Kim Laviolette sobbed and squeezed her boyfriend’s hand on the hourlong drive from Children’s Hospital of Wisconsin in Wauwatosa to their Kenosha home…
They had just learned their unborn son had heart failure.
Although the boy’s heart had beat normally during an ultrasound that morning and he kicked vigorously each day, doctors said a large tumor had cramped his heart into the right side of his chest and stopped his lung growth.
The baby’s name was going to be Aden. He likely would die inside her.
As a last resort, the couple allowed doctors to use a novel technique to shrink the tumor and give his lungs a final chance to develop.
The surgery would not be easy, and no one knew if it would work. Several doctors had performed a similar procedure at other hospitals, but nobody had attempted it here.
For the third time in less than a month, Laviolette and Jay Nelson grieved the possible loss of their son. This time felt different.
They prepared for the inevitable.
“We had to have the conversation we never wanted to have,” said the 34-year-old Laviolette. “What will we do if he dies? Do we have a funeral? I told Jay I’d have to have an immediate C-section because I couldn’t live with (Aden) not living inside me.”
In October, Laviolette and Nelson headed to the hospital for a routine ultrasound. Both were excited to finally learn whether they’d have a boy or girl.
Already a mother of three boys from a previous marriage, Laviolette knew what to expect at the visit. She knew the warning signs to look for during a pregnancy and hadn’t had any complications – not even a bout of morning sickness. Eighteen weeks into the pregnancy, she was feeling great.
But as the technician rolled the wand over Laviolette’s belly, she saw a bright spot in the baby’s chest that would normally appear gray.
The technician became silent. She then turned toward the couple.
“We knew right then that there was a problem,” said Nelson, 32.
The baby boy had a cyst the size of a small orange on his lung that was surrounded by several smaller ones. It was a rare condition called congenital cystic adenomatoid malformation; doctors refer to it as CCAM.
Although the majority of babies with the condition do well because the mass shrinks before birth or their lungs continue to grow, about 10% have tumors that interfere with heart and lung function. These babies develop a life-threatening condition called hydrops. Fluid seeps into the bloodstream and moves into the tissue, causing severe swelling and, ultimately, heart failure.
About half of fetuses with hydrops die.
“Initially, doctors told us (we could) end the pregnancy,” Nelson said. “They said, ‘This is bad, this is really bad.’ But we were like, ‘No,’ there was no way we would give up hope.”
When Froedtert Hospital perinatologist Thomas Wigton first met Laviolette a month after her ultrasound, the mass on her son’s chest already had grown.
Wigton, who’d seen only one severe CCAM patient in his practice, consulted physicians at The Children’s Hospital of Philadelphia who frequently treat the condition.
The Philadelphia hospital has performed nearly 80 shunt placements to drain large cysts since 1998, said Lori Howell, executive director of the Center for Fetal Diagnosis and Treatment at the Philadelphia hospital.
“We’re much better with calculating volumes of masses, and we can determine when someone will get into trouble,” she said. “We watch (patients) really well and don’t miss the window of opportunity for placing a shunt.”
Wigton, who is also an associate professor of medicine at the Medical College of Wisconsin, wanted to know viable treatment options and whether the Philadelphia doctors would be willing to see Laviolette at their hospital.
“When we approached the family and told them they could be on a plane tomorrow, they weren’t interested,” Wigton said. “So, we had to rattle the troops here and push the envelope a little bit.”
They had little time.
If the baby developed hydrops, Laviolette could experience a life-threatening condition called mirror syndrome and begin to show signs of preeclampsia, including swelling and high blood pressure.
A team of 12 specialists met to discuss what they could offer and gave Laviolette and Nelson the options.
They could do nothing, which would result in fetal death.
Laviolette could take two steroid shots. A few studies had suggested that steroids could slow tumor growth and decrease the complications of prematurity, should Laviolette deliver early. None of the doctors believed it would be beneficial.
The final two options would drain the tumor. One was similar to an amniocentesis, but would remove fluid from the cyst instead of the amniotic fluid. The other involved the placement of a catheter into the cyst to continuously drain it until birth, when the large mass could be surgically removed.
“Moms will want to do everything,” said Aimen Shaaban, an assistant professor of general surgery with the University of Wisconsin’s School of Medicine and Public Health in Madison. “You can’t lose the mother or let them sacrifice themselves for their baby.”
Many don’t realize that they are the primary patient, not their child, said Shaaban, who was not involved in the case.
In late November, Laviolette was given two steroid shots. The baby’s condition worsened. So Wigton tried to collapse the cyst by draining it. Despite removing about 12 tablespoons of fluid, the mass refilled.
About two weeks later, fluid was building up in the baby’s skin, scalp and abdomen. Laviolette also was feeling sick. She gained 15 pounds in two weeks and started swelling.
Froedtert perinatologist Mary Ames Castro called on the hospital’s interventional radiologists Sean Tutton and William Rilling for help in inserting a catheter to drain the tumor. Rilling is a professor of radiology and Tutton an associate professor of radiology at the Medical College.
First, the pair needed to tweak their child-sized instruments to fit into fetal space. They boiled water and bent them to the shape they needed. They drew pictures and studied ultrasound images to figure out how to insert the catheter.
On Dec. 20, the pair began their first attempt at placing a thoracoamniotic shunt. Laviolette was given anesthesia to keep her comfortable and lessen the movement of the baby during the procedure. Doctors didn’t want to keep him still – they needed him to move so that they could find the optimal placement for the shunt.
The doctors used the ultrasound image to locate the tumor and waited for the right opportunity to push the needle through the baby’s back and into his chest.
“There were four or five times when we were ready and then he moved,” Tutton said. “The heart was within a centimeter or two of where we wanted to go, and we didn’t want to go into it instead.”
Once the needle was inserted, the doctors carefully passed a wire through the needle into the cyst. They then removed the needle and slid a catheter over the wire and into the tumor to allow it to drain into the amniotic sac.
About four weeks later, Laviolette – now 30 weeks pregnant – returned for another routine ultrasound. Once again, she got a surprise.
Her cervix was thinning. She was now in preterm labor and would soon deliver.
Aden Lawrence Nelson was born Jan. 15. He weighed 4 pounds, 11 ounces. Although he was small, the swelling had subsided and the cyst now looked like a squished balloon. Surgeons removed the rest of the cyst and part of his left lung the next day.
“The expectation is that there’s probably no long-term complication, but only time will tell,” said Casey Calkins, a Children’s Hospital of Wisconsin pediatric surgeon involved in the case.
Aden remained in the hospital for six weeks.
He’s still active, but not fussy. And he looks just like his dad.
“When he was born, I was almost afraid to hold him because I didn’t want to be told an hour later that he was dead,” Laviolette said. “But as the machines started going away, I started feeling more confident. Now he’s just a normal preemie and we can all breathe easier.
I might be getting a gloomy bear plushie. i don’t know what colour yet. Red, Pink, Green, Blue, Yellow, Black and/or Grey. i got a few samples of these plushies.