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Table of Contents
CASE REPORT
Year : 2018  |  Volume : 9  |  Issue : 2  |  Page : 161-163

Fatal rupture of descending thoracic aortic aneurysm in a Nigerian patient


1 Department of Histopathology, Babcock University Teaching Hospital, Ilishan-Remo, Ogun State, Nigeria
2 Department of Pathology, University College Hospital, Ibadan, Oyo State, Nigeria

Date of Web Publication19-Nov-2018

Correspondence Address:
Dr. Mustapha Akanji Ajani
Department of Histopathology, Babcock University Teaching Hospital, Ilishan-Remo, Ogun State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/atp.atp_20_18

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  Abstract 

Aortic aneurysms are rare conditions in black patients and carry a high mortality if detected late. Unfortunately, the rarity of the condition makes detection a difficulty for many physicians as diagnosis in the early stages requires a high index of suspicion. The varied symptoms presented by the patient often mimic other less severe, but more common conditions and may cause a misdiagnosis. We report a 79-year-old male known hypertensive patient who presented a day before demise with a 4 h history of severe epigastric pain. He was managed for acute exacerbation of peptic ulcer disease to rule out pancreatitis. However, all investigations done were within normal limits. His clinical condition deteriorated, and he died 18 h after admission. An autopsy revealed massive left hemothorax and a tear in the descending aortic aneurysm. Microscopic examination confirmed complicated atherosclerosis in the descending thoracic aorta. The misdiagnosis, in this case, leads to wrong management with the patient receiving treatment for severe peptic ulcer. There is a need to consider this disease in patients who present with severe nonspecific abdominal symptoms.

Keywords: Aortic aneurysm, autopsy, left hemothorax, rupture


How to cite this article:
Ajani MA, Faniyi OS, Salami A. Fatal rupture of descending thoracic aortic aneurysm in a Nigerian patient. Ann Trop Pathol 2018;9:161-3

How to cite this URL:
Ajani MA, Faniyi OS, Salami A. Fatal rupture of descending thoracic aortic aneurysm in a Nigerian patient. Ann Trop Pathol [serial online] 2018 [cited 2018 Dec 14];9:161-3. Available from: http://www.atpjournal.org/text.asp?2018/9/2/161/245690


  Introduction Top


An aortic aneurysm refers to a pathological dilation of the normal lumen.[1] It is defined as a permanent localized dilation of the aorta, having a diameter at least 1.5 times of the expected normal diameter of that given aortic segment.[2] It is a vascular emergency. Risk factors include systemic hypertension, coronary artery disease, hypercholesterolemia, Ehlers–Danlos Type IV, and Marfan's syndrome.[3] The most common cause of aortic aneurysms is atherosclerosis in 80% of cases. They are usually asymptomatic except when dissection or rupture occurs, but occasionally present with symptoms such as abdominal and back pain.[4],[5],[6]

Thoracic aortic aneurysms (TAAs) and ruptures are less common than abdominal aortic aneurysms accounting for 25% of all aneurysms.[7],[8],[9] TAAs are more common in males with a male to female ratio of 1.7–3:1 and a mean age of 65 years at presentation.[7],[8],[9] The incidence of TAAs is estimated to be 5.9 compared with 350 cases for abdominal aortic aneurysms per 100,000 person-years.[10] Of the TAAs, the ascending aorta is affected in 50% of cases; the aortic arch in 10% and the descending thoracic aorta (DTA) in 40%.[9] Rupture of an aortic aneurysm has a high mortality even with prompt treatment only 10%–25% of patients survive. Globally, mortality figures from aortic aneurysms have risen from approximately 100,000 in 1990–152,000 in 2013.[11]


  Case Report Top


We report a 79-year-old male patient who was a known hypertensive for several years but has not been compliant with prescribed medications. He was also on medications for peptic ulcer diseases diagnosed years earlier. He presented a day before demise with a 4 h history of severe epigastric pain. He was managed for acute exacerbation of peptic ulcer disease to rule out pancreatitis and was subsequently placed on intravenous fluids, antibiotics, and antiulcer medications. Results of hematological and serological investigations were within normal limits. Eighteen hours later, his clinical condition deteriorated rapidly and he had a cardiopulmonary arrest and was confirmed dead.

At autopsy, general examination revealed only severe pallor. The thoracic cavity, however, revealed 3,300 ml of left hemothorax with collapse of the left lung and bulging left pleura [Figure 1] and [Figure 2]. There was a 6-cm aneurysmal dilatation of the DTA with a 4 cm × 2 cm tear/rupture [Figure 3] in the wall of the aneurysmal sac. Atheromatous plaques and extensive intimal hemorrhage were also seen.
Figure 1: Massive left hemothorax on opening the body up on the autopsy table

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Figure 2: Blood clots removed from the left thoracic cavity at autopsy

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Figure 3: Ruptured aneurysmal dilatation of the descending thoracic aorta

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Microscopic examination confirmed complicated atherosclerosis in the DTA. Hypovolemic shock from massive hemothorax due to rupture of a descending TAA was implicated as the cause of death.


