–7.
16. Dieter RA Jr, Kuzycz GB.Endovascular management of arterioen­teric stulas: a systemic review and meta-analysis of the literature. Letter to the editor. J Endovascul Ther. 2011;18:611–2.
17. Valentine RJ, Timaran CH, Modrall GH, Smith ST, Arko FR, Clagett GP. Secondary aortoenteric stulas versus paraprosthetic erosims: is bleeding associated with a worse outcome? J Am Coll Surg. 2008;207:922–7.
18. Walker WE, Cooley DA, Duncan JM, Hallman GL Jr, Ott DA, Reul GJ.The managment of aortoduodenal stula by in situ replacement of the infected abdominal aortic graft. Ann Surg. 1987;205:727–32.
19. Wilson WR, et al. Vascular graft infections, mycotic aneurysms, and endovascular infections. A scientic statement from the ameri­can heart association. Circulation. 2016;134:e412–60.
org/10.1161/CIR.0000000000000457. TBD.2016 c1-c49.
20. Maurel B, Mastracci TM. Aortoenteric Fistula. How to select a proper sealing zone. Endovasc Today. 2016;15(3):64–8.
21. Na KJ, Jung JC, Hwang Y, Lee HJ, Park IK, Kang CH, Jang J-Y, Kim YT.Minimally invasive surgical repair for congenital broncho­biliary stula in an adult. Ann Thorac Surg. 2016;101:1584–7.
22. Kohlberg A.MAEFISTO study highlights characteristics of late aortoenteric stulae after EVAR. London: Vascular News (BIBA Publishing); 2015. p.32.
23. Heyligers J, Vriens P.Spiral saphenous vein reconstruction of the infected aorta: a strategy to adopt. London: Vascular news (BIBA Publishing); 2015. p.28.
24. Zeller T.DISRUPT PAD trial shows “sustained success at six months for lithoplasty to treat calcied, synoptic infrainguinal peripheral arteries”. London: Vascular news (BIBA Publishing); 2016. p.28.
25. Moon MA. No harm from restrictive threshold. Chest Phys Cardiothor Surg. 2015;30
26. Kovach RL. Aortic aneurysmal disease: new techniques for management of complex anatomy. Global Vasc Dig (GVD). 2016;2016(3):4–7.
27. Fedakar A, Findik O, Kalender M, Balkanay M.Unusual emergent presentations of abdominal aortic aneurysm: can simple blood tests predict the state of emergency. Ulus Travma Acil Cerrahi Derg. 2011;17(6):525–32. https://doi.org/10.5505/tjtes.2011.65983.
28. Church JM, Lavery IC, Beven EG.Colonoscopic diagnosis of an aortoappendiceal stula presentation colonic hemorrhage. Anz J Surg. 1988;58(6):516–8.
29. Keripe S, Slavek S, Oshode T.Primary aortoappendicular stula arising from an infected, chronic, contained, ruptured abdominal aortic aneurysm. Ann Vasc Surg. 2006;20(6):820.
30. Kawakami H, Okamoto M, Kumatani M, Kubota Y, Kawakuloo K, Abe Y, Kawalata S, Kubo K.Endoscopic placement of a fully covered self-expandable metallic stent to treat an arteriobiliary stula. Endoscopy. 2014;46(Suppl 1 VCTN):E566–7. https://doi.
org/10.1055/s-0034-1377939. E pub 2014 Nov 19.
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Malignant andBenign Aortic Tumors
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RaymondA.Dieter Jr., GeorgeB.Kuzycz, RaymondA.Dieter III, JessicaGulliver, andRobertS.Dieter
28
Malignancy represents one of the more common afictions of the human body, but benign or malignant tumors of the aorta are very rare. Several causes or etiologies of tumors have been dened and related to various human habits or acquired infec­tion. Smoking and herpes infections represent two of the etio­logic agents related to tumor inducement in the adult human. Neither of these, however, has been associated with primary vas­cular tumor development nor has any other dened risk factor.
Malignancy of the vascular tree is very uncommon to rare. Rarely, if ever, are primary or secondary malignancies of the major arterial or venous systems encountered by the average vascular or thoracic surgeon. Certainly, as tumors develop, nutrition for the growth and multiplication of the malignant cells must be provided by the surrounding arterial system. But the vascular network providing such necessities is usually of the smaller arteriole and venous system.
