Is Hughes-Stovin syndrome Behçet's disease?

Hughes-Stovin syndrome (HSS) is a rare clinical disorder, which has been described as the presence of pulmonary artery aneurysm in the setting of systemic thrombosis. The term "Incomplete Behçet's Disease" has also been used to describe this syndrome due to the clinical and histopathological similarities between Behçet's disease and HSS. Indeed, pulmonary involvement can be indistinguishable between these two conditions of unknown pathophysiology. We describe an HSS patient who presented with a recurrent pulmonary artery aneurysm, review the clinical and pathological manifestations of HSS, discuss its similarities to Behçet's disease, and finally make the argument that HSS is in fact Behçet's disease.

Transjugular liver biopsy is safe and diagnostic for patients with congenital bleeding disorders and hepatitis C infection.

The prevalence of chronic hepatitis C virus (HCV) infection among patients with severe congenital bleeding disorders is as high as 98%. Advances in HCV treatment currently result in sustained virological response rates of > or =50%. Recent recommendations have reaffirmed that liver biopsy, which provides a direct histological assessment of liver inflammation and fibrosis, is still important for accurate diagnosis and therapeutic decision making. Percutaneous liver biopsy is a simple, standardized procedure that can be performed rapidly and relatively inexpensively, and has been safely performed in patients with congenital coagulopathies. However, the safety and efficacy of the transjugular approach (transjugular liver biopsy, TJLB), recommended for patients with acquired coagulopathies, has only been minimally studied in the congenital bleeding diathesis population. We now report our institutional experience with TJLB in 13 such adult patients (mean age 33 years) with severe/mild haemophilia A/B (10); von Willebrand disease (1); factor V deficiency (1) and factor XIII deficiency (1). Data were collected by retrospective chart review and the TJLB was performed according to institutional protocol as described. Haemostasis prophylaxis was given for 1-5 days. Patients were hospitalized for < or =48 h and all tolerated the procedure without bleeding. Three patients experienced self-limited abdominal discomfort; one episode was accompanied by transient transaminaemia. Diagnostic specimens were obtained from all patients and were instrumental in the therapeutic decision-making process. We suggest that with a co-ordinated multidisciplinary approach to care, TJLB is a safe, effective and potentially cost-effective alternative to the percutaneous approach in the congenital bleeding disorders population.

Ostial renal artery stent placement in patients 75 years of age or older.

Renal artery stent placement has been shown to improve blood pressure (BP) and stabilize renal function in patients with atherosclerotic renovascular disease. However, limited data are available in patients > or = 75 years of age. We analyzed the prestent characteristics and clinical outcomes of patients aged > or = 75 years who underwent renal artery stenting at our institution. We compared these data with those from the remainder of our stent cohort. Nineteen of 89 (21.3%) stent patients were > or = 75 years old. Before intervention, those > or = 75 years were significantly more likely to be women (84.2% v 55%; P = .02), current or former smokers (78.6% v 36.8%; P = .002), and on a greater number of antihypertensive medications (3.68 v 2.80; P = .048). Average clinical follow-up was similar in both groups (23.9 v 23.2 months; P > .05). At last available follow-up, there were more deaths in those > or = 75 years (7/19 v 5/70; P = .038). No significant difference was found in the incidence of dialysis after intervention (3/19 v 7/70). Seventy-four percent of those > or = 75 years had improved BP, 21% were stable, and 5% were worse. Renal function was improved in 26%, stable in 53%, and worse in 21%. Among those > or = 75 years, there was a significant decrease in systolic BP (186.9 to 144.4; P < .01). There was a trend toward decreased diastolic BP and medications. These clinical results did not differ significantly from patients <75 years. Patients > or = 75 years of age with atherosclerotic renovascular disease have a higher incidence of mortality 2 years after renal artery stent placement, but they seem to derive clinical benefit comparable to younger patients.

Transcatheter interventions for the treatment of peripheral atherosclerotic lesions: part I.

