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Case Abstracts

Cerebral Hypoperfusion as a Cause of Ischemic Stroke in a 46 Year-Old Female with Stanford Type A Acute Aortic Dissection: A Case Report
A Rare Case of Lower Limb Arterial Thrombosis in Antiphospholipid Syndrome
Piriformis Inflammation as a Cause of Deep Venous Thrombosis
Aortic Dissection in a Filipino patient with Human Immunodeficiency Virus: A Case Report
A Rare and Fatal Case of Aortobronchial Fistula Secondary to Disseminated Tuberculous Aortitis and Takayasu Arteritis
A Rare Case of Single Coronary Artery and Concomitant Aberrant Right Subclavian Artery in a Patient with Bicuspid Aortic Valve Complicated with Painless Aortic Dissection
Close Call....Stent-assisted Retrieval of Extruded Embolic Agent During Transcaval Endoleak Embolization
Pathology is Still the Gold Standard - Unusual Case of Multiple Visceral Artery Aneurysms
Flow-induced Petechiae During Cardiac Surgery in a Patient with a De Novo Fibrillin-1 Gene Mutation
Takayasu’s Arteritis with Giant Left Ventricular Pseudoaneurysm Presenting as Heart Failure in a 36-year-old female: A Case Report and Literature Review
Noninvasive Diagnosis of Arterial Impairment in Phlegmasia Alba Dolens
Congenital Subclavian Steal Syndrome
Drug Coated Balloon Angioplasty in Subclavian Artery Stenosis Adjacent to Vertebral Artery
Rare Venous Compression Syndrome
A Rare Case of Penile Raynaud’s Phenomenon due to Giant Cell Arteritis
Use of Thrombolysis in Conjunction with Bivalirudin in Sub-massive Pulmonary Embolism in a Patient with Severe Thrombocytopenia Resulting from HIT
Pseudoxanthoma Elasticum Presenting with Advanced Atherosclerosis
A Rare Cause of Lower Extremity Hyperpigmentation
Beaded Arteries: Consider Fibromuscular Dysplasia Mimickers
Improvement in Vasomotor Function does not Correlate with Reduced Pain in CRPS
Pylephlebitis in a 58 Year-Old Male with Fever: A Case Report
Mesenteric Artery Dissection and/or Inflammation
A Rare Case of Eagle Syndrome Presenting with Vascular Symptoms

Cerebral Hypoperfusion as a Cause of Ischemic Stroke in a 46 Year-Old Female with Stanford Type A Acute Aortic Dissection: A Case Report
Katherine Rose Acosta

Description of background: We report a case of a 46 year-old hypertensive female, current 7-pack year cigarette smoker with sudden, severe retrosternal chest pain associated with vague, intermittent epigastric pain as the initial presentation of Stanford type A acute aortic dissection (AAD), who developed decrease in sensorium and verbal output and focal extremity weakness few hours into optimal BP and heart rate (HR) control as a manifestation of ischemic stroke probably secondary to cerebral hypoperfusion.

Type A AAD is a life-threatening disease that requires urgent surgery and medical management directed at decreasing the aortic wall stress by controlling systolic BP (SBP) to 100-120 mmHg and HR to 60 bpm or less.
Approximately 6% of elderly male patients with type A AAD have been reported to have concomitant ischemic stroke due to extension of dissection to the cervicocerebral arteries or emboli from the site of dissection. Few studies state the lack of clear evidence of cerebral hypoperfusion as a cause of ischemic stroke in patients with type A AAD. One study involving type B AAD proposed that a higher mean arterial pressure (MAP) on presentation and greater decline in MAP, as what happened to our patient, are associated risk factors.

Methods: Cranial MRI/ MRA of our patient revealed recent infarcts in the watershed distribution with normal carotid and intracranial vessels, without involvement of the arch vessel.

Results: She was successfully managed with beta-blockers and other antihypertensive medications maintaining SBP between 120-140 mmHg following the stroke, from a previous target of 100 mmHg, and HR of 60 bpm. Her neurologic and cardiac status improved after two weeks.

Conclusion: While strict BP and HR control is the cornerstone in the medical management of AAD, caution should be observed in lowering the MAP to prevent debilitating sequelae, such as ischemic stroke due to cerebral hypoperfusion.

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A Rare Case of Lower Limb Arterial Thrombosis in Antiphospholipid Syndrome
Rosario Soledad Neri

Description of Background: CAPS is an aggressive form of antiphospholipid syndrome causing multi-organ thrombosis and failure. A treatment approach has been adopted which consisted of anticoagulation (AC), glucocorticoids (GC), plasma exchange (PEX) and/or intravenous immunoglobulins (IG). CAPS mortality rates remain high despite this approach. Several reports have demonstrated the primary and salvage activity of Rituximab (RTX) and Eculizumab (ECL). We conducted this analysis to provide an updated description of the impact of current treatment approaches on the CAPS outcomes.

Methods: We compiled a pooled CAPS database. Frequency tables, Chi-squared tests, and Fisher's exact tests were used to assess the effect of therapeutic approaches on the clinical outcomes.

