April 1, 2014

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On February 23, 2012, the Fetal Center announced the birth of baby Charlotte, the first baby at Texas Children’s to receive in-utero surgery to treat spina bifida. Now, a little more than two years later, the center is celebrating another milestone with 20 cases completed.

After years of treating spina bifida by a neurosurgical closure of the spine defect during the first days of life, research breakthroughs prompted the Texas Children’s Fetal Center team to begin performing in-utero surgery on patients with a prenatal diagnosis of spina bifida.

Myelomeningocele, also known as spina bifida or open neural tube defect (NTD), is a developmental defect in which the spine is improperly formed and the spinal cord is open to and fused with the skin; it is usually associated with hydrocephalus, which requires surgical treatment to drain the fluid via a shunt. Myelomeningocele occurs in 3.4 out of every 10,000 live births in the U.S. and is the most common permanently disabling birth defect for which there is no known cure.

Advancements in fetal imaging and early prenatal diagnosis allow the team to gain access to a fetus while still inside a mother’s womb. Texas Children’s Fetal Center has developed extensive screening and diagnostic algorithms for pregnancies with fetal spina bifida. These algorithms determine which patients are appropriate candidates for the in-utero surgery.

The surgery is a huge, multi-disciplinary effort, bringing together a team of specialized experts, who all play a vital role in the medical treatment of both mom and baby. The team includes maternal-fetal specialists, led by Dr. Michael Belfort, fetal surgeons, led by Dr. Darrell Cass and Dr. Oluyinka Olutoye, and a neurosurgery team, led by Dr. William Whitehead. Once the fetus is exposed, Dr. Whitehead and his team surgically close the spinal defect before fetal surgeons return the baby to the womb. This procedure reduces the risk of hydrocephalus and may improve motor function in select patients.

A NICHD-funded study entitled the Management of Myelomeningocele Study (MOMS) published in the New England Journal of Medicine demonstrated a significant decrease in the risk of hydrocephalus for select patients undergoing fetal closure of the spine, as well as possible improvement in lower extremity function, compared to patients who underwent standard closure after birth. The MOMS trial is the second fetal intervention that has proved beneficial through a multi-center randomized clinical trial. The first was the Euro FETUS trial for laser ablation in the treatment of twin-to-twin transfusion syndrome (TTTS).

“Breakthrough studies like the MOMS trial are exciting and reaffirm our commitment to advancing fetal medicine and giving babies with complications and anomalies the healthiest possible start to life,” said Dr. Darrell Cass, co-director of Texas Children’s Fetal Center. “Our program has grown exponentially since we completed our first NTD repair in 2012, and we remain cautious, but it seems as though our center’s results are exceeding even those of the trial.”

The final piece to this puzzle is Dr. Kathryn Ostermaier, clinic chief of the Spina Bifida Clinic. It is under her guidance that the hospital provides long term care to surgical patients. The hospital’s Spina Bifida Clinic is a specialized program that serves only those patients with spina bifida, and includes several specialty services: orthopedics, neurosurgery, pediatrics, physical medicine and rehabilitation and urology.

The team that cares for NTD patients before birth, after birth and into childhood includes physicians from maternal fetal medicine, pediatric surgery, neurosurgery, anesthesiology, neonatology, pediatric radiology, cardiology, orthopedics, urology, physical therapy, and a highly dedicated group of specialized nurses, ultrasound technologists and genetic counselors.

“The confirmation that fetal surgery may decrease the physical challenges some of these babies face is not only a ray of hope for families, it is also a significant achievement for fetal medicine,” said Cass.