The Young's procedure for severe epistaxis from hereditary hemorrhagic telangiectasia.

Background: Surgical treatment of epistaxis in hereditary hemorrhagic telangiectasia (HHT) has historically been managed with the laser procedure or the septodermoplasty procedure. For transfusion-dependent patients with severe epistaxis we have been performing the Young's procedure or surgical closure of the nostrils. The objective of this study was to report treatment of severe epistaxis related to HHT with the Young's procedure and assess patient outcome.

Brain arteriovenous malformations associated with hereditary hemorrhagic telangiectasia: gene-phenotype correlations.

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disease with a wide spectrum of vascular malformations (VMs) involving multiple organs. Nine to 16% of patients with HHT harbor brain arteriovenous malformations (AVMs), which can cause intracranial hemorrhage (ICH). Our objective was to study clinical manifestations of brain AVMs in patients with HHT and correlate these with the specific gene mutated.

Reproducibility of oxygen saturation monitoring during six-minute walk test and exercise stress test in patients with pulmonary arteriovenous malformations associated with hereditary hemorrhagic telangiectasia.

Patients with pulmonary arteriovenous malformations (PAVMs) are at risk for multiple complications and require close follow-up. We investigated the reproducibility of the 6-minute walk test (6MWT) and exercise stress test (EST) for the evaluation of low oxygen saturation in patients with PAVMs. Twenty-two patients with PAVMs, most of whom had hereditary hemorrhagic telangiectasia (HHT), participated in a Human Investigations Committee-approved protocol.

Hemothorax due to rupture of pulmonary arteriovenous malformation: an interventional emergency.

Spontaneous hemothorax as a result of a ruptured pulmonary arteriovenous malformation (PAVM) is a life-threatening event and requires immediate interventional therapy. We present two patients who survived following emergent embolization. Definitive thoracentesis was delayed until embolization was performed.

Overlapping spectra of SMAD4 mutations in juvenile polyposis (JP) and JP-HHT syndrome.

Juvenile polyposis (JP) and hereditary hemorrhagic telangiectasia (HHT) are clinically distinct diseases caused by mutations in SMAD4 and BMPR1A (for JP) and endoglin and ALK1 (for HHT). Recently, a combined syndrome of JP-HHT was described that is also caused by mutations in SMAD4. Although both JP and JP-HHT are caused by SMAD4 mutations, a possible genotype:phenotype correlation was noted as all of the SMAD4 mutations in the JP-HHT patients were clustered in the COOH-terminal MH2 domain of the protein.

Results of exercise stress testing in patients with diffuse pulmonary arteriovenous malformations.

Patients with diffuse pulmonary arteriovenous malformations (PAVMs) are subject to frequent complications and need to be followed closely. As part of this follow-up, we have employed exercise stress testing (EST) as an aid to assess their status. Twenty patients from a cohort of 35 with diffuse PAVMs have undergone EST using a standard cycle ergometer test.

Comparison of four embolic materials at uterine artery embolization by using postprocedural MR imaging enhancement.

Purpose: 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).

Materials And Methods: Approval and a waiver of consent from the institutional human investigations committee was received for this study. The study was HIPAA compliant.

Pulmonary arteriovenous malformations: how do I embolize?

Pulmonary arteriovenous malformations (PAVM) are high-flow, low-pressure shunts, consisting of a single feeding artery connecting via an aneurysmal sac to a draining vein. The aneurysmal connection is referred to as an aneurysmal sac. The "filter capacity" of the pulmonary capillaries is lost and results in predisposition to brain abscess, stroke, and transient ischemic attack and, when multiple, dyspnea, because of right-to-left shunting and hypoxemia.

Comparison of MRI outcomes of uterine artery embolization for uterine leiomyoma using tris-acryl gelatin microspheres, polyvinyl alcohol spheres, and polyvinyl alcohol particles.

Objective: Comparison of recurrence of magnetic resonance imaging (MRI) enhancement of uterine fibroids treated with uterine artery embolization (UAE) among embolic agents: polyvinyl alcohol (PVA), spherical PVA (SPVA), and tris-acryl gelatin microspheres (GM).

Methods: Pre/post-UAE gadolinium-enhanced MRIs were evaluated for residual enhancement of fibroids after UAE. Data were analyzed using 2-tail Fisher exact test to determine the likelihood of recurrence of enhancement post-UAE among embolic agents.

Long-term complications of septal dermoplasty in patients with hereditary hemorrhagic telangiectasia.

Objective: Septal dermoplasty has been recommended as the treatment of choice for life-threatening epistaxis in patients with hereditary hemorrhagic telangiectasia. This study evaluates the complications of septal dermoplasty in the management of transfusion-dependent epistaxis.

