Hemangioma, vascular malformations
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How does prognosis of cavernous hemangioma differ compared to capillary hemangioma?
- Cavernous may enlarge more rapidly
- Less chance of regression, though 60% resolve spontaneously by 4-6 years old.
This abnormality is absent at birth, appears during infancy, grows rapidly within the first year of life, and then undergoes a variable period of involution.
Hemangioma of infancy
What are the components of PHACES syndrome?
Posterior fossa abnormalities, hemangiomas, arterial anomalies, cardiac anomalies, eye abnormalities, sternal or abdominal clefting). CNS abnormalities include developmental delays, seizures, and congenital stroke.
Most vascular malformations, but particularly lymphatic malformations have what clinically and radiographically apparent effect on surround soft tissue and bone.
either local tissue or bony over-growth
Ways of categorizing hemangiomas
- Segmental vs focal. Segmental HOIs usually are superficial, variably involve cutaneous dermatomes, and have more associated morbidity. Focal HOIs tend to be at embryonic fusion lines
- Deep vs superficial.
In patients suspected to have PHACES syndrome what work up is appropriate?
- head imaging, ophthalmologic examination, and cardiac evaluation.
- Some clinicians recommend antiplatelet therapy for patients with abnormal cerebral vasculature, to prevent congenital stroke.
Congenital hemangiomas and HOI are histologically identical, though one will stain positive for this marker.
HOI stain positive for GLUT-1, congenital hemangiomas don't
Treatment of congenital hemangiomas.
- RICHs will spontaneously involute, medical therapy is ineffective
- NICHs require surgical/laser excision
The two most common nonhemangioma vascular tumors?
- Kaposiform hemangioendothelioma (KHE). Significant lymphatic component in addition to blood vascular endothelium. KHEs appear as violaceous cutaneous nodules extending into deep tissues locally.
- Tufted angioma (TA). More localized and may or may not involve the skin.
- Both benign, dx by incisional biops
- assoc with Kasabach-Merritt phenomenon (profound thrombocytopenia)
HOIs in which areas of the face are prone to complications and noninvolution?
midface lesions. Embryonic fusion lines
Which laser is used for treatment of superficial HOIs?
- pulsed dye laser. This device is advantageous because it spares the superficial epidermis and allows for treatment of cutaneous redness without scarring. Does not reduce HOI volume.
- When the HOI is actively proliferating, it can induce epithelial disruption, ulceration, and scarring. Not recommended in children under 6 months
What percentage of children with segmental HOIs in the lower face have associated airway lesions?
at least 50%
Lymphatic malformation with what characteristics are likely to resolve without intervention?
Macrocystic malformations in the posterior neck, without septations, that are infrahyoid can resolve without treatment.
Lymphatic malformations in these locations tend to be microcystic and resistant to treatment
- Midline (medial to lateral canthus)
Skin capillaries that are persistently dilated form malformations frequently seen in the mid- and upper face. These are known as ________ and are associated with _________ syndrome.
- port-wine stains
- Sturge-Weber syndrome, characterized by capillary malformations involving the eye, skin, and leptomeninges
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