Only on occasion does the neoplastic process involve the larger vessels. When this occurs, the patient may develop either acute or chronic symptoms related to the vessel(s) involved. When tumor involvement occurs in the lung, hemop­tysis may develop. When tumor involvement of the major ves­sels to the extremities occurs, either acute or chronic extremity ischemia may create major consequences for the patient [1]. In the latter group, major peripheral vessels, the heart, or the
R. A. Dieter Jr. (*) ∙ G. B. Kuzycz Cardiovascular and Thoracic Surgery, Northwestern University at Cadence Health Emeritus, Wineld, IL, USA
R. A. Dieter III University of Tennessee Medical Center, Cardiovascular and Thoracic Surgery, Knoxville, TN, USA
J. Gulliver Department of Pathology, University of Wisconsin Hospital and Clinics, Madison, WI, USA
R. S. Dieter Interventional Cardiology, Vascular and Endovascular Medicine, Loyola University Medical Center, Maywood, IL, USA
aorta may be the source for the ischemic process by either embolic, obstructive, or constrictive mechanisms.
Aortic tumors, either primary or secondary in origin, are very uncommon, and thus, many physicians, including car­diovascular surgeons, have never had the opportunity to treat patients with these concerns. The original presentation may be confusing and thus suggest other non-neoplastic disease entities as the cause for the patient’s complaints. Even initial diagnostic testing may be misinterpreted or suggestive of a non-neoplastic etiology for the patient’s complaints, as dis­cussed in the Critical Extremity Ischemia book [1]. However, both primary and secondary aortic tumor involvement does occur and, in most instances, creates major diagnostic and therapeutic challenges.
Primary Aortic Tumors
Lesions involving the aorta are common, and their symptom­atic results are familiar to both the healthcare profession and the lay public. To mention that an individual has an aortic process affecting their health immediately brings forth an image of possible risks and the possible result. The aorta is well recognized as a major structure by the medical and non­medical community to be necessary for the development and maintenance of one’s body and its function. The usual aortic aneurysm rupture or occlusive concepts are generally under­stood. However, discussion with a pathologist of the inci­dence of primary or secondary aortic neoplasm involvement is usually met with a pause and then, “I don’t believe I have ever seen such a tumor.” They then will discuss the occa­sional hemangiopericytoma, and similar small vessel tumors, or the benign/malignant endocardial tumors which may embolize. Both of these examples are much more frequent than the aortic neoplasm and still are not common.
Aortic tumors may be classied in a variety of manners depending on the type of classication to be utilized. Lesions may be categorized as malignant or benign. They may be categorized according to their location in the aorta, or they
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may be grouped according to symptomatology. The major pathologic tumor types that one may encounter directly involving the aorta are as follows.
Types of Benign and Malignant Aortic Tumor Classications
I. Primary
A. Malignant (sarcoma—most common patho-
logic diagnosis)
1. Angiosarcoma (25%)—epitheloid
2. Pleomorphic sarcoma
3. Intimal sarcoma (27%)—actually dene location
4. Malignant brous histiocytoma (15%)
5. Leiomyosarcoma (14%)
6. Undifferentiated/high-grade sarcoma (9%)
7. Fibromyxosarcoma
8. Myobrosarcoma
9. Hemangiopericytoma
10. Chondrosarcoma
B. Benign
1. Myxoma
2. Lipoma
II. Secondary/metastatic involvement
A. Lung
1. Non-small cell
(a) Squamous cell (b) Adenocarcinoma (c) Large cell
2. Small cell
B. Esophageal C. Retroperitoneal lymphoma D. Mediastinal
1. Malignant thymoma
2. Malignant germ cell
III. Associated tumor
A. Fibrous histiocytoma
B. Cardiac embolic
IV. Benign lesions—only a small percentage of the
total aortic tumors A. Localized polypoid lesions B. Thrombotic mass-like lesions
The most frequently reported tumor involving the aorta is the sarcomatous group. Primary epithelioid angiosar­coma is the most frequently reported primary malignancy of the aorta, and non-small cell lung carcinomas (squa­mous cell and adenocarcinoma) are the most frequent met-
astatic lesions involving the aorta. Less frequent malignancies and other very uncommon lesions are also diagnosed. The primary aortic tumor may metastasize to other locations.
