Chapter 173 - congenital vascular malformations endovascular management

Question 1

Classification of vascular anomalies by the International Society for the Study of Vascular Anomalies

Answer 1

TABLE 173.1

Question 2

Vascular anomalies types

Answer 2

Vascular malformation 2/3 Vascular tumors 1/3

Question 3

SE Mitchell vascular anomalies flow chart

Answer 3

FIGURE 173.1

Question 4

TWIST sequence of MRI

Answer 4

contrast-enhanced Time-resolved angiography with interleaved stochastic trajectories

Question 5

MRI features of vascular anomalies

Answer 5

TABLE 173.2

Question 6

Hereditary venous malformations

Answer 6

1-2% of all venous malformations 1) glomuvenous malformation 2) cutaneous venous malformation 3) cerebral cavernous venous malformation 4) blue rubber bleb nevus syndrome

Question 7

Histology of venous malformations

Answer 7

post-capillary endothelium lined venous channels with abnormal mural smooth muscle

Question 8

Histology of lymphatic malformation

Answer 8

dysplastic endothelium-lined lymphatic channels

Question 9

Hereditary lymphatic malformations

Answer 9

1) Turner syndrome 2) Noonan syndrome

Question 10

VM and LM on MRI

Answer 10

Isointense to skeletal muscle on T1 hyperintense on T2 Intralesion hemorrhage will change signal depending on age of hemorrhage LM micro solid, macro cystic VM void areas corresponding to phlebolith

Question 11

first line treatment for low flow vascular malformation

Answer 11

Sclerotherapy

Question 12

Preoperative assessment for sclerotherapy for vascular malformation (low flow)

Answer 12

1) MRI to identify lesion and nearby structures 2) coagulation status (CBC, PTT, INR, fibrinogen, D-dimer) 3) LMWH if LIC for 10 days before and after treatment 4) status of deep venous system

Question 13

Sclerosant used for vascular malformation treatment

Answer 13

1) ethanol 2) sodium tetradecyl sulfate 3) ethanolamine oleate 4) bleomycin 5) OK-432 Picibanil (LM)

Question 14

Treatment of hematuria after sclerotherapy

Answer 14

Sclerosant-induced hemolysis hydration and urinary catheter

Question 15

Complications of sclerosant injection

Answer 15

1) inflammatory complications 2) skin necrosis and wound infection due to extravasation 3) hematuria 4) acute compartment syndrome

Question 16

Other endovascular therapy for low flow VM

Answer 16

1) MRI guided sclerotherapy 2) coil/glue embolization 3) laser therapy 4) sirolimus (mTOR inhibitor)

Question 17

Schobinger staging of AVM

Answer 17

TABLE 173.3

Question 18

Yakes AVM classification

Answer 18

TABLE 173.4

Question 19

Difference between AVM and AVF

Answer 19

AVM has an intervening nidus

Question 20

Example of congenital AVF

Answer 20

Vein of Galen malformation in deep cerebral venous system

Question 21

Recurrence of AVM after surgical + embolic therapy

Answer 21

57% in 1 year 98% in 5 years

Question 22

Benefit of arterial vs venous side embolization of AVM

Answer 22

Arterial 1) reduce bleeding during subsequent surgery 2) higher chance of success Venous 1) less chance of causing ischemia 2) balloon occlusion distally to prevent emboli

Question 23

Syndromes associated with low flow malformations

Answer 23

1) KTS 2) BRBNS 3) unilateral limb VM 4) mucocutaneous VM 5) Surge-Weber syndrome 6) Proteus syndrome 7) congenital lipomatous overgrowth 8) vascular malformation 9) epidermal nevi CLOVE syndrome 10) Maffucci and Gorham-Stout syndrome

Question 24

Syndromes associated with high flow malformations

Answer 24

1) Parkes weber 2) HHT 3) Cobb 4) Wyburn-Mason 5) Bannayan-Riley-Ruvalcaba 6) Cowden

Question 25

Diagnosis of KTS

Answer 25

2/3 of 1) port wine stain 2) low flow malformation 3) soft tissue bony overgrowth

Question 26

Define pseudocapillary malformation

Answer 26

Part of the high-flow vascular malformation Not a true capillary malformation Seen in Parkes Weber

Question 27

Osler-Weber-Rendu

Answer 27

1) autosomal dominant 2) AVM in multi organs (brain, lungs, liver, bowel) 3) Curacao criteria

Question 28

Curacao criteria

Answer 28

Diagnosis for HHT needs 3/4 1) epistaxis 2) telangiectasias 3) visceral lesions 4) family history 1st degree relative HHT

Question 29

HHT with pulmonary AVM treatment

Answer 29

Embolization if > 2-3 mm