Results: Our database consisted of 453 cases. Mortality rates for the following combinations were: 37% for ACGC, 20% for ACGCPEX, 27% for ACGCIG, 31% for ACGCPEXIG, 17% for ACGCPEXIGRTX, and 20% for ACGCPEXIGRTXECL. Mortality of all RTX-based combinations was 17% whereas that of ECL-based combinations was 6%. Compared to ACGC, ACGCPEX, RTX-based, and ECL-based combinations had lower mortality. When compared to ACGCPEX+/-IG, ECL-based combinations demonstrated significant lower mortality unlike RTX-based combinations. When mortality was analyzed separately by whether or not the combinations included PEX, IG, or GC, only those that included PEX had significant lower mortality as long as RTX and/or ECL were not included.

Conclusion: Early use of ECL and RTX improves CAPS mortality. We recommend a new CAPS frontline treatment approach consisting of ACGCECL+/-RTX+ therapies targeting the underlying medical condition.

Learning Objective: Highly active agents should be used upfront in CAPS to significantly lower mortality and morbidity.

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Piriformis Inflammation as a Cause of Deep Venous Thrombosis
MerryBarnett

Description of background: Hypercoagulability, stasis, and endothelial injury are well-documented contributing factors to development of deep venous thrombosis (DVT). With regards to stasis, anatomic abnormalities that cause venous compression are often what predisposes patients to the development of iliofemoral DVT. The most commonly described anatomic abnormality is May-Thurner Syndrome: the left iliac vein is compressed between the right common iliac artery and the lumbar vertebrae.  Other examples of venous compression are more rare, but can occur in the right circumstances.

Methods: This case report describes a 20-year-old athletic female who developed signs and symptoms suggestive of piriformis syndrome, but continued to run regularly and ultimately presented with an acute right-sided iliofemoral DVT.

Results: The patient was treated with catheter directed thrombolysis. Follow up CT venogram at 6 weeks revealed complete recanalization of right common iliac without residual thrombus, normalization of the piriformis muscle, and chronic occlusion of the internal iliac vein and it’s branches.

Conclusion: Regular distance running can cause repetitive microtrauma of the piriformis muscle, leading to inflammation, edema, and compression of the associated blood vessels and nerves. Hence, an inflamed piriformis can cause an iliofemoral DVT.

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Aortic Dissection in a Filipino patient with Human Immunodeficiency Virus: A Case Report
Armand Delo Tan

Description of background: The Philippines currently has the fastest growing number of Human Immunodeficiency Virus (HIV) cases worldwide. Patients with HIV have become a demographic of concern in health care, including cardiovascular health. Aortic pathologies are just one of the many varied presentations of cardiovascular problems in patients with HIV. While Aortic Aneurysm has been discussed frequently in the literature, very few reports have presented cases of Aortic Dissection among patients with HIV. There has been no local published data on this subject matter.

Methods: We report a case of a 37-year old male with HIV infection and Stanford A, DeBakey 1 Aortic Dissection. The patient presented with dyspnea, easy fatigability, and bipedal edema. Computed Tomographic Aortogram revealed aortic dissection from the level of the sinus of Valsalva to the aortic arch. The patient acquired several morbidities preoperatively including pneumonia, acute kidney injury, and hepatic failure. Once he recovered, he underwent Modified Bentall’s surgery. Histopathologic report revealed an aneurysmal aortic wall with chronic dissection and moderate medial degeneration. The patient was stable upon the time of writing.

Results: Our patient displayed several similarities to the 3 other cases of aortic dissection in HIV reported in the literature. He was a male with hypertension and chronic kidney disease. The distribution of his dissection was similar in classification to the previous studies. Description of aortic pathology among patients with HIV is based on studies of aortic aneurysms in this population. Pathology was associated with atherosclerosis, vasculitis, or infections. Previous case reports on aortic dissections have not discussed the histopathology of the specimen lesions. Criteria to consider for surgical intervention include a high CD4 count, absence of AIDS, good nutrition, and good functional status. These were all present in our patient and solidified our decision for surgery.

Conclusion: As the number and lifespan of patients with HIV increases, it is important to understand the various non-AIDS related health issues these patients deal with. In the field of cardiology, more research is needed to help us characterize the pathophysiologic mechanisms of aortic dissection in patients with HIV. Doing so will help us develop better management plans and goals for these patients.

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A Rare and Fatal Case of Aortobronchial Fistula Secondary to Disseminated Tuberculous Aortitis and Takayasu Arteritis
Bryan Rene Toledano

Description of background: Aortobronchial fistula (ABF) is a rare complication of a chronic thoracic aneurysm. It occurs due to longstanding inflammation with subsequent erosion and connection between the aorta and pulmonary parenchyma or bronchial tree. The reported etiologies are atherosclerosis and prior aortic surgical intervention in the elderly while infectious or inflammatory disease in the young.

Methods: We report a case of a  29-year-old Filipino female, married, who was admitted to our institution due to massive hemoptysis. Computed tomography of the chest and aorta, showed the following findings pulmonary tuberculosis (TB) with lymph node involvement, aneurysms of the mid to distal descending thoracic aorta, distal abdominal aorta, superior mesenteric artery and stenoses of the subclavian and left renal arteries hence she was referred to at our institution.

Results: The pertinent physical examinations are Blood pressure differential of 20mmhg of upper extremities, painless multiple nodular tongue, multiple cervical, and supraclavicular lymphadenopathies. She was immediately started on anti-TB medications, corticosteroids and was scheduled for aortic aneurysmal repair. However, there was a recurrence of massive hemoptysis leading to her demise. An autopsy of the thorax and abdomen revealed an Acute pulmonary hemorrhage, Aortobronchial fistula secondary to Disseminated (TB) Aortitis and Takayasu Arteritis (TA).

