EP21.02: Intrauterine open fetal surgery for cervical spina bifida correction: case report

Abstract

Spina bifida is characterised by the incomplete formation of structures that protect the spinal cord and the inappropriate closure of the spinal bones. It presented a high rate of associated comorbidities (hydrocephalus, neurological deficits, motor deficiency in the lower limbs, vesical e and intestinal dysfunctions) secondary to the herniation of the structures of the posterior fossa by the foramen magno (Arnold-Chiari II malformation) and spinal cord injury and anchorage. Currently, there is the possibility of intrauterine open fetal surgery for correction of spina bifida lumbosacral which showed benefits in relation to the postnatal treatment according to MOMS clinical trial. Spina bifida in cervical topography is rare (3.5% of cases) and may be associated with Chiari II, hydrocephalus and cognitive deficits. Although it does not meet the criteria for intrauterine correction, we present a case with an isolated diagnosis of cervical myelomeningocele submitted to open fetal surgery for correction of spinal dysraphism. Pregnant LMOC, 37 years old, primigravida, no comordidades, ultrasound showing cervical myelomeningocele (C5-C7), no ventriculomegaly, chiari II (with anchoring of the cervical spine), no other malformations in the central nervous system. Fetal magnetic resonance imaging confirming findings of the ultrasound. Normal fetal karyotype. After clarification and consent of the couple and approval of our institution's ethics committee, patient underwent open fetal surgery at 26 weeks. Post-surgical ultrasound showed complete reversal of cerebellar herniation and absence of ventriculomegaly. Caesarean delivery at 37 weeks, newborn weight 3035g, female, apgar 9/10. Postnatal exams normal. Immediate postnatal follow-up adequate for the age. Cervical spina bifida is rare, but may present sequelae such as hydrocephalus and cognitive deficits. In carefully-selected cases, intrauterine surgery may be considered as therapeutic option.