a case of massive aortic thrombosis with catastrophic ... · increased density of the left middle...

IJCRI – International Journal of Case Reports and Images, Vol. 4 No. 6, June 201 3. ISSN – [0976-31 98]

IJCRI 201 3;4(6):294–298.www.ijcasereportsandimages.com

A case of massive aortic thrombosis with catastrophiccerebrovascular embolization and infarctionAleksandra MamorskaDyga, Doru Paul, Ch. Khalil,Ghulam Sajjad Khan

ABSTRACTIntroduction: Antiphospholipid syndrome (APS)is the most common type of acquiredthrombophilia. Lupus anticoagulant (LA) ismore commonly associated with venous thanwith arterial thrombosis and accounts for 6–8%of thrombotic events. Case Report: A 50yearold AfricanAmerican male presented to theemergency department with right sidedhemiparesis, facial droop and impaired speech.First symptoms were noted about 10–12 hoursbefore presentation to the emergencydepartment. During two weeks precedingadmission, patient experienced severaltransient episodes of similar neurologicaldeficits. Computed tomography (CT) scan ofbrain done on admission showed ischemicstroke involving the area of distribution of theleft middle cerebral artery. The CT angiographyof brain revealed a large thrombus in the aorticarch, left common carotid and internal carotidartery. Laboratory studies were positive forlupus anticoagulant. Serial brain CT scandemonstrated increasing brain edema andmidline shift eventually leading to transtentorialherniation. Several days after admission,neurological examination revealed loss of brainstem reflexes. Conclusion: Antiphosphotipidsyndrome (APS) is the most common type ofacquired thrombophilia. Lupus anticoagulant is

mainly associated with venous thrombosis andaccounts for 6–8% of thrombotic events inotherwise healthy patients. Prompt diagnosisand treatment, with longterm anticoagulationfor survivors is crucial. The most common siteof arterial thrombosis in APS is central nervoussystem, presenting with stroke and transientischemic attacks in 50% of the cases. Patientswith diagnosed APS and prior thrombosis havehigh risk for recurrent events. Due to this factsome authors postulate lifelong anticoagulationtherapy.Keywords: Antiphospholipid thrombosissyndrome, Acquired thrombophilia, Aorticthrombosis, Systemic embolization,Cerebrovascular embolization

*********MamorskaDyga A, Paul D, Khalil C, Khan GS. A case ofmassive aortic thrombosis with catastrophiccerebrovascular embolization and infarction.International Journal of Case Reports and Images2013;4(6):294–298.

*********doi:10.5348/ijcri201306316CR1

INTRODUCTIONStroke is one of the leading causes of morbidity andmortality in the United States, with an incidence of over790,000 per year. Eighty seven percent of strokes areischemic and 25% affect patients younger than 65 yearsof age. Before the age of 65 years more women than mensuffer from stroke, however the opposite is true for age65 years and older, as reported by American Heart andAmerican Stroke Association.

CASE REPORT OPEN ACCESS

Aleksandra Mamorska-Dyga1 , Doru Paul1 , Ch. Khali l 1 ,Ghulam Sajjad Khan1

Affi l iations: 1 Lincoln Medical and Mental Health center,Department of Hematology OncologyCorresponding Author: Aleksandra Mamorska-Dyga 234East 1 49th, Bronx, New York, USA, 1 0451 ; Ph: 71 8 5794937; Fax: 71 8 579 5722; Email : amdyga@gmail .com

Received: 1 5 March 201 2Accepted: 04 June 201 2Published: 01 June 201 3

Mamorska-Dyga et al. 294


IJCRI 201 3;4(6):294–298.www.ijcasereportsandimages.com Mamorska-Dyga et al. 295

Antiphospholipid syndrome (APS) is the mostcommon type of acquired thrombophilia, associatedwith venous and/or arterial thrombosis. Left atrial isresponsible for 6–8% of thrombotic events affectingotherwise healthy individuals and is most commonlyassociated with venous thrombosis [1, 2].We present a case of massive aortic thrombosis withsubsequent catastrophic cerebrovascular embolizationand infarction.