Conclusion: A masked ABF should be suspected in a patient with massive hemoptysis and a pseudoaneurysm of the descending aorta. In a young female with disseminated TB aortitis, a concomitant TA should be investigated. Both can share the same clinical, radiological and pathologic features. It is fatal and needs prompt surgical or endovascular intervention.

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A Rare Case of Single Coronary Artery and Concomitant Aberrant Right Subclavian Artery in a Patient with Bicuspid Aortic Valve Complicated with Painless Aortic Dissection
Nestor Subong

Description of background: A case of 67 year old female, hypertensive, diabetic female presented with bizaare symptoms of easy fatigability and body weakness with associated shortness of breath.  Three years prior to consult, patient started to complain of intermittent chest discomfort and was found to have widened mediastinum on chest radiograph. A 2 D echochardiogram was requested however patient was unable to comply and then lost to follow up.  Interval history revealed sporadic symptoms of chest pain but no subsequent consult was done until the patient suddenly experienced unexplained generalized body weakness, headache and shortness of breath. Physical examination revealed unappreciable blood pressure, tachycardia, thready pulses with distended neck veins and cold clammy extremities. Work up revealed persistent widened mediastinum and cardiomegaly on chest radiograph. 2 D echocardiogram showed a large hemopericardium compressing the right ventricle. CT aortogram revealed ascending thoracic aneurysm with dissection from the aortic root extending to the ascending aorta until before the origin of an apparent aberrant right subclavian artery which coursing behind the esophagus and trachea. A single coronary ostium was also noted bifurcating into two separate vessels. Patient was immediately subjected for emergency surgery replacing the aortic root and aortic valve.  Intraoperatively, the aortic valve was to be bicuspal in morphology and only one coronary ostium was indeed appreciated. Patient tolerated the procedure well.

Methods: N/A

Results: N/A

Conclusion: The rare coexistence of these clinical entities has not been reported yet in literature. Hence we report the probable first case of painless acute thoracic aortic dissection in a patient with bicuspid aortic valve, aberrant right subclavian artery and single coronary ostium.

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Close Call....Stent-assisted Retrieval of Extruded Embolic Agent During Transcaval Endoleak Embolization
Minhaj Khaja

Description of background: Endoleaks are a common complication following endovascular aortic repair of aneurysm, especially in the abdominal aorta. Traditional treatment methods include embolization via trans-arterial or direct aneurysm sac puncture. Recently, transcaval access to the aneurysm sac has been employed, usually without many complications and with reasonable success in the appropriate patient.

Methods: A 70-year-old male with an EVAR procedure in 2002 presented with a type 2 endoleak 17 years later. An initial attempt at trans-arterial endoleak embolization failed after multiple attempts. The patient was referred to an interventional radiologist for direct sac or transcaval attempt at endoleak embolization.  Transcaval access into the juxtcaval endoleak sac was obtained for embolization. Access was obtained using a BRK trans-septal needle from right common femoral vein (CFV) access through an 8 Fr sheath, under intravascular ultrasound (IVUS) guidance. Angiography confirmed needle access into the nidus with lumbar artery outflow. Through the sheath, a 4 Fr catheter and microcatheter were advanced. Two vials of ethylene vinyl alcohol-18 (Onyx) were injected into the nidus. Upon completion of the embolization, the sheath was gently advanced while the catheters were removed. Immediately, Onyx was noted to extrude from the aneurysm sac into the IVC with the potential to cause a large pulmonary embolism.

Results: The patient was immediately heparinized to a therapeutic ACT. Suction was placed on the sheath using a 20-mL syringe in order to stabilize the caval Onyx. A 14 Fr x 30 cm sheath was advanced via right IJ access. A 24x70 mm Wallstent was advanced and partially deployed rostral to the Onyx. The CFV sheath with Onyx cast was advanced into Wallstent and both were oversheathed. The Onyx cast and Wallstent were removed successfully with no residual onyx or distal embolization. The patient was discharged the following morning and is without endoleak recurrence at 6 months. The authors believe the Onyx tracked back along the catheter and while advancing the sheath to account for this, it may have enlarged the hole in the aneurysm sac.

Conclusion: Foreign body retrieval may be an unfortunate complication associate with many procedures, including endoleak embolizations. It is imperative for the operator to be aware of such complications and to be able to manage them in a safe and quick manner in order to avoid a devastating consequence for the patient.

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Pathology is Still the Gold Standard - Unusual Case of Multiple Visceral Artery Aneurysms
Stanislav Henkin

Description of background: Visceral artery aneurysms are rare, but can be life-threatening due to risk of rupture. They are associated with multiple conditions, including infection, fibromuscular dysplasia, or arteritis. We present an unusual case of multiple visceral artery aneurysms in a young woman.

