Loma Linda University Children’s Hospital team of experts accomplish rare feat.
Published on: 07-09-2018
Jethro Quach, an infant diagnosed with a rare, congenital birth defect that would inhibit his ability to breathe independently at birth, was saved during birth thanks to a specialized procedure by a multidisciplinary team at Loma Linda University Children’s Hospital (LLUCH). LLUCH is the only tertiary care facility equipped to handle such a complex case in the Southern California region known as the Inland Empire.
Jethro suffers from congenital high airway obstruction syndrome, or CHAOS, which is an extremely rare fetal condition. Even before his birth, Jethro’s mother, Jennifer Quach, was monitored by the maternal-fetalmedicine team to manage her pregnancy, undergoing seven ultrasound procedures and three Magnetic Resonance Imaging (MRI) scans in the months leading up to her delivery.
Members of the medical team say that Jethro’s prognosis is hopeful but he has a long road ahead, with at least one more surgery necessary before he will be able to breathe completely on his own. Doctors are keeping a close watch on the baby, now almost two months old.
Ciprian Gheorghe, assistant professor of obstetrics and gynecology and a physician at the maternal-fetal medicine center at LLUCH, says Jethro’s condition could have caused abnormal lung development in utero, potentially leading to other issues. “We were hoping this would be one of the instances where the problem resolves itself, which is a possibility,” Gheorghe said. “Unfortunately, in Jethro’s case, he required a fetal surgery and another at birth.”
CHAOS is such a rare condition that fewer than 50 cases have occurred in the U.S. since 1989, according to Jill Howie, MSN, who was the executive director of the Perinatal Institute at LLUCH at the time of the surgery.
The life-saving procedure done at birth, an ex uterointrapartum treatment, or EXIT procedure, is so uncommon that it is only the second time it has been performedat LLUCH.
At 21 weeks’ gestation, a fetal echocardiogram that showed abnormalities led a pediatric cardiologist to suspect Jethro had CHAOS. After a week of additional testing, including a detailed ultrasound and a fetal MRI, doctors confirmed that Jethro had CHAOS.
Just below the vocal cords, a web of tissue was blocking the trachea, which would have stopped Jethro from taking his first breath at birth.
It was not the news Jennifer and her husband, David, were hoping for halfway through the pregnancy, something they had wanted since before getting married in 2014.
“We were ready to start a family right away,” Jennifer said. But they faced many challenges in their efforts to get pregnant, and when they finally did,they were elated, calling Jethro their “miracle baby.”
“We prayed and prayed for him,” she said.
With more obstacles ahead, the Quachs were determined to become a family of three.
At 24 weeks, doctors at the Los Angeles Fetal Surgery Center performed the fetal surgery on Jethro, who, at the time, was roughly the size of a cantaloupe. A small incision was made in Jennifer’s abdomen to insert a scope containing a laser. The laser made a hole in the membrane, allowing the fluid to drain from Jethro’s lungs so they could thoroughly develop and limit any risk of heart or brain damage.
In the weeks following the fetal surgery, Jennifer said, Jethro’s lungs began to develop, and his overall health was improving in the womb. However, he still would not be able to breathe on his own at birth unless he had a tracheotomy, a breathing tube inserted in his trachea, to help him breathe.
Jennifer was scheduled to give birth to Jethro via a Cesarean section on May 17, 2018, although this would not be a standard C-section procedure.
While Jethro was in utero, Jennifer provided him with oxygen, which passed through the umbilical cord of the fetus. “Once the umbilical cord is cut,” Gheorghe said, “he will no longer receive oxygen since he is not able to breathe on his own.”
According to pediatric otolaryngology surgeon Robin Dyleski, the only way to have had enough time to do the tracheotomy would be via the EXIT procedure, since the baby was still connected to the placenta and receiving oxygen from his mother.
During an EXIT procedure, the baby is delivered about halfway, with everything below his chest still in utero so that he remains connected to the placenta. It is done at that time to minimize the chance of brain damage, which can occur if the baby is deprived of oxygen.
Because of the complex nature of the case, the Quachs had a team of specially trained physicians from maternal-fetalmedicine, NICU, pediatric ENT (ear, nose, and throat), and pediatric anesthesiology working together to deliver their baby boy.
The Maternal FetalMedicine Center at LLUCH uses a multi-disciplinary team approach to provide comprehensive diagnosis, treatment, and management of high-risk, complicated pregnancies within a single family-oriented center.
Gheorghe performed the EXIT procedure, and Dyleski completed the tracheotomy.
“As soon as we learned of the diagnosis, we initiated a team to begin the months of preparation needed to ensure that everything was smooth and successful,” Dyleski said.
Once the tracheotomy was complete, Jethro was born weighing 5 pounds, 9 ounces, and although it was assisted, he was breathing on his own.
On June 28, 2018, at exactly six weeks old, Jethro was discharged from the NICU at LLUCH. His trachea was still dilated. The date for his next surgery, which will repair the blockage, is not set, but until then the Quachs are taking things one day at a time, they said.
“We are thrilled to have our baby home,” said Jennifer, who spent every day at the hospital with Jethro and the group of nurses and staff caring for Jethro. “It feels like family is taking care of us,” she said.
Jethro’s team of physicians are optimistic about his future. “This was a collaborative effort that involved a lot of different people and departments, and everyone worked well together,” Gheorghe said. The most important thing about CHAOS is an early diagnosis, detailed fetal assessment, and an adequate postnatal intervention for establishing an airway, “and that’s exactly what we were able to provide,” he said.