Our First Visit with the Pediatric Surgeon

Since my last blog, I feel like I've become a lot more informed on my little peanut's condition which has allowed me to gain some comfort with our situation. My mom had come across the blog of another CCAM mom who is only a few weeks ahead of me and her little girl was also diagnosed with a CCAM just a few weeks before me. After reading her blog, I felt like I was reading my own story. In addition to the little girl that they have on the way, her and her husband also have a three year old little girl. It was just all too familiar. Not knowing what to expect, I commented on her blog to let her know how I had come across her story and asked her if she would like to correspond regarding our similiar pregnancy situations and concerns. She responded the next day, inviting me to email her further. Of course I did just that and we have been corresponding through email and messaging ever since. It's always odd meeting people over the internet but there are some people that you feel like you have an instant connection with. In this case, it has been so comforting to me to talk with someone who is going through the same feelings I am and can relate to our situation and the fears that accompany it. Additionally, she is a few weeks ahead of me and has been able to provide me with lots of resources and support groups that she has encountered in her research. . .two of which she came across on Facebook. Whoever pokes fun at social networking has no idea how important a part of my life it has become. With multiple cross country moves thanks to the military, we've been able to receive daily updates on family and friends. And now, I've made connections with several other women all over the world who are pregnant with babies who have CCAMs. Some have already given birth and underwent surgery and have pictures and stories to share. Others have opted out of surgery based upon their experiences. It's interesting (and sometimes a little scary) to hear the different scenarios but mostly, I feel like it is raising my awareness to the different possibilites that we may encounter.

Additionally, since my last blog, I was instructed by my doctor to give a 24 hour urine test sample. The test results came back on Tuesday and were in the normal range with regard to protein. They were a little on the high side but still within the normal range. My OB called to tell me that they would use that as my baseline test and would be testing me again in a few weeks to ensure that my levels had not doubled or shown any significant jump that would insinuate preeclampsia. As I believe I mentioned previously, women who are pregnant with CCAM babies are more likely to get preeclampsia. Additionally, since I've already had preeclampsia with a previous pregnancy, I'm also at further risk to get it again. That being said, it's nice to know that my doctors are all watching me closely to ensure that it does not go undetected as long as it did with my first pregnancy.

Today, Tim and I went for our first visit with the pediatric surgeon, Dr. Marion Henry. We were a little thrown off when we arrived because we were expecting to meet with Dr. Romeo Ignacio. After speaking with my perinatologist, Dr. Richards, the week before, he had made me confident in Dr. Ignacio's surgical capabilities and experience with CCAMs. Similarly, when I met with one of my OBs on Friday, he had also reassured me that Dr. Ignacio was "the best" and he would trust his own family's care in Dr. Ignacio's hands. While I was a little surprised by Dr. Henry's introduction, she did reassure me that her and Dr. Ignacio were the only two pediatric surgeons at the hospital and they hold monthly formal meetings with the Fetal Assement Unit (also known as Maternal Fetal Medicine) doctors. They discuss all of their patients across the board in order to ensure that all four of them are on the same page regarding these patients. That also doesn't account for the dozens of interactions that Dr. Ignacio and Dr. Henry have on a daily basis working out of the same office. I felt a little more comfortable after hearing that all four doctors would have a complete understanding of our journey prior to surgery. In addition to Dr. Henry, we were also introduced to Margie Gabriel who was a Pediatric Surgery Physician Assistant to Dr. Henry as well as another resident.

