Paget–Schroetter Syndrome Treated with Cutting-Balloon Angioplasty

Here, a case of Paget-Schroetter Syndrome in a 25-year-old guitar player is reported. After thrombolysis, conventional angioplasty failed to dilate the underlying subclavian stenosis both before and after first-rib excision with scalenus anterior and medius division. For the third attempt at angioplasty, a cutting balloon was used, which immediately produced a good result. Venography at 4-year follow-up showed no restenosis and no functional deficit. This case report demonstrates that cutting-balloon angioplasty may be considered when conventional balloon fails and may have greater durability than conventional balloon angioplasty in the treatment of Paget-Schroetter syndrome.     

Giant-cell-rich pseudotumour in Paget’s disease

Paget’s disease (PD) of the bone is a disorder of bone remodelling that may be polyostotic or monostotic. Although development of a sarcoma in PD is well-recognised, it is less well recognised that pseudosarcomas in bone and soft tissue can also arise in this condition. In this report we document the case of a large giant-cell-rich pseudotumour that developed in the tibia and overlying soft tissues in a case of polyostotic PD. Bone and soft tissues were highly vascular and contained abundant haemorrhage with focal areas of new bone formation and a diffuse infiltrate of osteoclastic giant cells. The lesion has not recurred or produced metastases 3 years after removal. Clinicians should be aware that a benign giant-cell-rich pseudotumour can develop in PD and that it needs to be distinguished from other giant-cell-rich tumours.     

The spine in Paget’s disease

Paget’s disease (PD) is a chronic metabolically active bone disease, characterized by a disturbance in bone modelling and remodelling due to an increase in osteoblastic and osteoclastic activity. The vertebra is the second most commonly affected site. This article reviews the various spinal pathomechanisms and osseous dynamics involved in producing the varied imaging appearances and their clinical relevance. Advanced imaging of osseous, articular and bone marrow manifestations of PD in all the vertebral components are presented. Pagetic changes often result in clinical symptoms including back pain, spinal stenosis and neural dysfunction. Various pathological complications due to PD involvement result in these clinical symptoms. Recognition of the imaging manifestations of spinal PD and the potential complications that cause the clinical symptoms enables accurate assessment of patients prior to appropriate management.     

Endovascular Management of May–Thurner Syndrome in Adolescents: A Single-Center Experience

Purpose

To report a single-center experience in regard to the technique, safety, and clinical outcomes of endovascular therapy for treatment of May–Thurner syndrome (MTS) in adolescent patients.

Embolization of Life-Threatening Arterial Rupture in Patients with Vascular Ehlers–Danlos Syndrome

Purpose

To evaluate the safety and efficacy of transarterial embolization of life-threatening arterial rupture in patients with vascular Ehlers–Danlos syndrome (vEDS) in a single tertiary referral center.

Methods

We retrospectively analyzed transarterial embolization for vEDS performed at our institution from 2000 to 2012. The indication of embolization was spontaneous arterial rupture or pseudoaneurysm with acute bleeding. All interventions used a percutaneous approach through a 5F or less introducer sheath. Embolic agents were microcoils and glue in 3 procedures, glue alone in 2, and microcoils alone in 2.

Results

Five consecutive vEDS patients were treated by 7 embolization procedures (4 women, mean age 29.8 years). All procedures were successfully performed. Two patients required a second procedure for newly arterial lesions at a different site from the first procedure. Four of the five patients were still alive after a mean follow-up of 19.4 (range 1–74.7) months. One patient died of multiple organ failure 2 days after procedure. Minor procedural complications were observed in 3 procedures (43 %), all directly managed during the same session. Remote arterial lesions occurred after 3 procedures (43 %); one underwent a second embolization, and the other 2 were observed conservatively. Puncture site complication was observed in only one procedure (14 %).

Conclusion

Embolization for vEDS is a safe and effective method to manage life-threatening arterial rupture.     

Multifocal osteogenic sarcoma in Paget’s disease

The most serious complication of Paget’s disease is sarcomatous degeneration of pagetic bone. Multifocal sarcomatous degeneration occurs mainly in polyostotic Paget’s disease. Multifocal Paget’s sarcoma is uncommon and can arise in any site. We report two cases of synchronous multifocal sarcomatous degeneration. The two patients were elderly women (aged 77 and 86 years, respectively) who developed sarcomatous lesions concomitantly, in the first case report in left ilium, left tibia, and first lumbar vertebra and in the second case report in the skull, right ilium, and sacrum. Whether these cases are due to the simultaneous development of several primaries or to metastases from a single primary remains unclear. Received: 3 August 1998 Revision requested: 14 September 1998 Revision received: 6 March 2000 Accepted: 14 March 2000     

Pharmacomechanical thrombectomy with the Castañeda brush catheter in thrombosed hemodialysis grafts and native fistulas

