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

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

Publication History

Received: 05 June 2022

Accepted: 30 September 2022

Article published online:
16 November 2022

• References

• 1 Cavalheiro S, Silva da Costa MD, Nicácio JM. et al. Fetal surgery for occipital encephalocele. J Neurosurg Pediatr 2020; 26 (06) 605-612
• 2 Stevenson RE, Allen WP, Pai GS. et al. Decline in prevalence of neural tube defects in a high-risk region of the United States. Pediatrics 2000; 106 (04) 677-683
• 3 Kasprian GJ, Paldino MJ, Mehollin-Ray AR. et al. Prenatal imaging of occipital encephaloceles. Fetal Diagn Ther 2015; 37 (03) 241-248
• 4 Siffel C, Wong LY, Olney RS, Correa A. Survival of infants diagnosed with encephalocele in Atlanta, 1979-98. Paediatr Perinat Epidemiol 2003; 17 (01) 40-48
• 5 Cavalheiro S, da Costa MDS, Mendonça JN. et al. Antenatal management of fetal neurosurgical diseases. Childs Nerv Syst 2017; 33 (07) 1125-1141
• 6 Adzick NS, Thom EA, Spong CY. et al; MOMS Investigators. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011; 364 (11) 993-1004
• 7 Moron AF, Barbosa MM, Milani H. et al. Perinatal outcomes after open fetal surgery for myelomeningocele repair: a retrospective cohort study. BJOG 2018; 125 (10) 1280-1286
• 8 Novoa Y Novoa V, Shazly S, Araujo Júnior E, Tonni G, Ruano R. Tocolysis for open prenatal repair of myelomeningocele: systematic review. J Matern Fetal Neonatal Med 2020; 33 (10) 1786-1791
• 9 Cochrane D, Aronyk K, Sawatzky B, Wilson D, Steinbok P. The effects of labor and delivery on spinal cord function and ambulation in patients with meningomyelocele. Childs Nerv Syst 1991; 7 (06) 312-315
• 10 Mahapatra AK. Giant encephalocele: a study of 14 patients. Pediatr Neurosurg 2011; 47 (06) 406-411