Follow-up to landmark spina bifida study could influence future treatment

1/30/2013

HOUSTON — Almost 10 years ago, the Management of Myelomeningocele (MOMS) study began comparing two approaches to treatment for a serious form of spina bifida: prenatal surgery versus the standard postnatal repair.

This breakthrough study, funded by the Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) and published by the New England Journal of Medicine in 2011, found that children whose spina bifida defects were repaired surgically before birth were more likely to walk without the assistance of orthotics or devices.  NICHD is one of the Institutes of the National Institutes of Health.

Now, a follow-up study, also funded by NICHD and informally known as MOMS2, is being conducted to determine whether prenatal repair done in the original study influenced the adaptive behavior of these children, now 5 to 9 years of age, compared with those who underwent postnatal repair.

One of the effects in question is the brain development of these children. Jenifer Juranek, Ph.D., a neuroimaging expert with the Texas Fetal Center and the Children’s Learning Institute at The University of Texas Health Science Center at Houston (UTHealth), is performing high-resolution magnetic resonance imaging (MRI) brain sequences on 177 of the children from the original study to investigate if those who underwent prenatal surgery experienced greater structural modification in their brains than those infants who underwent postnatal surgery.

The follow-up brain imaging protocol was set up by Juranek at each of the three original MOMS study sites: Vanderbilt University in Nashville, Tenn., The Children's Hospital of Philadelphia, and the University of California at San Francisco. Each follow-up image will be analyzed and quantified by Juranek for such key development indicators as brain volume, matter integrity, synaptic pruning (i.e., when excess connections between cells are eliminated) and myelination, which enables nerve cells to transmit information faster and allows for more complex brain processes.

“Researchers have demonstrated that many neurodevelopmental disorders like spina bifida may be linked to poorly-timed cellular events during brain development. These events lead to specific structural and functional brain development,” said Juranek. “With this follow-up study, researchers can evaluate the impact different intervention strategies have on brain structure, function and behavior.”

The results of the MOMS2 study could influence future surgical procedures for babies diagnosed with myelomeningocele, says KuoJen Tsao, M.D., associate professor of pediatric surgery at UTHealth Medical School and a co-director of the Texas Fetal Center.

“The MOMS study gave us data that goes two or three years out from surgery, but we know there is a lot of development beyond that,” said Tsao. “We know there are certain short-term outcomes, but there may be some long-term neurological effects we don’t know about. What’s most exciting about the MOMS2 study is they are going to follow these patients at school age.”

One common risk associated with myelomeningocele is the buildup of fluid inside the skull that leads to brain swelling. This swelling is repaired with shunts inserted into the brain to relieve pressure. The original MOMS study found that prenatal surgery reduced the need for shunts, which may improve long-term brain development.

“Once you put in a shunt you have risks,” said Juranek. “Getting into the center of brain isn’t easy. If you put in a shunt, you are likely to cut through gray and white matter, both of which are responsible for certain brain functions.”

Tsao added, “The important thing about the MOMS2 study is it will answer questions that we are asking now. That’s where Dr. Juranek’s work is very important.”

Other follow-up testing will look at other development factors such as attention, executive function and fine and gross motor skills.

American College of Obstetricians and Gynecologists recommends counseling for prenatal spina bifida surgery

American College of Obstetricians and Gynecologists recommends counseling for prenatal spina bifida surgery

Published On: 1/21/2013 2:48:37 PM

BY MARY ELLEN SCHNEIDER
IMNG Medical News
http://www.imng.com

Breaking News

Certain pregnant women who are carrying a fetus with severe spina bifida should be counseled about the potential benefits and risks of maternal-fetal surgery, according to the American College of Obstetricians and Gynecologists.

The ACOG Committee on Obstetric Practice recently recommended counseling about maternal-fetal surgery for myelomeningocele, the most severe form of spina bifida, following promising results from the procedure in an 8-year randomized controlled trial (Obstet. Gynecol. 2013;121:218-9).

In the Management of Myelomeningocele Study (MOMS), prenatal repair before 26 weeks of gestation reduced the need for a shunt at 12 months and also decreased the rate of hindbrain herniation by one-third at 12 months of age. The procedure also doubled the rate of independent walking and produced higher levels of functioning than were expected based on anatomic levels (N. Engl. J. Med. 2011;364:993-1004).