PURPOSE: To evaluate the safety and efficacy of the Casta?eda brush catheter in the treatment of thrombosed hemodialysis fistulas and grafts. MATERIALS AND METHODS: Twenty-six revascularization procedures with the Casta?eda brush catheter combined with urokinase were retrospectively analyzed in 21 patients (mean age, 69 years; range, 35-87 y). Hemodialysis shunts were native arteriovenous (AV) fistulas (n=15; 16 procedures) or polytetrafluoroethylene grafts (n=6; 10 procedures). Major outcomes included procedure time, anatomic and clinical success rates, complication rate, and primary, primary assisted, and secondary patency. RESULTS: In 26 procedures, the brush catheter was used in combination with a mean dose of 239,792 IU urokinase (range, 60,000-300,000 IU). Additional angioplasty was performed in all procedures; five procedures (19%) required additional stent implantation. Mean procedure time was 99.2 minutes (range, 49-261 min). Anatomic and clinical success rates were 100% and 96.2%, respectively. Two minor complications (8%) occurred, neither of which was device-related: one case of extravasation treated by balloon tamponade and one hematoma at the distal puncture site without the need for surgery or transfusion. Primary patency rates were 87%, 62%, and 50% at 3, 6, and 12 months, respectively, for AV fistulas, and 50%, 33%, and 17%, respectively, for grafts. Assisted primary patency rates were 93%, 77%, and 70% at 3, 6, and 12 months, respectively, for AV fistulas, and 50%, 33%, and 17%, respectively, for grafts. At 3, 6, and 12 months, secondary patency rates were 93%, 85%, and 80%, respectively, for AV fistulas, and 83%, 67%, and 50%, respectively, for grafts. CONCLUSIONS: The Casta?eda brush catheter is a safe and effective pharmacomechanical thrombectomy device for the treatment of thrombosed hemodialysis grafts and native fistulas.     

Changes in Liver Congestion in Patients with Budd–Chiari Syndrome following Endovascular Interventions: Assessment with Transient Elastography

Purpose

Transient elastography (TE) is routinely used for noninvasive staging of hepatic fibrosis. The objective of the present study was to investigate the role of TE (FibroScan) in determining changes in liver congestion in patients with Budd–Chiari syndrome (BCS) treated by endovascular interventions and determine the effects of pretreatment Meta-analysis of Histological Data in Viral Hepatitis (METAVIR) fibrosis score on posttreatment liver stiffness (LS).

Dynamic contrast-enhanced MRI in Paget’s disease of bone–correlation of regional microcirculation and bone turnover

The purpose of this study was to evaluate regional microcirculation in Paget’s disease of bone (PD) with dynamic contrast-enhanced MR imaging (DCE-MRI). Additionally, we correlated regional bone perfusion with alkaline phosphatase as serum marker of bone turnover. We examined 71 patients with PD (27 men, 44 women, 67±10 years) localized at the axial and appendicular skeleton. Contrast uptake was analyzed using a two-compartment model with the output variables amplitude A and exchange rate constant k_ep. Color-coded parametric images were generated to visualize microcirculation. Serum levels of alkaline phosphatase (AP) were compared with DCE-MRI parameters. Amplitude A and exchange rate constant k_ep were significantly increased in PD compared to unaffected bone (A_PD 0.81±0.24 vs. A_control 0.34±0.1 and k_{ep PD} 4.0±2.86 vs. k_{ep control} 1.73±0.88, p<0.001). There was a significant correlation (r_s=0.5–0.7) of DCE-MRI parameters and AP at the axial (pelvis, spine) and appendicular skeleton (femur, tibia). The long bones showed increased circulation of the advancing peripheral zones and no vascularization of the central part, which had been replaced by fatty tissue. Regional microcirculation in PD is inhomogeneous with focal areas of excessive hypervascularity, especially in the advancing peripheral zone. There is a significant correlation of bone circulation and bone turnover in PD. DCE-MRI might therefore be a diagnostic tool for monitoring therapeutic effects of bisphoshonates in Paget’s disease of bone.     

Synchronous Paget’s sarcoma of tibiae in which Paget’s disease was limited to these bones

A 51-year-old native of Rio de Janeiro presented with bilateral synchronous Paget’s sarcomas in the tibiae, which developed in the upper right tibia and in the distal third of the left tibia. There were no other areas of Paget’s disease. The largest tumor spread to the right inguinal nodes and also soft tissue. The tumor in the left tibia spread dramatically in the soft tissues up the leg and only involved the medullary cavity in its inferior portion. The patient died, but there was no autopsy. Comments are made about the prevalence of Paget’s disease in Rio de Janeiro. Received: 8 April 1999 Revision requested: 10 May 1999 Revision received: 23 June 1999 Accepted: 1 July 1999