Primary Aortic Tumor Metastatic Sites
I. Intraluminal—embolic
A. Legs B. Mesentery C. Kidneys
II. Periaortic—direct
A. Thoracic B. Retroperitoneal
III. Hematogenous
A. Bones B. Skin
C. Organs IV. Liver V. Spleen VI. Lung
Most reports of primary aortic tumors are of single or a
small number of malignancies involving the aorta [219]. The paucity of series reports further demonstrates the rarity of this disease. The sarcoma groupings (epithelioid; pleo­morphic; angio-, bro-, myobro-, leiomyo-, and undiffer­entiated sarcoma groupings) are varied in frequency, occurrence, and appearance. Less commonly reported are the brous histiocytomas [18]. But, all these sarcomas are highly malignant with a guarded prognosis. Lipomas are the most common benign aortic tumor [6, 9].
Similarly, the secondary or metastatic malignancies of the
aorta also carry a guarded or unfavorable prognosis. These lesions usually occur in the 50- to 70-year age group and may result primarily from the pulmonary or bronchopulmonary group of carcinomas (CA of the lung) or esophageal carcino­mas. All cell types of the lung have been involved. These include both the small cell and non-small cell malignancies, including the large cell, the adenocarcinoma, and the squa­mous cell tumors [2031]. Malignant thymomas and germ cell involvement have also been known to involve the aorta.
The primary aortic tumors also metastasize in a fashion
similar to many of the other malignant tumors which an indi­vidual may develop. The primary tumor, especially the sar­comas, initially may present with both local and distant spread. One of the more common aortic tumor presentations is the distant embolic phenomenon of the tumor cast or sec­ondary clot with occlusion of the iliofemoral or enteric sys­tems, especially creating sudden secondary ischemic
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symptoms of the abdomen or legs [3]. Also, periaortic direct local extension of the malignant thoracic or retroperitoneal aortic lesions is a natural consequence of these malignancies. Similar to many other malignancies, hematogenous spread to the bones, liver, and spleen is reported [3, 7, 17, 19]. Some patients have also developed multiple palpable skin or sub­cutaneous metastatic nodules [7, 10].
Aortic Tumor Symptomatology
As with so many diseases, development of one or more symptoms may direct an individual to nding the primary disease entity almost immediately after development of the symptom. But as with many disease entities, the pre­sentation, the symptom complex, and the patient’s history may be both confusing and misleading. The literature demonstrates that both the diagnosis and the potential sug­gested treatment routes by the physician have frequently been led astray due to the lack of specicity of symptoms and diagnostic studies. Such a dilemma occurs with both primary and secondary tumor involvement of the aorta.
The symptomatology most commonly revolves around four main areas: (1) embolic, (2) occlusive, (3) pain, and (4) generalized or systemic.
Potential Aortic Tumor Symptomatology
I. Embolic symptoms
A. Acute B. Chronic C. Loss of function
II. Occlusive—progressive
A. Abdominal aorta B. Coarctation/dissection type
III. Pain—sudden or progressive
A. Abdominal B. Extremities C. Thoracic
IV. Generalized/systemic
A. Weight loss/fatigue B. Fever C. Night sweats D. Nausea
E. Anorexia V. Renal—infarction VI. Clubbing—hand VII. Neural
A. Horner’s syndrome
B. Hemiplegia VIII. Extremis
Embolic symptoms may develop in both the upper and lower extremities and in the abdomen. Most commonly, the embolic tumor or clot may produce acute symptoms with sudden and total occlusion of the recipient distal artery.
Symptoms Due to Embolic Aortic Tumor
I. Extremity
A. Ischemia B. Claudication C. Acute or chronic pain D. Loss of function
II. Skin
A. Pallor B. Hematogenous lump or rash
III. Stroke
A. Unable to speak
B. Major paralysis IV. Coronary occlusion V. Buttock pain VI. Gastrointestinal—ischemic bowel
Depending on the artery involved, the patient may develop
stroke, severe acute leg pain, renal concerns, or skin lesions [2, 3, 8, 11, 18, 32, 33]. In the legs, the acute embolic pain may be sudden and excruciating with pallor and loss of func­tion. Or, with smaller emboli, the pain may be slow and insidious in onset depending on the vessel occluded and size of the clot or tumor embolus. Abdominal symptoms also may be sudden and catastrophic with embolization to the enteric (e.g., superior mesenteric artery) or renal vessels. The patient who develops a sarcoma in the abdominal aorta may have progressive and confusing discomfort similar to that of an aortic aneurysm.
