Chapter 173 - congenital vascular malformations endovascular management Flashcards
Classification of vascular anomalies by the International Society for the Study of Vascular Anomalies
TABLE 173.1
Vascular anomalies types
Vascular malformation 2/3 Vascular tumors 1/3
SE Mitchell vascular anomalies flow chart
FIGURE 173.1
TWIST sequence of MRI
contrast-enhanced Time-resolved angiography with interleaved stochastic trajectories
MRI features of vascular anomalies
TABLE 173.2
Hereditary venous malformations
1-2% of all venous malformations 1) glomuvenous malformation 2) cutaneous venous malformation 3) cerebral cavernous venous malformation 4) blue rubber bleb nevus syndrome
Histology of venous malformations
post-capillary endothelium lined venous channels with abnormal mural smooth muscle
Histology of lymphatic malformation
dysplastic endothelium-lined lymphatic channels
Hereditary lymphatic malformations
1) Turner syndrome 2) Noonan syndrome
VM and LM on MRI
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
first line treatment for low flow vascular malformation
Sclerotherapy
Preoperative assessment for sclerotherapy for vascular malformation (low flow)
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
Sclerosant used for vascular malformation treatment
1) ethanol 2) sodium tetradecyl sulfate 3) ethanolamine oleate 4) bleomycin 5) OK-432 Picibanil (LM)
Treatment of hematuria after sclerotherapy
Sclerosant-induced hemolysis hydration and urinary catheter
Complications of sclerosant injection
1) inflammatory complications 2) skin necrosis and wound infection due to extravasation 3) hematuria 4) acute compartment syndrome
Other endovascular therapy for low flow VM
1) MRI guided sclerotherapy 2) coil/glue embolization 3) laser therapy 4) sirolimus (mTOR inhibitor)
Schobinger staging of AVM
TABLE 173.3
Yakes AVM classification
TABLE 173.4
Difference between AVM and AVF
AVM has an intervening nidus
Example of congenital AVF
Vein of Galen malformation in deep cerebral venous system
Recurrence of AVM after surgical + embolic therapy
57% in 1 year 98% in 5 years
Benefit of arterial vs venous side embolization of AVM
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
Syndromes associated with low flow malformations
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
Syndromes associated with high flow malformations
1) Parkes weber 2) HHT 3) Cobb 4) Wyburn-Mason 5) Bannayan-Riley-Ruvalcaba 6) Cowden
Diagnosis of KTS
2/3 of 1) port wine stain 2) low flow malformation 3) soft tissue bony overgrowth
Define pseudocapillary malformation
Part of the high-flow vascular malformation Not a true capillary malformation Seen in Parkes Weber
Osler-Weber-Rendu
1) autosomal dominant 2) AVM in multi organs (brain, lungs, liver, bowel) 3) Curacao criteria
Curacao criteria
Diagnosis for HHT needs 3/4 1) epistaxis 2) telangiectasias 3) visceral lesions 4) family history 1st degree relative HHT
HHT with pulmonary AVM treatment
Embolization if > 2-3 mm
