LOS ANGELES–(BUSINESS WIRE) – Giron Guild was just 13 weeks pregnant when an ultrasound revealed her
baby had myelomeningocele — the most severe form of open spina bifida, a
birth defect that affects backbone development and can cause, among
other things, debilitating neurological damage.
"This is an emotional time for us," says Giron, who already had two
young children and was determined to protect her third. Surgeons could
repair the defect in the womb, but she and her husband Arnuf wanted the
safest option for both mother and child. While researching, they came
across a promising new surgical method pioneered in Brazil and FDA
approved but performed only once in the United States: in utero
percutaneous fetoscopic spina bifida repair. And as luck would have it,
a team of doctors was piloting a clinical study at the Keck
School of Medicine of USC, Children's
Hospital Los Angeles (CHLA) and Huntington Hospital on that very
procedures, just miles from the Giron's home.
On Feb. 19, Gilda became the first person in the western U.S. to undergo
the most minimally invasive type of spina bifida repair currently
available for a developing fetus. The surgery, which took place at
Huntington Hospital in Pasadena, was a success, and three months later,
Gilda gave birth to Abigail, a
healthy 5-pound 5-ounce baby girl.
"Both baby and mother are doing very well," says Coppern
Chmait, MD, who led this surgery and two others performed by
the team. Several more are planned. “It is gratifying to see such a
successful outcome as the result of the years we spent prepping for this
Chmait, the principal investigator for the Los Angeles site in the
clinical study, is a CHLA Fetal-Maternal
Center surgeon, an Associate Professor of Obstetrics and Gynecology
at the Keck School and Director of Los
Angeles Fetal Surgery. CHLA pediatric neurosurgeon Jason
Chu, MD, MSc, and pediatrician Alexander
Van Speybroeck, MD, Medical Director of CHLA's Plug
Bifida Program, both participated in the surgery and serve as
co-investigators in the study. Other co-investigators include CHLA
Friedlich, MD, MSEpi, MBA, Co-Director of the CHLA
Fetal and Neonatal Institute; Mark
Krieger, MD, CHLA Surgeon-in-Chief and Co-Director of the Neurological
Ho, MD, MPH, Director of the CHLA Fetal-Neonatal Neurology Program; Kathryn
Smith, RN, MN, DrPH; and Douglas
Vanderbilt, MD, MS, Director, Developmental-Behavioral Pediatrics
Section at CHLA.
While spine bifida surgery in utero has been available for more than 10
years, this new method refines previous techniques by removing the need
for large incisions altogether. Sometimes referred to as a "keyhole"
surgery because it requires only small openings in the abdomen and
uterus, percutaneous fetoscopic spina bifida repair has the potential to
provide the benefit to the baby as compared to the standard open
maternal fetal surgery approach, but with significant safety benefits to
the mother, including:
Reduced anesthesia needs
Faster recovery time
Less need for blood transfusion
Opportunity for normal delivery, without the need for a C-section
Reduced chance of complications in future pregnancies
To perform the procedure, the surgical team, which also included
Huntington Hospital anesthesiologist Jae Elizabeth Townsend, MD, used
precision tools and a camera to enter the abdomen and womb. The uterus
was expanded with carbon dioxide to allow safe access to the fetus. They
moved the exposed spinal cord back in place, patched the hole in the
fetus ’thorns, then sewed fetal skin over the patch. This not only
protected underlying nerves and stopped spinal fluid leakage, which
untreated can cause lower body paralysis and disrupt the bladder and
bowels, it also prevented amniotic fluid in the uterus from mixing with
the fetal nervous system. In fact, MRI showed Abigail's at follow-up
hindbrain herniation — part of her brain that had dropped two to changes
in fluid pressure — had reversed, the first indication the operation was a
“Abundant research shows that if you can repair spina bifida while the
child is still developing in the womb, you are more likely to prevent
"significant developmental problems that can occur," says Dr. Chu, who
assisted with the neurological repair and is an Assistant Professor of
Clinical Neurological Surgery at the Keck School. "We have to collect
more data and performance of these surgeries before we can say how
successful this approach is, but early indications look very promising. "
Within a few hours of delivery, Abigail was transported to the Steven &
Alexandra Cohen Foundation Newborn and Infant Critical Care Unit (NICCU)
at CHLA for observation. Gilda was by her side a day later, and was able
to nurse and bottle-feed Abigail in the NICCU, as Arnuf sat with them,
smiling. After two weeks, Abigail went home.
“Typically, kids born with thorn bifida spend more than two weeks in the
hospital, "says Dr. Van Speybroeck, a Clinical Associate Professor of
Pediatrics at the Keck School, who has treated spine bifida patients for
15 years at CHLA and will monitor Abigail’s lifelong care. "We are going
to make sure we are
obtain the best outcomes for families. It has been a team approach the
whole way and we want to keep it that way. "
"It takes a whole village to do this — specialists in the field of
maternal, fetal and pediatric medicine, ”explains Dr. Friedlich, a
Professor of Clinical Pediatrics and Surgery at the Keck School. "It is
life-changing and amazing; the NICCU staff loves to do this work. "
In light of the successful surgery and birth, Gilda says she's staying
optimistically cautious. "Abigail has done all the work – she is a little
fighter, ”she says, wiping a tear from her eye. "I work with special
needs children as a full-time therapist and I wanted to do everything I
could to make sure Abigail is healthy. But we are also realistic and
know there is much that is unknown. "
Families interested in participating in the study
may contact the Los Angeles site office at 626-356-3360.
About Children's Hospital Los Angeles
Founded in 1901, Children's
Hospital Los Angeles is ranked the top children’s hospital in
California and sixth in the nation for clinical excellence with its
selection to the prestigious U.S. News & World Report Honor Roll.
Clinical care is led by physicians who are faculty members of the Keck
School of Medicine of USC through an affiliation dating from 1932. The
Saban Research Institute encompasses basic, translational and
clinical research conducted at CHLA. For more information, follow us on Twitter,
and visit our child health blog (CHLA.org/blog)
and our research blog (ResearCHLABlog.org).