title: In utero surgery rescues neurological function at birth in sheep with spina bifida.
abstract: We hypothesize that the neurologic deficit associated with open spina bifida is not directly caused by the primary defect but rather is due to chronic mechanical and chemical trauma since the unprotected neural tissue is exposed to the intrauterine environment. We report here that exposure of the normal spinal cord to the amniotic cavity in midgestational sheep fetuses leads to a human-like open spina bifida with paraplegia at birth, indicating that the exposed neural tissue is progressively destroyed during pregnancy. When open spina bifida was repaired in utero at an intermediate stage, the animals had near-normal neurologic function. The spinal cord was deformed but largely preserved. These findings suggest that secondary neural tissue destruction during pregnancy is primarily responsible for the functional loss and that timely in utero repair of open spina bifida might rescue neurologic function.
authors.affiliation: Fetal Treatment Center, University of California, San Francisco 94143-0570, USA.
authors.forename: M
authors.initials: M
authors.lastname: Meuli
authors.forename: C
authors.initials: C
authors.lastname: Meuli-Simmen
authors.forename: G M
authors.initials: GM
authors.lastname: Hutchins
authors.forename: C D
authors.initials: CD
authors.lastname: Yingling
authors.forename: K M
authors.initials: KM
authors.lastname: Hoffman
authors.forename: M R
authors.initials: MR
authors.lastname: Harrison
authors.forename: N S
authors.initials: NS
authors.lastname: Adzick
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D004195:Disease Models, Animal; D005260:Female; D005315:Fetal Diseases / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D005333:Fetus / Q000601:surgery*; D009460:Neurologic Examination; D011247:Pregnancy; D012756:Sheep; D013116:Spinal Cord / Q000196:embryology / Q000503:physiopathology* / Q000601:surgery; D016135:Spinal Dysraphism / Q000196:embryology / Q000503:physiopathology / Q000601:surgery*; D014599:Uterus / Q000601:surgery