Recurrent Arterial Thrombosis as a Presenting Feature of a Variant M3-Acute Promyelocytic Leukemia.

Acute limb ischemia (ALI) is a common vascular emergency. Hematologic malignancies are commonly associated with derangement of normal hemostasis and thrombo-hemorrhagic symptoms during the course of the disease are common. However, ALI as an initial presenting feature of acute leukemia is rare. Due to the rarity of this presentation, there is a scarcity of prospective randomized data to optimally guide the management of these patients. Current knowledge is mainly based on isolated cases. We report our experience managing a patient who presented with ALI and was found to have occult leukemia. A review of all cases with ALI as a presenting feature of acute leukemia is also presented.

An avulsed radial artery with a high take-off.

A 63-year-old female was diagnosed with severe aortic stenosis, who underwent a diagnostic coronary angiography via transradial approach prior an aortic-valve replacement. After imaging the left coronary system, entrapment of the diagnostic catheter was encountered as a result of spasm of the radial artery. An arteriogram of the arm revealed an anatomical variation in the radial artery (high take-off). Several attempts to remove the entrapped catheter resulted in avulsion of the artery, which was managed successfully with coil embolization. To our knowledge, no such complication has been reported.

An interesting anatomic variant of inferior vena cava duplication: case report and review of the literature.

Congenital anomalies of the inferior vena cava (IVC) occur in roughly 4% of the population. We report an interesting case of an atypical variant of duplicated IVC. A 20-year-old man presented with orthopedic injuries and intracranial hemorrhage following a motorcycle accident. He was taken to the fluoroscopy suite for IVC filter placement; duplication of the IVC was noted. The right and left iliac veins shared a normal confluence but two IVCs drained independently into renal veins before reuniting into a single structure. Both IVC filters were placed via a single puncture in the groin. We performed a search of the PubMed database using' inferior vena cava duplication' and reviewed common anomalies of the IVC. Several variants of duplicated IVC exist; the most common of which is two distinct IVCs that arise from each iliac vein without a normal confluence. Our patient had a unique anomaly which allowed filter placements from a single puncture.

Coral reef aorta: case report and review of the literature.

Coral reef aorta (CRA) is a rare form of atherosclerosis that affects the paravisceral and pararenal aorta and its branches. Patients typically present with arterial insufficiency of the bowels, kidneys and lower extremities. The current mainstay of treatment is operative, typically involving transaortic endarterectomy. Herein, we describe a 54-year-old woman with incapacitating lower extremity claudication secondary to a paravisceral coral reef atheroma treated successfully with transaortic endarterectomy via a left retroperitoneal approach. In addition, we present a complete review of modern English literature on CRA.

Results of polytetrafluoroethylene-covered nitinol stents crossing the inguinal ligament.

OBJECTIVE: Placement of arterial endoprostheses across the inguinal ligament is generally thought to be contraindicated for fear of device kinking, fracture, or occlusion and possible obliteration of the deep femoral artery (DFA). We present a series of selected patients who underwent insertion of polytetrafluoroethylene-covered nitinol stents (Viabahn stent grafts. W. L. Gore and Associates Inc, Flagstaff, Ariz) crossing the middle common femoral artery (CFA) on an emergency basis or who were considered high risk for open surgery. METHODS: We treated 16 patients with 17 lesions adjacent to or within the CFA with stent grafts that originated in the common iliac (two) or external iliac (15) artery and terminated in the distal CFA (12), DFA (three), or superficial femoral (two) artery. Stent grafts were placed on an elective (10) or emergency (seven) basis for arterial occlusive disease (10), bleeding (six), and aneurysmal disease (one). Comorbidities favoring endovascular treatment were high medical risk (10) previous scarring (four), morbid obesity (two), and dense arterial calcification precluding open surgical repair (one). RESULTS: The DFA was deliberately sacrificed in one of the 17 cases. No patient suffered major complications after the procedure. All grafts remained patent based on duplex ultrasound imaging during follow-up (mean, 12.3 months; range, 1-58 months). Two patients required an additional endovascular intervention to treat inflow or outflow stenoses during follow-up, yielding a 2-year primary patency rate of 93.8% and assisted primary patency rate of 100%. CONCLUSIONS: These results suggest that selective placement of Viabahn stent grafts across the inguinal ligament to treat arterial occlusive disease or bleeding may prove to be safe, effective, and associated with acceptable patency rates. This strategy helps avoid complicated open arterial surgery in high-risk patients with associated multiple medical risk factors or hostile scarred groins.

