It was a day Kasey Hilton had been anticipating from the beginning of her pregnancy – finding out whether she was having a boy or girl. But the ultrasound appointment in June, 2014 brought news she and her husband Mike weren’t ever expecting to hear. They were having a baby boy, but he had a serious spinal cord defect called myelomeningocele that is associated with lifelong disabilities.
There was, however, hopeful news. University of Michigan’s C.S. Mott Children’s Hospital had recently become the only hospital in the region and among just over a dozen in the country to offer fetal surgery to correct the defect months before birth. The procedure prevents further damage to the spinal cord and improves neurologic function.
The Hiltons spent the day meeting with many experts from the Fetal Diagnosis & Treatment Center at C.S. Mott Children’s Hospital and Von Voigtlander Women’s Hospital. After extensive testing, doctors determined that Kasey and the baby were potential candidates for the procedure. The Hiltons then underwent multi-disciplinary counseling by the team to understand the relative maternal and fetal risks and benefits.
It takes a team
“A prenatal diagnosis of myelomeningocele can be overwhelming for families, but this new procedure offers hope for better outcomes,” says Marcie Treadwell, M.D., director of U-M’s Fetal Diagnostic Center.
“Although not a cure, fetal surgery prevents ongoing damage to the spinal cord and is associated with improved neurologic outcomes,” says Cormac Maher, M.D., associate professor of pediatric neurosurgery.
Last July, when Carter was only 23.5 weeks gestation, Kasey underwent open fetal surgery with the hope of improving her baby’s outcome. This procedure required an experienced multi-disciplinary team working seamlessly together. Maternal anesthesia was provided by Dr. Baskar Rajala, pediatric anesthesia by Dr. Paul Reynolds, and fetal monitoring by Drs. Sarah Gelehrter and Rebecca Jane Vartanian. Five surgeons were involved in the procedure – Treadwell, Maher, pediatric surgeon George Mychaliska, M.D. and maternal fetal medicine specialists Deborah Berman, M.D., and Clark Nugent, M.D.
The doctors performed open fetal surgery which involved deep maternal anesthesia, hemostatic hysterotomy, extensive maternal and fetal monitoring and partial exposure of the fetus. Once they had access to the one-pound fetus, Dr. Maher performed the delicate, 30-minute operation to repair his spinal canal and cover the defect with skin. The uterus was closed using a specialized technique to incorporate the membranes and ensure a watertight seal.
Hilton was closely monitored in the hospital by the maternal fetal medicine team and then sent home on bed rest. She underwent serial ultrasound examinations and for signs of preterm labor.
At 34 weeks, Carter Hilton was born via C-section. Although Carter did require a ventriculoperitoneal shunt, he is currently six months old and doing well.
Innovative and promising
“Fetal surgery for myelomeningocele is innovative and promising,” says Mychaliska, director of the Fetal Diagnosis and Treatment Center. “Although fetal surgery improved outcomes, children with myelomeningocele still require long-term care. In addition, the potential fetal benefits must be weighed with an understanding of the potential maternal and fetal risks.”
For nearly a decade, U-M doctors have been working together to develop the Fetal Diagnosis and Treatment Center which provides comprehensive prenatal diagnosis and cutting edge fetal surgery for carefully selected patients. “We are fortunate to have a talented and experienced team dedicated to our maternal and fetal patients,” says Mychaliska.
“The team carefully explained to us the risks involved for both me and my unborn son but we did not hesitate to say yes in light of the potential to improve his quality of life,” Kasey Hilton says. “I had concerns, as anyone would, but knew I was in good hands.
“We’ll have to watch Carter for various milestones in his life and we won’t know if he will have challenges with things like walking or with his bowels and bladder until he’s old enough to walk and potty train. For now, he’s doing great and we are grateful to the entire fetal team at U-M for a procedure that could help give him a better future.”
The MOMs trial
The landmark randomized prospective clinical trial comparing prenatal versus postnatal repair of myelomeningocele, known as the MOMS trial, closed early due to efficacy, and the results were published in the New England Journal of Medicine. By 12 months, children who had fetal surgery had a decreased need for shunting and less hindbrain herniation. At 30 months of age, those who received fetal surgery also scored better on mental and motor function tests and were more likely to walk independently. However, fetal surgery was also associated with an increased risk of preterm delivery and uterine dehiscence at delivery.
- Mothers ≥ 18 years of age
- T1—S1 with hindbrain herniation
- Normal fetal karyotype
- Gestational age between 19 to 25 weeks
- Fetal anomaly unrelated to myelomeningocele
- History of spontaneous preterm birth
- Maternal BMI > 35
- Maternal co-morbidities
- Inability to comply with follow-up