A Good Life for Gavin
On October 18 and 19, 2007, a multidisciplinary team of 15 highly trained medical staff at Children’s Memorial Hermann Hospital used a rarely required but critical procedure to transform a potentially disastrous delivery into a safe birth. Today, the infant who benefited from the Ex-Utero Intrapartum Treatment (EXIT) procedure is approaching his third birthday – and is on track physically and developmentally.
“The EXIT procedure uses surgical techniques that were first developed to treat tracheal occlusion,” says pediatric surgeon KuoJen Tsao, M.D., who performed the procedure with other members of the multidisciplinary team. “It has a narrow range of indications that include congenital high airway obstruction syndrome, large fetal neck masses with tracheal compression, cystic hygroma and large masses in the chest. This particular case involved an infant with a large neck mass characterized on ultrasound and suspected to be a teratoma.”
The mass on Gavin Meredith’s neck was initially detected during a routine sonogram at Memorial Hermann Southeast Hospital by Edward Yeomans, M.D., a maternal-fetal medicine specialist and associate professor in the department of Obstetrics, Gynecology and Reproductive Sciences at The University of Texas Medical School at Houston. The patient was transferred to the Texas Center for Maternal and Fetal Treatment at Children’s Memorial Hermann Hospital and the UT Medical School, where a second ultrasound was performed by maternal-fetal medicine specialist Joan Mastrobattista, M.D., who assumed Gavin’s mother’s care and coordinated her inpatient management.
“We identified the fetus as one who may benefit from airway management while still on placental support and tagged the case early on as a candidate for the EXIT procedure,” says Dr. Mastrobattista, who is a professor in the department of Obstetrics, Gynecology and Reproductive Sciences at the UT Medical School. “Our goal in cases with high airway obstruction is always the same – to manage the pregnancy carefully using fetal sonographic monitoring and other tests of fetal wellbeing, to watch for signs of excessive amniotic fluid, fetal compromise and early labor and to plan delivery as near to term as possible to ensure the best chance of survival with the least number of complications.”
From diagnosis to delivery, management of fetal high airway obstruction requires close coordination between the mother’s obstetrician and the team of specialists caring for the fetus. Depending on the diagnosis and characteristics of the case, the surgical team will include one or more obstetricians or maternal-fetal medicine specialists, one or more pediatric surgeons, one or more neonatologists, an adult anesthesiologist for the mother, a pediatric anesthesiologist for the fetus, a fetal radiologist, a team of experienced labor and delivery nurses, the operating room team of nurses, managers and techs and the neonatal transport nurses.
At 37 weeks gestation, following a fetal MRI for more accurate confirmation of the extent of the mass and location of the airway, mother and baby were scheduled for surgery. “Prior to the procedure, we confirmed fetal presentation and the location of the placenta. The patient was intubated, and we entered the abdomen through a Pfannensteil incision,” Dr. Mastrobattista says. “We used ultrasound to confirm placental location, because it is critical to avoid entering into the placenta to preserve blood flow through the umbilical cord and to maintain uterine relaxation, while allowing enough time to establish an airway. We entered the uterus through a lower transverse incision, well away from the placenta and utilized a uterine stapler on both sides of the hysterotomy for hemostasis. We delivered the head and neck, the anterior shoulder and the upper arm while maintaining uteroplacental circulation.”
Immediately following the partial delivery, a pulse oximeter was placed on Gavin’s hand to assess oxygenation. The surgical team was able to establish an airway by directly intubating the baby, avoiding an operative procedure.
“Once we’d intubated the baby, we could see an immediate increase in oxygen saturation on the pulse oximeter,” Dr. Mastrobattista says. “As soon as the airway was secured, we delivered the rest of the baby.”
Continuous close monitoring of anesthesia is critical to the success of the procedure. “In many ways EXIT is similar to a cesarean delivery but our goals are different,” says Dr. Tsao, who is an assistant professor in the department of Pediatric Surgery at the UT Medical School. “Like all fetal surgery, the EXIT procedure involves the treatment of two patients, the mother and her baby. In addition to maternal anesthesia, we maintain fetal anesthesia at the appropriate level for surgery while monitoring the baby closely to avoid cardiac depression. Because of the complexity of the procedure, we work with anesthesiologists who specialize in EXIT.”
Risks associated with the procedure include those that accompany a cesarean delivery – increased blood loss, infection, injury to surrounding organs, respiratory complications, a longer hospitalization and reactions to anesthesia. Because the uterus remains open for a longer period of time, mothers are at increased risk for infection and slightly higher risk for uterine atony. “There’s also the danger that we won’t be able to maintain the airway or hemodynamic stability before the baby comes off of placental support,” Dr. Mastrobattista says.
As with all fetal anomalies, high airway obstruction demonstrates the importance of a multidisciplinary medical team approach and accurate maternal-fetal diagnosis and testing, including the combination of fetal ultrasound and MRI in characterizing masses and other abnormal anatomy.
“The first step is identifying a problem,” Dr. Mastrobattista says. “If we can identify a problem prenatally, we’re ahead of the game. We can monitor the pregnancy closely with serial ultrasounds, fetal biophysical profiles and non-stress testing to monitor the health of mother and baby. In this case both of them did well.”
The day after Gavin’s delivery, Dr. Tsao resected the benign teratoma and performed a tracheostomy. “Gavin’s recovery went well,” he says. “We monitored him closely in the Neonatal Intensive Care Unit. He didn’t have many complications per se, but it took him some time to learn to breathe and feed on his own.”
Gavin went home to his grandparents and legal guardians Gail and Bruce Meredith, who have overseen his follow-up care. “Before we could take him home, I had to learn how to replace his trach tube and correctly use a suction machine, nebulizer, humidifier and pulse oximeter,” says Gail Meredith, whose grandson has inspired her to make a mid-life career change to nursing. “He had oxygen if he needed it, and I had tanks and hoses to hook up to the trach at night. He’s had a lot of different issues during the last two years, including laser surgery to treat subglottic stenosis caused by the trach tube. In the beginning it was really rough to get his weight up. Until he was about six months old, he was in the 10th percentile for his age, which was a big concern for us. As he began to put on weight, we got him to crawl and then walk. He was exactly 11 months old when he let go of the couch and walked to me on his own. From then on, his development has been nonstop and fast.”
The tracheostomy tube was removed in January 2010. “Gavin required the expertise of many specialists before, during and after his birth, from the time he was diagnosed as a fetus until he reached the point where he is today,” Dr. Tsao says. “Mentally and developmentally, he’s been nearly perfect. He was a little delayed in walking but caught up quickly. He’s currently working with a speech therapist to improve his speech.”
His grandparents are proud of his progress – and theirs. “Gavin gave me an entirely different outlook on life and health and illness,” says Gail Meredith, who after completion of an externship will graduate in September from Sanford Brown Institute as a registered medical assistant. “So many medical professionals have told me what Gavin might not be able to do because of his medical issues. None of those things have come true. He’s an amazing little miracle – and what we want for him is a good life. So when he dances around or climbs up on the couch, I say, ‘Keep going, baby. Do everything you can do.’”