Myelomeningocele (MMC), one of the most common congenital malformations, can result in severe lifelong disabilities, including paraplegia, hydrocephalus, Chiari II malformation (CM-II), incontinence, sexual dysfunction, skeletal deformations and mental impairment. MMC was the first nonlethal anomaly to be treated by fetal surgery. Experimental and clinical evidence suggests that the primary cause of the neurologic deficit associated with MMC is not simply incomplete neurulation but rather chronic mechanical injury and amniotic fluid-induced chemical trauma that progressively damages the exposed neural tissue during gestation. Case series and a prospective, randomized study show that fetal surgery for MMC before 26 weeks’ gestation may preserve neurologic function, reverse the hindbrain herniation of the CM-II and obviate the need for postnatal placement of a ventriculoperitoneal shunt. However, these studies also demonstrate that fetal surgery is associated with significant maternal and fetal risks. Consequently, further research is warranted to further expand our understanding of the pathophysiology of MMC, to evaluate the long-term impact of in utero intervention and to refine the timing and technique of fetal MMC surgery.
© 2011 S. Karger AG, Basel
- Spina bifida
- Hindbrain herniation
- Prenatal diagnosis
- Fetal surgery
- Management of Myelomeningocele Study
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N. Scott Adzick, MD
The Center for Fetal Diagnosis and Treatment, 5th Floor Wood Center
The Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard
Philadelphia, PA 19104-4388 (USA)
Tel. +1 215 590 2727, E-Mail email@example.com
Received: May 22, 2011
Accepted after revision: May 31, 2011
Published online: August 18, 2011
Number of Print Pages : 11
Number of Figures : 3, Number of Tables : 1, Number of References : 78
Fetal Diagnosis and Therapy (Clinical Advances and Basic Research)
Vol. 30, No. 3, Year 2011 (Cover Date: November 2011)
Journal Editor: Gratacós E. (Barcelona)
ISSN: 1015-3837 (Print), eISSN: 1421-9964 (Online)
For additional information: http://www.karger.com/FDT
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