Pain due to a thoracic aortic tumor may resemble that of
an expanding aortic aneurysm. The tumor may also embo­lize distally—downstream—or more proximally to cause a stroke [11, 32]. The distinction of a primary tumor-induced cerebral infarction from a stroke due to aortic dissection may be difcult even after diagnostic studies. Retrograde occlusive coronary artery involvement has also been dem­onstrated acutely in a patient [16]. The thoracic aortic tumor symptoms may also present with pain similar to an acute dissection [7]. Horner’s syndrome along with hemi­plegia was also reported in the patient with acute coronary [16]. Less common symptoms may result from stulization into the bronchus, vena cava, and esophagus along with gastrointestinal bleeding.
Other systemic symptoms include weight loss and fatigue
in many of the patients. Nausea, unilateral clubbing, night
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sweats, and fever may suggest other maladies in these patients.
Associated Symptoms and Risks of Aortic Tumor
I. Concommitant—atherosclerosis II. Age—40s, 50s, 60+ III. Sex—more common in males IV. Hypertension—resistant to diagnosis/treatment V. Aneurysm formation
A. Leakage—descending thoracic
B. Multiple VI. Smoker/cough VII. Death—acute
A. 1year—most
B. 3+ years—more than 90% deceased
The patients tend to be older, over 60years of age. A
few are in the 40- to 50-year age range, and the age range of occurrence is from 3months to 82years [5, 32]. It also seems that the male gender predominates, with only a few females reported with these tumors [16, 19]. Single or multiple aneurysm formations with suspected leakage present diagnostic concerns. Elevated blood pressure has been found in a number of these patients as has the smok­ing habit.
Morbidity is great in these patients, and the aortic
tumor diagnosis suggests a highly morbid outcome. As a group, these sarcomatous lesions are highly fatal. The individual may present acutely in a moribund state, and surgery in this acute state is very risky. By the end of 1 year, a large portion of the patients have passed away despite therapy. A few patients survive up to 3 years. However, there is 90% mortality by the end of 3years [7]. The survivors are primarily patients with low-grade malignant or benign tumors.
Aortic Tumor Diagnosis
As already mentioned the patient’s symptomatology and ndings may be difcult to sort out and confusing. The patient’s history may be classical for other more common lesions and lead the physician down the wrong diagnostic pathway. Thus diagnostic testing options and consultants may be led astray by the patient, the history, the physical ndings, and the diagnostic studies. The sorting out of perti-
nent historical and testing interpretations may prove difcult.
But, symptomatology and physical ndings may lead the diagnostic and treatment approach in the correct direction. Even then, the diagnosis may not be established until after therapeutic intervention has occurred. Such is the case, for example, when a thrombus has been removed from the lower extremity and the pathologist, a few days later, forwards the microscopic ndings and pathologic diagnosis [1].
The usual initial approach to the patient’s problem is to obtain a history from the patient or of an accompanying indi­vidual regarding the patient’s complaints. This is followed by a complete physical examination—head to toe. Having assessed the situation, this initial assessment will guide the number and type of studies to be obtained. Initially, a CBC (complete blood count) and x-ray may be ordered to help differentiate the problem. With the modern-day emergency room programs, most of these studies will be obtained in the emergency room (ER) along with other blood tests and radiologic scans.
The physical exam may demonstrate a relatively stable and less emergent situation or may dictate urgent or emer­gency diagnostic testing, blood typing, consultation with potential diagnosticians, and intravenous access which may all be required during the initial evaluation.
Laboratory testing may show a marked anemia, an ele­vated sed rate, an increased C-reactive protein (CRP), or an increase in the number of white blood cells (WBCs). All of these are nonspecic and may be misleading. The physical diagnosis may reveal a stable nondistressed individual or a patient in severe pain. He/she may be pale, febrile, and tachycardic and have a heart murmur. The legs may be pale and pulseless or immobile. The patient may have severe abdominal or chest pain and be in extremis.
The skin may contain multiple nodules and petechiae. The patient may be aphasic or hemiplegic. The abdomen may be tense and distended. A Horner’s syndrome or ocu­lar disturbance may be present. Urine may be abnormal and the BUN and creatinine elevated. The blood pressure may be elevated and vary between the right and the left arm [33]. Evidence of weight loss and clubbing may be obvious to all.
