Percutaneous trans-luminal managment of the vascular manifestations of neurofibromatosis type 1
1. Review the clinical manifestations of Neurofibromatosis type 1 (NF1). 2. Discuss the vascular pathology associated with NF1. 3. Review the clinical presentation, diagnosis, and percutaneous managment of NF1 related renal artery stenosis, middle aortic syndrome, and cerebral artery disease.
Neurofibromatosis 1 (NF1) is an autosomal dominant disorder found in approximately 1 out of every 3,000 individuals. The disorder results from a microdeletion in the NF1 gene on chromosome 17q11.2 that results in premature translation termination of the neurofibromin protein. The classic clinical pr[...]
Findings and procedure details:
Renal arterial involvement NF1 vasculopathy most commonly affects the renal artery. Renal artery stenosis (RAS) is a more common presentation than renal artery aneurysms. Patients with RAS typically present with hypertension and/or renal dysfunction. The initial diagnostic modality of choice is ultr[...]
The management of NF1 vasculopathy involves a combination of medical therapy with either surgical or percutaneous interventions. Specific indications for intervention are poorly defined; however, the overall goal is symptom alleviation. Appropriate patient selection based on the degree of vascular i[...]
Ahmed Kamel Abdel Aal , MD, PhD, FSIR University of Alabama at Birmingham (UAB). Department of Radiology Division of Interventional Radiology. United States , Alabama. Address: 619 19th Street South, Birmingham, AL 35249. Tel: (205) 975-4850 Fax: (205) 975-5257. Email: firstname.lastname@example.org Twitter: ahm[...]
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