Urological Involvement in Patients with OEIS

So, uh, one of the questions that we're seeing is from a family asking about what is the incidence of urological involvement in patients who have OIES, and OIS is a syndrome. It's a constellation of symptoms. The O stands for omphalocele, atrophy, imperfect anus, and spinal cord anomalies. So I would say that given the involvement of the spinal cord and the interectal malformation, the involvement of the urinary tract is almost always going to be present, so about 100% involvement, and it is important for any child with OEIS to be assessed by a urologist. And if they're deemed to be safe, then again at various age. Um, cutoffs, we want the urological involvement to be assessed again, and one of those age cutoffs would be at the age of normal continence, so that would be around 3, 3.5. Now it's important to understand that bladder training or being able to be toilet trained is not an age-based definition, but it's actually individual for each child, and it comes from the development and the involvement of their neurological development with their urological development. Their spinal cord has to be developed and all the synapses and connections have to be formed so that that brain bladder connection can be developed. The default nature for all human beings is what we call an infantile bladder. So the bladder fills with urine. There are stretch receptors in the bladder. When it gets to a certain volume, it's just going to trigger a contraction, and that contraction is going to make the bladder squeeze and the sphincter open up so the bladder can empty. And that's the default for all human beings. Toilet training is our brain's ability to impact and override that infantile contraction. And that's what toilet training's all about. So in order for that to happen, that brain bladder connection has to develop. All of the connections between the brain to the lowest part of the spinal cord, which is where our bladder's nerves come from, has to develop. Um, oftentimes it's around 3, 3.5, but a quick way to tell is when your child can walk upstairs like you do. So if you watch most children, they put both feet on the same step, it's because they don't have the neurological control on the leg to be able to pivot their entire body weight and go up the stairs. But when they can walk upstairs like you can. Their neurological connections are made and the toilet training will be a breeze for them.