Testing will proceed along the most likely and appropri­ate route to establish the diagnosis and course of treatment. Different ndings and results may lead to the best and most expeditious course of treatment. Less complicated studies such as an arterial Doppler, electrocardiogram (EKG), and emergency room (ER) echocardiogram may all be obtained with varying results.
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Diagnostic Studies to Delineate Aortic Tumors
I. Radiologic imaging
A. CT (computerized tomography)
1. May be misleading
2. CT angiogram
B. MRI (magnetic resonance imaging)
1. Gadolinium
2. MRA (magnetic resonance angiogram)
C. Angiography
1. Arterial—diagnostic
2. OR (operative room)
3. Venography
D. PET scan (positive emission)
II. Biopsy/tissue
A. Microscopic
1. Mass
2. Skin B. Immunohistochemical (best) C. Vascular system D. Surgical specimen
III. Lab—CBC—nonspecic
A. Increased sed rate B. CRP (C-reactive protein) increase C. WBC (leukocyte count) increase D. Video microscopy
IV. Echo
A. TEE (transesophageal echocardiography) B. Cardiac Doppler C. Arterial ultrasound
V. Autopsy
Many of the studies performed in these patients are pre­sented above. A fairly frequent approach may include an emergency CT (computerized tomographic) scan. These may be misleading or misinterpreted as an aneurysm or thrombus due to the rarity of a primary aortic tumor [2, 12]. A CT angio­gram, with intravenous contrast, may follow due to the lack of an established diagnosis [2]. Others have performed magnetic resonance imaging (MRI) in an effort to delineate the patient’s diagnosis and extent of disease. MR angiography and the use of gadolinium have been advocated in the diagnostic “tree” due to the inability to differentiate lumen from the arterial wall [3, 8, 16, 32]. None of these scans have proven sufcient in all situations, and thus, angiography including operating room (OR) angiography has also been required [8]. Distal aortic angiography has been utilized to rule out a Leriche syndrome [15]. Using these techniques and transvascular angiography, one must guard against distal embolization [16].
389
Due to the difculty in diagnosis of these patient’s prob­lems, other studies have included a TEE (transesophageal echo) and temporal artery biopsy [3, 16, 31]. PET (positive emission tomography) has further been utilized to delineate the extent of the patient’s disease—especially in suspected metastatic disease. Skin or nodule biopsy may prove confus­ing and diagnostic. Obviously tissue biopsy and associated immunohistochemical studies are the most accurate diagnos­tic studies available. But this requires obtaining tissue and the recognition that tissue is necessary [17]. Even video microscopy has been utilized.
Preoperative biopsy of aortic tumors or transarterial tumor biopsy has been discussed in the literature. In the past, 17 gauge 7 1/2 inch translumbar aortography was a routine and rapid testing procedure for aortic, renal, or iliofemoral dis­ease with little morbidity in our experience of its usage in hundreds of patients. This technique has been largely sup­planted by catheter angiography for diagnosis and treatment. Transvascular biopsy techniques have been developed for multiple disease entities such as liver and renal disease, or cardiac rejection [3436]. This transvenous approach for the biopsy of neoplasia has been utilized for cardiac tumors, ret­roperitoneal tumors, cavoatrial tumors, and pancreatic tumors [3739]. Despite the availability of transarterial and transvenous needle and catheter techniques, the use of these approaches is uncommon for aortic tumors. The nal tissue diagnosis usually does not occur until the surgical explora­tion and removal of the tissue specimen. The nal, but unfor­tunate, diagnostic approach may be an autopsy in the difcult situation. Utilization of transarterial or transaortic biopsy techniques has not gained favor for the diagnosis of primary aortic tumors due to the embolization concerns [16]. Also, arterial endoscopic techniques have not been utilized for diagnostic purposes in these patients.
Dierential Diagnosis
As already presented, diagnosing and differentiating the true nature of an aortic neoplasm may be very difcult and the correct pathway to the tumor denition tortuous. The patient’s symptoms may be confusing, misleading, and strongly suggestive of other disease entities.
