N. Tulipan et al., Reduced hindbrain herniation after intrauterine myelomeningocele repair: Areport of four cases, PED NEUROS, 29(5), 1998, pp. 274-278
Background: It has been theorized that fetal myelomeningocele repair may re
duce ongoing intrauterine injury and perhaps allow healing and regeneration
of dysplastic neural tissue. We report on the postnatal imaging studies of
the first 4 patients to have undergone intrauterine myelomeningocele repai
r at our institution. Methods: Each of the 4 patients underwent postnatal s
onographic and MRI. In addition, the postnatal ultrasounds of these 4 were
compared to a group of retrospective controls. Results: MRI scans of the 4
experimental subjects revealed no evidence of hindbrain herniation while ot
her stigmata of the Chiari-II malformation persisted. In comparison to the
retrospective controls this absence of herniation was distinctly unusual. C
onclusion: Intrauterine myelomeningocele repair may reduce the degree of hi
ndbrain herniation normally seen in patients with myelomeningocele. This ra
ises the possibility that intrauterine repair may decrease the morbidity as
sociated with the Chiari type-II malformation including brainstem dysfuncti
on, hydrocephalus and syringomyelia.