But the study, which was sponsored by the National Institutes of Health, also found significant maternal and fetal risks. For example, the surgery was associated with high rates of preterm birth, fetal bradycardia, oligohydramnios, placental abruption, pulmonary edema, maternal transfusion at delivery, and an increased incidence of uterine thinning.

ACOG cautions that the MOMS trial used stringent inclusion criteria and rigorous requirements for the experience of the surgeons involved. As a result, outcomes from the trial should be considered a "best-case scenario," the committee members wrote. They urged physicians to use the same inclusion criteria as the researchers when considering which women to counsel about the maternal-fetal surgery. The criteria were a singleton pregnancy, myelomeningocele with the upper boundary between T-1 and S-1, evidence of hindbrain herniation, gestational age between 19.0 and 25.9 weeks, and a normal karyotype. The major exclusion criteria included a fetal anomaly unrelated to the myelomeningocele; severe kyphosis; risk of preterm birth; a maternal body mass index of 35 kg/m² or more; and contraindications to surgery, including previous hysterotomy in the active uterine segment.

Women should also be counseled about the implications of the surgery for future pregnancies, the ACOG committee recommended.

"It is a highly technical procedure with potential for significant morbidity and possibly mortality, even in the best and most experienced hands," they wrote. "Maternal-fetal surgery for myelomeningocele should only be offered at facilities with the expertise, multidisciplinary teams, services, and facilities to provide the intensive care required for these patients."

The study authors declared that they received funding from the National Institutes of Health. One of the authors reported receiving funding from Vanderbilt University in Nashville, Tenn.

Why pre-natal surgery makes sense, for some, for spina bifida

 

Posted: Aug 24, 2012, 9:17 am

By Jeff Hansel
The Post-Bulletin, Rochester MN


Mayo Clinic surgeons in May performed surgery on a fetus before birth to decrease almost certain complications of spina bifida.

The baby was born by cesarean section in early August and is doing well, though she might face struggles.

In "myelomeningocele,"(the most severe form of spina bifida, the spinal cord is open to the amniotic fluid during pregnancy, and complications build as the pregnancy progresses. Almost all infants with this form of spina bifida have complications, such as the brain essentially sagging toward the spinal column.

Standard treatment has been surgery after birth, and babies often need a permanent shunt to drain fluid the brain isn't able to dispose of on its own.

Many believed surgery before birth would help because it would allow babies' neural systems more time to develop naturally. But no one knew for sure if this would keep more babies alive or, because of a higher risk of premature birth and other factors, would be harmful.

So researchers convinced every prenatal surgical center in the U.S. to cease early-intervention surgeries unless they were part of a national research study, says a March article in the New England Journal of Medicine.

From February 2003 to December 2010, women were offered the surgery at one of only three locations — Children's Hospital of Philadelphia, Vanderbilt University and the University of California at San Francisco. The data was analyzed at an independent center.

"Children in the prenatal-surgery group were more likely than those in the postnatal-surgery group to be able to walk without orthotic devices," the New England Journal article reported. Also, prenatal surgery before 26 weeks of gestation "decreased the risk of death or need for shunting by the age of 12 months and also improved scores on a composite measure of mental and motor function."

Results were so good that the trial, randomly assigning women to prenatal surgery or post-natal surgery, was stopped early after 183 of a planned 200 women pregnant with babies diagnosed with spina bifida were recruited for the study.

That doesn't mean spina bifida was cured, nor were all associated problems fixed. Two babies in each group died. The percentage of deaths overall was dramatically decreased in the prenatal group, but it remained high.

Because of the potential for fetal death, severe physical impairments, lifelong complications and mental deficits, parents of babies with severe spina bifida typically are asked whether they want to terminate the pregnancy.

Most of those who survive surgery after birth can't get a job without the presence of a vocational assistant, said Mayo Clinic pediatric neurosurgeon Dr. Nick Wetjen.

The hope with the surgery is to improve quality of life and decrease complications. Parents need to be fully informed of the possible risks and benefits of the procedures.

"There are some people that will be helped by this, and there are some people that we will end up harming, too, because of the nature of the procedure," said Mayo maternal fetal medicine specialist Dr. Norman Davies.

Health reporter Jeff Hansel writes the Pulse on Health column every Monday. Follow him on Twitter @JeffHansel.
 

