1 00:00:00,000 --> 00:00:14,960 Today our guest is Dr. Jose Pairo, a pediatric surgeon at Cincinnati Children's, which has 2 00:00:14,960 --> 00:00:17,920 the nation's highest volume photoscopic center. 3 00:00:17,920 --> 00:00:23,800 Dr. Peiro also specializes in neonatal and fetal malformations, and he is the endoscopic 4 00:00:23,800 --> 00:00:27,760 fetal surgery director in Cincinnati Children's Fetal Care Center. 5 00:00:27,760 --> 00:00:32,640 My first question is, why do we even think about fetal surgery and why do we offer it 6 00:00:32,640 --> 00:00:33,640 to our patients? 7 00:00:33,640 --> 00:00:35,480 The answer is very easy. 8 00:00:35,480 --> 00:00:38,560 It's just because we have prenatal diagnosis. 9 00:00:38,560 --> 00:00:43,160 So in other words, we can detect things in utero very early in gestation. 10 00:00:43,160 --> 00:00:47,100 And fortunately, we have very good tools for prenatal diagnosis. 11 00:00:47,100 --> 00:00:49,680 The most important is the prenatal ultrasound. 12 00:00:49,680 --> 00:00:55,160 The conventional ultrasound at mid-gestation, around 20 weeks, will be the best. 13 00:00:55,160 --> 00:01:01,400 So most of the obstetricians and MFMs can catch most of the malformation at that point. 14 00:01:01,400 --> 00:01:06,400 So the rate of the detection of this malformation is very high in the developed countries. 15 00:01:06,400 --> 00:01:12,200 Obviously, with ultrasound, we have the 3D and the 4D modalities that basically we can 16 00:01:12,200 --> 00:01:13,360 see the surface. 17 00:01:13,360 --> 00:01:16,960 In this case, we can detect if it's a cleft lip plate or things like that. 18 00:01:16,960 --> 00:01:22,040 And we can also see the movement, which helps us analyze how these babies move their legs, 19 00:01:22,040 --> 00:01:26,800 knees, and the flex and extension rates of the ankles, which is important for conditions 20 00:01:26,800 --> 00:01:27,800 like spina bifida. 21 00:01:27,800 --> 00:01:33,720 With Doppler, we can analyze the modinamic status of the baby, taking the flows in the 22 00:01:33,720 --> 00:01:38,800 umbilical artery, in the ductus venosus, and also in the middle cerebral artery. 23 00:01:38,800 --> 00:01:41,280 We can have a good assessment of how the baby is doing. 24 00:01:41,280 --> 00:01:43,520 Finally, we have a fetal MRI. 25 00:01:43,520 --> 00:01:48,400 MRI can contribute a lot to define the diagnosis after the ultrasound. 26 00:01:48,400 --> 00:01:53,360 Basically defining on the brain, chest, and abdomen, providing a good health for the find 27 00:01:53,360 --> 00:01:55,960 the condition and counseling of the parents. 28 00:01:55,960 --> 00:02:00,800 So we know that we will detect many of these malformations and we know that some of them 29 00:02:00,800 --> 00:02:04,960 will be lethal or will have postnatal severe disabilities. 30 00:02:04,960 --> 00:02:11,400 So that's the goal of fetal intervention, just try to rescue or improve these conditions. 31 00:02:11,400 --> 00:02:13,240 So what happens after the detection? 32 00:02:13,240 --> 00:02:17,480 For instance, in gastrosclerosis, I have good experience just moving a little bit earlier 33 00:02:17,480 --> 00:02:22,840 so we can reduce the injury on the bowel so we have less serositis and better recovery 34 00:02:22,840 --> 00:02:23,840 in these babies. 35 00:02:23,840 --> 00:02:27,760 Let's talk a little bit about what kind of surgeries they do at Fetal Care Center here 36 00:02:27,760 --> 00:02:29,320 at Cincinnati Children's. 37 00:02:29,320 --> 00:02:32,120 Open fetal surgery is the most similar to neonatal surgery. 38 00:02:32,120 --> 00:02:33,720 So we have the patient in our hands. 39 00:02:33,720 --> 00:02:37,880 We can operate in a fetus the same as we operate in a preemie. 40 00:02:37,880 --> 00:02:40,080 The only problem is we need to open the uterus. 41 00:02:40,080 --> 00:02:45,720 Fetal surgeons use dissolvable staples to limit the bleeding and maintain stability. 