27 Congenital - Vascular

Question 1

What are the clinical differences b/w a hemangioma and vascular malformation

Answer 1

Vascular malformations are present at birth, grow proportionately with the child, and are associated with distortion or destruction of surrounding bone or cartilage; hemangiomas generally emerge after birth, proliferate and then regress, and do not affect surrounding bone or cartilage.

Question 2

What are the typical clinical features of venous malformations

Answer 2

Soft, compressible, nonpulsatile mass m/c found on lip or cheek w/in H&N; also can be found w/in masseter muscle or mandible

Question 3

What are the clinical features of AVM

Answer 3

brightly erythematous lesions of skin w/ an assoc thrill and bruit

Question 4

What % of infants have a hemangioma by age 1

Answer 4

12%

Question 5

What is the incidence of hemangiomas in premature infants weighing < 1000 g

Answer 5

23%

Question 6

B/w what ages do hemangiomas grow most rapidly

Answer 6

8-18 months

Question 7

What percent of hemangiomas regress by age 7

Answer 7

70%

Question 8

Where exactly are most subglottic hemangiomas located

Answer 8

Posterolaterally and submucosally

Question 9

What % of pts w/ subglottic hemangioma have an assoc cutaneous hemangioma

Answer 9

50%

Question 10

What is m/c tx for hemangiomas

Answer 10

observation, parental reassurance

Question 11

When is intervention for hemangioma warranted

Answer 11

• For massive, ulcerative, disfiguring lesions
• Hematologic, CV, upper aerodigestive tract compromise
• Large periorbital lesions obstructing vision

Question 12

What syndrome is characterized by profound thrombocytopenia a/w hemangioma

Answer 12

Kasabach-Merritt syndrome

Question 13

What are the 4 main types of vascular malformations

Answer 13

Capillary, venous, lymphatic, and arteriovenous malformations

Question 14

What is m/c vascular malformation

Answer 14

Port wine stain (a capillary type)

Question 15

What syndrome is characterized by capillary hemangiomas along the distribution of V1 w/ concomitant capillary, venous, and AVM of the leptomeninges

Answer 15

Sturge-Weber syndrome

Question 16

What is the optimal tx for port wine stains

Answer 16

Argon laser in darker-skinned adults; flashlamp pulsed tunable dye laser in kids and lighter skinned adults

Question 17

Which lesions respond best to PDL

Answer 17

Lesions less than 20 cm2 in kids < 1 year old

Question 18

What are the 4 categories of lymphatic malformations

Answer 18

Capillary, cavernous, cystic (hygroma), and lymphangiohemangioma

Question 19

Which lymphatic malformation is a/w episodic bleeding

Answer 19

Lymphangiohemangioma

Question 20

Which lymphatic malformation is m/c found on tongue or floor of mouth

Answer 20

capillary

Question 21

Which lymphatic malformation is a/w location in posterior triangle of neck

Answer 21

Cystic hygroma

Question 22

Which lymphatic malformation is more likely to rapidly enlarge during an upper respiratory tract infection

Answer 22

Lymphangiohemangioma

Question 23

“An infant has a left-sided suprahyoid lymphatic malformation. Using the DeSerres classification system, which class is this?

Answer 23

II

Question 24

What are the indications for definitive tx of lymphatic malformations

Answer 24

When vital structures are endangered, when episodic hemorrhage occurs, or if macroglossia is present

Question 25

Which lymphatic lesions are less likely to respond to sclerosis with OK-432 (Picibanil)

Answer 25

Microcystic, previously operated on, and those with massive craniofacial involvement

Question 26

What condition is seen in adolescent pts with severe frequently recurring epistaxis and pulm AVMs

Answer 26

HHT (Auto Dom)

Question 27

What are the advantages of using argon plasma coagulation for tx of HHT

Answer 27

• Noncontact application
• Limited and controlled tissue penetration with low risk of septal perforation
• No safety measures required (i.e. for lasers)
• Low thermal damage to adjacent tissue
• Inexpensive