CCAM - my baby needs an op

Hi
My baby was born with CCAM (congenital cystic adenomatoid malformation) - a mass of abnormal tissue growing in the lung. He needs an operation at 3 months old (he is now 5 weeks old) and although it is not uncommon and the surgeon reassures us that everything will be ok and that he does at least 2 of these operations a year - it is still hard.
I know our little Lachlan is a strong boy but I still can't help but worry.
Is there anyone else out there who's baby has had this? I think I am just looking for reassurance that someone else when through what we are going through and everything turned out ok.

Hi - I know it's been quite some time since your post but I also have had a baby diagnosed with CCAM... After getting chickenpox (me and bub) when she was 7 weeks old, she came down with pneumonia and an abscess on her right lung. X-Ray revealed a mass which could not be seen until the infection had been treated with IV antiobiotics and a CT scan taken. This revealed the CCAM in her top right lobe. My bub had this lobe removed at 6mths of age (after numerous viral infections) and even though I still noticed laboured breathing (particularly when laying down) the surgeon said that as far as they were concerned 'she's cured'. Well, after more viral infections (brochitis, bronchiolitis, etc), further investigations, including a sleep study, revealed that her diaphragm on the right side was paralysed. This can happen during surgery as the phrenic nerve that stimulates the diaphragm can be damaged when stretched, pulled, impinged upon, etc. This has meant home oxygen 24 hours a day for the last couple of months. What a learning experience this has been! We have had so many hospital visits and I have been on alert for most of her first year. I thought everything would be fine after her lobectomy but now she's having another surgery to plicate (tie down) her paralysed diaphragm. I am keeping my fingers tightly crossed that this will restore her breathing so that she will no longer require oxygen (although this can't be predicted). In my hospital experience there have been so many what-if's and maybe's and questions and explanations (or explaining away's) so that my feelings have been left out and put on hold... my local early childhood nurse has been great support but there's nothing like being in contact with someone who's actually been through the same kind of experience. So, I'm curious how everything went for you and your baby boy (who by now must be 5) and whether you had any ongoing dramas...

Hi there Alisonjholmes and Tbird007

I am currently 24weeks pregnant with our second child. At my 20 week abnormality scan, the sonographer noticed a mass in our boy's lung. Initially it was beleived to be a lung sequestration, but after another scan today, they think it may be CCAM and it has grown slightly.

The information on these lung abnormalities seems to be pretty scarce, so I've been trowelling google, trying to syphon through the nasty details. It was a little of a god-send to come across some Australian posts where people have experienced this first-hand.

We are supposed to be meeting with a paediatric surgeon at around 28 weeks, so hopefully they will be able to provide more advice and understanding - especially with what to expect when our baby makes it into the world. We're getting used to the idea that he will require some form of surgery, but some information on at what age, etc would be nice.

I hope your children are doing well, and am also very curious whether any other health issues have arised for your children.

Hi there Alisonjholmes and Tbird007

I am currently 24weeks pregnant with our second child. At my 20 week abnormality scan, the sonographer noticed a mass in our boy's lung. Initially it was beleived to be a lung sequestration, but after another scan today, they think it may be CCAM and it has grown slightly.

The information on these lung abnormalities seems to be pretty scarce, so I've been trowelling google, trying to syphon through the nasty details. It was a little of a god-send to come across some Australian posts where people have experienced this first-hand.

We are supposed to be meeting with a paediatric surgeon at around 28 weeks, so hopefully they will be able to provide more advice and understanding - especially with what to expect when our baby makes it into the world. We're getting used to the idea that he will require some form of surgery, but some information on at what age, etc would be nice.

I hope your children are doing well, and am also very curious whether any other health issues have arised for your children.

Hi smile

My son doesn't have this problem, but a problem was found on his 20 weeks scan. After much monitoring they still didn't now exactly what it was and he had to have some tests done after he was born.
They finally had narrowed it down to two things and decided that it needed to come out.

So at 4 1/2 months he had to have surgery. Very scary, but he did really well.

Anyway, my point is that if you feel the need to talk to someone about how you are feeling I will have some idea what you are going through smile

Hello Caetlyn''''''''sMummy,

It is very scary when you hear about CCAMs and then search online for information - there isn't a lot out there and the info that is available is fairly negative.

Our daughter's CCAM and pulmonary sequestration (she had an additional major artery going from her aorta to the CCAM) was detected at my 19 week ultrasound. I had regular ultrasounds after that to monitor the CCAM but thankfully as her lung grew the CCAM didn't.

When she was born she had a chest X-ray and it appeared clear. Her paediatrition advised that the CCAM was gone but I asked for a referral to a specialist for a second opinion. I'm glad I did because after seeing the specialist we learnt that x-rays don't tend to show CCAMs. She had a CT scan at 4 months which showed the extent of the CCAM and PS and removal was recommended ASAP.

We were very fortunate to be referred to a surgeon who was looking at performing lobectomies through key-hole surgery. She was the 2nd baby to have the surgery thorascopically and it was a success. After 48 hours in hospital she was home (she had the surgery at 8 months) and 12 months down the track we have just been given the all clear. She and her lungs are doing so well that her surgeon could no longer detect any difference between her normal and smaller lung.