42 00:02:45,720 --> 00:02:50,380 But an opening in the uterus produces a scar and that also can activate the dynamics because 43 00:02:50,380 --> 00:02:52,560 the uterus is very irritable. 44 00:02:52,560 --> 00:02:58,240 And sometimes, even though we give a lot of tocolysis to the mom, preterm delivery happens 45 00:02:58,240 --> 00:03:02,040 a few days after the surgery even if the surgery was a success. 46 00:03:02,040 --> 00:03:06,840 For that reason, all the fetal surgeons were trying to go away from fetal surgery, trying 47 00:03:06,840 --> 00:03:09,060 to look for minimally invasive surgery. 48 00:03:09,060 --> 00:03:12,660 So in what conditions a fetal surgeon might offer fetal surgery? 49 00:03:12,660 --> 00:03:18,200 Open fetal surgery still is used for large solid masses in the chest, for sacro-cociate 50 00:03:18,200 --> 00:03:20,880 thratomas and also for spina bifida. 51 00:03:20,880 --> 00:03:22,640 Let's start with fetal lung lesions. 52 00:03:22,640 --> 00:03:24,380 For example, CPAM. 53 00:03:24,380 --> 00:03:28,520 It's very easy to identify because we can see a multi-cystic component in the lungs of 54 00:03:28,520 --> 00:03:30,520 these fetuses by the ultrasound. 55 00:03:30,520 --> 00:03:36,160 Obviously, we need to be very careful about the microcystic, the solid CPAMs, because 56 00:03:36,160 --> 00:03:37,800 the growth is very fast. 57 00:03:37,800 --> 00:03:43,000 And sometimes you can see how the mass can grow and make a shifting of the mediastinum 58 00:03:43,000 --> 00:03:45,920 compression on the other lung and also the heart. 59 00:03:45,920 --> 00:03:49,200 So the next consequence of this rapid growth will be hydropts. 60 00:03:49,200 --> 00:03:55,140 We can see ascites in the abdomen, pleural effusion in the lungs, the scalp edema. 61 00:03:55,140 --> 00:03:57,880 So it's going to be a complete onasarca. 62 00:03:57,880 --> 00:04:01,960 So hydrosfetalis is very close to be complete a fetal demise. 63 00:04:01,960 --> 00:04:05,320 That is the usual outcome of these babies if we don't do nothing. 64 00:04:05,320 --> 00:04:11,600 So how we can anticipate the babies are having risk for hydropts and demise and not. 65 00:04:11,600 --> 00:04:12,600 We use the CVR. 66 00:04:12,600 --> 00:04:20,760 CVR is calculated by the volume of the CPAM, width times height times length times 0.523 67 00:04:20,760 --> 00:04:22,880 and divided by the head circumference. 68 00:04:22,880 --> 00:04:25,600 The cut-off is 1.6. 69 00:04:25,600 --> 00:04:31,080 Everything that measures less than 1.6 is very, very low risk of developed hydropts. 70 00:04:31,080 --> 00:04:36,560 On the contrary, more than 1.6, we have high risk of hydropts and complications. 71 00:04:36,560 --> 00:04:41,360 And for this condition, the first line of therapy is steroids, maternal intramascular 72 00:04:41,360 --> 00:04:46,120 betamethasone, like the one we use for lung maturation in a preemie. 73 00:04:46,120 --> 00:04:50,620 Steroids can be injected to mom in one, two, or even three rounds weekly. 74 00:04:50,620 --> 00:04:52,960 So we can decrease the risk by decreasing the CVR. 75 00:04:52,960 --> 00:04:56,940 It's a good rescue in more than half of these babies. 76 00:04:56,940 --> 00:05:01,520 So the other half, or 40%, probably don't respond very well. 77 00:05:01,520 --> 00:05:07,920 The only possibility before 30 weeks is just to put a shunt if it's a cystic component. 78 00:05:07,920 --> 00:05:13,720 But if it's a solid, the only way is to do open fetal surgery and do a lobectomy. 79 00:05:13,720 --> 00:05:18,080 The other condition, sacro coccidial teratoma, is pretty similar to this. 80 00:05:18,080 --> 00:05:22,000 We can detect in the ultrasound and also in the MRI very easily. 81 00:05:22,000 --> 00:05:26,340 It's important to analyze if this component is more cystic or more solid. 