Meuli M, Moehrlen U. (2014) Fetal surgery for myelomeningocele is effective: a critical look at the whys Pediatric Surgery International. 2014 Jun 8. [Epub ahead of print]
Abstract
Formerly, the
disastrous cluster of neurologic deficits and associated neurogenic
problems in patients with myelomeningocele (MMC) was generally thought
to solely result from the primary malformation, i.e., failure of
neurulation. Today, however, there is no doubt that a dimensional
additional pathogenic mechanism exists. Most likely, it contributes much
more to loss of neurologic function than non-neurulation does. Today,
there is a large body of compelling experimental and clinical evidence
confirming that the exposed part of the non-neurulated spinal
cord is progressively destroyed during gestation, particularly so in
the third trimester. These considerations gave rise to the
two-hit-pathogenesis of MMC with non-neurulation being the first and
consecutive in utero acquired neural tissue destruction being the second
hit. This novel pathophysiologic understanding has obviously triggered
the question whether the serious and irreversible functional loss caused
by the second hit could not be prevented or, at least, significantly
alleviated by timely protecting the exposed spinal
cord segments, i.e., by early in utero repair of the MMC lesion. Based
on this intriguing hypothesis and the above-mentioned data, human fetal
surgery for MMC was born in the late nineties of the last century and
has made its way to become a novel standard of care, particularly after
the so-called "MOMS Trial". This trial, published in the New England
Journal of Medicine, has indisputably shown that overall, open prenatal
repair is distinctly better than postnatal care alone. Finally, a number
of important other topics deserve being mentioned, including the
necessity to work on the up till now immature endoscopic fetal repair
technique and the need for concentration of these extremely challenging
cases to a small number of really qualified fetal surgery centers
worldwide. In conclusion, despite the fact that in utero repair of MMC
is not a complete cure and not free of risk for both mother and fetus,
current data clearly demonstrate that open fetal-maternal surgery is to
be recommended as novel standard of care when pregnancy is to be
continued and when respective criteria for the intervention before birth
are met. Undoubtedly, it is imperative to inform expecting mothers
about the option of prenatal surgery once their fetus is diagnosed with
open spina bifida.
- PMID: 24908159
