Jason Chu, MD, an assistant professor of clinical neurological surgery at the Keck School of Medicine of USC, discusses fetal surgery for the repair of myelomeningocele, a severe form of spina bifida, including novel surgical techniques designed to improve patient outcomes.
Hi. My name is Jason Shoe, and I am a assistant professor of neurosurgery at Keck School of Medicine here at USC. I'm also a pediatric neurosurgeon that works the Childrens Hospital Los Angeles, and I'm excited to talk to you a little bit about some of our ongoing work and our experience with field surgery from vomiting seal. So, um, I lamenting SEAL is a central nervous system malformation that occurs extremely early in life, and it results from a failure of primary no relation where the coddle neuro poor fails to go from a sheet and roll over it to form a tube. The resultant malformation not only affect the spinal cord, but also the nerves, the bones, the muscles and the skin in the back. There are also secondary effects that can occur in the brain as a result of this malformation and lower down in the spinal cord. And in this picture, we can see, um, our mama *** seal in this newborn baby. But what's not immediately obvious, um, in this picture is the other malformations that can occur within the brain. Weakness in the legs or paralysis in the legs, bladder dysfunction, scoliosis as well as symptomatic hydrocephalus. The traditional way that um, vomiting seals is treated is to repair it after the babies were born. However, a lot of work done in the nineties had looked for ways to repair the vomiting. Silesian Prenatally and the researchers had shown that prenatal repair was effective in protecting the existing neural elements from continued injury in utero, a zwelling preventing spinal fluid leak from the mom eating seal itself with a secondary effect of restoration of more normal CSF hydrodynamics. All of this work had resulted in a large randomized control trial that was published approximately 10 years ago. This trial is called the Management of Mom, Monica Seal or Mom's Trial, and the investigators had found improvements in rates of symptomatic hydrocephalus that required treatment, as well as improved motor outcomes in a number of the patients that were repaired prenatally. The benefits to the infants, however, did not come without risks, and there was noted to be very significant eternal morbidity related to prenatal repair, including severe complications such as placental abruption, premature rupture of membranes resulting in prematurity within many of the baby's born, as well as the need for a C section for the current pregnancy as well as any future pregnancies. This is a picture of how the moms trials. Investigators had completed the prenatal repair. And what is what some of the maternal morbidity came about from was not only was a laparotomy required, but a history. Artemis was also required where the uterus is open in order to gain access to the mom Ningxia lesion. Despite the maternal risks, fetal surgery for my meeting, a seal has gained traction worldwide, and several centers within the United States have, um, started fetal programs to for my lamenting seal. In addition, several these programs have attempted to refine the procedure so that there's less maternal risk and less trauma to the uterus. So this is a technique that's described by Dr Belford and Dr Whitehead, um at Baylor College of Medicine in Texas Children's Hospital. And there there technique had involved ah hybrid approach where the uterus was externalized. However, the procedure was completed in a in a minimally invasive fashion where labra scopes were and laparoscopic instruments were placed within the uterus in order to complete the mining steel closure. Additionally, other groups, mainly actually in Europe as well as South America have attempted to complete the mom innings to repair through a purely per cutaneous approach involving the placement of the Labra scopes and working channels and laparoscopic instruments in a minimally invasive fashion through the skin. While this minimally invasive approach has mainly been done outside of the United States, here at USC, we've collaborated with a group in Miami to try and bring this technique here and to date. U. S. C and C H. L A. Um, is currently on Lee, one of two centers in the United States, to offer this perky Titanius Fetus Coptic repair from a limiting A sealed. We're currently conducting a Phase one study to evaluate its safety, and our goal is to try and achieve similar outcomes for our infants as the mom's trial while improving maternal outcomes. This is a picture of our team, and as you can see, it's a very multi disciplinary team that not only involves fetal surgeons O B G y n n's neurosurgeons, but also spina bifida, um, pediatrician's radiology as well as neonatologists, and you can see it really takes a village to try and complete this work. So we've done our first case, Um, in February of 2019, Andi as of October 2020 we've done 27 patients split between here and our collaborators over in Miami. This is a video of one of our cases, and here we have introduced our labra scopes and laproscopic instruments. You can see the neuro placko from before and using laproscopic scissors were able to carefully dissect out the neural placko and preserve any neural elements. After separating out the um, neural plaque owed and the malformed spine from some of the surrounding tissue, you can see that pops down into a more normal position within the spinal cord. And once that's complete, we proceed with closure. And we're trying to do a multilayered closure to provide as much coverage and protection to the spinal cord is possible. So here what? What we're doing is we're elevating and raising some of the muscles beside the spine to, um, provide an extra barrier called the Maya Fashion Flap. This is a piece of during repair, which we're using to reconstruct the fecal sack. And the mile fashion flap is closed over top of this. Yeah, here you can see the mile fashion flap is completed with complete coverage of our mom getting a Silesian. Following this, the skin is closed with a running future. Here's some of our early results in comparison to the mom's trial. Thus far, we've had very similar, um, gestational ages at delivery. One thing that's immediately apparent is, um, the lower rate for Caesarian section Andi other, um, 13 patients were actually delivered vaginally, which was something that was not seen in the mom's trial. We're still calculating our maternal risks, but thus far they've been, um, comparable to what was seen in the mom's trial. In terms of our infant results, we've seen very similar results of the moms trial as well. Of course, Mawr data analysis is needed. However, Um, what we've experienced, um, is that, um, we have had, um, pretty comparable rates for the need for hydrocephalus treatment, as well as those improvements in motor outcomes. So, in summary, um, fetal surgery for maligning seal has become an increasingly popular treatment modality worldwide. The results of the moms trial had demonstrated improved infant outcomes in terms of symptomatic hydrocephalus as well as motor outcomes. However, this did come with risks to Mom currently, um, in the United States. Um, USC is undergoing a phase one trial. A za collaborative work. Um, that's assessing the safety and feasibility of fetus Coptic surgery in the United States in our early data thus far has been very encouraging. Thanks for watching. And that's this picture. Here is a picture of our first patient, um, and that patients doing extremely well, they have not required treatment for hydrocephalus. And, um, are able thio ambulance independently on this is another picture of a patient here who's approximately six months old and has been doing quite well.
