Congenital Cystic Adenomatoid Malformation (CCAM) Surgery: Owen’s Story

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The Fetal Therapy Nurse Coordinator at CHOP’s Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment was instrumental in guiding Melissa and Rory through one of the scariest times of their lives. She continues to stay in touch with them to this day, checking in on their 3-month-old son, Owen, who is happy, healthy and doing well at home.

A few months into the COVID-19 lockdown, Melissa, 37, had exciting news for her husband, Rory, and their two children — ages 2 and 5: the family of four was soon going to be a family of five! It was unexpected but welcome news in a year that desperately needed a silver lining.

Owen smiling Due to social distancing measures, Melissa’s first few appointments with her obstetrician were done virtually. She was able to see her OB and a maternal-fetal medicine (MFM) specialist in person as her pregnancy progressed. Her 16-week anatomy scan with the MFM was uneventful, however, her 20-week scan revealed some issues. Rory wasn’t allowed to go with Melissa to the appointment, so he called her just as the MFM was coming into the exam room and listened in on speaker phone.

The information that followed hit the couple like a ton of bricks. The MFM said there was a mass in the fetus’s chest, and it had pushed the heart over to the other side of the chest cavity. Because the condition could be life-threatening, the couple needed to see a specialist immediately. Though the family lives in New York City, the MFM said the nearest center equipped to handle their care was Children’s Hospital of Philadelphia’s Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment.

Within 48 hours, Melissa and Rory were in the Center’s waiting room. The first person they met with was Fetal Therapy Nurse Coordinator, Cathy Mackell, BSN, RNC-NIC. The Center’s team worked to gather a detailed and thorough picture of the fetus’s condition using the most advanced prenatal imaging techniques and state-of-the-art technologies available. Cathy made sure the family was prepared for what was going to happen at each appointment and that their personal needs were met.

“Our expectations were managed in a way I’ve never experienced in medicine before,” says Rory. “What a well-oiled machine. It was patient first. Everything revolved around my pregnant wife. The doctors were waiting for us, not us waiting for the doctors. It was a totally different experience.”

Thorough diagnosis and collaborative prenatal monitoring

The fetus had a congenital cystic adenomatoid malformation (CCAM), a benign lung lesion that appears before birth as a cyst or mass in the chest. CCAMs (also called congenital pulmonary airway malformations, or CPAM) can vary in size and be either fluid-filled or solid. The vast majority of CCAM/CPAM lesions are small enough that they will not cause any problems to the baby during pregnancy and the CCAM/CPAM can be removed after birth. Some large lesions can cause serious and even fatal complications, including fetal heart failure (also called fetal hydrops) or maternal mirror syndrome. These cases may require treatment before birth.

The Center’s team used specialized tests to determine the type, size and location of the mass, where the blood supply was coming from, and if it was impacting the fetus’s heart. At the end of the day, Attending High-Risk Obstetrician, Shelly Soni, MD, and the rest of the care team met with Melissa and Rory to explain the condition, drawing illustrations to ensure they completely understood what was going on in the fetus’s body. They shared the positive news that the CCAM/CPAM was moderate in size. The Center’s team has experience managing lesions three times larger.

“We went from driving down there literally in tears and shaking to driving home with tears of joy,” says Rory. “Yes, there was a rough road ahead, but they knew how to handle it and said our baby was going to be just fine. That was really a relief.”

A treatment plan for CCAM/CPAM

The plan was to monitor the growth of the CCAM every two weeks for the remainder of the pregnancy. If things were normal, which the CHOP team expected, Melissa would be able to deliver at a hospital in New York City and then the family would return to CHOP for surgery after birth to have the lesion removed. Due to the family’s distance from CHOP — about a two-hour drive — half of the follow-up appointments were done at CHOP and half were done by their MFM near home, with the two care teams coordinating throughout.

Owen was born in New York City on December 26, 2020. He spent two days in the hospital with his parents and was discharged home. He was healthy enough that he got to enjoy a month at home with his family before surgery at CHOP to remove the CCAM.

Surgery for CCAM/CPAM at CHOP

Fetal and Pediatric Surgeon, N. Scott Adzick, MD, MMM, performed Owen’s surgery, removing the lesion as well as the upper left lobe of his lung. After surgery, Owen was admitted to CHOP’s Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU). It was hard for Rory and Melissa to see him connected to so many tubes and machines, but Owen wasn’t on them long. After just six hours, Melissa was already able to nurse him and he was recovering well.

“The nurses in the N/IICU were absolutely amazing,” says Rory. “Besides doing their job of caring for Owen, they were also really good at listening to us, sharing in our experiences and helping us get through those difficult first few hours.”

Home and happy

Owen Today, Owen is a happy, healthy 4-month-old who loves being held and adores his two older siblings. A chest X-ray since surgery shows that his heart is back in its rightful position.

“Everything was fantastic,” says Rory of the experience at CHOP. “The results speak for themselves.”

Cathy Mackell still checks in with the family by text or email every week or two. The family is looking forward to seeing her and the rest of their CHOP care team in person at Owen’s six-month follow-up visit.

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