Study Design: Consecutive retrospective study.

Initial experience with 3% sodium tetradecyl sulfate foam and fibered coils for management of adolescent varicocele.

Purpose: To report the results of treatment of adolescent patients with varicocele with use of 3% sodium tetradecyl sulfate foam (STS) in combination with pushable fibered coils.

Materials And Methods: From September 2004 to September 2006, 16 adolescent patients (age 12-19 y) with symptomatic varicocele, testicular atrophy, or surgical recurrence underwent embolization with STS foam and coils. The left internal spermatic vein (ISV) was coaxially catheterized from the right femoral vein.

New definition and natural history of patients with diffuse pulmonary arteriovenous malformations: twenty-seven-year experience.

Background: Patients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality rates.

Methods: Thirty-six patients (21 female and 15 male) with diffuse PAVM from a cohort of 821 consecutive patients with PAVM were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs.

Septectomy and septal dermoplasty for the treatment of severe transfusion-dependent epistaxis in patients with hereditary hemorrhagic telangiectasia and septal perforation.

Background: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by abnormal angiogenesis with resultant telangiectasia formation in mucocutaneous tissues, visceral organs, and the central nervous system. The most common manifestation of HHT is epistaxis resulting from trauma to thin-walled telangiectasias. Many patients with HHT experience worsened epistaxis due to the presence of a septal perforation.

Value of Micronester coils in port-catheter implantation for continuous hepatic arterial infusion chemotherapy with fixed catheter tip method.

To retrospectively evaluate the use of Micronester coils in port-catheter implantation with the fixed catheter tip method in comparison with other previously used coils. The cohort of this study was 143 consecutive patients with unresectable advanced liver cancer for whom a port-catheter system was percutaneously implanted. In the most recent 32 patients, Micronester coils were used for catheter tip fixation.

Liver involvement in hereditary hemorrhagic telangiectasia: consensus recommendations.

Study Purpose: To formulate recommendations about clinical management of liver involvement in hereditary hemorrhagic telangiectasia (HHT), using a formal consensus development process.

Consensus Process: A nominal group technique was used. A list of main clinical, diagnostic and therapeutic issues about liver involvement in HHT was generated by the organizing committee.

Liver involvement in hereditary hemorrhagic telangiectasia: CT and clinical findings do not correlate in symptomatic patients.

Objective: The purpose of our study was to report the multiphasic CT findings in patients with symptomatic liver involvement by hereditary hemorrhagic telangiectasia (HHT) and to correlate the CT findings with the type of clinical presentation.

Conclusion: Patients with symptomatic HHT liver disease have diffuse hepatic telangiectases, a dilated common hepatic artery, and a high incidence of biliary abnormalities. Multiphasic CT is useful in diagnosing liver involvement due to HHT; however, no strong correlation was seen between CT findings and the clinical subtype of HHT liver disease.

Clinical and anatomic outcomes after embolotherapy of pulmonary arteriovenous malformations.

Purpose: To assess long-term clinical and imaging results of technically successful pulmonary arteriovenous malformation (AVM) embolization.

Materials And Methods: One hundred fifty-five patients with pulmonary AVMs underwent embolization during a period of 3 years. Recommended follow-up included clinical assessment, helical computed tomography, and physiologic evaluation within 1 year and then every 5 years.

Outcome of septal dermoplasty in patients with hereditary hemorrhagic telangiectasia.

Objectives/hypothesis: Septal dermoplasty has been recommended as the treatment of choice for life-threatening epistaxis in patients with hereditary hemorrhagic telangiectasia. The purpose of the study was to evaluate the effectiveness and outcomes of septal dermoplasty for management of transfusion-dependent epistaxis.

Study Design: Retrospective study.

Enteroscopic evaluation of the gastrointestinal tract in symptomatic patients with hereditary hemorrhagic telangiectasia.

Objectives: Hereditary hemorrhagic telangiectasia is an autosomal dominant disease in which 25% to 30% of patients will develop gastrointestinal bleeding from telangiectases. The extent of telangiectases has not been previously evaluated. This cross-sectional study compared the presence, number, and size of telangiectases in the stomach and duodenum to those in the jejunum using enteroscopy.

Pulmonary arteriovenous malformations in children: outcomes of transcatheter embolotherapy.

Objective: To describe outcomes of transcatheter embolotherapy (TCE) in children with pulmonary arteriovenous malformations (PAVMs).

Study Design: Chart and imaging review of all children (age
Results: All 42 treated patients were included, with a mean age of 12 years (range, 4 to 18).