Vascular Malformations

Front 1

What findings are associated with Klippel-Trenaunay syndrome?

Back 1

Port wine stain of one extremity overlying a venous or lymphatic malformation with limb hemihypertrophy

Front 2

What is the inheritance pattern of Rendu-Osler-Weber syndrome?

Back 2

Autosomal dominant

Front 3

How does Rendu-Osler-Weber syndrome present?

Back 3

epistaxis, hematuria, hematemesis and melena

Hint 3

bleeding

Front 4

What is Rendu-Osler-Weber syndrome?

Back 4

Multiple ectatic vessels involving skin, mucous membranes and visceral organs?

Hint 4

Ectatic vessels

Front 5

What is Parkes-Weber syndrome?

Back 5

Port wine stain overlying venous and lymphatic malformations of upper extremity with AV fistulas and skeletal hypertrophy

Front 6

What syndrome is associated with vascular malformations, mental retardation and seizures?

Back 6

von Hippel-Lindau disease

Front 7

What vascular malformations are found in von Hippel-Lindau disease?

Back 7

Hemangioms of retina and hemangioblastomas of cerebellum and visceral organs

Hint 7

retina, cerebellum

Front 8

What is proteus syndrome?

Back 8

Partial gigantism of exremities, hemifacial hemihypertrophy, macrocephaly, macrodactyly, localized exostoses, subcutaneous lipomas and vascular malformations.

Front 9

In Sturge-Weber syndrome a large vascular malformation is found in the distribution of what nerve?

Back 9

Trigeminal (V1, V2)

Front 10

What is the most common vascular malformation found in Sturge-Weber syndrome?

Back 10

port wine stain

Front 11

What neurological findings are associated with Sturge-Weber syndrome?

Back 11

focal motor seizures, hemiparesis, visual field defects, glaucoma and mental retardation

Hint 11

nevr0, visual, psych

Front 12

What is the cause of mental retardation in Sturge-Weber syndrome?

Back 12

Leptomeningeal venous malformations and intracranial calcifications

Hint 12

Calcification

Front 13

What vascular malformation contains a central blanching arteriole?

Back 13

Spider angioma (arteriolar malformation)

Front 14

What is the treatment for spider angiomas?

Back 14

Obliteration of the central feeding arteriole with laser or electrocautery.

Front 15

What percentage of hemangiomas are noted within the first year of life?

Back 15

80%

Front 16

What is the natural history of hemangiomas?

Back 16

Present at (or near) birth, proliferate for several months then spontaneously regress

Front 17

Hemangiomas contain an increased quantity of what type of cells?

Back 17

mast cells

Front 18

The mast cells in hemangiomas produce what protien?

Back 18

Heparin

Front 19

What are the histologic findings of hemangiomas?

Back 19

Proliferative primitive endotheilial cell nests in syncytial masses with or without vessel lumens. Hyperplasia

Front 20

What is the most common tumor of infancy?

Back 20

Hemangioma

Front 21

Which vascular malformation is characterized by compressiblility and fills with blood when body position changes?

Back 21

Venous malformation

Front 22

How can a venous malformation affect the function of an extremity?

Back 22

By intertwining with neurovascular structures

Front 23

How do venous malformations cause pain?

Back 23

thrombosis

Front 24

Which vascular malformation is limited to capillaries in the dermis?

Back 24

Port wine stain (capillary malformation)

Front 25

If left untreated what will happen to a port wine stain?

Back 25

Cobblestoning, ectasia and progressive darkening (from deoxyhemoglobin)

Front 26

What syndrome is associated with a port wine stain?

Back 26

Sturge-Weber syndrome

Front 27

What is the treatment for port wine stains?

Back 27

585nm laser

Front 28

What are the physical findings associated with Klippel-Feil syndrome?

Back 28

short neck and low posterior hairline with fusion of cervical vertebrae

Front 29

What syndrome is associated with fusion of cervical vertebrae?

Back 29

Klippel-Feil

Front 30

What are the physical findings associated with an AV malformation?

Back 30

pulsation, temperature changes, bruit, thrill. Does not decompress fully with arm elevation

Front 31

How do you make the diagnosis of an AV malformation?

Back 31

MRA (MRI with iv gadolinium)

Front 32

What is the treatment for an ulcerated or bleeding AV malformation?

Back 32

Aggressive resection with immediate flap coverage

Front 33

How do superficial lymphatic malformations present?

Back 33

Clear vesicles

Front 34

Bone overgrowth is associated with which vascular malformations?

Back 34

Deep lymphatic malformations

Front 35

What is the treatment for lymphatic malformations?

Back 35

Erbium laser (2940 nm)

Front 36

What vascular malformation occurs secondary to trauma?

Back 36

pseudoaneurysm

Front 37

What is Kassabach-Merritt syndrome?

Back 37

Profound thrombocytopenia associated with solitary hemangioma or diffuse hemangiomatosis

Hint 37

thrombocytopenia

Front 38

What percentage of patients with Maffucci syndrome develop chondrosarcoma?

Back 38

20%

Front 39

What is Maffucci syndrome?

Back 39

Multiple enchondromas and cavernous hemangiomas associated with limb foreshortening.