Concurrent preexistent disease and treatments may con­fuse the issue and mask the underlying sarcoma. Depending also on whether the aortic tumor is primary or metastatic from a non-arterial primary tumor may cloud the diagnosis and even be difcult to diagnose under the microscope. Also, a benign primary aortic tumor may present differently than a malignant aortic tumor.
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Consulting radiologists or cardiovascular surgeons may misinterpret the history, physical, laboratory studies, or radio­graphic evidence as to the true etiology. Cardiac murmurs or aortic bruits may suggest underlying vascular disease, such as mitral regurgitation with valvular prolapse or a chronic Leriche syndrome. A lateralizing stroke can suggest aortic dissection or cardiac emboli. Abdominal pain with distention and bruit may imply atherosclerotic disease—especially if previous his­tory is compatible. Some of the disease entities that may be misapplied to the patient’s symptoms are the following.
Dierential Diagnosis of Aortic Tumors
I. Valvular heart disease—mitral regurgitation
with prolapse II. Aortic occlusion—abdominal/Leriche III. Primary intra-aortic thrombus IV. Horton’s disease V. Infections
A. Mycotic aneurysm
B. Positive cultures VI. Lung cancer—in association VII. Anatomy: congenital
A. Right aortic arch
B. Confusing VIII. Multiple malignancies
The presence of chronic symptomatology due to other
nontumor lesions may suggest superimposed acute progres­sion of a vascular process, such as arterial or aortic athero­sclerosis of the aortoiliac vessels, rather than an embolic tumor thrombus. A suspected mycotic aneurysm may be pur­sued with blood cultures, angiography, and antibiotic therapy [3, 7]. When other lesions exist, such as bronchopulmonary malignancy, the aortic association or involvement may be difcult to differentiate. Bronchoscopy may yield the tumor diagnosis, but not dene the aortic concern. Similarly, con­genital aortic arch and branch formation such as the right arch and descending aorta with aneurysm formation may fur­ther cloud the diagnosis [25, 27].
Multiple malignancies may also confuse the physician
[32]. Development of primary angiosarcoma in a previous aortic graft will be confusing [40]. Other surgical proce­dures, such as cholecystectomy, have been performed while attempting to improve the patient’s condition—without ben­et [41]. Presence of exophytic-calcied atheroma may, but not correctly, suggest the presence of a benign papillary broelastoma [42]. Further, oating thrombus or polypoid thrombus may also be found distal to the aortic valve and produce confusing distal systemic symptoms.
Similarly, one must carefully review the CT scan, MRI, or
other studies to eliminate the possibility of another non­aortic origin site of tumor such as the left ventricle or other
cardiac locations—even in infants [43]. The diagnosis may be further confusing when infectious complications develop during the course of the disease.
Aortic Tumor Location/Classication
The aortic tumors (mostly of mesenchymal origin) may occur in any location from the aortic valve to the aortic bifurcation. The type of tumor, benign or malignant, denes to some degree the primary location of the tumor. If the broadest tumor denition includes a mass of any type, then the oating throm­bus or polypoid aortic thrombus seen in the ascending aorta, especially just above the aortic valve, probably represents the most common aortic tumor [44]. The subsequent distal embolic symptoms may also be confusing as to their origin.
Echocardiography has proven helpful in this determina­tion as to site of origin. These lesions demonstrate one of the growth patterns of aortic tumors, namely, the intraluminal form. Three forms of aortic tumor growth have been dened morphologically: (1) intraluminal, (2) intimal, and (3) mural (to include adventitial) [41].
Location of the tumor has also been delineated anatomi­cally as to the level of the aorta involvement. Forty-six per­cent of the tumors are located in the chest, 25% as thoracoabdominal, and 27% in the abdominal aorta [7]. Further localization to the aorta is shown below. The trans­verse aorta and arch seem to be particularly prone to the pri­mary aortic tumor and also may be confusing when they create a dissection. The descending aortic lesions may be subclassied into proximal, mid, and distal thoracic lesions and include both primary and metastatic malignancies.
