Two-Port Endoscopic Fetal Closure of Myelomeningocele

Abstract

Introduction: Open neural tube defects are a common congenital abnormality with significant consequences for affected children and their families. Issues include paraplegia, walking difficulties, bowel and bladder incontinence, and hydrocephalus. The 2011 Management of Myelomeningocele Study established fetal closure as a treatment option.1 Fetal closure reduces the incidence of hydrocephalus and improves motor outcomes compared with conventional postnatal closure. Open prenatal closure, however, necessitates hysterotomy and significantly increases the risk of preterm delivery and uterine dehiscence.2 Endoscopic approaches eliminating the need for hysterotomy have been described.3–5 Here we demonstrate our two-port endoscopic technique. Materials and Methods: The mother is at 26 weeks gestation and ultrasonography work-up has revealed a myelomeningocele defect of the fetus at the L3-S1 levels. A transverse abdominal opening is made for uterine exposure. Two ports are placed 6 cm apart with ultrasound guidance into the fundus of the uterus. A total of 300 cc of amniotic fluid are removed and the uterus is insufflated with humidified CO2 gas. The fetus and myelomeningocele defect are observed under endoscopic guidance. Endoscopic closure of the myelomeningocele is done in two major steps. First, the neural placode is released through sharp circumferential dissection of the arachnoid lying between the neural placode margin and the junctional zone. Second, the defect is closed primarily with interrupted vertical mattress sutures. Closure is facilitated with the use of relaxing flank incisions and retention sutures. Results: The child was born term at 39 weeks through vaginal delivery. Her neurologic examination demonstrates intact spinal function inclusive of the S1 level. Conclusions: Endoscopic fetal closure of myelomeningocele is technically feasible and can be accomplished through a two-port technique utilizing single layer closure. The avoidance of hysterotomy with this method may reduce the risk of preterm delivery and uterine dehiscence. No competing financial interests exist. Runtime of video: 6 mins 19 secs Related Presentations: Podium Presentation (ID 83697), IPEG's 26th Annual Congress for Endosurgery in Children, July 20, 2017, London, England.