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cavernous hemangioma (cavernoma, capillary venous lymphatic malformation, CVL, nevus cavernosus, vascular erectile tumor)
Deep vascular malformations, characterized by soft, compressible deep tissue swelling.
Epidemiology: 10% of vascular malformations
Genetics: may occur as autosomal dominant condition
Pathology:
1) dilated, blood-filled vascular spaces with flattened endothelium
2) vessels may be capillary, venous, lymphatic or cavernous
Clinical manifestations:
1) lesions not present at birth, but become apparent during childhood
2) soft tissue swelling, dome shaped or multinodular
3) nodules blue to purple
4) borders poorly defined
5) when vascular malformation extends into the epidermis, surface may be verrucous
6) easily compressed, fills rapidly when pressure is released
7) may be tender
8) may result in diffuse swelling & enlargement of extremity
9) may involve skeletal muscle with muscle atrophy
10) dilated tortuous cutaneous veins & arteriovenous fistulas may be present
Laboratory:
1) clinical diagnosis
2) angiography will not demonstrate cavernomas in the brain
3) magnetic resononce imaging of brain
a) small cavernomas may appear as black dots on T2-weighed images
b) larger cavernomas have central variegated appearance with a decreased rim of signal intensity
Complications:
1) ulceration, bleeding, scarring & secondary infection
2) high output heart failure with large lesions
3) platelet sequestration & destrucion may result in thrombocytopenia (Kasabach-Merritt syndrome)
4) interference with food intake or breathing if located in mucus membranes of mouth, pharynx or larynx
5) if located on eyelids or vicinity of eyes may instantly obstruct vision & may lead to blindness
6) lifetime risk of hemorrhage is 0.25% per person-year
Management:
1) no satisfactory treatment except compression
2) for larger lesions or compromise of organ function
a) surgery
b) intravascular coagulation
3) high dose systemic corticosteroids
4) interferon alpha
Related
angiomatous nevus; strawberry nevus; capillary hemangioma of infancy
cerebral cavernous malformation
Kasabach-Merritt syndrome
verrucous; vericose
Specific
blue rubber bleb nevus
General
nevus
hemangioma (strawberry nevus)
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