Methods: N/A

Results: A 26 year old woman presented with abdominal pain. She had no significant past medical history. Computed tomographic imaging detected multiple large visceral artery aneurysms. She underwent endovascular embolization of splenic and celiac artery aneurysms and open resection of a SMA branch aneurysm. Comprehensive laboratory and genetic evaluation were unremarkable. The pathology of the specimen demonstrated medial lympho-histocytic inflammation with rare giant cells, concerning for arteritis. She was lost to follow-up for 6 years, returning to vascular clinic prior to elective termination of pregnancy. Repeat CT showed multiple new SMA and IMA branch aneurysms without evidence of aneurysms, dissections, or beading elsewhere. PET-CT showed no active inflammation. After multidisciplinary discussion, she underwent exploratory laparotomy with resection of these aneurysms. Pathology showed active inflammation with histiocytes and giant cells within media of vessels, compatible with Takayasu arteritis. The patient was started on methotrexate. She will undergo regular imaging to monitor for new aneurysms.

Conclusion: Takayasu arteritis most commonly occurs in young women and classically affects large arteries, causing luminal narrowing or occlusion. Histopathology is not readily available in most cases. We present a case of a young woman with multiple visceral artery aneurysms where pathology was key in making the diagnosis.

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Flow-induced Petechiae During Cardiac Surgery in a Patient with a De Novo Fibrillin-1 Gene Mutation
Sae Jang

Description of background: A 24-year-old male with a dilated ascending aorta and de novo 15(del)q15.3-23.2 fibrillin-1 gene mutation underwent elective Bentall procedure. Intra-operatively, he developed diffuse head-and-neck petechiae.

Methods: During the distal anastomosis of the ascending aorta, retrograde cerebral perfusion was started via the snared superior vena cava cannula with cold oxygenated blood at a standard flow rate of 300 mL/min and central venous pressure of less than 20 mmHg.

Results: Before weaning off cardiopulmonary bypass, bilateral diffuse petechiae appeared on his face and neck. His extremities and trunk were spared. There were small conjunctival petechiae.

His pre-operative laboratory tests were within normal limits.  His intra-operative laboratory tests were hemoglobin 6.9, platelets 68, INR 1.6, fibrinogen 145, aPTT 42s. Peripheral smear did not show schistocytes. He received a unit of pRBCs, a unit of platelets, two units of FFP, a unit of cryoprecipitate, and several cell-saver auto-transfusions with normalization of laboratory tests. His petechial rash did not progress post-operatively.

Conclusion: Patient has a de novo fibrillin-1 mutation resulting in aortopathy similar to Marfan's. This is the first report of diffuse head-and-neck petechiae from aortic surgery. We hypothesize that the increased venous pressure during retrograde cerebral perfusion, combined with vascular fragility from his fibrillin-1 mutation  triggered capillary damage -- in a similar mechanism to facial and peri-orbital bruising seen in amyloidosis due to periorbital vascular infiltration by amyloid fibrils. In amyloidosis, mild stress such as increasing venous pressure with coughing or bearing down can damage the fragile capillaries resulting in petechial rash.

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Takayasu’s Arteritis with Giant Left Ventricular Pseudoaneurysm Presenting as Heart Failure in a 36-year-old female: A Case Report and Literature Review
Frederick Rivera

Description of background: Takayasu's arteritis (TA) is a rare chronic granulomatous vasculitis with a predilection for young females. It primarily affects the aorta and major branches. Left ventricular pseudoaneurysm (PSA)  in the setting of TA is an extremely rare phenomenon.

Methods: We report a 36-years old Filipina who presented with heart failure symptoms. Years prior, she had recurrent fever, headache, myalgia and left arm claudication. An “error” reading on the digital BP apparatus was noted when it was used on her left arm. On work up, a transthoracic echocardiogram revealed a left ventricular PSA with mural thrombus and moderate mitral regurgitation. Cardiac MRI further characterized the PSA arising from the basal to mid anterolateral wall with a sac diameter of 8 x 7.5 x 8.4 cm (CCxTxAP). A nearly transmural late gadolinium enhancement (LGE) was also noted at the base of the PSA neck that may represent fibrosis or inflammation. Carotid Duplex Scan revealed total occlusion of the mid to distal right common carotid artery and left subclavian artery as well as an increased intima-media thickness of the left common carotid artery suggestive of active inflammation. Near total occlusion of both ulnar arteries and stenosis of the left vertebral artery was demonstrated on arterial duplex scan. Coronary angiogram was normal.

Results: She was started on heart failure medications and anticoagulation. Because the TA was active, steroid therapy was initiated. After a month of treatment, she underwent successful endoventricular patch closure and mitral valve repair.

Conclusion: This case highlights the importance of actively searching for cardiac complications of TA which albeit very rare, can dominate the clinical picture and may carry a dismal prognosis if left untreated.

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Noninvasive Diagnosis of Arterial Impairment in Phlegmasia Alba Dolens
Robert Scissons

Description of background: Phlegmasia alba dolens (ALBA) is a rare, limb-threating condition.  A serious sequela of this condition is impaired arterial flow caused by compartment syndrome yet noninvasive data documenting this effect is scarce.  We present the noninvasive evaluation of a 58-year-old female with limb pain, swelling, milky skin discoloration and blue toes.

Methods: This uterine cancer patient with recent pulmonary embolus was referred from the emergency room for right lower extremity venous duplex.  Duplex and clinical findings prompted arterial waveform analysis and ankle-brachial index (ABI) followed by venography.