Transcatheter endovascular procedures are increasingly used to treat symptomatic peripheral atherosclerosis. This two-part review identifies the existing evidence supportive of the application of transcatheter treatments for peripheral atherosclerotic lesions. The first part addresses the treatment of obstructive lesions that cause limb claudication and critical ischemia, renovascular hypertension and azotemia, and mesenteric ischemia. Studies were identified via a search of MEDLINE (January 1993 through April 1999) and reference lists of identified articles. When multicenter prospective randomized trials or other high-quality studies were unavailable, a preference was given to studies with at least 50 patients per treated group and a minimum mean follow-up duration of 6 months. Data presented in tables are proportionally weighted averages from included studies. For each application, the authors assessed the quality of evidence (QOE; efficacy, safety, and, where available, cost-effectiveness) and made recommendations with appropriate caveats. There is higher QOE supporting the more established treatments such as lower limb percutaneous transluminal angioplasty (PTA) with stent placement and thrombolysis. Treatments such as renal artery PTA and stent placement and mesenteric and brachiocephalic PTA are in wide use, but high QOE supporting general application is lacking. Blanket recommendations based on established efficacy and cost-effectiveness cannot be made. However, the use of transcatheter therapies can be supported in specific circumstances based on an expected reduction in procedure-related morbidity and/or mortality rates. It is hoped that the identification of deficiencies in the literature will inform and inspire critically needed research in this area.

Type B aortic dissection complicating renal artery angioplasty and stent placement.

Percutaneous renal artery stent placement has been demonstrated to improve blood pressure control and stabilize renal function in patients with atherosclerotic renal artery disease. However, this procedure is not without risk of significant morbidity, and its effectiveness, as compared to alternative treatments, has not been adequately established. The authors report a case of acute type B aortic dissection complicating renal artery stent placement. The authors postulate that an intimal disruption occurred during initial balloon angioplasty, and that repeated application of radial, shear, and torque forces during stent placement may have extended the injury. The diagnosis of acute aortic dissection should be considered in patients with suggestive symptoms immediately after stent placement.

Contrast-enhanced peripheral MR angiography from the abdominal aorta to the pedal arteries: combined dynamic two-dimensional and bolus-chase three-dimensional acquisitions.

UNLABELLED: Wang Y, Winchester PA, Khilnani NM, et al. Contrast-enhanced peripheral MR angiography from the abdominal aorta to the pedal arteries: Combined dynamic two-dimensional and bolus-chase three-dimensional acquisitions. Invest Radiol 2001;36:170-177. RATIONALE AND OBJECTIVES: To obtain reliable contrast-enhanced peripheral MR angiography for imaging peripheral vascular disease from the abdominal aorta to the pedal arteries. METHODS: A protocol consisting of contrast-enhanced, dynamic two-dimensional (2D) acquisition at the feet and calf and bolus-chase three-dimensional (3D) acquisition from the abdominal aorta to the calf was developed and applied in patients with peripheral vascular disease. The performance of this integrated protocol was assessed in 89 consecutive patients. RESULTS: The bolus-chase 3D acquisition was of diagnostic quality in 100% of the acquisitions in the abdomen, 96% in the thigh, and 43% in the calf. The poor quality of the calf acquisitions was due to insufficient spatial resolution, poor arterial signal, and venous contamination. Diagnostic-quality images were obtained in 100% of the dynamic 2D acquisitions of the calf and 98% of the feet. CONCLUSIONS: The combined dynamic 2D and bolus-chase 3D contrast-enhanced MR angiography technique provides diagnostic images of the entire lower extremity.

Delayed epistaxis resulting from external carotid artery injury requiring embolization: a rare complication of transsphenoidal surgery: case report.

