"We were devastated when we first learned that our daughter was not 'perfect' and diagnosed with spina bifida," Song said. "But the specialists we consulted with at Cincinnati Children's were so compassionate, supportive, and enthusiastic – and gave us so much hope."
When Song was 20 weeks pregnant, doctors diagnosed her baby with myelomeningocele, the most serious form of spina bifida. In myelomeningocele, a portion of the baby's spinal cord and surrounding nerves protrude through an opening in the spine into a flat disc or sac that is visible on the back. This exposes the baby's spinal cord to amniotic fluid in the mother's womb and can be harmful to fetal development.
Song was referred to Cincinnati Children's Fetal Care Center, a collaboration with TriHealth and UC Health, which provides comprehensive care for healthy moms expecting a baby with known medical conditions. The baby may require specialized care at the hospital's Level IV neonatal intensive care unit (NICU) or cardiac intensive care unit (CICU).
"Children who are born with myelomeningocele can experience serious health problems such as hydrocephalus, which is a potentially fatal excessive accumulation of fluid in the brain, and leg weakness or even complete paralysis. The severity of leg weakness depends on where the opening occurs in the spine," said Charles Stevenson, MD, a pediatric neurosurgeon and co-creator of the Fetal Myelomeningocele Surgery Program at Cincinnati Children's. "To help these babies have better functional outcomes, we began more than a decade ago performing and refining prenatal surgical techniques to optimize safety and efficacy for both mother and baby."
In 2011, a landmark clinical research effort called the "Management of Myelomeningocele Study (MOMS)" showed that babies who received prenatal repair of myelomeningocele can significantly benefit. The study compared the effects of fetal surgery versus surgical repair after birth. It followed 183 women and found that if a baby is operated on while still in the womb, some serious complications of myelomeningocele, such as the incidence of hydrocephalus, can be lessened. In addition, follow-up studies demonstrated that babies undergoing fetal repair of their myelomeningocele frequently exhibited improved leg strength and walking ability.
Shortly after the MOMS study was published, surgeons at Cincinnati Children's completed the first open repair surgery for a baby with spina bifida. In addition to the fetal surgeons, the care team includes maternal-fetal medicine specialists, pediatric, neurosurgeons, neonatologists, anesthesiologists, genetic counselors, developmental pediatricians and social workers.
"Our team works closely with each family to determine whether fetal surgery is a possible solution," said Foong-Yen Lim, MD, surgical director of the Fetal Care Center. "Fetal surgery for myelomeningocele can offer significant benefits to the baby, but it also carries serious risks and potential complications."
Lim and Stevenson performed the first myelomeningocele surgery at Cincinnati Children's in 2011 and also the 100th repair almost 10 years later, along with fetal surgeon Jose L. Peiro, MD, PhD, fetal endoscopic director of the Fetal Care Center, and Kara Markham, MD, a maternal-fetal-medicine specialist at UC Health.
During the surgery, the doctor makes an incision across the mother's abdomen and opens the uterus. The neurosurgeon removes the myelomeningocele sac, if one is present, and repairs the spinal defect in layers before closing the skin to protect the spinal cord from exposure to amniotic fluid. After the procedure, the uterus and abdomen are closed.
"At Cincinnati Children's, we provide extensive testing and counseling to ensure that every patient meets the criteria for fetal myelomeningocele surgery," Lim said. "We make sure parents have all the information they need to make an informed decision whether to undergo the procedure. We also understand if a parent decides to wait until after the baby is born. It can be a parent's choice, and we will support it."
In 2016, Peiro introduced a less invasive procedure at Cincinnati Children's. Fetoscopic repair of myelomeningocele can provide surgical outcomes for the fetus that are similar to those of open surgery while lessening the risk of maternal complications.
Once the mother is under anesthesia, surgeons make an incision in the abdomen to expose the womb. An ultrasound confirms the location of the fetus and shows the best location to insert the first port. Doctors then expand the amniotic cavity using humidified and heated carbon dioxide, which creates more space to do the repair. To see inside the womb, a camera is inserted into the port. Two additional ports are then inserted to allow the instruments to make the repair.
"We are starting to do more fetoscopic repair surgeries because it allows the mother an opportunity to have a vaginal delivery versus open surgery which requires a Caesarean," Peiro said. "Fetal repair offers a number of benefits for the baby. It can lower the rate of hydrocephalus and decrease the need for brain shunts. Plus, it can reduce the risk of damage to the spinal cord and nerves caused by exposure to amniotic fluid. Our goal is to repair the damage as soon as we can."
To date, Cincinnati Children's has performed 56 open repairs and 51 fetoscopic repairs. How well a child with myelomeningocele does after fetal surgical repair depends on many factors. Some children will require minimal ongoing care, while others will require medical and educational support for many years.
The Center for Spina Bifida Care at Cincinnati Children's provides comprehensive, interdisciplinary and family-centered medical care to infants, children and adolescents living with repaired myelomeningoceles and other neural tube defects.
Kelsey Song was the 100th patient to undergo prenatal surgical repair at Cincinnati Children's. Her daughter, Flora, spent close to a month in the NICU due to her premature birth but is now home.
"Flora had a head MRI while in the NICU, which showed significant improvement in her Chiari Malformation. Chiari is a complication of spina bifida where the cerebellum is dragged downward due to the spine opening. The repositioning of the cerebellum inhibits the normal flow of fluid around the brain and causes pressure buildup (hydrocephalus)," Song said. "However, in Flora's case, fetal surgery allowed her brain time to reverse back to a more normal position before birth. Her MRI showed a very healthy brain with no pressure buildup, which is amazing and, for us, means that her surgery was successful!"
Every 3 months, Flora will come back to the Spina Bifida clinic at Cincinnati Children's to meet with all of her specialists. They will continue monitoring her growth and development.
"We are so very thankful for Cincinnati Children's and for everyone we worked with over the past several months," Song said. "Our fetal surgeons, fetal nurse coordinator, maternal-fetal-medicine doctors, neonatologists, NICU therapists and NICU nurses are incredible. They truly have changed our lives and our daughter's life."
About Cincinnati Children's Fetal Care Center:
Cincinnati Children's Fetal Care Center is one of few comprehensive fetal care centers in the world. Led by physicians from Cincinnati Children's, TriHealth and UC Health, our team specializes in treating complex and rare fetal conditions. We provide mothers and their babies with a continuum of care from prenatal diagnosis and fetal treatment to delivery, postnatal care, and long-term follow-up. Since 2004, our team has evaluated more than 6,800 high-risk pregnancies. This experience enables us to provide the most sophisticated, effective therapies available and strong outcomes for the most complex conditions.
SOURCE Cincinnati Children's Hospital Medical Center
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