82 00:05:26,340 --> 00:05:30,880 It are more dangerous because they're more rapid growth and also the vascularization 83 00:05:30,880 --> 00:05:32,480 that we can analyze with the dopplers. 84 00:05:32,480 --> 00:05:37,980 The classification of Altman that we use postnatally can also serve in the analysis of these MRIs. 85 00:05:37,980 --> 00:05:41,880 Just to tell you what type of teratoma is. 86 00:05:41,880 --> 00:05:45,320 And also we can decide if we need to do something or not. 87 00:05:45,320 --> 00:05:49,840 The other condition that they're working a lot at Cincinnati Children's is spina bifida 88 00:05:49,840 --> 00:05:51,640 and myelomeningocele. 89 00:05:51,640 --> 00:05:53,680 So open fetal surgery is the gold standard. 90 00:05:53,680 --> 00:05:58,120 Still we are offering, but you will see that there are other innovations on the road. 91 00:05:58,120 --> 00:06:02,480 It is required to operate on the first day of life, just to avoid infections and protect 92 00:06:02,480 --> 00:06:04,660 the spinal cord as much as possible. 93 00:06:04,660 --> 00:06:10,040 The problem came from early in the embryo, usually at four weeks of embryonic time. 94 00:06:10,040 --> 00:06:14,920 Normally, the spinal cord is protected by the different layers, but in spina bifida, 95 00:06:14,920 --> 00:06:16,520 it is not protected. 96 00:06:16,520 --> 00:06:20,480 It's completely facing the outside and also open like a book. 97 00:06:20,480 --> 00:06:23,120 That's the first hit, the malformation itself. 98 00:06:23,120 --> 00:06:25,280 But the most important thing is the second hit. 99 00:06:25,280 --> 00:06:29,280 This neural tissue is not prepared to be in contact with the amniotic fluid. 100 00:06:29,280 --> 00:06:34,520 We know the amniotic fluid is rich in meconium, in enzymes that progressively will damage, 101 00:06:34,520 --> 00:06:39,200 will destroy this supposed spinal cord and the nerves. 102 00:06:39,200 --> 00:06:42,860 Also during the delivery, the spinal cord can almost be destroyed. 103 00:06:42,860 --> 00:06:47,100 So it is very important to say that this condition is very progressive in utero. 104 00:06:47,100 --> 00:06:51,680 So we are losing all the neurons that should help this baby to move their legs. 105 00:06:51,680 --> 00:06:55,080 So what happens when we do neonatal surgery for spina bifida? 106 00:06:55,080 --> 00:07:00,120 We need to assume the sequela of this malformation, that basically in the lower part of the body. 107 00:07:00,120 --> 00:07:05,800 We have fecal incontinence, urinary incontinence, sexual dysfunction, orthopedic anomalies, 108 00:07:05,800 --> 00:07:10,040 and also motor impairments, even needing a wheelchair at some point, depending on the 109 00:07:10,040 --> 00:07:11,260 level of the injury. 110 00:07:11,260 --> 00:07:15,480 But also in the head, because the compression of the brain stem produce obstruction. 111 00:07:15,480 --> 00:07:19,360 You can see like a plaque in a bottle, the brain retains that fluid and the build-up 112 00:07:19,360 --> 00:07:20,360 produce hydrocephalus. 113 00:07:20,360 --> 00:07:24,960 At 24 weeks, half of the fetuses have already hydrocephalus. 114 00:07:24,960 --> 00:07:30,480 And for the others, 95% of the time, they will have hydrocephalus later in the pregnancy. 115 00:07:30,480 --> 00:07:34,960 Hydrocephalus is important because these babies will need derivation, some kind of ventricle 116 00:07:34,960 --> 00:07:37,800 peritoneal chanting with all the problems that produce. 117 00:07:37,800 --> 00:07:42,040 And also that generates migration disorders, atherotopia, second in these babies. 118 00:07:42,040 --> 00:07:47,160 To summarize, we can say that the pathophysiology of spina bifida makes the spinal cord exposed 119 00:07:47,160 --> 00:07:51,640 to amniotic fluid, have second anural damage and hydrocephalus. 