Fetal Surgery for Congenital Cystic Adenomatoid Malformation: Elijah’s Story

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To see 6-year-old Elijah run around and play, it’s hard to believe he needed a lifesaving surgery while he was still in his mother’s womb.

Elijah Elijah, now 6, had a CCAM tumor removed from his lung before he was born When April was just 21 weeks pregnant with Elijah, an ultrasound revealed that he had a mass called a congenital cystic adenomatoid malformation (CCAM) on his lung.

The physicians near the family’s home in Wisconsin referred them to the Center for Fetal Diagnosis and Treatment (CFDT) at Children’s Hospital of Philadelphia (CHOP), home to pioneers in fetal surgery. Two days later, April and her husband Jason flew out to Philadelphia for an evaluation.

“We needed CHOP,” says April. “That was really our only option.”

Early diagnosis and close monitoring are extremely important for fetal lung lesions because they can have serious implications on the health of both mother and baby.

The growth was pushing on Elijah’s lung and his heart. His esophagus was being kinked, which prevented him from swallowing amniotic fluid. As a result, amniotic fluid built up in April’s uterus.

“I looked like I was 40 weeks pregnant,” she says.

Despite treatment at CHOP with maternal steroids which can often stop or slow down the growth of CCAM lesions, the growth on Elijah’s lung got larger, resulting in heart failure known as hydrops. Fetal hydrops is a sign the baby will not survive if left untreated, and it can also make the mother sick with maternal mirror syndrome, a condition in which the mother develops toxemia of pregnancy. The team at the center offered three alternatives: abort, let the pregnancy continue and hope the lesion stops growing, or undergo fetal surgery. The Leffingwells knew the only option was fetal surgery to remove the tumor.

“Fetal surgery was the only hope to save the baby,” says N. Scott Adzick, MD, CHOP’s Surgeon-in-Chief and Founder and Director of the CFDT. “This is an operation that I developed years ago, and there are many success stories — happy, healthy children — as a result.”

Surgery before birth to remove the lung mass

Dr. Adzick and his fetal surgery team performed the delicate fetal pulmonary lobectomy procedure, removing a tumor 3.5 times the size of Elijah’s head and returning him to his mom’s womb. Before they could operate, the team had to remove one liter of amniotic fluid from the uterus. At one point during surgery, Elijah’s heart, then the size of a dime, stopped. Dr. Adzick had to massage the heart to bring Elijah back to life. The entire complex fetal surgery took 82 minutes.

After the surgery, April and Jason stayed in Philadelphia until delivery. The next few months were a rollercoaster. April’s uterine membranes separated and she needed to be briefly admitted back to the hospital for monitoring. Then, the lung that had had the mass collapsed due to a diaphragmatic hernia.

Elijah was born prematurely at 33 weeks in CHOP’s Garbose Family Special Delivery Unit (SDU), the first delivery unit within a children’s hospital designed for healthy mothers carrying babies with known birth defects. He weighed 5 lbs 9 ounces. The day after Elijah was born, he had to have surgery for his diaphragmatic hernia. He then spent nine days in CHOP’s Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU) on extracorporeal membrane oxygenation (ECMO), a form of heart-lung life support.

Two months after his birth, he was finally able to go home and meet his grandparents and older sister.

Life after CCAM surgery

Elijah smiling Elijah returns for follow-up visits with the Pulmonary Hypoplasia Program Elijah needed a feeding tube until he was 1 year old and took a little longer to achieve early milestones like crawling and rolling over. But he has since caught up. Now 6, he loves school and playing with his sister. While he can’t play hard contact sports like football for fear of a punctured lung, he plays basketball and T-ball, goes swimming, rides his bike, and rock climbs.

He wears a vest that inflates with air and pulsates his chest twice a day for 20 minutes. It helps keep his lungs clear. Prior to using the vest, Elijah had chronic respiratory issues. Now, his mother says he goes to the doctor less than his sister.

Elijah is now a patient of CHOP’s Pulmonary Hypoplasia Program, led by Holly Hedrick, MD. He returns to CHOP every two years for follow-up visits to monitor his lung health, evaluate his neurodevelopmental growth, and help meet his nutritional needs. Each time the family returns for follow-up, they are excited to see the nurses and doctors who cared for them during a scary time in their lives.

“We are blessed to be part of the CHOP family,” says April. “Everyone was so kind and gentle. They held our hand and walked us through the process. We have the utmost appreciation and gratitude for everything that they’ve done for us. To go above and beyond to try to help unborn fetuses so their outcome is a little bit better is absolutely amazing.”

The Leffingwells are forever grateful.

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