Nurses from The Children’s Hospital of Philadelphia Review History and Recent Advances of Fetal Surgery for Spina Bifida

Nurses from The Children’s Hospital of Philadelphia Review History and Recent Advances of Fetal Surgery for Spina Bifida

August 10, 2012 

PHILADELPHIA, Aug. 10, 2012 /PRNewswire-USNewswire/ — Thirty years ago the idea of performing fetal surgery seemed more like science fiction than reality. Today, however, highly sophisticated surgical teams regularly repair spina bifida and other birth defects before birth, and fetal therapy is recognized as one of the most promising fields in pediatric medicine.
(Photo: http://photos.prnewswire.com/prnh/20120810/DC56010)

Spina bifida is the most common birth defect of the central nervous system, affecting about 1,500 babies born each year in the United States. In 2011, research co-led by The Children’s Hospital of Philadelphia (CHOP) and published in the New England Journal of Medicine, showed that performing surgery in the womb, months before birth, can substantially improve outcomes, such as mobility, for children with this common, disabling birth defect of the spine.

In a new article in the August 2012 issue of the AORN Journal, published by the Association of periOperative Registered Nurses, nurses from CHOP’s Center for Fetal Diagnosis and Treatment review the history of fetal surgery for spina bifida from inception to current practice.

“Our Center’s multidisciplinary team has the world’s greatest amount of experience performing fetal surgery and as a result, CHOP nurses have had a great opportunity to help advance the field of fetal medicine,” said Susan M. Scully, BSN, RN, CNOR. “Specifically, as participants in the landmark Management of Myelomeningocele Study (MOMS), CHOP nurses contributed to making fetal surgery for spina bifida a standard-of-care option for families.”

In their article, “Fetal Myelomeningocele Repair: A New Standard of Care,” Scully and her co-authors, Maureen Mallon, MBA, BSN, RN, CNOR, Joy C. Kerr, BSN, CNOR, and Allison Ludzia-DeAngelis BSN, RN, provide an overview of the field of fetal surgery, the rationale to prenatally repair spina bifida, the landmark MOMS trial, CHOP’s fetal surgery program and the important role perioperative nurses have played in this pioneering treatment.
To read the full article visit: http://bit.ly/ObW4J0
 
About the Center for Fetal Diagnosis and Treatment at CHOPThe Center for Fetal Diagnosis and Treatment at The Children’s Hospital of Philadelphia is an internationally recognized leader in fetal surgery and fetal care. One of the only programs of its kind in the world, it offers a comprehensive breadth of services, including fetal therapy, to support patients from prenatal evaluation through delivery, postnatal care, and long-term follow-up. Established in 1995, the Center has welcomed more than 12,000 expectant parents and received referrals from all 50 states and more than 50 countries. Its multidisciplinary team brings decades of experience to the care and treatment of the fetus and the expectant mother. The Center has performed over 900 fetal surgeries, including complex open procedures for birth defects such as spina bifida; less invasive fetoscopic or ultrasound-guided surgeries for conditions such as twin-twin transfusion syndrome; and specialized coordinated delivery approaches for babies that require surgical intervention while still on maternal-placental life support (EXIT delivery). For more information visit http://fetalsurgery.chop.edu.

About The Children’s Hospital of Philadelphia: The Children’s Hospital of Philadelphia was founded in 1855 as the nation’s first pediatric hospital. Through its long-standing commitment to providing exceptional patient care, training new generations of pediatric healthcare professionals and pioneering major research initiatives, Children’s Hospital has fostered many discoveries that have benefited children worldwide. Its pediatric research program is among the largest in the country, ranking third in National Institutes of Health funding. In addition, its unique family-centered care and public service programs have brought the 516-bed hospital recognition as a leading advocate for children and adolescents. For more information, visit http://www.chop.edu.
Contact: Ashley Moore
The Children’s Hospital of Philadelphia
Phone: (267) 426-6071
MooreA1@email.chop.edu
SOURCE The Children’s Hospital of Philadelphia

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press release

Feb. 23, 2012, 3:00 p.m. EST

Texas Children's Fetal Center Performs Successful Fetal Surgery to Treat Spina Bifida

HOUSTON, Feb. 23, 2012 /PRNewswire via COMTEX/ -- As one of the country's leading medical centers diagnosing and treating fetal anomalies, Texas Children's Fetal Center is proud to announce the birth of Baby Charlotte, the team's first patient to undergo in-utero surgery for the treatment of spina bifida. Baby Charlotte's mother went into labor nearly 11 weeks after fetal closure was performed, and delivered on Saturday evening by cesarean section. Mother and baby are doing well.

