Saturday, January 31, 2015

Study group discussion: Osler Weber Rendau syndrome

I had once taken a case of Osler Weber rendau syndrome.

It's an autosomal dominant disease..Where AV malformation occur throughout the body.

The case i had was of a 26 yr old lady G6P3L1D2A2
And in her most recent pregnancy, during her 24th week.. She had sudden onset cough and breathlessness.
Later on pulmomary tapping..Blood was withdrawn.

The AV malformation in her lungs had bursted. She was taken for surgery.

Which surgery?

I forgot the name of it. Under angiographic control, the bleeding is sought out and the bleeder clamped.

The baby was taken out by C section at 37th week..Cause it was having an IUGR lag of four weeks.

But from what I reserched was angiogenesis occurs our entire life time.
So as you grow the number of av malformation go on increasing.
In children it presents as epixtasis.
In young adults..bleeding in lungs.
As you grow they may rupture in the brain as well.

I had a case of Osler Weber Rendau syndrome who presented with Haematemesis.
The attending told us to suspect it in patients who do not have signs of liver cell failure (Since portal hypertension is the most common cause of Haematemesis!)

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