Monday, June 29, 2009

Fetal myelomeningocele: Natural history, pathophysiology, and in-utero intervention.

Adzick NS. (2009) Fetal myelomeningocele: Natural history, pathophysiology, and in-utero intervention. Seminars in Fetal & Neonatal Medicine. 2009 Jun 17.

Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, 34th Street & Civic Center Blvd, Philadelphia, PA 19104, USA.

Myelomeningocele (MMC) is a common birth defect that is associated with significant lifelong morbidity. Little progress has been made in the postnatal surgical management of the child with spina bifida. Postnatal surgery is aimed at covering the exposed spinal cord, preventing infection, and treating hydrocephalus with a ventricular shunt. In-utero repair of open spina bifida is now performed in selected patients and presents an additional therapeutic alternative for expectant mothers carrying a fetus with MMC. It is estimated that about 400 fetal operations have now been performed for MMC worldwide. Despite this large experience, the technique remains of unproven benefit. Preliminary results suggest that fetal surgery results in reversal of hindbrain herniation (the Chiari II malformation), a decrease in shunt-dependent hydrocephalus, and possibly improvement in leg function, but these findings might be explained by selection bias and changing management indications. A randomized prospective trial (the MOMS trial) is currently being conducted by three centers in the USA, and is estimated to be completed in 2010. Further research is needed to better understand the pathophysiology of MMC, the ideal timing and technique of repair, and the long-term impact of in-utero intervention.

PMID: 19540177


Anonymous said...

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Anonymous said...

My husband is a doctor in xl pharmacy , there he met a professional in Spina bifida , he told me that Robert Nesta (the professional in Spina bifida) d said him that The spinal cord lesion or the scarring due to surgery may result in a tethered spinal cord.