Results: Venous duplex findings included limited visualization of the inferior vena cava and iliac veins with right femoropopliteal, profunda, and deep calf DVT; great and small saphenous veins were patent and compressible.  Waveform analysis of the common femoral artery (CFA) displayed abnormal systolic deceleration.  Posterior tibial (PT) and dorsalis pedal (DP) artery waveforms had transient systolic acceleration with prolonged flow reversal.  Antegrade and retrograde blood flow volume (Q) in the CFA was 1131 and 182 ml/min, PT 63 and 37 ml/min, and DP 3.5 and 1.4 ml/min, respectively; PT and DP ABI were 0.59 and 0.52 and 1.38 and 1.36 in the contralateral extremity, respectively. Venography demonstrated right iliofemoral and femoropopliteal DVT with right iliac vein tapering.  Tissue plasminogen activator (TPA) was introduced at 1mg/ hour with iliofemoral balloon angioplasty.  Post-TPA Q was antegrade without flow reversal: CFA 94, PT 35 and DP 9.2 ml/min; PT and DP ABI were 0.71 and 0.73 respectively.

Conclusion: ALBA represents a small percentage of acute DVT patients.  Arterial impairment is a severe limb-threatening progression of this condition.  In addition to venous duplex imaging, we believe arterial waveform analysis, Q and ABI data may offer supportive data for monitoring and management of phlegmasia.

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Congenital Subclavian Steal Syndrome
Alexandra Moran

Description of background: Congenital arterial anatomic variants can remain clinically silent until physical demands in adulthood cause symptoms.

Case: A 27-year-old man without significant past medical history presented with left arm pain, syncope and dizziness with exertion. He is active in the military. During a drill he began to notice left arm pain with exercise which progressed into left arm paresthesia. Two months later, with the acquisition of an aggressive drill sergeant, his work load increased. He was carrying a heavy object and reported severe chest pain and right arm pain which was followed by dizziness. He subsequently had a syncopal episode. Physical exam reveals upper extremity blood pressure discrepancy. Upper extremity brachial and radial artery pulses were 1+ on the left and 2+ on the right. Chest magnetic resonance angiogram demonstrated a right sided aortic arch (Figure; arrow heads), an absent left subclavian artery aortic origin (Figure; bracket), and a subclavian artery that arises from the left vertebral artery (Figure). Carotid ultrasonography showed retrograde flow from the left vertebral to the left subclavian artery.

Decision Making: This patient presented with worsening exertional symptoms with a physically demanding occupation. Work up showed subclavian steal. Given his dramatic presentation, he was referred for carotid to subclavian bypass grafting. One month following surgery, he denies recurrence of symptoms with plans to return to active military duty.

Conclusion: This case demonstrates the importance of a thorough vascular exam, the potential for congenital vascular anomalies to present in adulthood, and the role for surgery to correct vascular anomalies when symptoms are severe.

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Drug Coated Balloon Angioplasty in Subclavian Artery Stenosis Adjacent to Vertebral Artery
William Lang

Description of background: Percutaneous transluminal angioplasty (PTA) with stenting is an accepted modality for the treatment of symptomatic subclavian artery stenosis. Lesions with proximity to the vertebral artery or a left internal mammary artery graft pose a particular challenge, as stent ‘jailing’ may result in cerebrovascular or coronary ischemia. We report a case with stand alone drug coated balloon angioplasty.

Methods: n/a

Results: A 65-year old gentleman with a history of atrial fibrillation, stroke and tobacco abuse presented with progressive left arm claudication. Exam revealed severely diminished left brachial and radial arterial pulses. Carotid duplex revealed a focal left subclavian stenosis with peak systolic velocity of 520 cm/s and evidence of vertebral steal phenomenon. Angiography revealed a high grade focal left subclavian stenosis with minimal calcium adjacent to the vertebral artery. Percutaneous intervention was performed with a 5mm x 20mm x 135cm Balloon followed by drug coated balloon angioplasty with a 7.0mm x 40mm balloon. Post-intervention angiography revealed brisk antegrade flow. The patient was discharged on the same day and completed 1 month of DAPT. At 2-year follow-up, the patient remained asymptomatic and follow up carotid duplex revealed antegrade flow in the vertebral artery and triphasic left brachial artery Doppler waveforms.

Conclusion: In this case of left subclavian artery stenosis adjacent to the vertebral artery we obtained satisfactory intermediate-term results following drug coated balloon angioplasty alone. Use of stand-alone drug coated balloon angioplasty is feasible to avoid stent-induced compromise of branch vessel flow.

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Rare Venous Compression Syndrome
Scherisse Baldeo

Description of background: The most common presentation of venous compression syndrome (VCS) i.e. May–Thurner syndrome (MT) is stenosis or occlusion of the left common iliac vein (CIV) from compression between right common iliac artery (CIA) and underlying vertebral body. We report 3 unusual site venous compression syndromes.

Methods: A 49yo male with presented with right leg swelling. MRV revealed non-thrombotic right CIV compression by right CIA. Stenting of the vein led to symptom resolution.

An 87yo male with history of atrial fibrillation off anticoagulation presented with left leg swelling and shortness of breath after long flight. CT reported acute pulmonary embolism. US revealed acute left common femoral to popliteal vein thrombus. CT showed tortuous iliac arteries with thrombus extending up to distal left external iliac vein just distal to compression of the distal left external iliac vein by the left external iliac artery. He was treated with anticoagulation alone without endovenous interventions.

An 81yo male with history of provoked R calf DVT, colitis s/p gastrectomy and colon resection was admitted for abdominal pain. CT abdomen noted a filling defect in right internal iliac vein which due to variant anatomy drained into L CIV and was compressed between R CIA and sacrum. He was also only anticoagulated without endovenous interventions.