OBJECTIVE AND IMPORTANCE: Delayed epistaxis resulting from trauma to branches of the external carotid artery is an infrequent but potentially serious complication of transsphenoidal surgery. We report two cases of severe, delayed epistaxis in patients who had undergone transsphenoidal surgery. In both cases, noninvasive treatment failed, necessitating endovascular intervention. CLINICAL PRESENTATION: The first patient, a 52-year-old woman with a prolactinoma, underwent a second transsphenoidal resection 18 months after the first surgery. She was readmitted on postoperative Day 15 with massive epistaxis. The second patient, a 40-year-old woman, had undergone two transsphenoidal surgeries, 14 years apart, for an adrenocorticotropic hormone-secreting adenoma. She was readmitted with massive epistaxis on postoperative Day 17. INTERVENTION: Both patients were initially treated with nasal balloon packing but experienced recurrent hemorrhage when the balloon was deflated, necessitating referral to the interventional radiology department for embolization. At arteriography, the first patient was found to have a pseudoaneurysm of the medial branch of the left internal maxillary artery, which was subsequently embolized. Arteriography in the second patient revealed an abnormally dilated midline branch of the right internal maxillary artery in the nasal septum; this vessel was occluded at arteriography. CONCLUSION: Delayed massive epistaxis is a rare but significant complication of transsphenoidal surgery. Injury to branches of the external carotid artery, along with injury to the internal carotid artery, should be suspected in patients who present with delayed epistaxis after transsphenoidal surgery. Angiography performed in patients with refractory bleeding should include selective external carotid injections. Epistaxis that is refractory to anterior and posterior nasal packing may be effectively treated with endovascular embolization.

Prevention of recurrent pulmonary edema in patients with bilateral renovascular disease through renal artery stent placement.

Pulmonary edema and congestive heart failure (both referred to here as PE) have been reported to be complications of bilateral renal artery stenosis or unilateral stenosis in a solitary functioning kidney (both referred to as BRAS). The goals of this study were to determine whether a history of PE was more common in patients with BRAS than in those with unilateral stenosis and a normal contralateral kidney (URAS), and whether recurrent PE could be prevented by renal artery stent placement. We evaluated 90 consecutive patients with renovascular disease who were treated with percutaneous renal artery stent placement. History and clinical follow-up were obtained through chart review and phone contact with referring physicians. Mean follow-up was 18.4 months after stent placement. Twenty-three of 56 (41%) subjects with BRAS had a history of PE before revascularization, compared with four of 34 (12%) subjects with URAS (P = .05). Twenty-five of the 27 patients with history of PE had adequate clinical follow-up. Seventeen of the 22 (77%) subjects with BRAS and history of PE had no further PE after stent placement in one or both renal arteries. The five BRAS subjects with recurrent PE after stent placement had evidence of stent thrombosis or restenosis. In contrast, only one of three (33%) URAS subjects with a history of PE remained free of PE after stent placement. We conclude that PE is a common complication of BRAS, but not of URAS. In patients with BRAS, recurrent PE can be prevented by successful stent placement in one or both renal arteries.

[Congenital cardiovascular malformations and chromosome microdeletions in 22q11.2]. / Angeborene kardiovaskuläre Fehlbildungen und chromosomale Mikrodeletionen in 22q11.2.

