Abstract
Occipital encephalocele is a rare neural tube defect characterized by an opening in
the occipital bone, resulting in herniation of meninges and brain tissue. Current
treatment consists of surgical repair in the postnatal period. The main objective
of intrauterine surgery for encephalocele is to decrease/stop the progression of the
brain herniation and reversal of microcephaly, contributing to better perinatal outcomes.
We reported the first experience of a fetal open surgery for occipital encephalocele
in a Brazilian Public Health Service. The surgery took place with gestational age
of 27 + 2 weeks of pregnancy. Careful dissection was performed between the skin and
the dura that herniated through the bone defect, and the redundant tissue was removed
and the dura was opened for access to neural tissue. Cesarean section was indicated
prematurely at 34 + 4 weeks due to significant thinning of the myometrial wall with
risk of uterine rupture. The newborn was discharged from the neonatal intensive care
unit at 21 days after delivery and from the hospital at 30 days in good conditions.
Fetal open surgery for encephalocele is still an experimental therapy with maternal–fetal
risks; however, the postnatal follow-up is critical for the assessment of the real
benefits of this surgery.
Keywords
fetal surgery - encephalocele - hysterotomy - cesarean section