Results: VCS can occur beyond established compression sites especially in the setting of anatomical variants and can place the patient at risk of recurrent thrombosis or morbidity from persistent swelling and pain. It is of grave importance to identify these findings as treatment may vary based on its presence.

Conclusion: As clinicians it is important to recognize uncommon venous compression syndromes as a cause for recurrent thrombosis and its associated morbidity.

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A Rare Case of Penile Raynaud’s Phenomenon due to Giant Cell Arteritis
Arvind Pandey

Description of background: Giant cell arteritis (GCA) typically affects the aorta and large vessel branches in the head, neck, and upper extremities. GCA of the lower extremity vasculature is distinctly rare.

Methods: A 71-year-old male with a history of hyperlipidemia presented with one week of worsening discoloration of the penile tip, associated with intermittent coolness and pain. He reported no recent injuries and had not been sexually active for the last five years. On exam, he was normotensive and in regular rhythm. The glans penis appeared gangrenous at the distal tip with tenderness to light palpation. ESR and CRP were elevated at 86 mm/h and 204.4 mg/L, respectively.

Results: The initial evaluation raised concern for vasculitis with penile Raynaud’s phenomenon. Penile pulse volume recording showed baseline blunted waveforms that nearly normalized with warming (Figure 1A). CTA of the abdomen/pelvis showed no abrupt vessel cutoffs but was notable for multi-focal areas of circumferential vascular wall thickening in the pelvic arteries, suggestive of vascular edema. PET scan showed increased radiotracer uptake in these vessels, confirming an inflammatory process (Figure 1B). On further questioning, the patient reported jaw pain for the preceding six weeks. Ultrasound demonstrated acute arteritis in the bilateral temporal arteries (Figure 1C). Temporal artery biopsy confirmed giant cell arteritis. The patient was started on prednisone taper, transitioning to tocilizumab, sildenafil, and amlodipine. After 2 months of therapy, his penile gangrene resolved.

Conclusion: GCA can present with diffuse, upper and lower extremity vascular inflammation. A high index of suspicion and multi-modal imaging can identify and confirm involvement of atypical vascular beds.

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Use of Thrombolysis in Conjunction with Bivalirudin in Sub-massive Pulmonary Embolism in a Patient with Severe Thrombocytopenia Resulting from HIT
Chirag Sheth

Description of background: Heparin induced thrombocytopenia (HIT) can result in both arterial and venous thrombosis, DVT and PE being the most common complications.

A 69 year old female with history of provoked DVT presented with acute onset of dyspnea. Patient underwent left TKA two weeks prior and was discharged on prophylactic enoxaparin. She was normotensive and requiring supplemental oxygen. A CTA chest revealed a saddle PE (Figure A). A 2D Echo revealed severe RV dysfunction. A venous duplex showed bilateral femoral and popliteal DVT. Her platelet count was 23,000/uL, so HIT was suspected. She was started on a direct thrombin inhibitor, bivalirudin. Her anti-platelet factor 4/heparin antibody assay resulted positive. On day 3, she went into atrial fibrillation with rapid ventricular rate with worsening of hypoxia. Due to persistent hemodynamic instability, despite platelet count of 24,000/uL, the patient was given systemic alteplase 50 mg over 2 hours with concomitant bivalirudin. After thrombolysis, her hemodynamics improved significantly. By day 8, her platelet count increased to 155,000/uL and she was started on warfarin. Patient was subsequently discharged. At 3 months follow up, she remained asymptomatic and repeat echocardiogram revealed normal RV function.

Thrombosis associated with HIT has a high mortality, approximating 20-30%. Systemic thrombolysis in the setting of sub-massive PE associated with severe thrombocytopenia is considered a contraindication. In our patient with such low platelet count, we opted to use reduced dose tPA, per the MOPETT trial. Our case suggests that a patient with significant pro-thrombotic condition like HIT can be successfully thrombolysed even in the setting of profound thrombocytopenia.

Methods: n/a

Results: n/a

Conclusion: n/a

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Pseudoxanthoma Elasticum Presenting with Advanced Atherosclerosis
Nicholas McDonald

Description of background: Patients who present for evaluation of premature atherosclerosis in the absence of traditional risk factors present a unique challenge for the clinician and require further assessment to look for unusual arteriopathy or underlying systemic disease. We present a patient with Pseudoxanthoma elasticum who typifies this disorder with diffuse advanced atherosclerosis involving multiple vascular beds.

Methods: A 55-year-old man presented for vascular medicine consultation in the setting of lower extremity claudication.  Previous vascular involvement included his retina, having gone blind at age 35 and found to have angioid streaking, bilateral internal carotid arteries presenting with TIA and diffuse CAD including a 90% obstructed left main after presenting with unstable angina.  Despite this diffuse vascular involvement, he had no identifiable risk factors. He was a lifelong non-smoker, non-diabetic, without hypertension or hyperlipidemia and had an unremarkable family history.

He had noted chronic yellow colored nodules of his posterior neck and the flexor surfaces of his arms. Biopsy from a femoral endarterectomy showed fibrocalcific atherosclerosis, consistent with Pseudoxanthoma elasticum.

