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.

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.

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.

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.

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.

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.

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.