Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 3  |  Page : 277-279

Multiple penetrating descending thoracic aortic ulcers and intramural hematoma with a right massive hemothorax in a young male


1 Department of Cardiothoracic Surgery, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
2 Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India

Date of Submission16-Aug-2020
Date of Acceptance31-Aug-2020
Date of Web Publication6-Jul-2021

Correspondence Address:
Sreekanth Yerram
Department of Cardiology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijves.ijves_119_20

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  Abstract 


Penetrating aortic ulcer and intramural hematoma is one of the causes of acute aortic syndromes and can present as an emergency. It is common in older individuals with multiple risk factors and rarely presents as hemothorax on the right side. We present a young male without significant risk factors who presented acutely with the right massive hemothorax. On evaluation, he had multiple ulcers in the descending thoracic aorta, which was successfully managed with a stent graft. This case illustrates the rarity of presentation at a young age and rarity of manifestation with the right hemothorax.

Keywords: Acute aortic syndrome, aortic stent graft, hemothorax, intramural hematoma, penetrating aortic ulcer


How to cite this article:
Palanki SS, Yerram S, Bhyravavajhala S, Malempati AR. Multiple penetrating descending thoracic aortic ulcers and intramural hematoma with a right massive hemothorax in a young male. Indian J Vasc Endovasc Surg 2021;8:277-9

How to cite this URL:
Palanki SS, Yerram S, Bhyravavajhala S, Malempati AR. Multiple penetrating descending thoracic aortic ulcers and intramural hematoma with a right massive hemothorax in a young male. Indian J Vasc Endovasc Surg [serial online] 2021 [cited 2021 Jul 25];8:277-9. Available from: https://www.indjvascsurg.org/text.asp?2021/8/3/277/320615




  Introduction Top


Acute aortic syndromes (AAS) encompass multiple entities, including aortic aneurysm, intramural hematoma (IMH), penetrating atherosclerotic ulcer (PAU), and aortic dissection and share some commonality in etiopathogenesis. PAU is formed when an ulcerating atheromatous plaque extends through internal elastic lamina into aortic media[1] and can be associated with IMH which is clotted blood in the aortic wall presumably due to ruptured vasa vasorum of the aorta. PAU accounts for 2%–7% of AAS[2] and may complicate to form IMH, saccular pseudoaneurysm, or rupture. They usually present in older individuals with multiple risk factors.[1] We report a patient who was a young male without any risk factors who presented with the right massive hemothorax, which is a rare complication,[3] and only a single such patient has been described in the literature.


  Case Report Top


A 32-year-old man presented to us with shortness of breath and vague abdominal pain of 1-week duration. He had diminished breath sounds on the right side and was hemodynamically stable at presentation. His hemoglobin was 11.8 mg/dl, renal, hepatic, and coagulation parameters were normal. On echocardiography and color Doppler study, he had an ejection fraction of 65%, no aortic regurgitation, and no regional wall motion abnormality. Computerized tomography (CT) revealed a nonenhancing false lumen from just beyond the isthmus of the descending thoracic aorta suggestive of an IMH. There were multiple craters in the intima with contrast filling them suggestive of penetrating ulcers along the length of the descending thoracic aorta [Figure 1]. There was gross hemothorax on the right side of the chest, and communication between the ulcer and the thoracic cavity could be demonstrated [Figure 2]. There were minor splenic and left renal infarcts, probably due to distal embolization from the aortic thrombus, but a thrombus was not demonstrated. He was negative for antineutrophilic antibody, lupus anticoagulant, anticardiolipin antibodies, and Factor V Leiden genotype were normal. Homocysteine was elevated, and the lipid profile was normal.
Figure 1: Computed tomography aortography in the coronal section showing non opacified thickening of the wall of the descending thoracic aorta and multiple craters with a filling of the contrast suggestive of penetrating aortic ulcers. The blood in the right side of the chest suggestive of massive hemothorax

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Figure 2: Computed tomography, axial section illustrating one of the ulcers extending into the media with contrast filling it and hemothorax on the right side of the chest

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As the patient had a diffuse disease of the descending aorta with a massive hemothorax, the endovascular repair was considered. The procedure was done under general anesthesia, with endotracheal intubation and mechanical ventilation. Intraoperative pressure monitoring was done through the right radial artery. A 6 French sheath was inserted in the left femoral artery, and the right femoral artery was exposed. An aortogram was done with a marker pigtail catheter from the left femoral artery to identify the proximal and distal landing zones and the extent of the disease process. Under fluoroscopic guidance, Ankura (Lifetech, Shenzhen, China) aortic stent graft system of size 26 mm × 26 mm × 160 mm (21 F) was advanced over a super stiff guidewire. Then the stent graft was deployed, and the final arteriogram was done to confirm the position of the stent graft, and the presence of endoleaks. Then, the sheath was removed, and arteriotomy was repaired with 6-0 Prolene suture. The postoperative period was uneventful, a CT scan after a few days revealing a well-positioned aortic stent with complete resolution of the right-sided hemothorax. The patient is asymptomatic, with a normal CT scan after 1 year [Figure 3]a and [Figure 3]b.
Figure 3: (a) Chest X-ray frontal projection showing the aortic stent graft and complete resolution of hemothorax. (b) Computed tomography 3D reconstruction showing the aortic stent graft and good filling of the branches of the aorta