Contained rupture of a pseudoaneurysm of the descending thoracic aorta related to remnant outflow graft of left ventricular assist device after heart transplantation.

The Jarvik 2000 left ventricular assist device is inserted via a left thoracotomy with the outflow graft anastomosed to the descending thoracic aorta. Removal of the device during heart transplantation involves division of the outflow graft, resulting in a retained remnant. We describe the first reported case of a mycotic pseudoaneurysm of the descending thoracic aorta related to the remnant of a left ventricular assist device outflow graft in an immunosuppressed heart recipient complicated with systemic Pseudomonas infection. The pseudoaneurysm was temporarily treated with endovascular stent grafting followed by delayed thoracotomy, pseudoaneurysm excision, and placement of an aortic interposition graft using an aortic allograft.

Robotic-assisted median arcuate ligament release.

Median arcuate ligament syndrome results from external compression of the celiac axis by attachments of the diaphragmatic crura. It has been treated with open or laparoscopic surgical decompression of the celiac axis with neurolysis. We describe our initial experience treating three patients using a robotic-assisted technique with median arcuate ligament release and celiac neurolysis. Average operative time was 2.2 hours. No intraoperative complications occurred. At an average of 11 months postoperative (14, 11, and 8 months), two patients continue with resolution of preoperative symptoms. Our experience affirms that further study using the robotic approach appears warranted.

An unusual cause of venous hypertension after dialysis access creation.

Venous hypertension after creation of arteriovenous fistula or arteriovenous shunt occurs in approximately 10-15% of patients (Kojecky et al., Biomed Papers, 2002;146:77-79; Criado et al., Ann Vasc Surg 1994;8:530-535). Its etiology is commonly stenosis and/or thrombosis of the central venous system secondary to previous catheterization with subsequent development of venous hypertension after the arteriovenous connection is made. Treatment strategies often involve venography to determine the site of venous stenosis and/or occlusion centrally and subsequent endovascular recanalization of the stenotic or occluded veins. In this article, we report a case of venous hypertension in a 76-year-old man who presented with a swollen arm after placement of an arteriovenous fistula. In this circumstance, venography revealed extrinsic compression of the subclavian vein at the level of the first rib, the anatomic abnormality seen in venous thoracic outlet syndrome. In this report, we describe surgical and endovascular management of this patient, and review the literature on the causes of central vein stenosis discovered after creation of dialysis access.

Long-term outcomes of internal carotid artery dissection.

OBJECTIVE: The natural history of acute carotid artery dissection is poorly characterized. The purpose of this study is to report on single institutional long-term outcomes. METHODS: A retrospective review of patients treated for acute spontaneous or posttraumatic carotid artery dissection over a 20-year period from August 1989 to July 2009 was performed. RESULTS: Twenty-nine patients with a mean age of 47 ± 19.6 years were identified with acute carotid dissection. Six (25%) were related to trauma, while 23 (79%) were spontaneous. Neurologic symptoms included contralateral limb weakness (55%), facial pain (35%), and Horner's syndrome (21%). Eight patients (28%) presented with an acute hemispheric stroke. Diagnostic imaging modalities used included computed tomography angiography (52%), magnetic resonance angiography (41%), and conventional angiography (48%). Twenty percent of patients had complete carotid occlusion and 25% had near occlusion. Most dissections (65%) had intracranial extension, and 35% were limited to the extracranial cervical internal carotid. The majority (96%) of patients were treated conservatively with anticoagulation or antiplatelet therapy or both. One patient underwent stenting for persistent symptoms resulting in complete recovery. There were two deaths, one from unrelated traumatic injuries and the other from unknown causes. Long-term follow-up was available for 20 patients: 14 had complete symptom resolution (70%) and five (25%) had partial clinical symptom resolution. Two patients had initial resolution of symptoms, with subsequent recurrence that was successfully managed conservatively. Follow-up imaging revealed luminal patency in 79% of patients with minimal residual stenosis. Two patients developed a small asymptomatic internal carotid aneurysm that did not require treatment. Mean follow-up was 1133.2 days. CONCLUSIONS: Most cervical carotid dissections can safely be conservatively managed, with the majority achieving anatomic and symptomatic resolution, with low rates of recurrence over long-term follow-up.

Cerebral reserve is decreased in elderly patients with carotid stenosis.

