All right, I think in the interest of keeping things on time, we will start on time. We're going to start with Dr. Roberts talking about lipomyeloma negatio. Good afternoon. My name is Tullo Roberts. I'd like to welcome all of you here today. I'd like to thank the organizers for including me and thank you all for attending. The discussion is about lipomyeloma negatio management. I think my colleagues and I have put together a very interesting lecture and we're looking forward very much to hearing what your comments might be. Next slide please. I don't have any disclosures. And I do want to take a moment to thank my esteemed colleague, Dr. Clarence Green. He's my local JEDI master and he contributed some slides to this lecture. So the objectives of the discussion are to review presentation, workup, patient selection and surgery and follow-up of patients with lipomyeloma negatio. There's a lot of controversy in the field, not least of all what exactly is meant by lipomyeloma negatio. Some corners of the literature emphasize that lipomyeloma negatio usually have a negatio associated with them, the placode exists outside the spinal canal. I think what's more commonly encountered on the front line is a congenital spinal lipoma with a dural defect. And I have a few examples of that in the discussion. Because the goals of management are the same and the techniques for treating are the same, I'm not sure the distinction poses a hindrance to including those examples in the lecture. If you extrapolate from information obtained and reviewing the incidence of lipomyeloma negatio in Hawaii, you can estimate that it occurs at about a rate of 3 to 6 over the course of 2 years in Louisiana. In our practice here, we've had 11 patients over 5 years. Three of those were patients who had been operated on before who required surgery, two were patients who had surgery for the first time around, and two were patients that we're observing. Of those 11 patients, I was primarily responsible for 7. And I listed them here. Some of them present simply because a sacral mass was discovered. Others present with actual symptoms. And I'll go into further detail about the workup. So again, patients present either as infants whose families discover shortly after birth or at birth that they have a lumbosacral mass. Another group of patients are older children who present with concerning signs or symptoms. Those symptoms can be musculoskeletal, neurologic, urologic, things like scoliosis, leg length discrepancy, foot deformities, motor sensory deficits, back pain, urinary tract infections and the like. And then there's another group of patients who are retethered patients who've had surgery previously and present with similar symptoms. In working up those patients, I perform a thorough history and physical. My goal is to document the baseline as clearly as I can so I have something to compare to after surgery if they become surgical candidates. I want to especially rule out the presence of any other disorder or dysfunction affecting other systems. That's not unheard of in patients who have lipomyelomeningoceles. And then I move on to imaging. If it's an infant who presents who's asymptomatic, they usually have an ultrasound that their primary care provider's obtained. And I like to get an MRI of the entire spine. I am also keen on getting a CT scan if it's an older patient who's been operated on previously. It allows me to get a better sense of what the anatomy looks like if I'm taking a patient to the operating room. I like to get post-void residuals on the patients as a screening study if they're asymptomatic. In the event that they do have some evidence of urinary dysfunction, my urology colleagues often encourage me to get a renal bladder ultrasound and send them for urodynamics. I make use of subspecialists as indicated, referring patients to urology, orthopedics, and if it's a patient who's getting reoperated on, I make use of my plastic surgery colleagues as well. They're very good at helping me get in. And if the patient develops a leak, they're very good at taking responsibility and fixing it afterwards. This is a picture that was provided by my esteemed colleague, Dr. Green. It's a patient with a lipoma. It's not unusual for them to have dystrophic signs. In addition to the lipoma, in this case, the hemangioma, it's not unusual for them to have hypertrichosis or even digits. And this is an MRI of a patient with a caudal spinal lipoma. This one appears to be of the transitional variety. My colleagues will be going into further detail about describing the different kinds that occur. You can also see what looks like a terminal ventricle or a serincin association with this. This, I think, is the closest thing I have to a true lipoma, myelomeningocele, in that the placode looks like it's outside the spinal canal. Can't really appreciate the meningocele in association with it, but in the course of the operation, it was evident, and I have pictures to show that. And then this is an older patient who has what looks like a terminal caudal spinal lipoma in association with a serincin-associated scoliosis. It's not unusual for patients to have hemivertebrae. This is fairly evident on this coronal view. Okay. One of the areas of controversy encountered in managing patients with caudal spinal lipomas is when to operate. So if you have an asymptomatic patient who has an incidentally discovered caudal spinal lipoma, is this a patient one should operate on? I generally choose to operate on patients who have evidence of symptoms or appear to be declining. If they're asymptomatic, I may operate if the patient has a large syrinx or evidence of significant compression from the lipoma. For surgery, after consenting the parents, I perform a standard preparation. The patient's taken to the operating room. I'm their position prone. I make sure they're very adequately padded. I usually place a Foley. One other area of controversy is whether or not to make use of neuromonitoring. I choose to make use of neuromonitoring. I use somatosensory evoked potentials, motor evoked potentials, rectal EMG, and electrical stimulation as well. The anatomy can be very humbling when you get in there, especially in retethered patients. And I think any aid that is an investment in trying to make sure you have a good outcome is worthwhile. Although I understand we'll be looking at some interesting cases where it doesn't always work out as intended. The goals of surgery are to detether, decompress, and I like to perform a duroplasty afterwards. One of the risks of surgery is that the patient may eventually retether again. In order to try