CCAM — Ryan | The Children's Hospital of Philadelphia

Center for Fetal Diagnosis and Treatment

Surgery at Birth — Surgeons Use EXIT Procedure to Remove Large CCAM from Logan Ryan Before Cutting Umbilical Cord

Logan Ryan has a bubbly personality, a dazzling smile, sparkling eyes... and a little scar than runs from under his armpit to his spine that will make a great story some day. It’s a story that includes a frightening diagnosis, hundreds of miles of travel and life-saving surgery performed the day he was born.

Logan’s story begins in Chicago in early February of 2005 when his mother, Jennifer, went for a routine 20-week ultrasound. Two days later, the doctor called to say that the ultrasound showed “something wrong with the baby’s lungs.” It was a congenital cystic adenomatoid malformation (CCAM) – a large mass – in the baby’s left lung.

CCAM can cause a myriad of life-threatening problems for the baby, including fetal hydrops, an accumulation of fluid surrounding internal organs caused by congestive heart failure. Not only was the baby in danger from the mass, but Jennifer was also at risk. CCAM can cause massive fluid retention in the mother, which can lead to preeclampsia.

“My whole life flashed before me,” remembers Jennifer. “All the visions I had of having a perfect baby disappeared.”

Jennifer and husband Jeff started scouring the Internet for information on CCAM, but came up with very little – until they found The Children’s Hospital of Philadelphia site.

“It had the most information we could find anywhere,” says Jennifer. The couple called Children’s Hospital on February 16, and arrived in Philadelphia the following week.

After undergoing tests and meeting with N. Scott Adzick, M.D., surgeon-in-chief and director of the Center for Fetal Diagnosis and Treatment, it was determined that the baby’s condition was good and no complications were anticipated. If all continued as expected, Jennifer would be able to deliver in Chicago, then bring the baby to Children’s Hospital at the age of 6 weeks for surgery to remove the mass.

However, those plans changed at around 32 weeks when regular ultrasounds showed that the mass was continuing to grow. The Ryans hopped a plane back to Philly for a consultation.

“We were scheduled to leave a few hours after meeting with the doctors,” says Jennifer. “But they told us that it was so much bigger that they felt we should stay in Philadelphia.”

At 36 weeks, the ultrasound showed the mass had grown even larger.

“We were concerned that the mass was so large there was a very high risk of it causing heart failure or fetal hydrops,” said Dr. Adzick. In addition, Dr. Adzick was concerned about extreme respiratory problems the growing mass could cause for the baby.

He told the Ryans that Logan’s birthday – as well as his surgery – would be in four days.

On June 7, 2005, Dr. Adzick and his medical team removed the mass using the EXIT procedure. During EXIT, which stands for ex-utero intrapartum therapy, the baby is delivered through an abdominal incision, but remains connected to the umbilical cord to allow the baby to maintain placental support.

“With Jennifer under general anesthesia, we went in, removed the mass on the left side, and closed the chest... all before clamping the umbilical cord,” explains Dr. Adzick. Once the umbilical cord was cut, baby Logan, weighing in at approximately 6 pounds, was whisked away to the neonatal intensive care unit.

As Dr. Adzick escorted baby Logan down the hall to the Newborn/Infant Center, Jeff could see he was smiling. Ten days later, Logan was so strong that he was released from the hospital.

For the Ryans, little Logan’s story has a happy ending.

“The kid is cured,” says Dr. Adzick, adding, “He’s perfect.”

Jeff and Jennifer couldn’t agree more.

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Learn more about congenital cystic adenomatoid malformation»

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