OBJECTIVES: Octogenarians and even patients over 70 years old have unexplained poor outcomes with carotid angioplasty and stenting (CAS). We sought to evaluate whether older patients may have compromised intracranial collaterals and cerebral reserve and be intolerant to otherwise clinically silent emboli generated during CAS. METHODS: One thousand twenty-four cerebral blood flow (CBF) studies performed between 1991 and 2001 with stable xenon computed tomography scans (Xe/CT) were reviewed. CBF was measured before and after 1 gm intravenous acetazolamide (ACZ), a cerebral vasodilator. The normal response to ACZ is an increase in CBF. In areas of significant compromise of cerebral reserve (CR), CBF drops, representing a "steal" phenomenon. CBF changes were categorized as normal or abnormal and correlated with age, gender, cerebral symptoms, and with intracranial, carotid, or vertebral artery disease. Logistic regression was used to determine the effect of age on CR in the entire group and a subgroup of 179 patients with significant carotid stenosis of >50%. RESULTS: Nine hundred sixteen studies were suitable for analysis. Carotid occlusion was predictive of decreased reserve (OR, 3.9; P = .03) regardless of age. There was also a trend toward lower reserve with severe carotid stenosis >70% (OR, 3) and in women (OR, 1.8; P = .08). Age >or=70 had no effect on reserve in the overall heterogeneous population with and without carotid disease and neither did a history of stroke, carotid, or intracranial stenosis. However, in 179 patients with significant carotid stenosis, age >or=70 was predictive of poor reserve (OR, 2.7; P = .03) and so was the presence of peripheral vascular disease (OR, 3.7; P = .03). A trend toward decreased reserve was also seen in women (OR, 2.3; P = .08). CONCLUSIONS: Age >or=70 is associated with poor cerebral reserve in patients with significant carotid stenosis as measured by CBF response to an ACZ challenge. Thus, patients >or=70 may be more sensitive to minor cerebral emboli, which may be one factor explaining their higher risk of stroke during CAS.

A novel percutaneous solution to limb ischemia due to arterial occlusion from a femoral artery ECMO cannula.

PURPOSE: To describe a novel percutaneous technique for distal limb perfusion in the face of femoral artery occlusion secondary to extracorporeal membrane oxygenation (ECMO) cannula placement. TECHNIQUE: The technique is described in a 59-year-old man who presented with an inferior wall myocardial infarction and a large ventricular septal defect (VSD) requiring the initiation of ECMO via right femoral artery and vein cannulae. He subsequently developed right lower limb ischemia secondary to cannula occlusion of the femoral artery. Percutaneous transfemoral placement of a flush catheter in the right common femoral artery was performed angiographically. Ischemic symptoms resolved, and the patient was subsequently able to undergo repair of his VSD without any further lower limb ischemic sequelae. CONCLUSION: Percutaneous transfemoral placement of a flush catheter in the common femoral artery distal to ECMO cannula insertion has not to our knowledge been reported and carries the added benefit of restoring perfusion to both superficial and profunda femoris arteries.

Malignant melanoma presenting as obstructive jaundice secondary to metastasis to the Ampulla of Vater.

CONTEXT: Malignant melanoma commonly metastasizes to the small intestine where it can cause pain, bleeding, and obstruction. However, jaundice from metastatic melanoma is relatively uncommon. CASE REPORT: A case of known malignant melanoma presenting as new onset obstructive jaundice as a result of a rarely reported metastasis to the ampulla of Vater. CONCLUSION: Multidisciplinary management of patients with metastatic melanoma is essential.

Pharmacomechanical thrombectomy for iliofemoral deep vein thrombosis: an alternative in patients with contraindications to thrombolysis.