and avoid that or at least prolong the period before retethering, a duroplasty that increases the ratio of the canal diameter is a worthwhile investment. In order to decompress the lipoma, there are a variety of techniques that are available. I choose to use ultrasonic aspiration and bipolar cautery. I've also been impressed with the use of the YAG laser. And I make use of the operative microscope as well. I put this slide in just to remind everyone that even when everything goes well, one can still encounter problems. In this case, this was from a BIS monitor that was placed on a patient when she was position prone and she developed a grade two pressure ulcer. And she did fine. The technique I employ for surgery, I like an elliptical incision. This is not the best diagram, but I think it conveys the essence of the information I'm trying to pass along. A straight incision and then in the area of the lipoma, I like to create a small ellipse. And this makes it easier for me to manipulate the lipoma as I dissect towards the fascia. I like a wide fascial opening. It makes it easier to identify the fascia when closing. And that's an important step in trying to make sure you don't develop a complication like a CSF leak. The goals of surgery, once the fascia is exposed, you identify the normal anatomy and work towards the anomaly, starting with a normal spinous process and then a normal dura. Attack of stitch in the dura, dissect and open up the dura. It's important where the lipoma articulates with the neraxis to be sure that while you're opening the dural sac, you take every measure to avoid injuring dorsal nerve roots, which could be in the close vicinity to that point of articulation. I like to place the patient in an increased Trendelenburg because it helps the CSF flow away from the area of operation. I put cotinoids in the area to siphon off fluid. And I'll show you pictures of that as well. After debulking the lipoma, I cut the phylum. I re-approximate the pia. I place gel film over the pia and I perform a duroplasty. And I'll show you pictures of all that going into further detail. This is a lovely picture provided by Dr. Green. The purple arrow shows you the orientation of the patient towards the head. This is the edge of the laminectomy. You're looking at normal dura here. This is lipoma. At this point, one would open the dura, taking care to avoid any injury to dorsal nerve roots that might be located in that area. You can see some evidence of that here. Dura's been opened. You've got tachypseutras next to it. Next step would be to debulk the lipoma, which you can see being done here, or having been done here. This is the CUSA in place. Once the lipoma's satisfactorily debulked, identify the pial edges that'll be re-approximated. Make sure that there's no distal tethering phylum. Forgive me. And then close up. This is a different patient. The patient's oriented in that direction towards the head. This is a patient who presented with re-tethering after previously having a lipoma laminate seal resected. The dura's been opened. You're looking at the lipoma here. It's scarred all around on the inside of the dura. It dissect around that lipoma to make sure it's free. Try to access the lateral gutters, which you can see occurring here. Nerve roots occur in the strangest locations. One has to try and preserve them. It's very important to expect the unexpected when you're operating on anomalous anatomy like this. And again, you're looking at the lipoma here. Normal spinal cord is in this direction. This is just more scar on the inside of the dura. I'm dissecting to try and free it up. Once this is accessed and I can see the anterior or the ventral portion of the fecal sac on both sides laterally, then I make sure that there's no distal tethering portion there. And re-approximate the peel edges. So here again, patient's oriented towards that direction. We've opened up the lateral gutter here. You can actually see motor nerve roots distally there. And then this is more distal on the core, the same thing. On this side, you can see a whole plethora of nerve roots. Makes it difficult to try and access that area. But once again, you've identified the peel edge. That's one of the edges that'll be re-approximated at the end. This is the CLUSA being used to perform a debulking of the lipoma. Peel edges are being re-approximated. That's what it looks like at the end. I put a layer of gel film down. This is denatured collagen. It's supposed to try and reduce arachnoiditis and hopefully prolong the duration before re-tethering occurs, and then a nice duroplasty. You can do duroplasty with autologous grafts. You can do it with synthetic grafts. I tend to use synthetic grafts. This is a very nice picture of a lipomyelomeningocele. You see the meningocele there. The placoid very likely exists outside the spinal canal. It's the definition, the strictest definition. This was a picture that was provided by Dr. Green. The peristion is oriented towards your left. The dura has been opened. You can see where the lipoma articulates within your axis. Once again, accessing the lateral aspects of the tethered portion of the cord and then confirming the same on the opposite side. Make sure it's re-tethered distally. Re-approximate the peel edges, and you're done. Post-operatively, it's standard management. The patient gets 24 hours of antibiotics, adequate pain control. I favor alternating doses of Tylenol and Motrin or IV Tylenol and Toradol. I tend to keep my patients flat for 24 to 48 hours. If they're infants, it's very hard to convince them to do that. I tell the families not to fight with them because crying is just as burdensome for the incision. Once the Foley catheter is removed, I confirm they're voiding within eight hours. I monitor them closely for any evidence of complications like a CSF leak or burgeoning infection. So in summary, managing patients with lipomyelomeningoceles or caudal spinal lipomas with a dural defect. Perform a detailed preoperative assessment. I'm trying to make sure I have a clear and succinct baseline exam. I look for any evidence of other congenital disorders that might complicate surgical management if they become a candidate. It's targeted patient selection. I operate not on site, but on patients who have concerning or worsening symptoms or perhaps a searing switch is growing larger. It's a standard approach. The goals are to detether, debulk, and perform a duralplasty at the end, and then vigilant postoperative care. That's the last picture from Dr. Green. Thank you.

lipomyelomeningocele

management

Dr. Tullo Roberts

surgical techniques

postoperative care