CMN - Congenital Melanocytic Nevus
At Bogota’s new Children’s Hospital, head paediatrician Dr Jaime Cespedes thought long and hard before accepting Didier’s case. Dr Cespedes “It is not an easy surgery, the lesion covers more than 60% of the body.”
Didier will be operated on by a team led by Dr Santiago Merchan. Dr Santiago Merchan “What’s at stake here is not whether the mole is pretty or ugly but.. But its potential of becoming malignant.”
As they draw up a treatment plan the doctors want to consult others on this uniquely challenging operation. They find a key source of support in London’s Great Ormond Street Hospital. Didier’s medical history has been sent to surgeon Neil Bulstrode.
Neil Bulstrode “It’s the worst case that I have seen due to the bulk of the lesion, because often you can have ones that are much flatter and, therefore, they’re much easier to deal with. you can see the other satellite lesions all around his body when I saw the pictures of Didier one of my first feelings was that by removing this it would significantly improve his quality of life.”
Neil regularly operates on CM cases in Great Ormond Street Hospital. Removing a mole, even a few inches across, requires delicate surgery. A mole such as Didier’s would be a huge undertaking.
Neil Bulstrode “Obviously, he has to go through a number of painful operations but I feel that, on balance, it would be worth it.”
Because Didier’s case is so severe, Neil volunteers to join the surgical team in Bogota
Neil’s colleague Dr Veronica Kinsler has been researching CMN for years working towards a non-surgical cure. She’s not convinced that surgery is always the best option.
Her research has led to the breakthrough that CMN is caused by a gene giving those affected characteristics in common. Doctors have found another hidden CMN symptom. In 25% of cases, moles develop in the brain.
Veronica Kinsler “These moles become malignant either in the skin, or more commonly in the brain, and in those cases it can be fatal.”
It’s the day of departure for Luz and Didier, when they reached the Colombian capital, doctors must find out if Didier’s condition has affected his brain. This complex treatment means he’d be away from his family for many months.
Jose “Our child is going faraway without his family by his side.. I don’t know when he’ll be back”
Luz “My father is stressed he’s staying away from the house.. He is really unhappy.”
Today they are meeting British surgeon Neil Bulstrode. He’s treated CMN mole’s before but never on this scale.
Next, all the surgeon’s get-together to plan what will be a pioneering operation. Santiago Merchan “We’re going to intervene in two surgical teams. Four plastic surgeons are going to operate at the same time.”
The plan is set. For surgeons in theatre will work simultaneously against the clock. The longer Didier is under the knife the greater the risk of blood loss is their major concern.
Neil Bulstrode “Hopefully by taking it at a gentle but safe pace we will be able to excise the whole lesion.”
Jaime Cespedes “How much we remove depends on blood loss. But let’s hope it’s as much as possible”
The surgeons now have a clear plan for this dangerous procedure. But they can’t move forward unless Didier passes some crucial tests. Hospital staff must check if he’ll be strong enough to withstand the huge physical ordeal of surgery.
There is one risk we must rule out above all. In some cases of CMN syndrome, moles can develop in the brain. This would cause major problems and change all their plans.
“Enemy number one.. Is a mole that has developed in the brain. Around 20% of cases cause moles in the brain. That’s why we need to watch out. The excellent news is that Didier doesn’t have any. We’re going to go ahead and operate.”
The operation to remove Didier’s mole can go ahead. With the operation green-lit, Luz has finally got the chance for Didier that she’s been battling for all his life.
Jaime Cespedes “Didier’s mother is a great mother, she’s a fighter. She obviously has come across all the difficulties of class and poverty. And yet, she’s been dreaming of doing something for her child. And dreams of being able to offer him the very best will now become reality.”
The last preparations for surgery go-ahead. Because of the worries about blood loss, staff must line up a blood transfusion in case of emergencies. With the operation now scheduled for tomorrow morning Luz’s hopes and fears come to a head. She brings Didier to Montserrat church high above Bogota to pray that he’ll make it safely through the surgery. At times Didier’s problems have caused Luz to doubt her Christian faith.
Luz “I want to get him baptised. I’m proud of my son he’s my child, my baby. The thing is, when he was born with his mole, it was so ugly. I prayed a lot to God that I would be able to find a solution.”
Now Luz can only hope that she’s made the right decision for her son.
The day of the operation has arrived, it’s a 5 AM start, but Didier is refusing to go to hospital. In desperation Luz calls Didier’s grandfather. The call home has worked, Didier is on his way.
Neil Bulstrode “He’s going to need repeated surgeries, they may span 6 to 8 weeks. He does have to go through a lot. He will be in pain and discomfort for the period of time to achieve the ultimate benefit.”
The doctors can’t give Luz any false hope, this operation is uncharted territory.
Santiago Merchan “Without a doubt, this is a surgery with very great risks and with the great challenges for us. I hope the family understands how risky and complicated this surgery is.”
The surgeon’s first take measures to prevent the greatest worry, that Didier will lose a dangerous amount of blood. With safety measures in place the first cut is made. The two surgical teams must now work together in a race against blood loss. The electric knife cuts through the soft tissue of the mole with ease. It’s the muscles and blood vessels beneath the need to watch out for. They must seal each blood vessel they expose. Luz is hoping for updates, but after a while the wait is too much. Didier is losing more blood than the surgeon asked for another adrenaline transfusion. It takes effect and the surgeons pick up the pace.
Luz has been told that the surgery could take five hours, Didier is only halfway there. The surgeons have hit a problem. The mole has grown deep into Didier’s body. The final pieces are removed from each side. The operation now takes on the next crucial phase, closing the wound with synthetic skin. This layer will keep Didier’s back safe until ready for skin grafts. After five hours of operating surgeons have successfully removed more than 5 kg of mole, a fifth of Didier’s bodyweight.
Neil Bulstrode “They’re going to wake him up gently, he’ll have regular dressing changes for three weeks, then you’ll be able to have the grafting”
So far, his mother’s prayers have been answered but this is only the start of a long and painful process. Didier must go under general anaesthetic 20 times as surgeons make new skin to cover the two thirds of his exposed torso. They graft skin from all over his body.
Updates on Didier’s progress are sent to London for Neil Bulstrode.
Doctors can’t be sure how long the recovery process will take, but Didier’s new body is slowly emerging.
It’s four months since Didier’s big operation and today is a big day for him. He’s finally well enough to look in the mirror. Doctors are pleased with his progress and he is ready to leave the hospital.p
Jaime Cespedes “Didier has recovered very well, and we are now happy that Didier can now return home. The surgery has been a success. We have taken all large nevus. His mother’s dream has been accomplished.”
Luz now wants to give thanks for Didier’s recovery. She’s arranged to have them baptised to mark his new start in life.p>
Didier “I felt bad after the surgery because my back hurt a lot. But now I want to see my family because I miss them a lot. I feel light without my patch. Now I don’t feel anything. No weight at all ”
Didier’s family are now making plans for the future. It’s time for Didier is to take another big step, he’s ready for his first day at school.
Additional Reading
[amazon template=template&title=-&artist=-&asin=1441977031] Epidermal Cell Tumors - Bruce R. Smoller, Kim M. Hiatt
[amazon template=template&title=-&artist=-&asin=0387403264] Pathology of Melanocytic Nevi - Michael Piepkorn, Raymond L. Barnhill
