Capillary malformation-arteriovenous malformation (CM-AVM) syndrome is an autosomal dominant condition characterized by multifocal, noncontiguous pink patches on the skin that often have a surrounding pale halo. In some cases, an association with a fast flow, arteriovenous malformation (AVM) can be identified. Here, we describe a case report of a 16-year-old woman with CM-AVM syndrome and significant cardiac compromise successfully treated with trametinib, a mitogen-activated protein kinase (MEK) inhibitor.

Download full-text PDF

Source
http://dx.doi.org/10.1111/pde.14912DOI Listing

Publication Analysis

Top Keywords

arteriovenous malformation
12
syndrome cardiac
8
cardiac compromise
8
cm-avm syndrome
8
successful management
4
management arteriovenous
4
malformation
4
malformation trametinib
4
trametinib patient
4
patient capillary-malformation
4

Similar Publications

BACKGROUND Arterioportal fistulas (APFs) are abnormal connections between the arterial and portal venous systems, leading to portal hypertension (PH) and symptoms such as gastrointestinal bleeding, splenomegaly, and hepatic pain. Symptoms typically appear by the age of 2 years in about 75% of cases. CASE REPORT A 7-year-old boy with an asymptomatic APF developed life-threatening complications following a Clostridium difficile infection.

View Article and Find Full Text PDF

Background: Lung transplantation is a viable lifesaving option for patients with diffuse pulmonary arteriovenous malformations (AVMs). We present a case of diffuse pulmonary AVMs associated with juvenile polyposis and hereditary hemorrhagic telangiectasia (JP-HHT) that was successfully managed by lung transplantation.

Case Presentation: A 19-year-old woman developed severe hypoxemia due to pulmonary AVMs diagnosed at 4 years of age.

View Article and Find Full Text PDF

Arteriovenous malformation (AVM, arteriovenous dysplasia) is one of the variants of congenital vascular defects formed as a result of a defect in the development of the arterial and venous systems during ontogenesis with the formation of direct messages between vessels of different diameters. In this regard, high-speed shunting of blood from the arterial part of the vascular system to the venous through fistulas of various calibers occurs. This disease is characterized by a variety of clinical manifestations.

View Article and Find Full Text PDF

Indocyanine green video angiography, integrated into the operative microscope, is frequently used in cerebrovascular surgery. This technology is often preferred, for cost or availability, to Doppler or intraoperative DSA (digital subtraction angiography). With the same assumption it was possible, in our preliminary experience, to partially vicariate the aforementioned devices using the SPY mode of the Stryker endoscope; it allowed the visualization of fluorescence in high definition.

View Article and Find Full Text PDF

Objective: This study initiated a preliminary computational fluid dynamics (CFD)-based study to investigate the relationship between quantitative hemodynamics of arteriovenous malformation (AVM) draining veins and rupture.

Methods: The quantitative hemodynamics of AVM draining veins were generated from computed tomography angiography (CTA)-based steady-state CFD models. Morphological and hemodynamic parameters were compared between the ruptured and unruptured groups.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!