  Discussion Top


A high index of suspicion is needed in diagnosing aortic aneurysm due to its ability to mimic other more common conditions as was seen in this case.[12],[13] The disease has been dubbed “a silent killer” due to the fact that only about 5% present with symptoms while 95% of emergency patient with aortic aneurysm were previously asymptomatic. The previous history of peptic ulcer disease had totally masked any suspicion of an aortic aneurysm. The lack of evidence of peptic ulcer in the stomach at autopsy suggests that the epigastric pain may be due to the weakening aneurysmal sac.[14]

Cystic medial degeneration, usually due to aging, has been implicated as the main cause of aortic dissection and aneurysm in the ascending part of the thoracic aorta while atherosclerosis is the most common cause in the descending part of the thoracic aorta.[15] This is due to the weakening of the tunica media by deposition of atherosclerotic plaques causing derangement of layers of elastic fibers and smooth muscle cells as was seen in the index patient with complicated atherosclerosis. Although many connective tissue diseases, such as Marfan and Ehlers–Danlos syndrome, are known causes of aortic aneurysm, familial clustering of diseases have also been reported with several genes which code for fibrillin being implicated particularly MYH11 and ACTA2.[16]

The silent nature of the disease stresses the importance of doing appropriate examinations and tests in the elderly and in patients with family history of the disease. Despite the high fatality rate in the event of a rupture, the disease is curable using surgical and medical therapy if discovered early.[16] Typically, most aneurysms are treated surgically when the diameter of aneurysmal sac exceeds 5 cm.[15],[16] Medical treatment is usually instituted before the aneurysm getting to the critical value. The aneurysmal sac in our index patient was 6 cm which indicates that the aneurysm had gone beyond the critical value and was already a candidate for a catastrophic event as at the time of presentation.

Over 50% of aortic aneurysms are undetected before death which suggests that the actual incidence of this disease is not truly known.[17] The autopsy that was done in the index patient was key to arriving at the definitive cause of death. The importance of autopsy is further illustrated by this case and will again remind the medical community about the need to be aware of a “silent killer” and take appropriate preventive measures in their patients.


  Conclusion Top


The findings at the autopsy of this index patient is a reminder of the postmortem examination in determining the cause of death and thus making us have a true incidence of many “Silent killers” such as aortic aneurysms which are underreported. There is a need to look out for this disease in susceptible patient populations.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Johnston KW, Rutherford RB, Tilson MD, Shah DM, Hollier L, Stanley JC, 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;13:452-8.  Back to cited text no. 1
    
2.
Evangelista A. Aneurysm of the ascending aorta. Heart 2010;96:e979-85.  Back to cited text no. 2
    
3.
Pomianowski P, Elefteriades JA. The genetics and genomics of thoracic aortic disease. Ann Cardiothorac Surg 2013;2:271-9.  Back to cited text no. 3
    
4.
Kent KC. Clinical practice. Abdominal aortic aneurysms. N Engl J Med 2014;371:2101-8.  Back to cited text no. 4
    
5.
Hannuksela M, Stattin EL, Johansson B, Carlberg B. Screening for familial thoracic aortic aneurysms with aortic imaging does not detect all potential carriers of the disease. Aorta (Stamford) 2015;3:1-8.  Back to cited text no. 5
    
6.
Upchurch GR Jr., Schaub TA. Abdominal aortic aneurysm. Am Fam Physician 2006;73:1198-204.  Back to cited text no. 6
    
7.
Canon C. Radiology. McGraw-Hill Speciality Board Review. New York City: McGraw-Hill Education; 2009.  Back to cited text no. 7
    
8.
Dăhnert W. Radiology Review Manual. 7th ed. Philadelphia: Williams and Wilkins, Lippincortt; 2011.  Back to cited text no. 8
    
9.
Adekanmi AJ, Olusunmade D. Giant ascending aortic aneurysm: Are there peculiarities in the developing world? Open J Clin Diagn 2014;4:105-11.  Back to cited text no. 9
    
10.
Bickerstaff LK, Pairolero PC, Hollier LH, Melton LJ, Van Peenen HJ, Cherry KJ, et al. Thoracic aortic aneurysms: A population-based study. Surgery 1982;92:1103-8.  Back to cited text no. 10
    
11.
GBD 2013 Mortality and Causes of Death Collaborators. Global, regional, and national age-sex specific all-cause and cause-specific mortality for 240 causes of death, 1990-2013: A systematic analysis for the Global Burden of Disease Study 2013. Lancet 2015;385:117-71.  Back to cited text no. 11
    
12.
Behera C, Ravi R, Lalwani S, Dogra TD. Sudden death due to aortic rupture while swimming – A case report. J Indian Acad Forensic Med 2008;30:79-81.  Back to cited text no. 12
    
13.
Pannag SK, Sapeco SD, Wiseman Pinto RG, Couto F. Fatal traumatic rupture of ascending aortic aneurysm having idiopathic cystic medial necrosis: An autopsy case. Indian Acad Forensic Med 2010;32:339-42.  Back to cited text no. 13
    
14.
Verma S, Sheikh WR, Jaiswal S. An atypicaal presentation of acute aortic dissection in the emergency department. J Case Rep 2017;7:177-9.  Back to cited text no. 14
    
15.
Prakash P, Patni R, Asghar NM, Chan KM, Antanas M. Ascending aortic aneurysms: Pathophysiology and indication for surgery. E J Eur Soc Cardiol Counc Cardiol 2011;10:1-4.  Back to cited text no. 15
    
16.
Lavall D, Schäfers HJ, Böhm M, Laufs U. Aneurysms of the ascending aorta. Dtsch Arztebl Int 2012;109:227-33.  Back to cited text no. 16
    
17.
Elefteriades JA, Sang A, Kuzmik G, Hornick M. Guilt by association: Paradigm for detecting a silent killer (thoracic aortic aneurysm). Open Heart 2015;2:e000169.  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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