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t m c » p u l s e | j u n e 2 0 1 5 30 Closing the Gap in Fetal Surgery Texas Children's Hospital Fetal Center develops new minimally invasive repair technique to treat spina bifida in-utero performed the in-utero spina bifida fetoscopic closure on Grayson—their first patient. It is believed that this is the first time this type of two-port feto- scopic procedure has been performed in the United States. Prior techniques used a three-port method. Utilizing an approach developed by Michael Belfort, M.D., Ph.D., obste- trician and gynecologist-in-chief at Texas Children's Hospital, and William Whitehead, M.D., pediatric neurosur- geon at Texas Children's Hospital, the surgery was over three years in the making. At 25 weeks gestation, the team successfully closed the opening in Canezaro's unborn baby's spine. "The real innovation here is that we're doing this repair through two ports with a full neurosurgical closure, using a new kind of repair that Dr. Whitehead has developed with a differ- ent suture material from what has been used before," said Belfort, who is also professor and chairman of the depart- ment of obstetrics and gynecology at Baylor College of Medicine. "We're not compromising on the closure of the spinal cord defect, because the only way that this becomes solidified as a success is if the repair is as good as— or better—than an open fetal repair." Myelomeningocele, or open neural tube defect (NTD), is a form of spina bifida that 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. A developmental defect in which the spine is improperly formed and B y A l e x O r l a n d o the spinal cord is fused with the skin, myelomeningocele is usually associ- ated with hydrocephalus—a buildup of cerebrospinal fluid in the brain and a leading cause of morbidity in patients. "Somewhere between 24 and 28 days from conception, an area of the developing spinal cord doesn't close properly, and the neural tube defect is formed," said Belfort. "The lesion may have a covering of mem- brane or be totally open, but either way the nerve tissue is still exposed to the surrounding amniotic fluid. In addition, the defect allows the leakage of the fluid that surrounds the brain causing the lower part of the brain to descend towards the top of the spine. It's our belief that the exposure of these nerves to the amniotic fluid, compounded by the trauma of them bumping up against the side of the uterus, leads to ongoing damage." Previously, closure of the defect occurred either after the birth of the baby or in the first days of life, which had the unfortunate consequence of an 80-90 percent chance that a shunt, a medical device that drains fluid from the brain to the abdomen, would be required and remain for life. In February of 2003, the National Institutes of Health launched the Management of Myelomeningocele Study (MOMS), a landmark clinical trial in the treatment of spina bifida. Probing the benefits of prenatal surgery, the study demonstrated that a fetal surgical repair leads to lower rates of hydro- cephalus, decreases the need for a Our technique may lower the rate of prematurity—by implementing the two-port procedure, the babies may have a longer gestational age before they're born, which would be an extraordinary benefit. — WILLIAM WHITEHEAD, M.D. Pediatric Neurosurgeon at Texas Children's Hospital W hen Althea Canezaro discovered she was pregnant, she couldn't wait to add a new member to her family. Unfortunately, her routine 22-week ultrasound revealed something she didn't anticipate—spina bifida. In 2014, she and her son, Grayson, became part of a pivotal moment in fetal medicine as the first patients at Texas Children's Fetal Center to undergo a minimally invasive, two-port procedure to repair spina bifida in-utero. "After the diagnosis, it was like sit- ting on a roller coaster ride, except you were sitting still and everything else was moving at a fast pace," Canezaro recalled. "My physicians in Baton Rouge immediately referred me to Texas Children's Fetal Center." On July 30th, 2014, aided by an expert operating room team and nurs- ing staff, a multidisciplinary team of specialists from Texas Children's Fetal Center and Baylor College of Medicine Grayson was the recipient of the first in-utero spina bifida fetoscopic closure.