First Fetal Surgery Cases Set the Course for a Full Spectrum of Intrauterine Therapies

When pediatric and fetal surgeon Darrell Cass, MD, joined Cleveland Clinic in October 2017, his mission was to establish Cleveland Clinic as a global leader in fetal surgery. In only a few short years, Dr. Cass has made great strides toward this goal. Thanks to carefully timed and expert operations, fetuses that would have died can be saved and expected to develop normally, and fetuses with other anomalies can be repaired antenatally to minimize their risk for life-long disability.

The beginning of an era

A multidisciplinary team of Cleveland Clinic surgeons, led by Dr. Cass, performed the institution’s first fetal surgery in February 2019: a complex in utero repair of a neural tube defect in a nearly 23-week-old fetus. Historically, myelomeningocele (MMC) repair was completed postnatally, limiting the opportunity to prevent neurologic loss.

In December 2019, Dr. Cass and team successfully established an airway in a partially delivered fetus with a large trachea-obstructing neck mass, enabling safe birth and subsequent mass removal. The procedure, known as EXIT (ex-utero intrapartum treatment)-to-airway, takes advantage of uteroplacental blood flow and maternal-fetal gas exchange while the fetus’ trachea is secured. This minimizes the risk of life-threatening asphyxia or hypoxia at birth due to airway obstruction. In this first case at Cleveland Clinic, the fetus had a giant neck mass at the anterior aspect of the trachea that was compressing the trachea and esophagus, which posed a risk of death and need for risky emergency airway interventions immediately after birth. Instead, the airway was safely secured, allowing successful resection of the neck mass the day after delivery.

In June 2020, the Cleveland Clinic team successfully performed an EXIT-to-resection procedure on a partially delivered hydropic fetus with a large, right-sided lung mass. In the procedure, Dr. Cass exteriorized the lung mass, enabling safe birth and subsequent removal of the tumor. Although it was the first time Dr. Cass performed the operation at Cleveland Clinic, he has unique experience with this intervention, having performed it successfully 18 times previously. In total, Cleveland Clinic’s team has completed 15 open fetal surgeries through the end of October 2020.

Expert team

Cleveland Clinic Children’s Fetal Care Center is a collaborative team of highly skilled and compassionate healthcare professionals who offer state-of-the art techniques and interventions with a family-centered approach. Dr. Cass has high praise for the team and the outcomes they achieve.

“Each of these complex procedures requires the involvement of more than a dozen physicians and nurses who specialize in pediatric surgery, maternal-fetal medicine, fetal cardiology, pediatric and obstetric anesthesiology and neonatal intensive care. Additional specialists, like pediatric neurosurgeons for MMC, may be required depending on the procedure. At Cleveland Clinic, our patients benefit from a growing team of top subspecialists in their fields who work together seamlessly to help the mother and the fetus as it transitions from the in utero environment to life as a neonate,” Dr. Cass says.

The fetal surgery team includes maternal-fetal medicine specialists Amanda Kalan, MD, and Jeff Chapa, MD, pediatric cardiologists Francine Erenberg, MD, Rukmini Komarlu, MD, and Holly Nadorlik, DO, obstetric and pediatric anesthesiologists McCallum Hoyt, MD, MBA, Tara Hata, MD, and Yeal Dahan, MD, pediatric otolaryngologist Samantha Anne, MD, and pediatric neurosurgeons Violette Recinos, MD, and Kaine Onwuzulike, MD, PhD.

Timing matters

When it comes to fetal surgery, timing matters. In the MMC repairs, the mothers tend to present in the first trimester. Although the optimal fetal age is still being determined, at Cleveland Clinic the procedures were conducted around 22-25 weeks gestation.

“If you go too early, the fetus is too delicate and complications aren’t as well-tolerated,” Dr. Cass says. “If you go too late, the fetus risks further nerve damage from the amniotic fluid.” Upon completion, the hysterotomy is closed, the uterus returned to the mother’s abdomen and the laparotomy incision is closed. The mother is discharged in 4-5 days and is monitored closely until her delivery.

“We schedule EXIT procedures around gestational week 37-38 if possible to provide maximum time for fetal lung maturation. We monitor the mother for polyhydraminos which may prompt earlier intervention. Spontaneous labor should be avoided in these cases,” Dr. Cass.

Future plans

Dr. Cass expects the program’s volume and diversity of cases to continue to grow.

“I’m very pleased with the progress our program has made,” he says. “The complexity that we’ve been dealing with is already high. We’re going to do more and different types of procedures, and work to expand our outreach beyond the local region to our campuses in Florida and then to our international campuses. Giancarlo Mari, MD, a maternal-fetal medicine specialist with international expertise in fetoscopy and fetal treatment of twin-twin transfusion syndrome, has recently joined our Fetal Care Center team. He brings complementary expertise that will help take our program to the next level. In partnership with Dr. Mari and the entire fetal surgery team, our goal is to become one of the world’s leading fetal care programs. I’m very excited about the future,” Dr. Cass concludes.