So...although it is incredibly stressful, outcomes can be positive and with medicine developing the way it is, surgeries can be much more successful with less long term side effects. Talk to who ever you can, try to find out who is the best specialist/surgeon in your area if need be and go with your gut instincts! I hope everything works out for you.

I am also the mum to a baby with a CCAM. I also have an older son who is fine. The CCAM was first spotted at our 20 week scan on his lower right hand lobe. It continued to grow and pushed the heart over to one side. But after 28 weeks it got smaller in proportion to the rest of his lung. We had multiple scans throughout my pregnancy. I delivered him normally with a paediatrician present to check him immediately. He was really strong at birth, had a quick trip to NICU and an xray and 18 hours later we were home.

At 3 weeks we met with the surgeon who said “he’s well and we don’t mess with well” so referred him for a CT scan at 4 months. When we met with him a month later he decided we needed to operate within a few weeks. So now we are waiting for his surgery which is NEXT WEEK!

So I’m obsessively researching the internet, specifically interested in how babies cope with the surgery and how they recover. It’s really nerve-wracking waiting for the big day. But I think each second while he’s away from me in the operating room will be the hardest of all.

If anyone has any advice on what I should take to the hospital or what I should expect during his recovery (e.g. how long should I try and keep him away from other kids) I’d love to hear from you.

Hi GMcJ,

Probably the best advice I can give you is to look after yourself and get the support you need to get through the surgery and the days after. Take some books or magazines to read, some music and also a pillow to try and make sleeping in hospital easier. Also take some snacks and things like small packets of cereal, milk and coffee sachets for your stay.

When our daughter came out of surgery she was in intensive care for a day - that was the hardest because we couldn't sleep by her bedside and she was fairly restless as she had a c-pap machine to help her breathe as well as multiple tubes connected to all sorts of machines. Seeing her like that broke my heart BUT she recovered extremely well and was home a day later. When they are that little they are so resilient and even better don't remember a thing as they get older.

I was also breastfeeding her and the nurses were great at supporting me to feed her as soon as possible and as frequently as possible - it helped when she was unsettled in helping her sleep. I was lucky in that the nurses sent me to see the hospitals lactation consultant and she set things up so I could express during surgery.

Trust that the nurses are doing everything they can to make your baby comfortable and ask them any questions you have. They will probably be only to help you through it so rely on them if you need to.

I hope everything goes well with the surgery - let us know how things go if you can and feel free to ask any more questions whenever needed.

Hi I am glad I found this thread. I too have been looking online tonight. My 3rd son is nearly 4 months old, or really 3 months corrected (was born 3 weeks 5 days early) and had respiratory distress syndrome from the birth as no fluid left from the delivery. The blessing of this was he stayed in NICU and a chest x-ray to see extent of fluid in lungs revealed a shadow in lower right zone, no one was too concerned at the time as there was so much fluid there was no certainty what it was. We left hospital and then at 2 1/2 weeks old my son suffered from an acute life threatening event and had interrupted SIDS - interrupted as he was awake at the time it happened by having an acute obstructive apneoa where I gave CPR while the ambulance was on its way. Further x-rays continued to reveal a shadow in the lung still the same size. Possible pulomonary sequestration. 3 months down the track we had had a further chest x-ray last Tuesday and today revealed at paediatric appointment it is still there and paediatrician is confirming pulomonary sequestration. So it's not CCAM but I was wondering if I could discuss with you who have been and are going through this. We go for a CT scan soon and meet with a paediatric surgeon to discuss when his operation will be - between 9 - 15 months of age. I have read about the length of operating time and a little of afterwards and feeling quite overwhelmed. Only 2 operations for pulmonary sequestrations happen per year at our hospital, while the specialist says that is common being 2 I feel overwhelmed knowing my son is in that 2 total for next year. We've been told he will make a full recovery and can do a marathon if he is that way inclined when older, but I am stuck on the idea that what if they have to remove more than the sequestration.

I am studying midwifery and the specialist has told me he will write out medical letters so that I can give to the university to have compassionate consideration and hopefully extensions during the surgical time next year. He is also writing a letter so that if our son ever gets sick and we need to take him to hospital we do not have to wait in a&e, as he is suspectible to pneumonia.
I hope you are all doing well and that you have positive news.

Hello Jei,

I'm also glad you found this thread - the little amount of info out there about CCAMs and pulmonary sequestration makes it very difficult to feel prepared and positive about the conditions.

Our daughter had a pulmonary sequestration with a CCAM. The sequestration meant that she had an extra artery coming off her aorta that went straight to the CCAM providing it with a blood supply. Because the CCAM was not normal lung tissue the blood then left that part of her lung unoxigenated. From what the doctors explained (we were lucky to have been referred to a CCAM/sequestration expert at the Childrens Hospital) when you have a sequestration you tend to always have a CCAM. The surgery that my daughter had via keyhole removed the lower lobe of her left lung as well as clamping the extra artery. Although it sounds dramatic to remove a whole lobe, apparently it has better outcomes then a partial lobectomy - it's cleaner and has less complications.

