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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.
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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
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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.
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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
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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.
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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.
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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
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Treatment of congenital hemangiomas.
- RICHs will spontaneously involute, medical therapy is ineffective
- NICHs require surgical/laser excision
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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)
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HOIs in which areas of the face are prone to complications and noninvolution?
midface lesions. Embryonic fusion lines
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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
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What percentage of children with segmental HOIs in the lower face have associated airway lesions?
at least 50%
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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.
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Lymphatic malformations in these locations tend to be microcystic and resistant to treatment
- Suprahyoid
- Midline (medial to lateral canthus)
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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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