Results: n/a

Conclusion: Pseudoxanthoma elasticum is an inherited autosomal dominant or recessive disorder effecting the connective tissue of elastic fibers. The prevalence is 1 in 160,000 and it predominantly affects the skin, eyes and vasculature. Patients commonly develop blindness from chorioretinal angioid streaks and may develop myocardial infarction or gastrointestinal hemorrhage.  Yellow or off-white skin lesions are common. The diagnosis is confirmed by histopathology showing elastic fiber calcification and a supportive clinical history.

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A Rare Cause of Lower Extremity Hyperpigmentation
Tara Holder

Description of background: Sneddon Syndrome (SS) is a rare non-inflammatory thrombotic vasculopathy characterized by the occurrence of Livedo Racemosa (LR) and cerebrovascular disease.

Methods: Case: A 66-year-old male presented with four years of progressive bilateral lower extremity discoloration. Skin biopsy early in his course revealed scant chronic dermal inflammation and no evidence of vasculitis. These skin findings in conjunction with a history of arthralgias led to a diagnosis of Livedo Racemosa. An extensive serologic workup for autoimmune diseases, including rheumatoid arthritis, lupus, vasculitis and other inflammatory conditions was negative. On Antiphospholipid panel, anti-cardiolipin antibody was positive despite no personal or family history of thrombosis. Sneddon Syndrome was diagnosed based on his positive antiphospholipid antibody and Livedo Racemosa.

Results: Discussion: SS is typically observed in women, with an estimated incidence of 4 per 1 million per year in the general population. This disorder occurs sporadically, though a few familial cases have been reported. Circulating antiphospholipid antibodies are seen in almost 80% of patients with SS. Some patients may also present with Raynaud’s phenomenon in addition to LR. The onset of LR typically occurs prior to cerebrovascular events. Patients with suspected SS should undergo a complete rheumatologic evaluation, cerebral MRI, and skin biopsy. The patient was started on apixaban to prevent thrombotic events.

Conclusion: This case demonstrates the importance of identifying rare causes of LR, such as SS. In this instance, early diagnosis allowed providers to initiate anticoagulation before the occurrence of cerebrovascular events.

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Beaded Arteries: Consider Fibromuscular Dysplasia Mimickers
Rohan Bhandari

Intro: Fibromuscular dysplasia (FMD) is a non-inflammatory and non-atherosclerotic disorder that can lead to arterial stenosis, aneurysm, dissection, and tortuosity. The clinical diagnosis relies upon careful history taking and characteristic findings on imaging.

Case: A 77 year-old woman with history of an intracranial aneurysm with catheter-directed coiling and migraine headaches presented to clinic for evaluation of possible FMD. CTA Head and Neck showed multifocal beading in left ICA (Image 1). During the months leading up to her visit, the patient experienced a 15 pound unintentional weight loss, persistent night sweats, cervical lymphadenopathy, temporal tenderness and bilateral jaw claudication. CTA Chest showed evidence of possible aortitis. Carotid duplex ultrasound showed left ICA tortuosity and elevated mid to distal velocities consistent with FMD. However, given her systemic symptoms the patient was referred to Rheumatology. She was diagnosed with biopsy-proven giant cell arteritis and started on immunosuppressive therapy.  On subsequent office visits her weight loss, night sweats, and jaw claudication symptoms subsided.

Results: N/A

Discussion: Multifocal FMD is a diagnosis of exclusion. The carotid and renal arteries are most commonly affected, but any arterial bed may be involved.  Ultrasound studies will characteristically show elevated velocities and tortuosity affecting the mid to distal segments of vessels. However, it is important to be aware of FMD mimickers including vasculitis. In this patient, systemic symptoms of weight loss, night sweats, temporal tenderness, jaw claudication and potential aortitis ultimately led to the diagnosis of giant cell arteritis.

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Improvement in Vasomotor Function does not Correlate with Reduced Pain in CRPS
Arvind Pandey

Description of background: A subset of chronic regional pain syndrome (CRPS) patients can exhibit significant vasomotor changes. However, the full spectrum and underlying etiology of this phenotype is poorly understood.

Methods: Two patients were evaluated for leg pain and intermittent cyanotic color changes. Both patients had a prior history of CRPS in the contralateral lower foot and leg, with palliative below-knee amputation (BKA) pursued in the year preceding the current presentation due to unrelenting symptoms. The patients underwent peripheral angiography and low-dose epoprostenol infusion (8-10 ng/kg/min) for workup and therapy, respectively.

Results: Patient 1 was a 22-year-old woman with a history of left BKA. Patient 2 was a 35-year-old woman with a history of right BKA. Both had no prior cardio-vascular disease history. Vasomotor changes at the time of presentation included dependent cyanosis (both patients), and intermittent erythromelalgia (patient 1). Peripheral angiogram showed no flow-limiting lesion but did demonstrate very slow filling of the distal vessels of the leg and foot, which did not improve with nitroglycerin. Oral vasodilators were ineffective; following epoprostenol infusion, skin color changes improved but there was no improvement in pain. Due to non-response to medical therapy, both patients underwent a second palliative BKA with resolution in symptoms.

Conclusion: CRPS can be associated with peripheral small vessel outflow obstruction. Intensive interventions aimed at the vascular symptoms do not appear to improve the neuropathic pain component. Further clinical and translational studies are needed to better understand the pathophysiologic link between these two phenotypes.

