Prenatal Repair of Myelomeningocele and School-age Functional Outcomes

Abstract

BACKGROUND AND OBJECTIVES: The Management of Myelomeningocele Study (MOMS), a randomized trial of prenatal versus postnatal repair for myelomeningocele, found that prenatal surgery resulted in reduced hindbrain herniation and need for shunt diversion at 12 months of age and better motor function at 30 months. In this study, we compared adaptive behavior and other outcomes at school age (5.9-10.3 years) between prenatal versus postnatal surgery groups. METHODS: Follow-up cohort study of 161 children enrolled in MOMS. Assessments included neuropsychological and physical evaluations. Children were evaluated at a MOMS center or at a home visit by trained blinded examiners. RESULTS: The Vineland composite score was not different between surgery groups (89.0 6 9.6 in the prenatal group versus 87.5 6 12.0 in the postnatal group; P = .35). Children in the prenatal group walked without orthotics or assistive devices more often (29% vs 11%; P = .06), had higher mean percentage scores on the Functional Rehabilitation Evaluation of Sensori-Neurologic Outcomes (92 6 9 vs 85 6 18; P , .001), lower rates of hindbrain herniation (60% vs 87%; P , .001), had fewer shunts placed for hydrocephalus (49% vs 85%; P , .001) and, among those with shunts, fewer shunt revisions (47% vs 70%; P = .02) than those in the postnatal group. Parents of children repaired prenatally reported higher mean quality of life z scores (0.15 6 0.67 vs 0.11 6 0.73; P = .008) and lower mean family impact scores (32.5 6 7.8 vs 37.0 6 8.9; P = .002). CONCLUSIONS: There was no significant difference between surgery groups in overall adaptive behavior. Long-term benefits of prenatal surgery included improved mobility and independent functioning and fewer surgeries for shunt placement and revision, with no strong evidence of improved cognitive functioning. WHAT'S KNOWN ON THIS SUBJECT: Prenatal surgery was found to reduce hydrocephalus and hindbrain herniation in infants with myelomeningocele as well as improve motor function at 30 months of age. WHAT THIS STUDY ADDS: Among school-aged children with myelomeningocele whose mothers were randomly assigned to surgery before birth versus standard postnatal repair, there were no significant differences in adaptive behavior, but motor function and quality of life were better.

Open Fetal Microneurosurgery for Intrauterine Spina Bifida Repair

“…Our study supports the current evidence demonstrating that fetal surgery for SB repair decreases the risk of hydrocephalus and the need of ventriculoperitoneal shunting, with a low risk of perinatal death [3, 11, 20, 21]. We recognize that our study has also some limitations.…”

Update on prenatal diagnosis and fetal surgery for myelomeningocele

“…In this example, the defect starts approximately in L4. 3 Joyeux et al, 68 Brock et al, 48 Mazzola et al, 6 and Houtrow et al 50 ).…”

Spina Bifida: alternative approaches and treatment, based on evidence through gait analysis

“…Spina bifida is the most common congenital defect, presenting in a wide range of severity and with poor postnatal treatment options. The resolution "in utero" [29][30][31] have shown beneficial results such as the absence of a sac over the lesion, an improvement in the functional level, per example: an L3 lesion is significantly associated with independent ambulation [32,33] . The decrease in the need for ventriculoperitoneal shunts remains controversial, as the incidence or not of Chiari malformation as well [31] .…”