Hi Ciba

Yup, sounds very familiar and can be quite scary to see. It's called a pseudomeningocele and is apparantly not that uncommon. Luca developed this same phenomena after her surgery, also at 13 months old.

Our first attempts to get the spinal fluid re-absorbed was to get her a beany/skull cap type thing originally for children with eczema. It goes over the head down past the ears and around the bottom of the chin so only her face was visible. It kind of looks like the thing astronauts put on their heads before putting on their helmets. We actually used to call her Cosmonaut Luca when she was wearing it. Anyway, this cap put only a very little bit of pressure on the pseudomeningocele and didn't do the trick.

We were quite desparate to get home to South Africa and this pseudomeningocele was delaying us. Had this not been the case, perhaps our surgeon would have suggested we persist with this non-invasive method.

Be this as it may, he gave us 7 surgical options to sort it out. I don't remember all of them, but after further discussion we decided to go with the option whereby he tapped into the spine near the lower back and ran a small tube from there to Luca's stomach subcutaneously (under the skin). This tube would drain the spinal fluid from her spine, therefore reducing the build-up at her surgery site, into her stomach and from there it would just pass through her, as does any waste product.

This worked very well. I don't remember exactly what the timing was, but after about a week the pseudomeningocele was gone.

Luca has a small scar near the bottom of her spine and one just to the right of her stomach where they inserted the tube, and the tube is still inside her but you would never know this from just looking at her. The doctor said there is no reason to take it out, but if we really wanted to, it can be surgically removed.

Our doctor was never overly concerned about her developing this pseudomeningocele and was always very positive that we'd sort it out and that it happens in a number of cases. I know how worrying it looks but I believe it's very fixable if it doesn't sort itself out.

I have photo's of Luca in her cosmonaut disguise if you would like to see what the thing looked like.

I see our daughters seem to be very similar cases. Luca is now 15 months past surgery and doing incredibly well. She still suffers the residual effects of her strokes which is mainly the weakness in her legs, she is struggling to walk but is getting stronger all the time. She's also recovered considerably from a number of other effects from her strokes.

My best advice to you right now is to give your daughter time, don't expect too much from her too soon.

Take care
Mark (Luca's Dad)

Hi Ciba

I'll try to remember to send the pictures later from home otherwise it will be tomorrow.

Before we found the cosmonaut cap our doctor suggested we wrap a bandage around her head to apply some pressure to the pseudomeningocele to aid the re-absorbtion. You might want to try this BUT don't wrap the bandage tight, keep it quite loose - gentle pressure.

I do seem to remember that Luca's first stroke was possibly brought on by an infection. But I believe she would have stroked at some stage anyway because of the moyamoya. She has also had further ear and throat infections since her surgery with no problems.

All the best
Mark

Oh, OK, so you are already applying pressure to it; this is good.

From what I can remember our doctor had had other cases who'd developed this pseudomeningocele. I can't tell you exactly how many, but he certainly wasn't overly surprised by it and explained to us that it happens sometimes and was always very calm and reassuring to us that it will sort itself out. He had never seen the cap before us. He had told us to wrap a bandage around her head to apply pressure so he had no previous experience with the cap. We were at the pharmacy buying bandages when we discovered the cap and after showing him he was quite pleased with it. But it didn't work very well.

The pseudomeningocele developed about 10 days after the bypass surgery and then Luca wore the cosmonaut cap for about 10 days without any improvement; in fact I seem to remember it still grew a bit. The cosmonaut cap provided very little pressure, maybe even too little pressure and this is one of the reasons it wasn't effective. But as I mentioned before, our surgeon might have been happy for us to continue with the cap (or some other form of pressure) had it not been for us wanting to get home to South Africa. He performed the procedure to reduce the pseudomeningocele and after about a week it was almost gone.

An interesting thing with the pseudomeningocele is that you will notice that it gets larger when Hania lyes down and will reduce when she is sitting upright. So try to keep her sitting up a bit more, this might help the reabsorbtion.

Take care
Mark (Luca's Dad)

I would like to add an update and a cautionary note. In February this year it was discovered that the LP shunt at the base of Luca's spine used to drain CSF to relieve the pseudomeningocele has continued over-draining for 4 years and actually pulled her brain down into the cavity near the top of her spine. She was having these 'episodes' more and more frequently that we thought were TIA's but turned out to be her brain slowly pressing harder against her brain stem - critical if not discovered. After numerous precautionary scans to check on the moyamoya over the 4 years, thankfully one of the radiologists checked beyond the moyamoya and noticed the herniation of her brain. We closed off the shunt and her progress has been remarkable since. No more 'episodes', she's far more energised and her mobility is going from strength to strength. We've had a 3 month scan since closing it off and there are already small signs that her brain is recovering to it's  natural position.

Please use this LP shunt method for draining CSF with extreme caution.

Take care everyone and stay strong
Mark (Luca's Dad)