Table Of Contents
Fibromuscular Dysplasia (FMD)
Description
- Fibromuscular Dysplasia (FMD) is a non-inflammatory, non-atherosclerotic condition primarily affecting young to middle-aged women.
- Characterized by proliferation of connective tissue and muscle fibers within arterial walls, leading to stenosis and ischemia of the affected organs.
Epidemiology
- Sex → More common in females (♀ > ♂, 8:1).
Disease Localization
- Renal arteries (75–80% of cases, often bilateral).
- Carotid and vertebral arteries (may cause TIA, stroke).

String of beads sign in fibromuscular dysplasia - Angiography
Clinical Features
- Renal FMD
- Symptoms ⇒ Secondary hypertension, abdominal/flank pain, chronic kidney disease.
- Findings ⇒ Abdominal bruit, costovertebral angle bruits.
- Cerebrovascular FMD
- Symptoms ⇒ Headache, neck pain, pulsatile tinnitus, TIA, stroke, Horner's syndrome, amaurosis fugax.
- Findings ⇒ Cervical (carotid) bruit.
Diagnosis
- Best initial tests ⇒ Duplex ultrasonography or CT angiography.
- Gold standard ⇒ Digital subtraction angiography.
- Key finding ⇒ “String of beads” sign on angiography.
Treatment
- Renal FMD
- Antihypertensive therapy
- Definitive treatment → Balloon angioplasty (without stenting).
- Cerebrovascular FMD
- Stroke prophylaxis → Antiplatelet drugs (e.g., low-dose aspirin).
Hereditary Hemorrhagic Telangiectasia (HHT)
Definition
- A hereditary, systemic vasculopathy characterized by telangiectasia on the skin and mucosa, particularly in the area of the face (nose, lips, tongue)
- Pattern of inheritance → autosomal dominant
Clinical features
- Recurrent epistaxis
- Telangiectasia
- Cyanosis
Diagnosis
- Diagnostic criteria for HHT (Curaçao criteria)
- Epistaxis
- Telangiectasias involving the skin and mucous membranes
- Family history of HHT
- Signs of visceral involvement (e.g., pulmonary, gastrointestinal, cerebral arteriovenous malformations or telangiectases)
- Genetic testing

Telangiectasia
💎 Small dilated blood vessels with a threadlike appearance are visible.
Management
Complications
- CNS
- Brain abscess → paradoxical bacterial embolization across pulmonary AVM
- Hernorrhagic CVA
- Cardio - Pulmonary
- High-output cardiac failure
- Pulmonary → hernoptysis
- Pulmonary arterial hypertension.→ right-sided heart failure