Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>The Management of Myelomeningocele Study (MOMS) was a multicenter randomized trial comparing the safety and efficacy of prenatal and postnatal closure of myelomeningocele. The trial was stopped early because of the demonstrated efficacy of prenatal surgery, and outcomes on 158 of 183 pregnancies were reported. Here, the authors update the 1-year outcomes for the complete trial, analyze the primary and related outcomes, and evaluate whether specific prerandomization risk factors are associated with prenatal surgery benefit.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>The primary outcome was a composite of fetal loss or any of the following: infant death, CSF shunt placement, or meeting the prespecified criteria for shunt placement. Primary outcome, actual shunt placement, and shunt revision rates for prenatal versus postnatal repair were compared. The shunt criteria were reassessed to determine which were most concordant with practice, and a new composite outcome was created from the primary outcome by replacing the original criteria for CSF shunt placement with the revised criteria. The authors used logistic regression to estimate whether there were interactions between the type of surgery and known prenatal risk factors (lesion level, gestational age, degree of hindbrain herniation, and ventricle size) for shunt placement, and to determine which factors were associated with shunting among those infants who underwent prenatal surgery.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>Ninety-one women were randomized to prenatal surgery and 92 to postnatal repair. The primary outcome occurred in 73% of infants in the prenatal surgery group and in 98% in the postnatal group (p &lt; 0.0001). Actual rates of shunt placement were only 44% and 84% in the 2 groups, respectively (p &lt; 0.0001). The authors revised the most commonly met criterion to require overt clinical signs of increased intracranial pressure, defined as split sutures, bulging fontanelle, or sunsetting eyes, in addition to increasing head circumference or hydrocephalus. Using these modified criteria, only 3 patients in each group met criteria but did not receive a shunt. For the revised composite outcome, there was a difference between the prenatal and postnatal surgery groups: 49.5% versus 87.0% (p &lt; 0.0001). There was also a significant reduction in the number of children who had a shunt placed and then required a revision by 1 year of age in the prenatal group (15.4% vs 40.2%, relative risk 0.38 [95% CI 0.22–0.66]). In the prenatal surgery group, 20% of those with ventricle size &lt; 10 mm at initial screening, 45.2% with ventricle size of 10 up to 15 mm, and 79.0% with ventricle size ≥ 15 mm received a shunt, whereas in the postnatal group, 79.4%, 86.0%, and 87.5%, respectively, received a shunt (p = 0.02). Lesion level and degree of hindbrain herniation appeared to have no effect on the eventual need for shunting (p = 0.19 and p = 0.13, respectively). Similar results were obtained for the revised outcome.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>Larger ventricles at initial screening are associated with an increased need for shunting among those undergoing fetal surgery for myelomeningocele. During prenatal counseling, care should be exercised in recommending prenatal surgery when the ventricles are 15 mm or larger because prenatal surgery does not appear to improve outcome in this group. The revised criteria may be useful as guidelines for treating hydrocephalus in this group.</jats:p></jats:sec>

Editorial: Does it pass the sniff test? Mining the NSQIP-P database for neurosurgical diseases

“…This number at 1 month is lower than the published 84% shunt rate at 12 months reported in the MOMS trial. 1,3 The timing of intervention on hydrocephalus after MMC closure has yet to be defined and studied in the literature. A preliminary retrospective review of our institution's experience reveals that shunt rates are indeed appreciably lower at 1 month than at 12 months, though this complex and interesting problem deserves dedicated multiinstitutional study in the future.…”

Fetal Surgery for Myelomeningocele: A Systematic Review and Meta-Analysis of Outcomes in Fetoscopic versus Open Repair

“…Another case report of fetal MMC repair was excluded as it lacked details regarding the operation performed and subsequent outcomes [21]. Four articles were excluded as they reported follow-up outcomes of the MOMS [8,9,22,23]. We included the outcomes of a recently published study on a modified approach to fetoscopic MMC repair through a maternal laparotomy and excluded a case report by the same authors to avoid duplication of data [24,25].…”

Neuropathic bladders: Investigation and treatment through their lifetime

“…Although controversial, in-utero closure of the open neural tube defect may offer some advantages to the child, but the risks of preterm labor (13% delivered at less than 30 weeks gestation) and maternal health remain significant concerns. [7][8][9] An ambitious randomized trial of 183 patients has demonstrated decreased need for ventriculoperitoneal (VP) shunts, 7 as well as improved mental and motor development, decreased hind brain herniation, and improved ambulation by 30 months. 9 Urological outcomes were not part of the initial assessment, but a 2015 review of the Management of Myelomeningocele Study (MOMS) demonstrated no difference in the need for clean intermittent catheterization (CIC), but patients appeared to have less bladder trabeculation and fewer open bladder necks.…”

“…6,26,27 It has been shown that the most important factor in successful outcomes is proper family selection. [7][8][9]28 Bladder neck procedures range from endoscopic injections of the bladder neck to outpatient suburethral slings, to bladder neck reconstruction, to ligation or closure of the bladder neck. 7,29 Many pediatric urologists prefer a moderate approach, to leave the patient with a "pop-off" mechanism in the case of inability or unwillingness to catheterize.…”

“…[7][8][9]28 Bladder neck procedures range from endoscopic injections of the bladder neck to outpatient suburethral slings, to bladder neck reconstruction, to ligation or closure of the bladder neck. 7,29 Many pediatric urologists prefer a moderate approach, to leave the patient with a "pop-off" mechanism in the case of inability or unwillingness to catheterize. If an aggressive approach is considered (bladder neck reconstruction, occlusive sling, bladder neck closure) the patient will require a continent cutaneous catheterizable channel.…”