Chapter 54 - Vascular Malformations

Question 1

Absolute indication for vascular anomaly treatment

Answer 1

ocular or airway obstruction

ulceration, hemorrhage, may lead to long-term cosmetic/functional problems (ear/nose), psychosocial trauma

Question 2

Which lab differentiates hemangioma from vascular malf

Answer 2

GLUT-1 (hemang +)

Rapid involuting congenital hemangioma and non-involuting congenital hemangioma may be negative

Question 3

Which type of laser should be used for a port-wine stain (cutaneous capillary malformation)

Answer 3

Pulsed-dye laser

Question 4

How does the growth of vascular tumors and malformations differ?

Answer 4

Tumor (hemangioma)- rapidly enlarging by endothelial proliferation, then involute spontaneously

Malf- no proliferation, but progressive dilation, grows in proportion to child

Question 5

Percentage of hemangiomas noted within first month of life

Answer 5

80%

Question 6

How long can proliferative phase last for hemangioma?

Answer 6

12 mo

Question 7

Difference between red and blue hemangiomas

Answer 7

Bright red/crimson - superficial

Bluish- deeper

Question 8

How does propranolol work for hemangiomas. Success rate. AE

Answer 8

Mechanism not fully understood but likely vasoconstriction –> reduce lesion volume/soften lesion/apoptosis

97% response rate

AE bronchospasm, hypoglycemia, GERD, hypotension, somnolence

May be able to use topical timolol rather than po propranolol

Question 9

Treatments other than propranolol for hemangioma

Answer 9

Steroid inject - constrict/downregulate VEGF, mainstay prior to propranolol, 50-90% response rate, multiple treatments, 6-8wk intervals, cannot do if near eye, AE growth retardation/cushing/gastric

IFNa-2a: angiostatic, second line only or for recurrent/refractory.

Photocoag - pulsed dye (superficial), argon (ulcerated/active bleeder), Nd:Yag (deep penetration of dermis)

Surgically excised - during involution phase or proliferative if unresponsive

Question 10

Side effect (major one) of IFN-a-2a

Answer 10

25% spastic diplegia

Question 11

Side effect of steroid injection for periorbital hemangioma

Answer 11

central retinal artery occlusion

Question 12

How is vincristine used for vascular malformations

Answer 12

For Kasabach Merritt
Mitotic inhibition
Neurotoxic, requires central access, 4-6mo tx

Question 13

Kasabach Merritt

Answer 13

Rapidly enlarging hemangioma, platelet trapping, hemolytic anemia, thrombocytopenia
Kaposiform hemangioendothelioma
Tufted angioma

Question 14

PHACES

Answer 14

posterior fossa
hemangioma (segmental/dermatomal)
arterial
cardiac (Coarc Aorta)
Eye coloboma
Sternal/ventral developental defect
Need 2/6

Question 15

How many beard distribution hemangiomas have an airway hemangioma?

Answer 15

30-65%, usually subglottic

Question 16

Describe port wine stain

Answer 16

superficial capillary VM

present at birth, shar demarcation, darkens over time, grows with child

Question 17

Sturge Weber components

Answer 17

port wine (V1)
glaucoma, seizures, mental retardation, dural involvement

Question 18

Types of lymphatic malformations

Answer 18

Macrocystic (hygroma) >2cm^3

Micro (lymphangioma) <2cm

Question 19

Sclerosing agents for lymphatic malformations

Answer 19

doxycycline, bleomycin, ethanol, OK-432 (picibanil)

Question 20

Imaging for lymphatic malformation

Answer 20

U/S

MRI w/ gad and fat suppression…lights up on T2, fluid/level

Question 21

How do AVMs present?

Answer 21

Often mild in childhood

Can cause local destruction of bone/ST in adulthood