OBJECTIVE: Venous lysis is usually reserved for symptomatic patients with acute deep vein thrombosis (DVT) and low risk for bleeding. This study reports the use of pharmacomechanical thrombectomy (PMT) in patients with contraindications to thrombolysis. METHODS: A retrospective review of all patients with symptomatic DVT treated between 2007 and 2008 with PMT was performed. All patients were treated by a combination of local tissue plasminogen activator (tPA) with the Angiojet (Possis Medical, Minneapolis, Minn) or Trellis device (Bacchus Vascular, Santa Clara, Calif). Catheter-directed lysis was used sparingly. RESULTS: Forty-three patients (mean age, 48.4 +/- 16.6 years) presented with symptoms averaging 13.6 +/- 9.6 days in duration. Nineteen (44%) had symptoms for >14 days, and 15 (35%) had a high risk for bleeding. Symptomatic subclavian thrombosis occurred in eight (19%), and 35 (81%) presented with disabling lower extremity DVT (4 phlegmasia) despite anticoagulation. Fifteen patients had a thrombosed indwelling permanent filter. Sixty-three percent were treated in one session, but 16 patients required a lytic infusion after suboptimal PMT. Iliac stenting was required in 35% of limbs treated. Successful lysis (>50%) was achieved in 95% of patients and symptom resolution in 93%. All patients became ambulatory with no or minimal limitation. There were no major systemic bleeding complications, but access site hematoma occurred in two patients and worsening of pre-existing rectus sheath hematoma requiring transfusion occurred in another two. Limb salvage was maintained in 100% of patients who presented with phlegmasia. Mean follow-up was 5.0 +/- 4.8 months. Freedom from DVT recurrence and reintervention was 95% at 9 months by life-table analysis. CONCLUSIONS: PMT can be safely and effectively used for subacute iliocaval and iliofemoral DVT and in patients with contraindications for lytic therapy, resulting in improved functional outcomes relative to their debilitated state before the procedure.

Coverage of the celiac artery during TEVAR: is it ever appropriate?

Adequate seal at the proximal and distal extent of stent grafts in the aorta is paramount to the success of thoracic endovascular aortic repair (TEVAR). Thoracoabdominal aneurysms pose a formidable challenge given their extension into the arch branches proximally and the visceral segment distally. Extension of the landing zone of even 3 to 5 mm can possibly increase the durability of the stent graft and may decrease the chances of future migration or collapse. Although coverage of the subclavian artery to extend the proximal landing zone has been met with initial success, the outcome of coverage of the celiac axis in order to extend the distal landing zone has not been as well studied. Because of the abundance of rich collateral vessels in the foregut, it has been perceived as a potentially safe practice. However, careful angiographic anatomic delineation and patient selection is vital to determine whether concomitant revascularization procedures are warranted.

Does intraoperative radiation therapy improve local tumor control in patients undergoing pancreaticoduodenectomy for pancreatic adenocarcinoma? A propensity score analysis.

BACKGROUND: Locoregional recurrence (LRR) is an important factor after pancreaticoduodenectomy (PD) for pancreatic cancer. Intraoperative radiation therapy (IORT) administered to the resection bed may improve local tumor control. METHODS: We performed a retrospective analysis of patients who underwent PD at Thomas Jefferson University Hospital (TJUH) between 1995 and 2005 to identify patients who underwent resection with and without IORT. Data collected included age, gender, complications, margin status, stage, survival, and recurrence. Unadjusted analyses of the IORT and non-IORT groups were performed using Fisher's chi-square method for discrete variables and Wilcoxon rank sum test for continuous variables. To account for biases in patient selection for IORT, a propensity score was calculated for each patient and adjusted statistical analyses were performed for survival and recurrence outcomes. RESULTS: Between January 1995 and November 2005, 122 patients underwent PD for periampullary tumors, including 99 pancreatic cancers. Of this group, 37 patients were treated with IORT, and there was adequate follow-up information for a group of 46 patients who underwent PD without IORT. The IORT group contained a higher percentage of Stage IIB or higher tumors (65%) than in the non-IORT group (39.1%), though differences in stage did not reach significance (P = .16). There was a nonsignificant decrease in the rate of LRR in patients who had IORT (39% non-IORT vs. 23% IORT, P = .19). The median survival time of patients who received IORT was 19.2 months, which was not significantly different than patients managed without IORT, 21.0 months (P = .78). In the propensity analyses, IORT did not significantly influence survival or recurrence after PD. CONCLUSIONS: IORT can be safely added to management approaches for resectable pancreatic cancer, with acceptable morbidity and mortality. IORT did not improve locoregional control and did not alter survival for patients with resected pancreatic cancer. IORT is an optional component of adjuvant chemoradiation for pancreatic cancer. In the future, IORT may be combined with novel therapeutic agents in the setting of a clinical trial in order to attempt to improve outcomes for patients with pancreatic cancer.

Epigenetic modulation of endogenous tumor suppressor expression in lung cancer xenografts suppresses tumorigenicity.

