Late Prenatal Development of Hindbrain Herniation in Open Spina Bifida

We describe 4 cases of open spina bifida initially presumed to be "closed" defects in which serial prenatal ultrasound examinations documented the occurrence of sac rupture and development of Chiari II malformation later in the pregnancy.

In each case, the ultrasound examinations starting from 19 to 24 weeks' gestation, showed spinal cystic lesions containing neural elements but no evidence of Chiari II malformation. The lack of hindbrain herniation rendered them ineligible for prenatal surgical repair, but follow-up imaging at 24-32 weeks' gestation demonstrated the cystic lesions to be no longer visible or visible but decompressed, with the additional development of de novo hindbrain herniation.

We conclude by recommending serial ultrasound surveillance of presumed closed spina bifida as these may be in fact open defects, with confirmatory diagnosis only possible later in pregnancy. This detection is important because it may change counseling since prenatal repair or planned delivery may be warranted.