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Pylephlebitis in a 58 Year-Old Male with Fever: A Case Report
Trifon Antonio Caballero

Description of background: We report a case of a 58 year-old hypertensive, diabetic male with known coronary artery disease with fever as the initial presentation of pylephlebitis. Pylephlebitis is a rare infective suppurative thrombosis of the portal vein that occurs in 0.16% of patients with intra-abdominal infection, diverticulitis being the most frequent with paucity of data on enteric fever, with high early mortality rate (23%) but with good prognosis for survivors. Besides thrombosis, it typically presents with fever, bacteremia, abdominal pain and hepatic dysfunction. In studies,  the true incidence of this condition is unclear.

Methods: Whole abdominal CT scan revealed a filling defect in the right portal vein near the confluence with non-enhancement of its distal branches in the right hepatic lobe. Saccular outpouchings in the ascending colon were also noted with no sign of inflammation. Inferior vena cava duplex scan shows a dilated mid to distal portal vein with hypoechoic intraluminal density and absent color flow. Blood tests revealed leukocytosis with neutrophilic predominance on complete blood count and mildly elevated liver enzymes. Stool culture showed few growth of Pseudomonas aeruginosa. All other diagnostic tests were unremarkable.

Results: He was successfully treated initially with piperacillin-tazobactam downgrading to oral ciprofloxacin and oral metronidazole. Apixaban was also started. He was then discharged on the 7th hospital day improved and stable. No recurrence has yet been reported.

Conclusion: Pylephlebitis is a rare and serious complication of an intra-abdominal infection. Left untreated, this could result to debilitating sequelae, such as sepsis and ultimately, death. Timely diagnosis is key and early antibiotic treatment is the standard treatment of choice. Anticoagulation is still debatable.

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Mesenteric Artery Dissection and/or Inflammation
Jenna Smith

Description of Background: Mesenteric artery dissection (MAD) and/or inflammation (MAI) are uncommon with scant literature, yet may have great clinical significance. Herein, we present our experience diagnosing and managing these rare vasculopathies.

Methods: From a single center retrospective database, we identified 110 patients who presented with mesenteric artery (celiac, superior (SMA) and/or inferior mesenteric (IMA)) D and/or I between January 2000 and March 2017.

Results: Average age was 55 ± 14 years, mostly affecting men (56.4%). There were 20 celiac D (18%), 18 celiac I (16%), 15 celiac D+I (14%), 15 SMA D (14%), 6 SMA I (5%), 6 SMA D+I (5%), 1 IMA D (1%), 3 IMA I (3%) and 26 with multiple arterial involvement (24%). Based on most supported diagnostic criteria, primary etiologies included isolated dissection (n=20), localized vasculitis of the gastrointestinal tract (LVGT) (n= 15), segmental arterial mediolysis (SAM) (n=15), isolated inflamed aneurysm (n= 13) and fibromuscular dysplasia (FMD) (n= 11).

Most (70%) patients presented with abdominal pain, although 21% were asymptomatic. Hypertension (51%), hyperlipidemia (32%) and tobacco use (28%) were prevalent. CT angiogram was the most frequently used imaging (91%). Management included conservative (28%), antithrombotics (48%), antihypertensives (38%), endovascular (19%) and open repair (14%) with a high survival rate (98%) and symptom relief (75%). Of those medically or conservatively managed, 92% had stable or improved imaging at follow-up.

Conclusion: MAD and MAI are rare and can be caused by various etiologies. Most patients improved with a conservative, medical, endovascular and/or surgical approach. Our project provides guidance for the diagnosis and management of different mesenteric arteriopathies.

Learning Objective: Our research project expands the literature for diagnosing and managing patients with mesenteric arteriopathies.

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A Rare Case of Eagle Syndrome Presenting with Vascular Symptoms
Patrick Sullivan

Description of Background: Eagle Syndrome is a rare disorder characterized by compression of the stylohyoid process and ligament into the cervical neurovascular structures. This disorder that can present with diverse clinical symptoms making it difficult to recognize. It is important for Vascular Medicine (VM) specialists to recognize Eagle syndrome as it can often present with vascular manifestations, such as carotid compression/dissection or internal jugular compression as illustrated in the case below.

Methods: We present the case of a 52-year-old male with a five-year history of right sided facial fullness, flushing, and pressure. He complained of right-sided sinus pressure and headache worsened by both leaning forward and lying flat. He reported globus sensation and dysphagia if not sitting completely upright.

Results: A supine duplex ultrasound found a 17x22 mm right IJ vein, with increased velocities when sitting and retrograde flow when leaning forward. His left IJ vein was 8x2 mm in caliber. CTA was initially unremarkable, and no diagnosis was reached. A CTA was repeated 2 years later and showed bilateral compression of the internal jugular veins in between elongated styloid processes and the lateral aspects of C1. A diagnosis of Eagle Syndrome was made. He underwent right styloid resection and postoperatively asymptomatic internal carotid dissection was discovered on CTA.

Conclusion: This case illustrates the variety of nonspecific symptoms that may be present in Eagle syndrome. It is important for VM specialists to be aware of the presenting symptoms, physical exam findings and diagnostic imaging used to diagnose Eagle Syndrome, as VM specialists may be called upon to help diagnose and recommend treatment for this condition.

Learning Objective: Awareness of the variety of presenting symptoms in Eagle Syndrome as well as the vascular components that can arise. By becoming familiar with imaging techniques used for diagnosis. Treatment options clinicians can recommend to patients.

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