Say hi to Bentley Hi Bentley Bentley’s a young boy who by all rights should be a normal little guy and should be growing up and playing, but he has an interesting problem with the way his head and brain developed. Before birth, they realized that he had this encephalocele, which is an opening in the top of the head. He has brain inside and then he has brain that’s outside. In addition to that, he has a lot of fluid causing that entire mass outside to slowly grow. Every month that mass, called the encephalocele is getting larger. This particular encephalocele is somewhat unique, a lot of the times with encephalocele the brain tissue is very abnormal looking. In Bentley’s case, this was all functional brain tissue that if we injure in any way could change him in some way. We can’t simply resect that brain tissue, we have to protect that brain tissue and get it covered up safely for the rest of his life. What we’ve be able to do here at Boston Children’s Hospital is use our Simulation Center to really simulate in advance what the operation is going to be. In the operating room we probably will avoid making a cut there. At this point we could start to try to bend those out a little bit. Okay, yeah. and see how that looks. Basically, just cantilevering them off of those little lower points. What I’m holding here is a skull model of Bentley’s head. The top here is actually the hole in the top of his cranium and about half of that brain is outside. This model is incredibly helpful because it allows me actually to see and feel in 3 dimensions where the tissue is outside of the skull. You can see the dura on the outside and you can see the brain on the inside. This space here is filled with cerebrospinal fluid and the ultimate goal will be to move all of this back inside the skull here. We’ve done some measurements in collaboration with the Simulation Center, in collaboration with the Simulation Center, which show that we have to expand his skull by at least 20 percent. Dr. Proctor and I think that what we’ll probably do is make linear cuts here and that will allow this part of the back of the skull to open up and allow this then, to re-enter the skull. You cut the bone, but they all remain connected at the base. And bone is living so that over time all the spaces here are going to fill in with new bone and it’s all going to be covered. This case is rare and somewhat different than almost all of the encephalocele cases that we’ve had. This is the first time that we’ve had so much good brain outside of the skull that we’ve attempted to replace it back into the calvarium. The special risks that we’re worried about in his case is one, taking all of this normal brain tissue and moving it quite substantially and having it still maintain all of its function. There’s definitely the risk of causing a stroke by injuring any of the blood vessels going to it. There’s going to be a significant risk of leaking fluid. Because it’s so unique, exactly how this encephalocele will impact his neurocognitive development as he gets older is going to be a question that only time is going to tell us. Our hope with Bentley is that if we can save and preserve this brain that is outside the calvarium and put it back inside that he’ll continue to function and hit his developmental milestones and do well in the future.