Osler-Weber-Rendu syndrome

Question 1

Osler-Weber-Rendu syndrome aka

Answer 1

hereditary hemorrhagic telangiectasia (HHT), AKA capillary telangiectasia
With 🧠 tissue 🔛 cavernous malformation

Question 2

HHT or Osler-Weber-Rendu syndrome flow

Answer 2

Enlarged capillary ⬇️ flow ➡️ risk of hemorrhage very low 🔛 AVM

Question 3

Other syndrom associations with osler Weber rendu

Answer 3

Osler-Weber-Rendu (see below), Louis-Barr (ataxia telangiectasia), Myburn-Mason, Sturge-Weber.

Question 4

High risk of embolization Strok

Answer 4

High risk of pulmonary AVF ➡️ ⬆️ paradoxical cerebral emboli’s

Question 5

Sx

Answer 5

Recurrent epistaxis

Question 6

CT

Answer 6

high density due to hematoma, calcification, thrombosis, hemosiderin deposition, alterations in BBB, and/or increased blood volume1) wedema

Question 7

MRI T2

Answer 7

Hemosidreing
➡️increased and decreased intensity, a prominent surrounding rim of reduced intensity may be present
GRE flow related signal dropout

Question 8

Surgery indication

Answer 8

Hematoma evacuation

Question 9

Capillary telangiectasias are characterized

Answer 9

absence of adjacent gliosis or hemosiderin deposition.

Question 10

📍 location

Answer 10

Pons

Question 11

second most commonly detected vascular malformation in autopsy

Answer 11

Capillary telangiectasia