Fetoscopic Myelomeningocele Repair with Complete Release of the Tethered Spinal Cord Using a Three-Port Technique: Twelve-Month Follow-Up—A Case Report

Abstract

Open spina bifida is one of the most common congenital defects of the central nervous system. Open fetal surgery, which is one of the available therapeutic options, remains the gold standard for prenatal repairs. Fetoscopic closure may lower the number of maternal complications associated with open fetal surgery. Regardless of the approach, the outcome may be compromised by the development of tethered spinal cord (TSC) syndrome. At 24.2 weeks of gestation, a primipara was admitted due to fetal myelomeningocele and was deemed eligible for fetoscopic repair. Fetal surgery was performed at 25.0 weeks of gestation. It was the first complete untethering of the spinal cord and anatomic reconstruction (dura mater, spinal erectors, skin) achieved during a fetoscopic repair of spina bifida. Cesarean section due to placental abruption was performed at 31.1 weeks of gestation. VP shunting, with no need for revision, was performed at 5 weeks postdelivery due to progressing ventriculomegaly. No clinical or radiological signs of secondary tethering were observed. Neurological examination at 11 months postdelivery revealed cranial nerves without any signs of damage, axial hypotonia, decreased muscle tone in the lower extremities, and absent pathological reflexes. Motor development was slightly retarded. Complete untethering of the neural structures should always be performed, regardless of the surgical approach, as it is the only course of action that lowers the risk for developing secondary TSC.

Implementation and Assessment of a Laparotomy-Assisted Three-Port Fetoscopic Spina Bifida Repair Program

“…To report on our experience and then based on the IDEAL recommendations confirm the replication of the safety of this hybrid fetoscopic method, as already reported by other centers [22,23]. Other studies report the outcome of small groups of patients (seven cases, one case) but do not describe the implementation of the surgical approach or the establishment of a multidisciplinary team [24,25]. As part of our training, we were supervised directly by experts from the fetal team of TCH; in addition, essential requirements had to be met prior to the start of the surgery to join the consortium [20].…”

On the Cutting Edge

“…The stable infant is able to briefly bond with parents before admission to a neonatal intensive care unit for ongoing monitoring, care, and evaluation by the pediatric neurosurgeon. The typical neonatal length of stay following prenatal MMC repair is approximately 2 weeks (Pastuszka et al, 2022).…”

“…Ultrasound guidance is utilized throughout the surgery to assess the fetus and uterine environment. Typically, a combination of oral and intravenous tocolytic medications is provided before, during, and in the immediate postoperative phase to reduce premature uterine contractions (Pastuszka et al, 2022).…”

“…The fetus is then externally rotated and positioned to allow access to the defect. A cocktail of an opioid and muscle relaxant is given intramuscularly in the buttocks of the fetus through one of the working ports (Pastuszka et al, 2022). The spinal defect is then repaired by the pediatric neurosurgeon with assistance of the fetal surgeon(s).…”