Epigenetic changes involved in cancer development, unlike genetic changes, are reversible. DNA methyltransferase and histone deacetylase inhibitors show antiproliferative effects in vitro, through tumor suppressor reactivation and induction of apoptosis. Such inhibitors have shown activity in the treatment of hematologic disorders but there is little data concerning their effectiveness in treatment of solid tumors. FHIT, WWOX and other tumor suppressor genes are frequently epigenetically inactivated in lung cancers. Lung cancer cell clones carrying conditional FHIT or WWOX transgenes showed significant suppression of xenograft tumor growth after induction of expression of the FHIT or WWOX transgene, suggesting that treatments to restore endogenous Fhit and Wwox expression in lung cancers would result in decreased tumorigenicity. H1299 lung cancer cells, lacking Fhit, Wwox, p16(INK4a) and Rassf1a expression due to epigenetic modifications, were used to assess efficacy of epigenetically targeted protocols in suppressing growth of lung tumors, by injection of 5-aza-2-deoxycytidine (AZA) and trichostatin A (TSA) in nude mice with established H1299 tumors. High doses of intraperitoneal AZA/TSA suppressed growth of small tumors but did not affect large tumors (200 mm(3)); lower AZA doses, administered intraperitoneally or intratumorally, suppressed growth of small tumors without apparent toxicity. Responding tumors showed restoration of Fhit, Wwox, p16(INKa), Rassf1a expression, low mitotic activity, high apoptotic fraction and activation of caspase 3. These preclinical studies show the therapeutic potential of restoration of tumor suppressor expression through epigenetic modulation and the promise of re-expressed tumor suppressors as markers and effectors of the responses.

Diagnostic and treatment modalities for autoimmune pancreatitis.

Since the recognition of autoimmune pancreatitis (AIP) as a clinical entity, many advances have been made in defining clinical, radiologic, histologic, and laboratory parameters to assist in a complete definition of the disease. Despite all these efforts, a preoperative diagnosis still remains a clinical challenge but is of paramount importance, as these cases have been reported to be steroid-responsive; therefore, early treatment may obviate the need for surgical resection. Although the utilization of recently proposed guidelines by the Japanese Pancreas Society and an Italian study group may further assist the clinician and prompt the initiation of steroid treatment, the response to therapy should be observed within 2 to 4 weeks and reflected in progressive resolution of the presenting radiologic and laboratory abnormalities. Should these fail to demonstrate improvement, the diagnosis of AIP should undergo re-evaluation, and consideration for surgical exploration should be made, as the patient may be harboring a malignancy. Surgical resection in the form of pylorus-preserving pancreaticoduodenectomy remains the optimal solution in the attempt to clarify the diagnosis and offer treatment with low complication rates.

Peritoneal perfusion with oxygenated perfluorocarbon augments systemic oxygenation.

BACKGROUND: Despite maximal ventilatory support, many patients die from hypoxia in the setting of potentially reversible pulmonary failure. There remains a pressing need for additional pulmonary supportive care measures, especially techniques that do not require systemic anticoagulation. The objective of our experiments was to determine whether systemic oxygenation could be increased in a large animal, with induced hypoxia, by perfusing the abdominal cavity with oxygenated perfluorocarbons. METHODS: Fifteen pigs with a mean (+/- SD) weight of 45 +/- 5 kg were intubated and rendered hypoxic by ventilating them with a blend of nitrogen and oxygen to achieve subatmospheric concentrations of inspired oxygen ranging from 18 to 10%, resulting in baseline mean Pao(2) range of 65.9 +/- 9.7 to 26.6 +/- 2.8 mm Hg, respectively. Peritoneal perfusion was performed in eight animals with oxygenated perfluorocarbon and in seven control animals with oxygenated saline solution. RESULTS: The average increase in Pao(2) with oxygenated perfluorocarbon perfusion, compared to oxygenated saline solution perfusion, ranged from 8.1 to 18.2 mm Hg. A common treatment effect was estimated across all fraction of inspired oxygen (Fio(2)) values, representing the average mean difference in oxygen uptake between oxygenated perfluorocarbon and saline solution, irrespective of the level of Fio(2). This average was 12.8 mm Hg (95% confidence interval, 7.4 to 18.2; p < 0.001). The most clinically relevant results occurred at an Fio(2) of 14%, resulting in a baseline mean Pao(2) of 39.4 +/- 5.0 mm Hg with oxygenated saline solution perfusion, and a mean Pao(2) of 55.3 +/- 7.6 mm Hg with oxygenated perfluorocarbon perfusion. This corresponded to an increase in arterial oxygen saturation from 73 to 89%. CONCLUSION: These results of our principle experiments demonstrate that the peritoneal cavity can be used for gas exchange and, in our model, yielded clinically relevant increases in systemic arterial oxygen levels. This technique may have the potential for the supportive care of patients dying from hypoxia in the setting of reversible lung injury.