Congenital Cystic Adenomatoid Malformation, EXIT and ECMO: Jocelyn's Story

Geneva and Andy Nino remember not being allowed to hold their newborn baby daughter for fear of increasing her heart rate and blood pressure to dangerous levels. 

Now, they just have trouble holding onto 2-year-old Jocelyn because she’s always busy running around and “getting into trouble.”

After an abnormality was found at 21 weeks during a routine ultrasound, Geneva Nino was told that her unborn baby had a congenital cystic adenomatoid malformation (CCAM) — a large mass in the lower part of her left lung.

The doctors in Indianapolis, IN, referred the couple to The Children’s Hospital of Philadelphia. Initially, the Ninos wanted to seek consultation about the baby’s condition from CHOP and then care for mom and baby in Indiana.

“But the doctors (in Indiana) told me that if the baby developed hydrops, we would have to go to CHOP,” says Geneva. A week later, they boarded the plane for Philadelphia.

CCAM, then fetal hydrops

Among the life-threatening problems that CCAM can cause is fetal hydrops, an accumulation of fluid surrounding internal organs caused by congestive heart failure. Not only does hydrops pose a threat to the baby, but it can also be a danger to the mother by causing massive fluid retention leading to preeclampsia.

At Children’s Hospital, the Ninos met with Holly Hedrick, MD, and the fetal surgery team at the Center for Fetal Diagnosis and Treatment. Once there, the team measured the size of the CCAM.

CCAMs are measured using a CCAM volume ratio technique, which gives doctors an idea of how severe the mass is. A CVR of higher than 1.6 is dangerous; baby Jocelyn’s was 3.9.

“They told us that because the baby had developed hydrops, there were no guarantees,” remembers Geneva. “But they believed they could do an in utero shunt process to shrink the CCAM.”

Shunt surgery, then EXIT birth

Using ultrasound guidance, a shunt was placed in the baby’s chest wall to drain the cyst and reduce its size. In Jocelyn’s case, the shunt was able to drastically reduce the CVR  from 3.9 to 1.1 in less than 48 hours.

After a two-week stay in Philadelphia, the Ninos took a quick trip home to Indiana to “put things in order” and pick up their toddler son, Michael. They returned to CHOP for follow-up care and lived at the Ronald McDonald House in Camden, NJ, for three months.

Doctors decided it would be best to bring Jocelyn into the world using the EXIT (ex utero intrapartum treatment) procedure. On March 10, 2004, Jocelyn was delivered through an abdominal incision, but remained connected to the umbilical cord to allow her to maintain placental support while the CCAM was surgically removed.

Extra help with ECMO

Jocelyn initially was put on a ventilator, but after eight days, she required ECMO (extracorporeal membrane oxygenation), a heart/lung bypass machine that provides temporary breathing and cardiac support.

On April 1, Jocelyn was taken off the ECMO and began to gradually and steadily improve. She remained in the Newborn/Infant Intensive Care Unit (N/IICU) for two and a half months, then was released to Riley Children’s Hospital in Indianapolis on May 20.

“At that point, we were working on weaning her off of the morphine and oxygen, and trying to get through the feeding issues,” recalls Geneva.

Going home

Three weeks later, Jocelyn was released from the hospital, and went home on oxygen, a heart monitor, a feeding tube, and five different medications.

“It was a lot of work,” says Geneva. “We were up pretty much around the clock at first.”

“But she did great,” adds Andy. “Once we got her home she was happier and smiled a lot more.”

Jocelyn began physical therapy and occupational therapy at 3 months of age. She has now graduated from PT and OT, and is starting speech therapy.

While she still gets breathing treatments as needed, Jocelyn is a healthy, happy little girl whose favorite pastime is “beating up her big brother.”

Originally posted: June 2009

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