CASE REPORTA 50yearold AfricanAmerican male presentedwith right sided weakness, facial droop and impairedspeech. Symptoms started about 10–12 hours beforepresentation to the emergency department and weregradually getting worse. During two weeks precedingadmission patient experienced several episodes ofsimilar symptoms, but less severe, resolvingspontaneously, for which he did not seek medical help.Past medical history was significant for hypertensionand stroke two years before, which resolved with noresidual deficits. Social history revealed active use oftobacco, alcohol and cocaine.On admission, patient had markedly elevated bloodpressure (189/120 mmHg), was tachycardic (120/min)and had increased respiratory rate (20/min).Neurological examination revealed equal and reactive tolight pupils with gaze deviated to the left, globalaphasia, right sided facial droop and right sidedhemiparesis.Computed tomography (CT) scan of brain revealedincreased density of the left middle cerebral artery(MCA) and ischemic stroke involving the area of itsdistribution (Figures 1 and 2). The angiography showeda large thrombus involving the aorta (Figures 3–5),extending to the left common and internal carotidartery and proximal left cerebral artery segments.Initial management did not include systemicthrombolysis due to prolonged time relapsed since thesymptoms were first noted. Patient was also not acandidate for local intervention due to extensiveness ofthe thrombus.Patient was intubated for airway protection andadmitted to the medical intensive care unit for furthermanagement. Treatment was supportive. His hospitalcourse was complicated by aspiration pneumonia,sepsis, acute kidney injury, hemolytic anemia andthrombocytopenia. Laboratory tests were scheduled andrevealed increased Ddimers and low haptoglobin levels(Table 1). Peripheral smear was remarkable for thepresence of schistocytes, which was suggestive ofdisseminated intravascular coagulation, which wasattributed to sepsis. In view of a possible underlyingautoimmune pathology additional blood tests wereadded, among them mixing studies, which were positivefor lupus anticoagulant.Transthoracic echocardiography was ordered anddid not reveal any underlying structural cardiovascularpathology.

Figure 1: Brain computed tomography showing increaseddensity of the left MCA, marked with an arrow.

Figure 2: Brain computed tomography showing effacement ofthe sulci and decreased attenuation in the left parietal lobe,consistent with an early left MCA infarct (arrow pointing tothat area).


IJCRI 201 3;4(6):294–298.www.ijcasereportsandimages.com Mamorska-Dyga et al. 296

Serial CT scans showed worsening demarcation ofthe infarct in the left brain hemisphere, initially withmild mass effect. After several days of hospitalizationneurological examination revealed absence of brainstem reflexes. The CT scan of brain obtained at that timeshowed massive brain edema, midline shift,transtentorial herniation and new brain stem infarct(Figure 6).

DISCUSSIONAntiphospholipid syndrome, is the most commontype of acquired thrombophilia. Within the spectrum ofthe APS, anticardiolipin antibody syndrome is mostcommonly associated with arterial thrombosis, as

Figure 3: Computed tomography angiogram showing a largethrombus in aortic arch marked with an arrow.

Figure 4: Computed tomography angiogram showingthrombosis in the ascending aorta marked with an arrow.

Figure 5: Helical computed tomography angiogram showingfilling defect in the left common carotid artery resulting in itscomplete occlusion. Right common carotid artery marked withan arrow. Left common carotid artery is not visualized (defectmarked with box and arrow).

Figure 6: Brain computed tomography showing acutebrainstem infarction evolving acute infarction in the leftmiddle cerebral artery distribution. Interval progression ofbrain edema and mass effect, subfalcine herniation andmidline shift to the left (arrow).

opposed to left atrial, which is mainly related withvenous thrombosis [3].According to the International Consensus Statementon Preliminary Criteria for the classification of APS(Sapporo Criteria), at least one clinical and at least one



laboratory criteria are required to consider the APSdiagnosis definite. The laboratory criterion is met whenthe positive test is reproduced in at least 12 weeksinterval. Our patient presented with unexplainedthromboembolic event and laboratory studies revealedthe presence of left atrial. However, the latter could notbe repeated due to the fatal outcome.The most common site of arterial thrombosis in APSis central nervous system, with half of the casesresulting in strokes and transient ischemic attacks(TIAs) [3–5]. The major source of arterial systemicemboli is the heart, whereas systemic thrombioriginating in the aorta are much less common. Theoccurrence of the thrombi without underlyingpredisposing conditions such as structural heart diseaseor atherosclerotic plaque would make the case evenmore unusual [6].In our patient, no underlying pathology was found intransthoracic echocardiography. While transesophagealstudy would be a better modality in search for thepathology, in view of his critical condition this test wasnot performed in our patient.According to reviewed literature [1], the presence ofcoexisting mixed aortic, carotid and cerebrovascularthrombosis and/or TIAs (reported in our case prior toadmission) is very uncommon among patients withAPS, contributing to less than 10% cases.The majority of patients developing APS is otherwisehealthy, and is classified to have primary APS.Secondary APS should also be considered, especially inthe context of medication/drug induced scenario,including cocaine use.

Patients with the diagnosis of APS and priorthrombosis are at high risk for recurrent events. Someauthors report, that about 70% of them will have anepisode in 5–6 years, with highest rate during the firstsix months after discontinuation of the therapy [4, 7, 8].Due to that fact lifelong anticoagulation therapy ispostulated.Thrombosis occurring at the level of capillaries,arterioles or venules may result in a clinical picturesimilar to hemolytic uremic syndrome or thromboticthrombocytopenic purpura [3, 7] which could explainsome of the clinical and laboratory findings in ourpatient.Initial management did not include systemicthrombolysis as eligible patients are those presentingwithin three hours of onset of symptoms [9].

