Term
| what are the two types of vascular diseases |
|
Definition
vascular malformations
vascualr tumors - mostly hemangomia |
|
|
Term
| what are the two types of vascualr malformations where do they stem from |
|
Definition
low flow: capillaries, venous, lymphatic, combinations
high flow: arteries |
|
|
Term
| describe the morphology of a vascular malformation |
|
Definition
dysplastic vessels with no endothelial proliferation
surrounded by fat |
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Term
| how does a vascular malformation behave over time |
|
Definition
growth is proportional to patient's growth
never regresses |
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|
Term
| what are the two types of vascular tumors |
|
Definition
hemangomias
kaposiform hemangioendthelioma/ruffled angioma |
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|
Term
| what are the two types of hemangomias |
|
Definition
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|
Term
| what are the two types of infantile hemangomia |
|
Definition
|
|
Term
| what is the morphology of a congenital hemangomia |
|
Definition
| do NOT have glut-1 transporters |
|
|
Term
| how does a congenital hemangomia behave over time |
|
Definition
| begins to go away very soon (lots of change on follow up visits) |
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|
Term
| what is the tx for congenital hemangomia |
|
Definition
| none, regresses on its own quickly |
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|
Term
| what are two types of congenital hemagomia |
|
Definition
rapidly involuting
non-involuting |
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|
Term
| what is the morphology of infantile hemangomia |
|
Definition
| GLUT-1 endothelial glucose transpoorters on blood endothelial barrier and placenta BBB |
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|
Term
| how does a infantile hemangomia behave over time |
|
Definition
| begins to go away after a year old in involution |
|
|
Term
| what are the three stages of a infantil hemangomia |
|
Definition
endothelial proliferation
involution in childhood
disspearance by adolescence |
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|
Term
| what does a hemangomia look like in the endothelial proliferation stage |
|
Definition
begins pale/purple and proliferates over a year
if cutaneous/superificial dermis: raised, bosselated, crimson color |
|
|
Term
| what cells, enzymes, etc are in a proliferating hemangomia and drive its activities (6) |
|
Definition
MATURE AND IMMATURE ENDOTHELIAL CELLS and mast cells with surface markers with aklakine phosphatase and factor VII antigen
mast cells with antiogenic factors or anti-antiogenic blockers
pericytes, fibroblasts
decrease in apoptosis |
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|
Term
| what does an involuting hemangomia look like |
|
Definition
| gray blue center, marbling meat |
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|
Term
| what cells, enzymes, etc are in a involuting hemangomia that drive its activities (3) |
|
Definition
decreased angiogenesis stimulators
increased angiogenesis inhibitors
increased apoptosis |
|
|
Term
| what if a hemangomia is not gone by adolescence |
|
Definition
| it might not be a hemangomia and is a big concern |
|
|
Term
| what is the most common tumor of childhood |
|
Definition
|
|
Term
| who least oftens gets hemangomias (4) |
|
Definition
| asians, african americans, boys, dark skin |
|
|
Term
| who most often gets hemangomias (3) |
|
Definition
| fair skin, 30% INCIDENCE IN PREMATURE INFANTS, 10% incidence with family history |
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|
Term
| what are the associates syndromes of hemangomias (which hemangomias have other related complications) (7) |
|
Definition
bearded distribution
lumbosacral hemangomias
multiple cutaneous hemangomia
GU hemangomias
liver hemangomias
diffuse hemangomias
PHACE syndrome |
|
|
Term
| what is a bearded hemangomia distribution |
|
Definition
| located in both pre-auricular areas and chin |
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|
Term
| why is bearded hemangomia distribution a concern, how can it be identified |
|
Definition
| CHECK TO SEE IF THERE ARE SOME ON THE NECK or if the baby has RSV, infection, etc because it could mean they have one in the trachea area causing airway obstruction |
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|
Term
| if a baby has genitourinary area hemangomia what is the concern |
|
Definition
| treat agressivly because there is a high complication of ulceration and pain |
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|
Term
| what abnormalities is a lumboscaral hemangomia associated with |
|
Definition
neurological
GU abnormalities
renal abnormalities
bony sacral abnormalities
leptomeningocele
imperforate anus |
|
|
Term
| what test should all babies with lumbosacral hemangomia get before 6 weeks, why |
|
Definition
ULTRASOUND TO LOOK FOR TEATHERED CORD
lumbosacral hemangomia is associated with it
if later than 6 weeks the posterior vertebrae fuse and it is difficult to see and you have to sedate baby and get MRI |
|
|
Term
| what defines multiple cutaneous hemangomias |
|
Definition
|
|
Term
| what complications are multiple cutaneous hemangomias associated with |
|
Definition
internal hemangomia especially in liver (also in eye, cns, pancreas, GI, lung, spleen, airway)
GI, bleeds, heart failure, liver damage |
|
|
Term
| what is the main condition associated with liver hemangomia |
|
Definition
| HYPOTHYROIDISM (DONT MISS THIS!) |
|
|
Term
| what causes hypothyroidism |
|
Definition
| T3/T4 are active but degraded by type 3 D3 enzyme |
|
|
Term
| why is a liver hemangomia a concern, when should you have this concern |
