[HSF] (urgent) Acute dissection/Osler-weber-rendu

[prasannasimha]

I have done one case with hereditary hemorrhagic telangiectasia aka OWR 
disease but it was an AVR. We used a bioprosthesis. His surgery was 
surprisingly uneventful.
Have you CT scanned his brain to rule out a cerebral AVM too ? They can 
have pulmonary AVM's too.
We started him on preop estrogen (supposed to help as they have estrogen 
endothelial receptors) and strictly avoid nasal pasage of any catheter. 
(This patient had h/o GI bleed and also epistaxis). Epistaxis can be 
fatal in them.
Prasanna
Tdmartin2000 at aol.com wrote:
> OK Guys tell me what to do
>
> elderly man with Osler-weber-rendu syndrome that has had multiple mucosal 
> bleeding problems his entire life and has had over 200 u of PRBC's in his life 
> that now has an acute type 1 dissection and a HCT of 23 and multiple blood 
> antibodies. At present he is about 18 hrs out from his acute event, is clinically 
> stable with no pain, BP in the low 100's, mild AI and no pericardial effusion. 
> At present our plan is to wait at least till tomorrow in order to get enough 
> compatible blood.
> Questions
> 1. Operative results??
> 2. Risk of bleeding post op from GI, lungs etc?
> 3. Any role for medical management only?
>
> Thanks for the prompt responses I know I will get. I have no experience with 
> Osler-weber-rendu and cardiac surgery and from calling around not many do.
>
> Tom Martin
> U of Florida
> Gainesville
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