"Texas Children's Fetal Center is now one of the very few centers in the country providing all aspects of fetal surgery, and the addition of this capability increases the options of our Texas and regional patients tremendously," said Dr. Michael Belfort, MD, Ph.D., obstetrician and gynecologist-in-chief at Texas Children's Hospital and professor and chairman of the Department of Obstetrics at Baylor College of Medicine. "We have a magnificent team of specialists from a number of departments working together in the best interests of our fetal and neonatal patients. I am incredibly proud to be a member of this outstanding team and to be able to play a role in this mission." 

Myelomeningocele, also known as spina bifida or open neural tube defect (NTD), 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. Myelomeningocele 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, or the buildup of cerebrospinal fluid in the brain, which requires surgical treatment to drain the fluid via an implanted device called a shunt. The standard of care is neurosurgical closure of the defect in the first days of life.

"A prenatal diagnosis of spina bifida can be daunting for families because it is often associated with a constellation of neurologic disabilities as well as hydrocephalus," said Dr. Robert Bollo, pediatric neurosurgeon at Texas Children's Hospital and assistant professor of neurosurgery at Baylor College of Medicine. "Closure of the spinal defect before birth reduces the risk of hydrocephalus and may improve motor function in select patients. Fetal surgery is an exciting new tool in our multidisciplinary commitment to life-long care of patients with spina bifida." 

The spina bifida program at Texas Children's Hospital includes a dedicated multidisciplinary team of physicians including pediatric specialists in neurosurgery, orthopedics, urology, physical medicine and rehabilitation, as well as physical therapists, social workers, and child-life experts, under the leadership of Dr. Kathryn Ostermaier, developmental pediatrician at Texas Children's Hospital and assistant professor of pediatrics at Baylor College of Medicine. 

Recently, 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 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 Dr. Darrell Cass, co-director of Texas Children's Fetal Center and associate professor, departments of surgery, pediatrics and obstetrics and gynecology at Baylor College of Medicine. "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." 

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). Texas Children's Fetal Center has performed almost 400 cases of laser ablation for TTTS. Very recently, a third in-utero intervention has been shown in a randomized clinical trial in Brazil to be of benefit in babies with congenital diaphragmatic hernia. This seminal study was published in February this year by Dr. Rodrigo Ruano, MD, Ph.D., who is now a member of the Texas Children's Fetal Center team. 

"We are excited to be able to offer these types of life-changing procedures to our patients through our one-of-a-kind Fetal Center," said Cris Daskevich, senior vice president at the new Texas Children's Pavilion for Women. "Our mission is about caring for women through every stage of their pregnancy and I am grateful we have a program that can provide hope for these mothers in such a scary time in their pregnancy."

The Fetal Center at Texas Children's Hospital has developed extensive screening and diagnostic algorithms for pregnancies with fetal spina bifida. It takes a large multi-disciplinary team to successfully complete these types of fetal surgeries. The team includes physicians from maternal fetal medicine, pediatric surgery and neurosurgery, anesthesiology, neonatology, pediatric radiology, cardiology and a highly-dedicated group of specialized nurses, ultrasound technologists and genetic counselors.

Texas Children's Fetal Center recently moved and is now open on the fourth floor of the landmark new facility, Texas Children's Pavilion for Women. Texas Children's Pavilion for Women ushers in a new era as the pediatric hospital expands into obstetrical and gynecological services, establishing one of the nation's premier facilities for women's, fetal and newborn health. 

For more information, please visit http://women.texaschildrens.org/ . 

About Texas Children's HospitalTexas Children's Hospital, a not-for-profit organization, is committed to creating a community of healthy children through excellence in patient care, education and research. Consistently ranked among the top children's hospitals in the nation, Texas Children's has recognized Centers of Excellence in multiple pediatric subspecialties including the Cancer and Heart Centers, and operates the largest primary pediatric care network in the country. Texas Children's is completing a $1.5 billion expansion, which includes the Jan and Dan Duncan Neurological Research Institute; Texas Children's Pavilion for Women, a comprehensive obstetrics/gynecology facility focusing on high-risk births; and Texas Children's Hospital West Campus, a community hospital in suburban West Houston. For more information on Texas Children's, go to www.texaschildrens.org . Get the latest news from Texas Children's by visiting the online newsroom and on Twitter at twitter.com/texaschildrens.

Available Topic Expert: For information on the listed expert, click appropriate link.Darrell Cass http://www.profnetconnect.com/Darrell_Cass

Contact: Christy Brunton832-824-2645clbrunto@texaschildrens.org

SOURCE Texas Children's Hospital

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