Unfortunately, since I have not had my MRI yet, there wasn't a ton of information to discuss with the pediatric surgeon and her physician assistant. While the initial ultrasound that our perinatolist conducted had provide quite a bit of information, it can not be considered totally reliable. The MRI will provide much more concrete evidence about the size of the CCAM and possibly the type of CCAM which will in turn define our course of action. I asked her if this would be our only meeting prior to surgery, which I was concerned about considering we didn't even have the results of my MRI yet. She assured me that I could schedule an appointment to meet with her as much as I needed to feel comfortable. She would definitely want to meet with me post MRI (more along the January time frame or so) since we would have a better understanding of the direction in which things were moving. She said that some people felt comfortable only meeting with her that one time while others felt more reassured with frequent meetings. She was comfortable with whatever I was comfortable with. Additionally, she presented me with her and her assistant's direct phone lines and email addresses in case I had further questions that needed answering.

Here are the results from the Q&A session that I conducted with the pediatric surgeon:

Q: At the 20 week ultrasound when the baby was originally diagnosed with a problem, the doctor had mentioned pulmonary sequestration. However, our perinatologist was convinced that it looks like a CCAM. What is the difference between the two and how are the they treated differently?

A: She didn't quite explain the difference between the two. Although she did explain that they both fit under the umbrella of CPAM (Congenital Pulmonary Airway Malformation). From my understanding, the two are very similar. A pulmonary sequestration can be introlobar (meaning inside the lung) or extralobar (meaning outside of the lung) but they also have an additional blood supply (usually from the aorta or other arteries). The surgeon did say that most pulmonary sequestrations are introlobar. Unfortunately, since they are in the same family of malformations as the CCAM, they are often hard to distinguish (and sometimes even misdiagnosed) between the two through ultrasounds or MRIs. They are easier to decipher after the baby is born but even then, it is sometimes difficult to tell which it is until they actually do the surgery to remove it. Either way, they are treated similarly with regard to surgery. In addition to pulmonary sequestration and CCAM, she also mentioned that Congenital Lobar Emphysema (which was also mentioned at the 20 week ultrasound) is another malformation that can also fit under this umbrella. However, she doesn't believe this to be the problem because the lesion occurs on the right lobe with is not characteristic of the emphysema.


Q: What is the CVR? What does this mean?

A: Unfortunately, the surgeon did not seem familiar with this term (or was confused as to what I was speaking to) which made me a little skeptical. Every information site, blog, group in which I've researched has always mentioned their CVR, which is provided by their doctor. The CVR, according to the web, is a CCAM volume ratio obtained by dividing the CCAM volume by head circumfrance to correct for fetal size. In other words, it seems to me that it is comparing the size of CCAM with comparison to the size of the overall fetus. Basically, as long as the lesion seems small with regard to the rest of the baby, it is not as much of a concern. However, as the baby grows, the lesion will continue to grow with it. If it continues to be small and manageable, they will continue to monitor it's growth until birth. If it growing larger and larger and starts to impede upon the growth of the heart and other organs, it becomes much more of a concern and more frequent ultrasounds will become necessary.

Q: Are there currently any signs of hydrops (a sign of heart failure)?

A: No, none yet. It's still really too early to tell though. She did not seem too concerned that hydrops would be a problem as it is more of a rare occurence with CCAMs.

Q: Are there any symptoms/signs of hydrops that I should be aware of? It just makes me nervous because my ultrasound appointments are every 4 weeks or so. I've noticed that a lot of the women in my groups are being monitored weekly. What happens if something goes wronge between my four week ultrasounds?

A. No, unfortunately there are not any symptoms that you will be able to detect. This early in the pregnancy, it's going to be too difficult to detect hydrops through the ultrasounds. Appointments every four weeks should be sufficient. In these appointments, we will be looking specifically for any extreme growth that may cause hydrops.

Q: Dr. Richards had presented the idea that I may be a good candidate for surgery in utero. This made me a little concerned (besides the obvious concerns) because it has to be conducted prior to 26 weeks gestation. I'm currently at 21 weeks and will be 22 weeks when the results of my MRI are returned.

A: She seemed a little confused as to why this option was even discussed with me at this point. It seemed to be getting a little ahead of ourselves. The probability that I would require this surgery is miniscule. However, they are accustomed to working on tight turnarounds and in the event that this surgery would be required, they can set it up within a week's turnaround. Additionally, in the event that I would require this surgery, I would most likely be referred out to UCSF which is one of the top hospitals in the country/world for CCAM surgeries.

