PALO ALTO, Calif.--(BUSINESS WIRE)--Elizabeth Rodriguez-Garcia was nearly six months pregnant and in a celebratory mood when she arrived for a routine ultrasound July 2013. It would be the first baby for Elizabeth and her husband, Salvador Alvarez.
Pictures of the fetus appeared on the monitor. It was male, but within minutes it was clear something was wrong. The ultrasound technician found a large, dark spot where the fetus’ left lung should be.
They were immediately referred to Stanford Children’s Health Perinatal Diagnostic Center in Salinas, Calif., where technicians would take a closer look. Even though Elizabeth was still 80 miles away from Lucile Packard Children’s Hospital Stanford, a group of experts at the hospital’s Center for Fetal and Maternal Health were already examining a transmission of her ultrasound images and medical records. The highly specialized center offers counseling, management and lifesaving medical services to mothers with high-risk pregnancies and their newborns. Given the situation, Elizabeth’s obstetrician referred her to the center for further evaluation and continuing care.
When the worried couple arrived for their hospital appointment a few days later, they were met by a team of obstetric, pediatric and surgical experts – including the center’s medical director Susan Hintz, MD, who is also a professor of neonatology at the Stanford University School of Medicine. The team gave them not just a diagnosis, but a plan to save the baby’s life.
“Even before all of us met with Elizabeth face to face, we had many meetings to review the ultrasounds, the literature and our experience, and to formulate a plan,” Hintz said of the team’s comprehensive and coordinated care approach. “We wanted to offer her the best and safest care.”
The fetus had a congenital pulmonary airway malformation, known as a CPAM, which meant he had a large, abnormal cyst in the lower left side of his lung. The fluid-filled cyst was not only impeding growth of his lung, it was also compressing his esophagus and pushing on his heart. Causing even more concern, a new ultrasound showed the cyst had grown larger and that the fetus was developing fluid retention, a condition known as hydrops. He was at high risk of dying in utero. The doctors told Elizabeth and Salvador that a fetal intervention, which involved inserting a shunt through her to her fetus and draining the cyst in the fetal lung, would offer the best chances of survival.
A week after the cyst was first found, Jane Chueh, MD, director of prenatal diagnosis and therapy at the hospital’s Johnson Pregnancy and Newborn Center and a clinical professor of obstetrics and gynecology at the School of Medicine, inserted a large needle into Elizabeth’s abdomen and into the fetus’ chest using ultrasound guidance, then threaded a small rubber shunt through the needle into the cyst. It was the first use of the procedure at Lucile Packard Children’s Hospital Stanford.
“It immediately started to drain,” Chueh said. “It’s like popping a water balloon. Most of the fluid came out in seconds.”
Relieving pressure from the cyst came at a critical time, said Chueh. The dangerous fluid retention that doctors worried was endangering the baby’s life improved dramatically.
Delivery and surgery
Elizabeth spent a couple more weeks as an inpatient at the hospital undergoing nearly daily ultrasounds. Afterward, she stayed nearby where she could be frequently monitored and seen quickly if something unexpected happened.
As Elizabeth got closer to her due date, Hintz and team discussed the next step in their plan – this one for delivery. Though the fetus was doing well in utero, ultrasounds showed that once he was born, the cyst might need to be removed by emergency surgery to allow him to breathe properly on his own.
To simplify the transition between delivery and surgery, the scheduled C-section was performed Nov. 25, when Elizabeth was 39 weeks pregnant, in an operating room instead of the hospital’s labor and delivery center. That’s because a vast team of surgeons, obstetricians, anesthesiologists, neonatologists and respiratory therapists would be needed immediately to save the baby’s life.
“We had everyone on deck,” said neonatologist and clinical assistant professor Alexis Davis, MD. “There were around three dozen specialists assembled. We had to be prepared because we knew the baby could have significant breathing problems at birth.”
The operating team, led by surgeon Karl Sylvester, MD, the center’s executive director as well as an associate professor of pediatric surgery, stood by. Within minutes of birth, the baby was quickly moved into Sylvester’s operating room, where he and the surgical team, including assistant professor of pediatric surgery Matias Bruzoni, MD, removed both the cyst and more than two-thirds of the baby’s lung that was adversely affected by the cyst.
“Our ability to provide all these subspecialists in two rooms to care for both the mom and the baby is what led to the successful outcome for this family,” Sylvester said. “It made a huge difference in this young family’s life; without it, he may not have survived at all.”
The baby, who was named Elijah, received care in the hospital for almost a month. On Christmas Eve, Elijah and his parents went home to Salinas. Sylvester said it’s too soon to tell what the longer-term effects will be, but his path so far is encouraging. Until a child is about 7, lungs continue to grow and remodel, so there is a good chance Elijah’s lungs will develop into a normal size.
His parents say the 5-month-old is a healthy, happy baby, whose only sign of his near-fatal start in life is the fading 7-inch scar on his chest. He can sit on his own, push himself up on his belly, and loves to laugh and make sounds for his parents.
“He looks completely normal,” Elizabeth said recently. “If you see him, you’d never know what he went through and that he doesn’t have most of his left lung. The cyst is completely gone. I feel blessed.”
A lifesaving network
For Elijah’s family, it’s a happy ending that resulted from early detection, expert collaboration and a network of care that connected patient and providers in time to make a lifesaving difference.
“Through our Perinatal Diagnostic Center in Salinas,” said Hintz, “Elijah and his family had access to the extraordinary expertise of the physicians and specialists at the Center for Fetal and Maternal Health.”
The Stanford Children’s Health network of pediatric and obstetric care is the largest in Northern California and the U.S. western region, with specialty centers, partnerships, collaborations and outreach at more than 100 locations in eight states.
“This was a fantastic outcome,” said Hintz. “Our multidisciplinary team carefully and thoughtfully considers the best treatment approach for each of these challenging cases. We are extremely fortunate to have the expertise and experience to assure the best possible outcomes for extremely complex fetal patients and their families.”