|
Definition
sometimes it just looks like a hemangomia but is really a hepatoblastoma
get concerned with abdominal distension and raise in AFP |
|
|
Term
| what are the two concerns with diffuse hemangomia |
|
Definition
could look like hemangomia but be a tumor
could change into a tumor over time |
|
|
Term
| what are the symptoms of PHACE syndrome |
|
Definition
| hemangomias down the center of the child |
|
|
Term
| what is the main concern with PHACE syndrome |
|
Definition
| 20% of infants with large cervicofacial hemangomias with have associations with PHACE syndrome |
|
|
Term
| what conditions are assoicated with PHACE syndrome (4), which is the biggest concern |
|
Definition
posterior fossa malformations: BIG CONCERN get MRI to exclude ALWAYS
arterial and cardiac malformations
eye abnormalities
sternal cleft or supraumbilical raphe syndrome |
|
|
Term
| what are the 9 complications of hemangomia |
|
Definition
disfigurment
altered development
ulceration
infection
local hemorrhage
compressed viral structrures
high output cardiac function
kassabach merrit phenomenon (KMP)
psychological issues |
|
|
Term
| what is the #1 reason to treat a hemangomia |
|
Definition
| compressed vital structures like airway, eye, developmental |
|
|
Term
| what is the #1 reason people come to doctor for hemangomia |
|
Definition
| psychological (parents are worse than kids) |
|
|
Term
| what is a warning sign ulceration of hemangomia might occur |
|
Definition
|
|
Term
| how is a hemangomia diagnosed (3) |
|
Definition
ACUTE DIAGNOSIS ESSENTIAL
coordnate cellular, clinical, and historical evidence
radiographs for deep SC, intramucular, visceral lesion
biopsy if malignancy is suspected |
|
|
Term
| who is needed to treat a hemangomia |
|
Definition
multi discipline
hematology, oncology, radiology, dermatology, surgery (plastic, pediatric, otolaryngology), endocrinology, cardiology, basic scientist, family |
|
|
Term
| what are 4 indications for hemangomia tx |
|
Definition
interferes with vital structures/life threatning
possability of permanent scaring
large facial hemangomias
ulcerations |
|
|
Term
| how should hemangomia tx be approached |
|
Definition
| depends on size, location, complications, age, rate of growth |
|
|
Term
| how are hemangomia ulcerations tx |
|
Definition
|
|
Term
| how are visual disturbances from hemangomia treated |
|
Definition
| patch good eye to increase stimulation in obstructed eye |
|
|
Term
| what are 7 venues of tx for hemangomia, what is the most common first line of action |
|
Definition
observation
vincristine
cytoxan
laser therapy
steroids - common first line
interferon
propranolol |
|
|
Term
|
Definition
|
|
Term
|
Definition
| anti-angiogenic decreased endothelial cells and causes apoptosis |
|
|
Term
| steroid side effects (13) |
|
Definition
cushing facies / acne
buffalo hump
personality changes
weight gain
decreased growth
immune supression: no live vaccines
non-systemic fungal infection
HTN
myositis
osteoperosis
cataracts
hypothalamic-pituitary dysfunction
pituitary adrenal dysfunction |
|
|
Term
| how must somone on hemangomia steroid therapy be monitored |
|
Definition
BP, urine, stool, physical exam every 2 weeks
temp |
|
|
Term
| interferon administration |
|
Definition
|
|
Term
|
Definition
| anti-angiogenic by down regulation of FGF |
|
|
Term
| how must someone on interferon for hemangomia be monitered |
|
Definition
| weekly neurological exam, CBC, LFT, thyroid tests |
|
|
Term
|
Definition
neurotoxicity, spastic dysplegia (permanent), developmental delay, flu
anemia, neutropenia, altered liver enzymes, mood changes |
|
|
Term
|
Definition
| non-se;lective B1/B2 antagonist that stabilizes the membrane causing vasodilation, angiogenesis, apoptosis |
|
|
Term
| why is propranolol an advance in hemangomia tx |
|
Definition
| can be used in older pt and at any stage of hemangomia |
|
|
Term
|
Definition
| rebound if withdrawl too early |
|
|
Term
| who usually gets kaposiform hemangioeddothelioma, when |
|
Definition
equal in males and females
usually at birth
sometimes post natal or young adult |
|
|
Term
| what do the lesions look like in kaposiform hemangioeddothelioma |
|
Definition
non focal: trunk, neck, head, extremities
retroperitoneal and deep tissue
red/purple, tight, shiny |
|
|
Term
| what is an non-lesion symptom fo kaposiform hemangioeddothelioma |
|
Definition
| can occur with KMP but platelet tapping is often noted |
|
|
Term
| how is kaposiform hemangioeddothelioma diagnosed |
|
Definition
| T2MRI with hyperintensive lesion with ill defined margins involving several tissue layers |
|
|
Term
| what vessel and lymph signs are there in kaposiform hemangioeddothelioma |
|
Definition
compressed endothelium spindled and dilated
dilated and hyperplastic lymphatic vessesl |
|
|
Term
| what serological / morphological factors can identify kaposiform hemangioeddothelioma |
|
Definition
CD21, CD34, FLI1 positive cells
LwY and HHV-8 negative cells
GLUT-1 NEGATIVE!!
epitheloid islands with CD61 positive fibrin thrombi |
|
|
Term
| what are the 4 tx for kaposiform hemangioeddothelioma, which is the best |
|
Definition
surgery - best if possible
embolization prevention
KMP tx and reoccurance prevention
rampamycin |
|
|
Term
|
Definition
| tk receptor inhibitor stops cellular growth and proliferation |
|
|
Term
| why does rampamycin work for kaposiform hemangioeddothelioma |
|
Definition
| CAN WORK THROUGH DIFFERENT MECHANISMS LIKE CANCER TX DOES |
|
|
Term
| what is the prognosis of kaposiform hemangioeddothelioma |
|
Definition
50% mortality
WILL NEVER FULLY REGRESS AND MAY COME BACK |
|
|
Term
| what are 5 complications of kaposiform hemangioeddothelioma |
|
Definition
embolization
lymphadema
chronic pain
orthopedic issues |
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