Following the promising multicenter randomized trial results of in utero fetal myelomeningocele repair; we anticipate that an increasing number of tertiary care centers may want to offer this therapy. It is essential to establish minimum criteria for centers providing open fetal myelomeningocele repair to ensure optimal maternal and fetal/pediatric outcomes, as well as patient safety both short- and long-term; and to advance our knowledge of the role and benefit of fetal surgery in the management of fetal myelomeningocele. The fetal myelomeningocele Maternal-Fetal Management Task Force was initially convened by the Eunice Kennedy Shriver National Institute of Child Health and Human Development to discuss the implementation of maternal fetal surgery for myelomeningocele. The decision was made to develop the optimal practice criteria presented in this document for the purpose of medical and surgical leadership. These criteria are not intended to be used for legal or regulatory purposes.
Fetal therapy involves weighing fetal benefit against maternal risk. Since its introduction 5 decades ago, in utero therapy has been reserved for conditions that left untreated would result in fetal or neonatal demise. Although myelomeningocele (MMC) is not a lethal condition, the goal of in utero treatment is to improve the long-term outcome for affected children. The results of the Management of Myelomeningocele Study (MOMS), comparing prenatal surgery with standard postnatal repair, revealed decreased need for shunting, decreased hind brain herniation, improved motor outcomes, and a higher rate of independent ambulation at 30 months in the fetal repair group. However, this group also had a higher rate of preterm delivery and resultant lower birthweight, increased neonatal respiratory distress syndrome, and higher rates of maternal/obstetric complications including pulmonary edema, placental abruption, spontaneous preterm labor, transfusion at delivery, and thinning or dehiscence of the hysterotomy site. It is anticipated that an increasing number of parents will be willing to accept the risks associated with prenatal repair for the potential to improve the quality of life of their unborn child.
The accompanying editorial published with the MOMS trial results raised concerns regarding the increased availability of fetal MMC repair. Three fetal centers with extensive experience in open maternal-fetal surgery participated in the MOMS trial that concentrated the surgical capabilities and management expertise to minimize maternal-fetal morbidity and maximize pediatric outcomes. As the demand for in utero MMC repair increases, numerous questions arise. How do we maintain expertise with expansion of participating centers? Are the MOMS trial results generalizable to interventions performed in other centers? What should be the standard for fetal centers that offer in utero repair for MMC?
Although the MOMs trial has demonstrated a benefit to children that underwent in utero surgery at 30 months, there is a need for continued assessment of these children to assure everyone that the benefit continues beyond the currently reported 30 months, through childhood into adolescence and beyond. The reproductive health implications to mothers who undergo in utero therapy also need to be determined.
With the need to address these issues, the MMC Maternal-Fetal Management Task Force was created. The Task Force is comprised of representatives of professional societies and organizations whose members are directly involved with prenatal diagnosis and perinatal management of mothers carrying fetuses with MMC or pediatric specialists involved in the immediate postnatal care of these infants, or in the lifelong management of individuals affected with MMC ( Table 1 ). The current state and future direction of prenatal MMC repair were reviewed, with the resultant expert opinion presented here.
Alan R. Cohen, MD: American Association of Neurological Surgeons/Congress of Neurological Surgeons Section on Pediatric Neurological Surgery James Couto, MA: American Academy of Pediatrics James J. Cummings, MD: American Academy of Pediatrics Anthony Johnson, DO: North American Fetal Therapy Network Gerald Joseph, MD: American College of Obstetricians and Gynecologists Bruce A. Kaufman, MD: American Society of Pediatric Neurosurgeons Ronald S. Litman, DO: American Society of Anesthesiologists and Society of Pediatric Anesthesia M. Kathryn Menard, MD: Society for Maternal-Fetal Medicine Julie S. Moldenhauer, MD: North American Fetal Therapy Network Kevin C. Pringle, MB, ChB, FRACS: International Fetal Medicine and Surgery Society Marshall Z. Schwartz, MD: American Pediatric Surgical Association William O. Walker Jr, MD: Spina Bifida Association Benjamin C. Warf, MD: Joint Section on Pediatric Neurological Surgery of the American Association of Neurologic Surgeons and Congress of Neurological Surgeons Joseph R. Wax, MD: American Institute of Ultrasound in Medicine |
This document details 6 key areas for teams performing in utero fetal MMC repair. These include:
- 1.
Defining a fetal therapy center
- 2.
Perioperative management for fetal MMC repair
- 3.
Long-term care
- 4.
Counseling
- 5.
Reporting and monitoring
- 6.
Access and regionalization
Defining a fetal therapy center
In a joint Committee Opinion from the American College of Obstetricians and Gynecologists and the American Academy of Pediatrics, many of the aspects involved in defining a fetal therapy center have been addressed. These tenets will serve as the basis for the general description of an established fetal therapy center and will be further expanded as relates to fetal MMC repair.
The expertise and services required to be considered a fetal center appropriately equipped to perform prenatal MMC repair involves tremendous institutional commitment. This should include an experienced fetal care team ( Table 2 ), a multidisciplinary spina bifida program ( Table 3 ), a Level IIIC Neonatal Intensive Care Unit, a Labor and Delivery unit capable of caring for perioperative complications and obstetric emergencies with around the clock availability of Maternal-Fetal Medicine (MFM) specialists/Obstetricians skilled in managing delivery of patients with a recent hysterotomy, an institutional review board, an ethics committee, a Maternal/Fetal advocate to ensure that counseling is nondirective and an institutional commitment to track long-term pediatric neurodevelopmental outcomes.
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