I’ve been meaning to make this post for a long time. I received a lot of messages last fall asking for more information on Violet’s intestinal malrotation and volvulus diagnosis, particularly from those who also had kids with unexplained vomiting or a cyclic vomiting syndrome diagnosis. I wanted to post more at the time, but just mentally couldn’t do it. I think I’ve finally got the energy for it, so here goes! (I’ve also had a lot of people ask about the symptoms she had, but will save that for a future post.)
Before we start, I want you to understand that although intestinal malrotation is technically present at birth, not everyone has symptoms in infancy the way Violet did. Issues can show up at any age. So don’t assume you or your child can’t have this just because they aren’t a baby or didn’t have symptoms as a baby.
Let’s start at the very beginning (a very good place to start). During a fetus’s development, the digestive system begins as a straight tube, then grows into the more complicated system of stomach and intestines that you’re probably familiar with. The process begins at around 5 weeks gestation, and finishes up at around 12 weeks. If all goes well, you end up with something like picture e.
But if something along the way goes wrong, it could potentially result in whats called intestinal malrotation. Below, picture A shows a normal intestine and B shows a malrotated intestine.
So that right there is intestinal malrotation. However, intestinal malrotation itself will not kill you, the dangerous part about malrotation is that the way the intestines are situated gives them the ability to twist (called a volvulus) or get blocked off by something called Ladds bands.
In a person with malrotation, Ladds bands run across the duodenum (which is the start of the small intestine right after the stomach). These bands can block passage into the intestines, essentially causing an obstruction. In addition to obstruction from Ladds bands, the entire small intestine and part of the large intestine can twist on themselves. This twist is another, very dangerous, kind of intestinal obstruction called a volvulus. A volvulus can potentially restrict blood flow to the intestines. Without blood flow, the intestines can die in a matter of hours. This can lead to a whole host of issues and does have the potential to be fatal (scary I know, but I want you to understand this is serious business).
The most common treatment for intestinal malrotation and volvulus is called the Ladd’s Procedure. The Ladd’s Procedure consists of manually untwisting the bowel (and removing dead tissue as needed), cutting the Ladd’s bands, removing the patients appendix (because leaving it would make future appendicitis difficult to diagnose), placing the small intestines on the right side of a patient’s body and the large intestines on the left side of their body. Although this position is not anatomically correct, it makes the chances of the intestines twisting again much less likely.
Before the Ladd’s Procedure was developed, nearly everyone with malrotation and volvulus died. But as this point in time, as long as a volvulus is found fast enough, the majority of patients are able to have surgery and go on to lead healthy lives.
If someone is found to have intestinal malrotation but does not have a volvulus, they will usually still undergo the Ladd’s Procedure as a precaution, however their surgery would not need to be performed quite as urgently as someone with a volvulus and would not involve manually untwisting the intestines.
And that’s that! I’m going to try really hard to make a post about the symptoms we saw over the years. My biggest hurdle seems to be going through old photos for the post. Looking back at her misery all those years hurts my heart. I will say this for now: If you or your child ever vomits green or bright yellow bile, head straight to the ER (seriously – don’t pass go, don’t collect $200, just HEAD TO THE ER).