cavernous hemangioma (cavernoma, capillary venous lymphatic malformation, CVL, nevus cavernosus, vascular erectile tumor)
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Introduction
Deep vascular malformations, characterized by soft, compressible deep tissue swelling.
Epidemiology
10% of vascular malformations
Pathology
- dilated, blood-filled vascular spaces with flattened endothelium
- vessels may be capillary, venous, lymphatic or cavernous
Genetics
may occur as autosomal dominant condition
Clinical manifestations
- lesions not present at birth, but become apparent during childhood
- soft tissue swelling, dome shaped or multinodular
- nodules blue to purple
- borders poorly defined
- when vascular malformation extends into the epidermis, surface may be verrucous
- easily compressed, fills rapidly when pressure is released
- may be tender
- may result in diffuse swelling & enlargement of extremity
- may involve skeletal muscle with muscle atrophy
- dilated tortuous cutaneous veins & arteriovenous fistulas may be present
Laboratory
- clinical diagnosis
- angiography will not demonstrate cavernomas in the brain
- magnetic resononce imaging of brain
- small cavernomas may appear as black dots on T2-weighed images
- larger cavernomas have central variegated appearance with a decreased rim of signal intensity
Complications
- ulceration, bleeding, scarring & secondary infection
- high output heart failure with large lesions
- platelet sequestration & destrucion may result in thrombocytopenia (Kasabach-Merritt syndrome)
- interference with food intake or breathing if located in mucus membranes of mouth, pharynx or larynx
- if located on eyelids or vicinity of eyes may instantly obstruct vision & may lead to blindness
- lifetime risk of hemorrhage is 0.25% per person-year
Management
- no satisfactory treatment except compression
- for larger lesions or compromise of organ function
- surgery
- intravascular coagulation
- high dose systemic corticosteroids
- interferon alpha
More general terms
More specific terms
Additional terms
- angiomatous nevus; strawberry nevus; capillary hemangioma of infancy
- cerebral cavernous malformation
- Kasabach-Merritt syndrome
- verrucous; vericose
References
- ↑ Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 154-55
- ↑ Medical Knowledge Self Assessment Program (MKSAP) 11, American College of Physicians, Philadelphia 1998
- ↑ NINDS Cavernous Malformation Information Page https://www.ninds.nih.gov/Disorders/All-Disorders/Cerebral-Cavernous-Malformation-Information-Page