To test the hypothesis that not all embolic materials are equivalent by using postprocedural magnetic resonance (MR) imaging enhancement of uterine fibroids in patients treated with uterine artery embolization (UAE). Approval and a waiver of consent from the institutional human investigations committee was received for this study. The study was HIPAA compliant. A total of 84 women who underwent 6-month MR imaging follow-up constituted this retrospective study. Within this group, 25 women were treated with Contour polyvinyl alcohol (PVA) particles, 23 were treated with Contour SE particles, 19 were treated with Embosphere microspheres, and 17 were treated with Bead Block microspheres. Pre- and postprocedural MR imaging results were analyzed for the total number of fibroids present in the uterus of each patient and for the percentage of individual fibroid enhancement. Enhancement of individual fibroids was measured with quartile intervals. Greater than 25% residual enhancement of a fibroid after embolization was considered an incomplete infarction. The overall percentage change in enhancement was calculated for each patient. Bivariate analysis by using generalized linear modeling and one-way analysis of variance was used to assess differences in infarction with different embolic materials. Among patients treated with Contour and Embosphere, there was a mean reduction in enhancement by 76.60% and 83.07%, respectively, compared with a mean reduction of 52.53% and 49.78% in patients treated with Bead Block and Contour SE, respectively. There was a significant difference in postembolization enhancement between Bead Block and Embosphere, Bead Block and Contour, Contour SE and Embosphere, and Contour SE and Contour. Patients treated with Bead Block or Contour SE demonstrated a reduced degree of infarction at follow-up MR imaging compared with patients treated with Contour or Embosphere.

Hereditary hemorrhagic telangiectasia (HHT), a rare autosomal dominant disease mostly caused by mutations in three known genes (ENG, ACVRL1, and SMAD4), is characterized by the development of vascular malformations (VMs). Patients with HHT may present with mucocutaneous telangiectasia, as well as organ arteriovenous malformations (AVMs) of the central nervous system, lungs, and liver. Genotype–phenotype correlations have been well described in adults with HHT. We aimed to investigate genotype–phenotype correlations among pediatric HHT patients. Demographic, clinical, and genetic data were collected and analyzed in 205 children enrolled in the multicenter Brain Vascular Malformation Consortium HHT Project. A chi-square test was used to determine the association between phenotypic presentations and genotype. Among 205 patients (age range: 0–18 years; mean: 11 years), ENG mutation was associated with the presence of pulmonary AVMs (p < 0.001) and brain VM (p < 0.001). The presence of a combined phenotype—defined as both pulmonary AVMs and brain VMs—was also associated with ENG mutation. Gastrointestinal bleeding was rare (4.4%), but was associated with SMAD4 genotype (p < 0.001). We conclude that genotype–phenotype correlations among pediatric HHT patients are similar to those described among adults. Specifically, pediatric patients with ENG mutation have a greater prevalence of pulmonary AVMs, brain VMs, and a combined phenotype.

The spontaneous rupture of pulmonary AVMs, resulting in pulmonary hemorrhage and hydrothorax, is a life-threatening complication. While this phenomenon has been previously reported, the true incidence is not yet known. This study retrospectively reviewed records of 801 HHT patients with pulmonary AVMs to identify a single lifetime episode of hemothorax or pulmonary hemorrhage secondary to pulmonary AVM rupture. The lifetime prevalence and incidence of pulmonary AVM rupture in HHT patients was 2.7% and 0.16% respectively. In these patients, AVM rupture represented the initial presentation of HHT in nine (40.9%) cases and was life-threatening in nine (40.9%) cases. All cases occurred in virgin lesions, and subsequent embolization was curative. While a feared complication, pulmonary AVM rupture is rare and is likely effectively prevented by existing embolization techniques and indications.

Hyperimmunoglobulin E syndrome (HIES) is a rare primary immunodeficiency disorder defined by high serum immunoglobulin E titers that is associated with recurrent respiratory infections, formation of pneumoatoceles, recurrent skin abscesses, and characteristic dental and skeletal abnormalities. We report a case of a 56-year-old male with a history of HIES, cavitary mycetomas, and allergic bronchopulmonary aspergillosis who presented with recurrent massive hemoptysis. Bronchial artery angiography and bronchoscopy failed to identify active hemorrhage, and two embolizations of the bronchial artery did not resolve the bleeding. Subsequently, selective pulmonary artery angiography was conducted that demonstrated a subsegmental pulmonary artery branch pseudoaneurysm with extravasation into an adjacent lung cavity. This was treated successfully with transcatheter embolization. To our knowledge, this is the first case reported of pulmonary artery pseudoaneurysm in HIES in the medical literature. Pulmonary artery pseudoaneurysm should be considered in the differential diagnosis in patients with HIES and massive hemoptysis.