Q: What specific information will the MRI provide? What can you tell from this?

A. It may possibly be able to to tell us what type of CCAM we are looking but may not. It will definitely give us a more accurate idea of the size as well as the placement with regard to the rest of the organs.

Q: One of my CCAM mommy friends is seeing a pediatric cardiologist for an echocardiogram? Is there a necessity for me to see one? What additional information would that provide us?

A: No, we would not send you to a cardiologist unless we thought that the lesion might start growing to the point that it could possibly start crowding the heart. We won't know that for sure until we get the MRI and continue to monitor the growth of the lesion. So if you would be referred out for an echocardiogram, that would not occur until later in the pregnancy.

Q: Do you recommend fetal surgery? If so, why? What are the risks associated with it?

A: Yes, eventually. If the baby is born and is asymptomatic, we will have a check up after 6 months and then begin to think about scheduling the surgery. However, if the baby is born in respiratory distress, surgery will be immediate after birth. Additionally, whether or not the baby is born asymptomatic or not, we do recommend surgery at some point to remove the lesion which will ensure less risk of infection and/or the potential for cancer in the future.

Q: Will I be able to go full term in my pregnancy?

A: Yes, we hope to see you go full term. A CCAM does not necessarily mean early labor unless the rate of growth suggests that it is necessary.

Q: How many CCAMs have you dealt with? What was your success rate?

A: She said that they see about five a year. This year alone, I would be their sixth. There success rate is excellent. With the ones that were diagnosed this year, most of them have not had their surgeries yet as they are not within that six month window. One was supposed to have surgery this week but was forced to delay due to illness.

Q: What does the surgery entail and is there a less invasive option?

A: There are two types, the open incision surgery and the other which is less invasive and usually leads to a quicker recovery time and less scarring. They will not be able to determine which surgery our little girl will require yet. The less invasive option will not be available to us in the event that she is born under respiratory distress and needs surgery immediately after birth. However, in the event that she is asymptomatic at birth and does not require surgery until 6 months or later, the chances become better that they will be able to operate using the less invasive technique. Here is an article that a friend of mine linked to regarding the different techniques: http://www.columbiasurgery.org/news/si/2008_mas.html

Q: Will any special precautions need to be taken between the time of birth and surgery? For example, will she be more prone to respiratory issues? Will we need to stay restricted to the house as much as possible? Would her older sister be able to attend daycare, knowing that they become more susceptible to germs?

A: Obviously daycare can assist in bringing more germs into the mix. However, there aren't any other precautions that we will need to take.

Q: After the surgery, how will her respiratory function be?

A: Should be normal unless there are any unforseen complications.

Q: Does the lung regenerate? When you remove the lobe, will a new lobe grow in it's place? Will it function with the rest of the lung?

A: The lobe does not regenerate. However, the remaining lobe will continue to grow and expand to fill the empty portion of the cavity.

Q: If she doesn't show any symptoms when she's born, or the CCAM shrinks, would you still recommend surgery?

A: Yes, eventually. Otherwise, it can lead to a more likely possiblity of infection and/or can become cancerous in the future.

Q: What kind of recovery time are we looking at post surgery?

A: Very, very good. Babies are very resilient and often bounce back from surgery a lot faster than adults. Best case scenario, the baby would be required to stay in the hospital for four days post surgery. For two of these days, it is required that they have a tube inserted to drain the liquid from their lungs. The additional time is for observation. The stay could potentially last up until a week.


Our next major milestone will occur on Monday, 12/10 when I will have my pediatric MRI. I'm not exactly sure how long it will take to receive the results but I plan on stalking my perinatoligst until I hear something. ; ) LOL! I'll be sure to update my blog within a few days of the MRI when I know more information.