BACKGROUND AND OBJECTIVE: Congenital cardiovascular (c-v) malformations are the leading signs of two syndromes of highly variable phenotypes, the DiGeorge syndrome (DGS) and the velo-cardio-facial syndrome (VCFS), both of which in the majority of cases are caused by microdeletion in the chromosome region 22q11.2. It was the aim of this study to ascertain the frequency of these chromosomal abnormalities in patients with unselected congenital cardiovascular malformation, and to assess the type of c-v malformation for which microdeletion analysis of the mentioned region would be indicated. PATIENTS AND METHODS: The cohort consisted of 90 patients with congenital c-v malformations (35 males, 55 females; mean age 3.6 years (19th week of pregnancy-36 years). Most of them were newborns. The c-v anomalies were: ventricular septal defect (n = 20), pulmonary atresia (10), Fallot's tetralogy (9), truncus arteriosus communis (6), aortic valve stenosis (6), atrioventricular canal (6), type B interrupted aortic arch (5), atrial septal defect (5), tricuspid atresia (4), hypoplastic left heart syndrome (4), persisting ductus arteriosus (3), pulmonary valve stenosis (3), complete (third degree) atrioventricular block (2), Ebstein's anomaly (1), tachycardia (1) and enlarged right atrium (1). Four of 14 fetuses included in this study had complex cardiac anomalies that could not be definitively classified. Cytogenetic karyotype analysis was unremarkable in all cases. Microdeletion detection was done by fluorescence-in-situ-hybridization (FISH). RESULTS: 14 of the 90 cases (about 16%) showed microdeletion in the examined chromosomal region 22q11.2. Among the group with microdeletion were aortic arch interruption (5/5), ventricular septal defect (2/20). Fallot's tetralogy (1/9) and atrial septal defect (1/5). All the deletion carriers had other signs of the DGS/VCFS complex. One parent each in two of the microdeletion patients had the same microdeletions. CONCLUSION: In patients with congenital c-v and associated malformations of dysmorphism microdeletion diagnosis of 22q11.2 by FISH is indicated in addition to conventional cytogenetic testing. The incidence of this microdeletion seems to be especially high among patients with type B interrupted aortic arch.

Fluoroscopic landmarks for optimal visualization of the proximal renal arteries.

PURPOSE: To accurately determine the in vivo orientation of the origin of the renal arteries from the aorta relative to a fluoroscopic bony landmark for optimal diagnostic arteriography and renal artery stent placement. MATERIALS AND METHODS: One hundred sixty abdominal computed tomography (CT) scans of patients in eight age groups (20-90 years) were reviewed to determine the angle of the origins of the renal arteries from the aorta relative to the long axis of the L-1 spinous process (L1SP). RESULTS: The right renal artery arises ventrally at an angle of 30 degrees (standard deviation [SD] = 15 degrees) from a plane orthogonal to the long axis of the L1SP. The left renal artery arises dorsally at an angle of 7 degrees (SD = 13 degrees) relative to the same plane. CONCLUSIONS: The optimal initial angle for angiographic evaluation of the origin of the renal artery and for renal artery stent placement is 30 degrees left anterior oblique (LAO) relative to the L1SP for the right renal artery and 7 degrees LAO for the left renal artery. Unfortunately, there is variability in the angle of the renal artery origins from the aorta which cannot be controlled for using this technique. In some patients, additional views will be necessary to optimally depict the origins of the renal arteries.

Comparison of two-dimensional MR digital subtraction angiography of the lower extremity with x-ray angiography.

PURPOSE: To perform a preliminary evaluation of the diagnostic accuracy of contrast-enhanced, two-dimensional (2D) magnetic resonance (MR) digital subtraction angiography (DSA) of the lower extremity by comparison with x-ray angiography (XRA). MATERIALS AND METHODS: Forty lower extremities in 22 patients were imaged at multiple levels with both XRA and 2D MR DSA. Images were retrospectively analyzed by three radiologists in a randomized blinded manner. Seventeen vascular segments were graded as an insignificant lesion, a significant lesion, or as an occlusion. With the use of segments well depicted with XRA as the gold standard, the sensitivity, specificity, and accuracy of 2D MR DSA, as compared with XRA, were evaluated. The McNemar-Stuart-Maxwell test was performed to determine the significance of any differences found. RESULTS: Three hundred eighty-three arterial segments were evaluated with both techniques. Three hundred one segments were well depicted with XRA. There was no significant difference between 2D MR DSA and XRA for assessing the degree of occlusive disease in these 301 segments (.25 < P < .5). The sensitivity, specificity, and diagnostic accuracy of 2D MR DSA were found to be 90%, 98%, and 93%, respectively. CONCLUSION: Two-dimensional MR DSA is an accurate method for assessing arterial lesions in the lower extremity.

