Surgeries allow baby -- and his parents -- to breathe easy

Elizabeth Rodriguez-Garcia was nearly six months pregnant and in a celebratory mood when she went in for a routine ultrasound in July 2013. It would be the first baby, a boy, for Rodriguez-Garcia and her husband, Salvador Alvarez.

But something was wrong. The ultrasound technician found a large, dark spot where the fetus’s left lung should have been. The family was immediately referred to Stanford Children’s Health Perinatal Diagnostic Center in Salinas. Although Rodriguez-Garcia 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 was already examining a transmission of her ultrasound images and medical records.

“Even before all of us met face-to-face, we had many meetings to review the ultrasounds, the literature and our experience, and to formulate a plan,” said Susan Hintz, MD, the center director and professor of neonatology at the School of Medicine. The fetus had congenital pulmonary airway malformation, 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 but also compressing his esophagus and pushing on his heart. Worse, a new ultrasound showed that the cyst had grown even 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 the parents that a fetal intervention, which involved inserting a shunt to the fetus and draining the cyst, would offer the baby the best chance of survival.

'Like popping a water balloon'

A week after the cyst was discovered, Jane Chueh, MD, director of prenatal diagnosis and therapy at the hospital’s Johnson Pregnancy and Newborn Center, inserted a large needle into Rodriguez-Garcia’s abdomen and into the fetus’s chest using ultrasound guidance, then threaded a small rubber shunt through the needle into the cyst. “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, a clinical professor of obstetrics and gynecology at the School of Medicine. 

As Rodriguez-Garcia got closer to her due date, Hintz and the medical team discussed the next step — this one for delivery. Though the fetus was doing well, 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, a C-section was performed in an operating room on Nov. 25, when Rodriguez-Garcia was 39 weeks pregnant. A large team of surgeons, obstetricians, anesthesiologists, neonatologists and respiratory therapists were on site to respond immediately to save the life of the baby, who could have significant breathing problems.

Within minutes of birth, the baby was moved into another operating room, where the team, led by Karl Sylvester, MD, the executive director of the Center for Fetal and Maternal Health and associate professor of surgery, along with Matias Bruzoni, MD, assistant professor of pediatric surgery, removed the cyst and more than two-thirds of the baby’s lung that was adversely affected by the cyst.

The baby, named Elijah, went home to Salinas on Christmas Eve, after a month in the hospital. While it’s still too soon to tell what the long-term effects will be, doctors say his path so far is encouraging. Until a child is about age 7, lungs continue to grow, so there is a good chance that Elijah’s lungs will develop to a normal size.

Today, the only sign of his near-fatal start in life is the fading 7-inch scar on his chest, his parents said.