Fetal Open Surgery for Occipital Encephalocele: First Experience in a Tertiary Public Hospital in Brazil

Abstract

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.