Aortic Tumor Location and Dierential Diagnosis
I. Ascending aorta
A. Lipoma—adventitial B. Polypoid thrombus—endoluminal
II. Arch—especially transverse aorta
A. Atheroma B. Dissection
III. Descending thoracic aorta
A. Proximal—primary aneurysm B. Mid-thoracic C. Distal thoracic
IV. Abdominal
A. Suprarenal B. Aneurysm C. Paravascular—renal occlusive D. Infrarenal
1. Leriche
2. Intra-aneurysm
V. Metastatic sites—bone, liver, adrenal, and lung
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Intra-abdominal aortic tumors may also be subclassi­ed according to the aortic lesion location. These tumors may be suprarenal, paravascular, or infrarenal, and symp­toms may vary according to location—for example, they may cause renal artery occlusion. The general anatomic locations for the tumor-originating site can thus have some effect on the type of symptoms a patient may develop. For example, the ascending and arch locations may produce pain and stroke type of symptoms while the descending or abdominal primary sites may produce renal, gastrointestinal, systemic, or lower extremity complaints.
Non-aortic location of aortic tumor metastases may develop in multiple organs throughout the body. These sites include the bones, liver, kidneys, adrenal glands, and lung. Non-aortic primary tumors involving the aorta further con­fuse the issue as to the symptoms and diagnosis. The most common of these invasive tumors causing aortic involve­ment include the bronchopulmonary carcinomas and esoph­ageal and the retroperitoneal tumors, which have a direct or contiguous contact with the aorta. This further confuses the diagnostic and treatment attempts. To date, however, we have not seen direct tumor invasion requiring aortic resec­tion due to mesothelioma nor malignant thymoma.
Aortic Wall Involvement
These tumors may involve any layer of the aorta. Microscopically, they may be difcult to diagnose and to dif­ferentiate as to the cell type and point of origin. We have already mentioned the primary locations from which these tumors originate. Additional classications exist as to the type and site of initial growth.
Location of Tumor in Aortic Wall
I. Intraluminal thrombus—polypoid
A. Obstructive
B. Embolize II. Endothelial III. Intimal
A. Tunica intima
B. Usually descending thoracic or abdominal
C. Prone to embolize IV. Intramural
A. Extravascular dissection
B. Media—extravascular growth may cause S
and S
C. Adventitia V. Aortic branches—subclavian aneurysm
The most common point of origin seems to be the intimal or tunica internal layer [3, 5, 19, 33]. Because of their origi­nation site, many of these tumors may grow into the aortic lumen and produce polypoid-type lesions that may further be classied as obstructive or nonobstructive. It seems that these lesions are more commonly located in the descending thoracic and the abdominal aorta. This tendency may lend itself more to the formation or creation of emboli in associa­tion with narrowing of the aorta. Primarily polypoid lesions may also be felt to originate in the endothelium [17, 19].
The intramural-originating aortic tumors (18%) may lead to dissection or a differential diagnostic concern resulting from dissection like ndings as a result of their media origin. Extravascular growth from tumors originating in the media or adventitia usually creates greater mass-like tumors [16]. Ten percent of the malignant aortic tumors involve all three layers of the aorta. The intraluminal benign mass lesions are usually of thrombus or plaque in origin. Whereas the benign lipomatous lesions usually arise in the adventitia or in the outer layer of the aorta, major luminal encroachment may and does occur with most of the malignant aortic tumors. Such encroachment may lead to vascular occlusion as a result of growth, thrombus, or embolus. When the tumor involves the vessel media in and about the major cerebral circulation, cerebral ischemia or infarction may then create preoperative uncertainty as to the diagnosis.
Secondary complications due to the tumor depend in many instances on the wall location of the primary tumor origin. Stroke, renal infarction, and other embolic sites may result from the intraluminal lesion embolus, intramural growth and dissection, as well as additional adventitial mass formation. Such mass formations may thus be primary or secondary in origin. Nonprimary malignant growths involv­ing the aorta primarily originate from contiguous invasive malignancies such as the pulmonary or retroperitoneal lesions. Periaortic contiguous tumor masses may be malig­nant—such as lymphoma or carcinoma of the lung—and dif­cult to diagnose and treat. Such lesions may be benign, partially calcied, and delineation from an actual aortic aneurysm, small vessel aneurysm, or benign tumor lesion vexing (Fig.28.1).
Treatment ofAortic Tumors
Successful treatment of the patient and his/her disease usu­ally depends on having a correct initial diagnosis. With knowledge of the type of process aficting the patient, appropriate therapy may then be initiated. Medical, onco­logic, and surgical treatments are all utilized when the diag­nosis indicates. Unfortunately, with the malignant tumors of the aorta, diagnosis is often not established early in the pro­cess and, in a large number of the patients, only established