120 00:07:51,640 --> 00:07:53,240 So how can we stop that? 121 00:07:53,240 --> 00:07:58,840 So we can avoid amniotic fluid touching the nerves and we can avoid this leak of CSF. 122 00:07:58,840 --> 00:08:04,520 That's a rationale of open fetal surgery and also whatever repair in spina bifida. 123 00:08:04,520 --> 00:08:08,800 That's why the mom's trial started just to try to compare prenatal surgery with postnatal 124 00:08:08,800 --> 00:08:12,160 surgery using the same technique they use in neonates. 125 00:08:12,160 --> 00:08:17,200 Using the dura, the layers and the skin, sometimes using patches of the skin. 126 00:08:17,200 --> 00:08:21,640 And then the results show that these babies have a significant benefit from having peritoneal 127 00:08:21,640 --> 00:08:23,180 surgery. 128 00:08:23,180 --> 00:08:26,540 Surgeons reduced at least half of the need for chanting. 129 00:08:26,540 --> 00:08:30,080 They improved the mental scores and the motor outcomes. 130 00:08:30,080 --> 00:08:34,260 In most of the cases, they revert completely the high brain herniation. 131 00:08:34,260 --> 00:08:37,660 For duraplasty, we introduce a rollet patch through the cannula. 132 00:08:37,660 --> 00:08:42,660 So with one stitch, we can maintain in position as a dura patch. 133 00:08:42,660 --> 00:08:48,280 After that, we try to close the skin primarily or sometimes we need to put a patch. 134 00:08:48,280 --> 00:08:53,480 What we can say is that Dr. Pera and his team are creating less maternal morbidity and they're 135 00:08:53,480 --> 00:08:58,800 able to allow the moms to deliver their babies vaginally. 136 00:08:58,800 --> 00:09:04,040 Next one is the exit procedure, which is another kind of open fetal surgery happens at the 137 00:09:04,040 --> 00:09:05,040 time of delivery. 138 00:09:05,040 --> 00:09:10,000 And it's a very interesting strategy that we use when we detect something that can produce 139 00:09:10,000 --> 00:09:14,000 neonatal asphyxia or difficulties for intubation. 140 00:09:14,000 --> 00:09:16,480 We have many indications and cases. 141 00:09:16,480 --> 00:09:20,600 Most likely neck masses that are compressing the trachea, but also others like congenital 142 00:09:20,600 --> 00:09:25,520 obstructions or severe micrognathia may have a benefit from this technique. 143 00:09:25,520 --> 00:09:31,120 We can achieve between one hour and three hours, maintaining the oxygenation from mom 144 00:09:31,120 --> 00:09:34,000 through the placenta to the core to the baby. 145 00:09:34,000 --> 00:09:35,520 So it's a controlled situation. 146 00:09:35,520 --> 00:09:37,920 We don't need to rush to secure the airway. 147 00:09:37,920 --> 00:09:42,160 Obviously we need to paralyze and anesthetize the fetus. 148 00:09:42,160 --> 00:09:45,480 Maternal monitoring and stability is also important. 149 00:09:45,480 --> 00:09:49,160 And we use inhalational anesthetics for uterine relaxation. 150 00:09:49,160 --> 00:09:56,280 Cases like dratomas in the neck, sometimes even in an exit, you can do a straightforward 151 00:09:56,280 --> 00:09:57,900 intubation. 152 00:09:57,900 --> 00:10:01,400 Sometimes you can use rigid instruments or laryngoscopy. 153 00:10:01,400 --> 00:10:03,800 And sometimes even with that, it's not possible. 154 00:10:03,800 --> 00:10:08,440 With the exit procedure, we have time to do a partial resection of the theratoma, identify 155 00:10:08,440 --> 00:10:10,480 the trachea and then intubate the baby. 156 00:10:10,480 --> 00:10:15,680 So then once the intubation is done, we don't need to be more on placental support. 157 00:10:15,680 --> 00:10:20,840 So we can clamp the core and do the surgery in next door OR so we can complete this theratoma 158 00:10:20,840 --> 00:10:21,840 resection. 159 00:10:21,840 --> 00:10:23,840 In summary, in summary, fetal surgery is a very important procedure. 160 00:10:23,840 --> 00:10:38,560 So we need to start, what it looks like to do in the inst Mountains, where we really