Fetal Care Center of Cincinnati

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Fetal Care Center of Cincinnati

 
 
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  • Myelomeningocele Fetal Surgery

    Myelomeningocele / Spina Bifida is a protrusion of the meninges and spinal cord through a defect in the:

    • Vertebral arches
    • Muscle
    • Skin

    Myelomeningocele is associated with significant postnatal morbidity and lifelong disability and a small but significant mortality rate related to complications.

    Myelomeningocele is a relatively common anomaly affecting 1 in 2000 live births. The disabilities associated with myelomeningocele include:

    • Paraplegia
    • Hydrocephalus 
    • Sexual dysfunction
    • Skeletal deformities
    • Bowel and bladder incontinence
    • Cognitive impairment

    Although most children with myelomeningocele have normal intelligence, 15% require some form of custodial care.

    Dietary folate supplementation has been shown to prevent myelomeningocele in some cases.

    To be effective folate must be supplemented soon after conception, but 50% of women of childbearing age do not take supplemental folate, and most pregnancies are unplanned.

    In addition, it is estimated that 30% of neural tube defects are refractory to folate supplementation. For these reasons, despite folate supplementation, myelomeningocele is an anomaly that likely will continue to affect children.

    Traditionally, efforts to treat myelomeningocele have focused on postnatal surgical correction to prevent infection and to improve physical disabilities that cannot be corrected or reversed. The rationale for prenatal correction of myelomeningocele is to repair the defect before neurologic damage has occurred or when there is still potential for recovery.

    The neurologic damage in myelomeningocele is hypothesized to result from the initial defective spinal cord development and the damage to the exposed spinal cord caused by failure of mesodermal migration. This has been referred to as the "two-hit hypothesis."

    Examination of the spinal cords of midgestation fetuses with myelomeningocele shows near-normal cord development in most cases. In addition, leg movement in fetuses with myelomeningocele has been observed as early as 16 to 17 weeks of gestation.

    In contrast, most myelomeningocele fetuses exhibit severe neurologic impairment of the lower extremities by the time of birth, suggesting that the neurologic injury may occur later in gestation. This injury may occur during labor or as a result of passage through the birth canal. The neurologic injury may also occur by direct abrasion of the exposed cord against the uterine wall during gestation.

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    Myelomeningocele References

    Adzick NS, Sutton LN, Crombleholme TM, et al: Successful fetal surgery for spina bifida. Lancet 352:1675, 1998.

    Adzick NS, Walsh DS: Myelomeningocele: Prenatal diagnosis, pathophysiology, and management. Semin Pediatr Surg. 12: 168-174, 2003.

    Bruner JP, et al: Endoscopic coverage of fetal open myelomeningocele in utero [letter]. American Journal of Obstetrics and Gynecology 176:256, 1987.

    Bruner JP, et al: Endoscopic coverage of myelomeningocele in utero. American Journal of Obstetrics and Gynecology 180:153, 1999.

    Bruner JP, et al: Fetal surgery for myelomeningocele and the incidence of shunt-dependent hydrocephalus. JAMA 282:1819, 1999.

    Copp AJ, et al: The embryonic development of mammalian neural tube defects. Prog Neurobiol 35:363, 1990.

    Drewek MJ, et al: Quantitative analysis of the toxicity of human amniotic fluid to cultured rat spinal cord. Pediatric Neurosurgery 27:190, 1997.

    Edmonds LD, James LM: Temporal trends in the prevalence of congenital malformations at birth based on the birth defects monitoring program, United States 1979–1987. MMWP Morb Mortal Wkly Rep 39:19, 1990.

    Filly RA: Ultrasound evaluation of the fetal neural axis. In Callen PW (ed): Ultrasonography in Obstetrics and Gynecology. Philadelphia, WB Saunders, 1994, p 189.

    Heffez DS, et al: Intrauterine repair of experimental surgically created dysraphism. Neurosurgery 32:1005, 1993.

    Heffez DS, et al: The paralysis associated with myelomeningocele: Clinical and experimental data implicating a preventable spinal cord injury. Neurosurgery 26:987, 1990.

    Hutchins GM, et al: Acquired spinal cord injury in human fetuses with myelomeningocele. Pediatr Pathol Lab Med 16:701, 1996.

    Katz VL, et al: Role of ultrasound and informed consent in the evaluation of elevated maternal serum alpha-fetoprotein. American Journal of Perinatology 8:73, 1991.

    Lary JM, Edmonds LD: Prevalence of spina bifida at birth -- United States, 1983–1990: A comparison of two surveillance systems. MMWP Morb Mortal Wkly Rep 45:15, 1996.

    Luthy DA, et al: Cesarean section before the onset of labor and subsequent motor function in infants with myelomeningocele diagnosed antenatally. New England Journal of Medicine 324:662, 1991.

    Meuli M, et al: The spinal cord lesion in human fetuses with myelomeningocele: Implications for fetal surgery. Journal of Pediatric Surgery 32:448, 1997.

    Meuli M, et al: In utero surgery rescues neurologic function at birth in sheep with spina bifida. Nat Med 1:342, 1995.

    Meuli M, et al: The spinal cord lesion in human fetuses with myelomeningocele: Implications for fetal surgery. Journal of Pediatric Surgery 32:448, 1997.

    Michejda M: Intrauterine treatment of spina bifida. Primate model. Z Kinderchir 39:259, 1984.

    MRC Vitamin Research Study Group: Prevention of neural tube defects: Results of the Medical Research Council Vitamin Study. Lancet 338:131, 1991.

    Platt LD, et al: The California Maternal Serum alpha-Fetoprotein Screening Program: The role of ultrasonography in the detection of spina bifida. American Journal of Obstetric Gynecology 166:1328, 1991.

    Rintoule NE, Sutton L, Hubbard AM, et al: A new look at myelomeningocele: Functional level, vertebral level, shunting, and the implications for fetal intervention. Pediatrics 109: 409-413, 2002.

    Scheller JM, Nelson KB: Does cesarean delivery prevent cerebral palsy or other neurologic problems of childhood? Obstetric Gynecology 83:624, 1994.

    Shaw GM, et al: Epidemiologic characteristics of phenotypically distinct neural tube defects among 0.7 million California births, 1983–1987. Teratology 49:143, 1994.

    Sutton LN, et al: Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA 282:1826, 1999.

    Use of folic acid for prevention of spina bifida and other neural tube defects—1983–1991. MMWR Morb Mortal Wkly Rep 40:513, 1991.


 
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    Myelomeningocele / Spina Bifida

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  • MOMS Trial Summary

    Download a summary of results from the Management of Myelomeningocele Study (MOMS Trial).

  • Contact Us

    If you would like to request an appointment or get more information about the Fetal Care Center of Cincinnati, please call us at 1-888-338-2559 (1-888-FETAL59).

 
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