CONCLUSIONAntiphospholipid syndrome is the most commontype of acquired thrombophilia. SecondaryAntiphospholipid syndrome should be considered inview of the history of recent cocaine use as a provokingfactor. Left atrial is mainly associated with venousthrombosis. Concomitant arterial, mixed aortic, carotidand cerebrovascular thrombosis represent a very rarephenomenon, which makes presented case unique.The prompt diagnosis and treatment, with longtermanticoagulation given to survivors is crucial, as the rateof recurrent episodes is high and the outcomes may befatal.

*********Author ContributionsAleksandra MamorskaDyga – Conception and design,Analysis and interpretation of data, Drafting the article,Critical revision of the article, Final approval of theversion to be publishedDoru Paul – Conception and design, Acquisition of data,Analysis and interpretation of data, Drafting the article,Critical revision of the article, Final approval of theversion to be publishedCh, Khalil – Conception and design, Acquisition of data,Analysis and interpretation of data, Critical revision ofthe article, Final approval of the version to be publishedGhulam Sajjad Khan – Acquisition of data, Analysis andinterpretation of data, Critical revision of the article,Final approval of the version to be publishedGuarantorThe corresponding author is the guarantor ofsubmission.Conflict of InterestAuthors declare no conflict of interest.Copyright© Aleksandra MamorskaDyga et al. 2013; This articleis distributed under the terms of Creative Commons

Test Results NormalHgb 13.4–9.2 13.5–17.5 mg/dLPLT 243–91 150–350x105/mm5TP/aPTT 11.4/35.8 11–13/25–35 secHPF4, SRA Negative NegativeLDH 1839 11–190 U/LHaptoglobin <10–50 30–200 mg/dLDdimers >5000 <500 mg/dLFibrinogen activity 557 150–400 mg/dLCD 55, CD 59 Negative NegativeG6PD 5.9 5–13 U/g HbRF Negative NegativeANA titer Positive, speckled <1:160patternAPL panel + inhibitor inmixing studies

Table 1: Laboratory results of the patient.


ABBREVIATIONSHb; Hemoglobin, PLT; Platelet, PT; Prothrombin time, aPTT;Activated Partial 120 Thromboplastin Time, INR;International Normalized Ratio, HPF4, SRA; HeparinPlatelet Factor 4, Serotonin Release Assay, LDH; LactateDehydrogenase, Fibgn; Fibrinogen, G6PD; Glucose 6phosphate dehydrogenase, RF; Rheumatoid factor, ANA;Antinuclear Antibody, APL; Antiphospholipid.



Attribution 3.0 License which permits unrestricted use,distribution and reproduction in any means providedthe original authors and original publisher are properlycredited. (Please see www.ijcasereportsandimages.com/copyrightpolicy.php for more information.)

REFERENCES1. Rodger L Bick. Antiphospholipid ThrombosisSyndromes. Clin Appl Thrombosis Hemostasis2001;7(4):241–58.2. Monica Galli. Antiphospholipid antibodies andthrombosis: do test patients identify the patient’srisk? Thrombosis Research 2004;114(56):597–601.3. Bick RL, Baker WF. Antiphospholipid syndrome andthrombosis. Semin Thromb Hemost1999;25(3);333–50.4. Ware Branch D, Eller AG. AntiphospholipidSyndrome and Thrombosis. Clinical Obstetrics andGynecology 2006;49(4):861–74.5. Rosove MH, Brewer PM. Antiphospholipidthrombosis: clinical course after first thromboticevent in 70 patients. Ann Intern Med1992;117(4):303–8.6. Song IW, Hong GR, Cho JH, et al. A case of hugethrombus in the aortic arch with the cerebrovascularembolization. J Cardiovascular Ultrasound2009;17(4):148–50.7. Khamashta MA, Cuadraro MJ, Mujic F, Taub NA,Hunnt BJ, Hughes GR. The management ofantiphospholipidantibody syndrome. N Eng J Med1995;332(15);993–7.8. Levine JS, Branch DW, Rauch J. TheAntiphospholipid Syndrome. N Engl J Med2002;346(10):752–63.9. Panetta T, Thompson JE, Talkington CM, GarrettWV, Smith BL. Arterial embolectomy: a 34yearexperience with 400 cases. Surg Clin North Am1986;66(2):339–53.


Access full text article onother devices Access PDF of article onother devices

a case of massive aortic thrombosis with catastrophic ... · increased density of the left middle...

Documents