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  Discussion Top


Penetrating aortic ulcers and IMH can present as an AAS with a complication which may sometimes exist together or may occur independently. PAU and IMH variants presenting in the ascending aorta need urgent surgery, whereas those in the Stanford type B distribution may be managed medically if asymptomatic. They tend to rupture and need treatment in patients with symptoms and high-risk features.[4] Endovascular stenting or surgery are considered if symptomatic, progressive disease or complications arise.[5] Repair of PAU with endovascular stent grafts have been reported previously and is used more often as the procedure is less invasive.[6] Surgical treatment of this complicated penetrating aortic ulcer carries a high risk of morbidity and mortality. Hence, endovascular stent grafting was considered in this case as it is easier and avoids the respiratory complications of thoracotomy.

This lesion can be easily missed.[7] PAU on CT angiography is diagnosed by the presence of focal outpouching of contrast through the intima extending into the media of the aorta.[4] With the increased use of imaging modalities, PAU and IMH are found incidentally in asymptomatic patients undergoing evaluation for other conditions. The natural history of such asymptomatic patients is not clear. In our patient, we doubted if the ulcer was responsible for the hemothorax. All other causes were ruled out, and on careful observation, on CT, we could see the communication between one of the ulcers and the thorax.

Hemothorax is a common manifestation with aneurysm and dissection, but penetrating ulcers causing hemothorax on the right side is rare, and only a single case has been documented in the literature.[3] These lesions present like aortic dissection; they can cause a leak into the pleural or pericardial cavity. However, leakage of blood into only the right side of the thorax is very uncommon. Complete resolution after aortic stenting without additional thoracic drainage further supports this assertion. As the patient was not dyspneic and was presumed to tolerate the anesthesia well, thoracic drainage was not done before the procedure.

Penetrating ulcers and IMH are usually described in old age in patients with multiple risk factors for atherosclerosis.[8] Our patient was a young male without any risk factors, which is atypical. Many genetic disorders are associated with aortic aneurysm and dissection, but he did not have any clinical manifestations of genetic or connective tissue disorders, even though he did not undergo genetic testing. Other possible etiologies such as syphilis can present as penetrating aortic ulcers,[9] which were ruled out by antibody testing. This patient highlights the uncommon presentation of this entity in terms of age, etiology, and clinical features.


  Conclusion Top


IMH and penetrating aortic ulcers are a rare cause of AAS in younger people. Hemothorax, particularly on the right side, is an uncommon manifestation.

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.

Acknowledgment

Dr. Sandeep Mahapatra, Associate Professor, Department of Vascular surgery, Nizam's Institute of Medical Sciences.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Stanson AW, Kazmier FJ, Hollier LH, Edwards WD, Pairolero PC, Sheedy PF, et al. Penetrating atherosclerotic ulcers of the thoracic aorta: Natural history and clinicopathologic correlations. Ann Vasc Surg 1986;1:15-23.  Back to cited text no. 1
    
2.
Vilacosta I, San Román JA, Aragoncillo P, Ferreirés J, Mendez R, Graupner C, et al. Penetrating atherosclerotic aortic ulcer: Documentation by transesophageal echocardiography. J Am Coll Cardiol 1998;32:83-9.  Back to cited text no. 2
    
3.
Siegel Y. Penetrating atherosclerotic aortic ulcer rupture causing a right hemothorax; a rare presentation of acute aortic syndrome. Am J Emerg Med 2013;31:755.e5-7.  Back to cited text no. 3
    
4.
Nathan DP, Boonn W, Lai E, Wang GJ, Desai N, Woo EY, et al. Presentation, complications, and natural history of penetrating atherosclerotic ulcer disease. J Vasc Surg 2012;55:10-5.  Back to cited text no. 4
    
5.
Oderich GS, Kärkkäinen JM, Reed NR, Tenorio ER, Sandri GA. Penetrating Aortic Ulcer and Intramural Hematoma. Cardiovasc Intervent Radiol 2019;42:321-34.  Back to cited text no. 5
    
6.
Eggebrecht H, Plicht B, Kahlert P, Erbel R. Intramural hematoma and penetrating ulcers: Indications to endovascular treatment. Eur J Vasc Endovasc Surg 2009;38:659-65.  Back to cited text no. 6
    
7.
Chua M, Ibrahim I, Neo X, Sorokin V, Shen L, Ooi SB. Acute aortic dissection in the ED: Risk factors and predictors for missed diagnosis. Am J Emerg Med 2012;30:1622-6.  Back to cited text no. 7
    
8.
Cho KR, Stanson AW, Potter DD, Cherry KJ, Schaff HV, Sundt TM, et al. Penetrating atherosclerotic ulcer of the descending thoracic aorta and arch. J Thorac Cardiovasc Surg 2004;127:1393-401.  Back to cited text no. 8
    
9.
Byer M, Celli D, Colombo R. Cardiovascular syphilis presenting as penetrating aortic ulcers. J Am Coll Cardiol 2020;75:2595.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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