The CT scan will show exactly what is going on so hopefully you will have some answers after that. I don't know where you live or if you could travel but if you are concerned about the surgery maybe you could ask about whether there are any other specialists in your area?

Good luck with it all and if you have any other questions please ask! I dont know it all but am happy to share our experience in the hope that it is helpful to others.

Hello Jei,

I'm also glad you found this thread - the little amount of info out there about CCAMs and pulmonary sequestration makes it very difficult to feel prepared and positive about the conditions.

Our daughter had a pulmonary sequestration with a CCAM. The sequestration meant that she had an extra artery coming off her aorta that went straight to the CCAM providing it with a blood supply. Because the CCAM was not normal lung tissue the blood then left that part of her lung unoxigenated. From what the doctors explained (we were lucky to have been referred to a CCAM/sequestration expert at the Childrens Hospital) when you have a sequestration you tend to always have a CCAM. The surgery that my daughter had via keyhole removed the lower lobe of her left lung as well as clamping the extra artery. Although it sounds dramatic to remove a whole lobe, apparently it has better outcomes then a partial lobectomy - it's cleaner and has less complications.

The CT scan will show exactly what is going on so hopefully you will have some answers after that. I don't know where you live or if you could travel but if you are concerned about the surgery maybe you could ask about whether there are any other specialists in your area?

Good luck with it all and if you have any other questions please ask! I dont know it all but am happy to share our experience in the hope that it is helpful to others.

Hi Bella
Thank you so much for your post. I am in Wellington, NZ. We have been told that the surgeons do 2 sequestration operations per year in Wellington. I'm not sure if there are any specialists in our area.
I did not know that a CCAM is usually associated with a sequestration, he did not tell us that only that you usually had one or the other.
It would be so good to hear about your story on what happened and how your little one is doing now. How old is she now and how is her health?
Thanks so much

Hi Jei,

I have posted some of our story earlier, but our daughter is now 21 months and has no ongoing lung problems (although I do still have a slight panic whenever she coughs!). Although she had her lower left lobe removed in entirety at 8 months, her Doctors can't detect any difference between her lungs which is a great sign that the remainder of her left lung has compensated for the removed lobe. She also just had chest and spine xRays and they are all normal.

Our daughter had the surgery via keyhole - was only the 2nd child to have it done in NSW but it meant her recovery was so much faster and she has 3 small scars under her left arm. She was out of hospital in 48 hours and was walking 6 weeks later! It may be worth asking whether Drs have started performing the surgery via keyhole in NZ? They may not be but you never know!

Although our daughter did not have any obvious signs of lung issues prior to her surgery I know how scary it is to know that something is wrong and not knowing what the final outcome will be. It isn't all as bad as what you read on the Internet though!

I'll be thinking of you and hope you will send an update following the CT scan.

Hi Jei,

I have posted some of our story earlier, but our daughter is now 21 months and has no ongoing lung problems (although I do still have a slight panic whenever she coughs!). Although she had her lower left lobe removed in entirety at 8 months, her Doctors can't detect any difference between her lungs which is a great sign that the remainder of her left lung has compensated for the removed lobe. She also just had chest and spine xRays and they are all normal.

Our daughter had the surgery via keyhole - was only the 2nd child to have it done in NSW but it meant her recovery was so much faster and she has 3 small scars under her left arm. She was out of hospital in 48 hours and was walking 6 weeks later! It may be worth asking whether Drs have started performing the surgery via keyhole in NZ? They may not be but you never know!

Although our daughter did not have any obvious signs of lung issues prior to her surgery I know how scary it is to know that something is wrong and not knowing what the final outcome will be. It isn't all as bad as what you read on the Internet though!

I'll be thinking of you and hope you will send an update following the CT scan.

Thank you so much for your information, I read above and that is so awesome about your daughter's recovery and operation. All I know is an incision is made on his right side not sure of anything else yet. And was told he will be in hospital for 5 days for recovery. Our doctor said after that he'll be fine to come home and that he'll be fine afterwards.
I understand you on the cough. My son gets a cough constantly, lasts for 1 - 3 days and what upsets me the most if you can hear his chest rattle I always think he will choke and get sick again. I keep with my positives that he is ok and he's been out of hospital for 3 months now and is doing ok with no major episodes. I just want to get to the operation and have it done. Does your daughter still get coughs often or is it now just a cold she will get? It seems like if we take our son out for even 10 minutes in weather that isn't hot and sunny, just a mild wind and with shade and he'll break out in a cough the next day.
I'm so glad to have found somewhere to talk to someone about it. I feel so isolated and that my child is just defected. First born early, then having apneoa's and had cpr, stayed in ICU for a week, and now confirming the sequestration. I just get so down about it like this isn't going to be a means to an end and having a healthy baby. I feel right now like forever he's going to be sick and unable to be like his brothers.