In vitro evaluation of the relative thrombolytic efficiency of forced intrathrombic injections: saline versus urokinase.

PURPOSE: To compare the rates of thrombolysis produced by forced intrathrombic injections of saline versus urokinase, as well as automated versus manual injections of urokinase, with use of an in vitro model of a vascular occlusion. MATERIALS AND METHODS: The rates of thrombolysis produced by forced intrathrombic injections of saline and urokinase were compared in an in vitro radiometric model utilizing I-125-labeled thrombus. Similar experiments were performed to compare manual and automated injections of urokinase. The dissolution of the thrombus was quantitatively monitored with use of a scintillation detector. Averaged time activity data for each type of experiment were fit to exponential functions and half times of lysis calculated. The differences in the half times for the experiments being compared were evaluated for significance with use of the Student t test. RESULTS: The half times of lysis produced by forced intrathrombic injections of urokinase were substantially and significantly shorter than those produced by forced saline injections. The half time of lysis produced by automated injections was not significantly different than that produced by manual injections. CONCLUSIONS: Forced intrathrombic injections of urokinase produce faster and substantially more thrombolysis when compared with similarly administered saline. Also, for forced intrathrombic injections of lytic agents, an automated injector is an equivalent alternative to manual injections.

Timing algorithm for bolus chase MR digital subtraction angiography.

To acquire multiple longitudinal locations in the lower extremity after a single contrast injection, appropriate table translation and contrast injection are required. An approximate model based on constant bolus velocity was developed to describe the space-time course of a contrast bolus in the lower extremity. This model was verified in dynamic MR angiograms acquired in a group of patients using time-resolved 2D MR digital subtraction angiography (MRDSA). From this contrast bolus passage model, a timing algorithm for table translation and contrast injection was developed for bolus chase MRDSA, subsequently validated in bolus chase 2D MRDSA experiments. All targeted major peripheral arteries were well depicted in bolus chase 2D MRDSA using this timing algorithm and a single 15-ml contrast dose.

Distal lower extremity arteries: evaluation with two-dimensional MR digital subtraction angiography.

PURPOSE: To test the hypothesis that magnetic resonance (MR) digital subtraction angiography is superior to two-dimensional time-of-flight (TOF) MR angiography for demonstration of patent arteries in the distal lower extremity. MATERIALS AND METHODS: Thirty-seven lower extremities in 23 consecutive patients were imaged with two-dimensional TOF MR angiography and two-dimensional MR digital subtraction angiography. Images were interpreted in a randomized and blinded manner. Each lower extremity was subdivided into seven potential arterial segments. The number of digital arteries visualized was also determined. Overall image quality of MR digital subtraction and TOF angiograms was compared. The relative ability of MR digital subtraction angiography and TOF MR angiography to demonstrate patent arterial segments was assessed. RESULTS: MR digital subtraction angiography was significantly superior to TOF MR angiography for demonstration of patent arterial segments and digital arteries (P < .001). MR digital subtraction angiographic images were qualitatively superior to TOF images (P < .001). CONCLUSION: Two-dimensional MR digital subtraction angiography is superior to two-dimensional TOF MR angiography for help in identifying patent segments in the distal lower extremity.

Bolus-chase MR digital subtraction angiography in the lower extremity.

A bolus-chase magnetic resonance (MR) angiographic technique performed with a prototypic stepping table and coil holder and a 15-20-mL injection of contrast material was developed to depict the entire lower extremity. Image acquisition was synchronized with passage of the contrast medium bolus through the lower extremity. Ten subjects underwent the examination, which was performed in less than 1 minute. All major arteries were well demonstrated in all cases.

Erroneous pressure measurement due to artificially deformed vessel during angioplasty.

Percutaneous transluminal angioplasty is a commonly performed procedure. The clinical success of such a procedure is determined by the combination of the post-procedure angiographic appearance and the pressure gradient across the lesion. This case demonstrates the importance of recognizing the anatomical variation if proper pressure measurements are to be obtained.