Fetal surgery for spina bifida

Abstract

The two-hit hypothesis of neural injury in the wake of open neural tube defects suggests an opportunity for preservation of function and potential reversibility of early morphological changes in the fetus diagnosed with myelomeningocele. The Management of Myelomeningocele Study (MOMS) demonstrated reduced need for shunting and improved neurological function in patients treated in utero relative to postnatally, thereby offering level 1 evidence supporting fetal repair. Subsequent studies have offered additional information about urological, orthopedic, radiological, and maternal factors surrounding fetal repair. The quest for robust long-term neurocognitive and motor function data is underway and poised to shape the future of fetal repair. In addition, technical innovations such as fetoscopic surgery aim to minimize maternal morbidity while conferring the beneficial effects observed with open intrauterine intervention.

Keywords: CHOP = Children’s Hospital of Philadelphia; CM-II = Chiari malformation type II; CPC = choroid plexus cauterization; ETV = endoscopic third ventriculostomy; IUR = intrauterine repair; MM = myelomeningocele; MOMS = Management of Myelomeningocele Study; PROM = premature rupture of membranes; TCS = tethered cord syndrome; VP = ventriculoperitoneal; congenital; fMMR = fetal MM repair; fetal; fetoscopic; intrauterine; myelomeningocele; spina bifida.