Neurosurgical procedures for children with myelomeningocele after fetal or postnatal surgery: a comparative effectiveness study

Abstract

Aim To compare the frequencies of neurosurgical procedures to treat comorbid conditions of myelomeningocele in patients who underwent fetal surgery versus postnatal surgery for closure of the placode. Method By utilizing the National Spina Bifida Patient Registry in a comparative effectiveness study, 298 fetal surgery patients were matched by birthdate (±3mo) and spina bifida clinic site with one to three postnatal surgery patients (n=648). Histories were obtained by record review on enrollment and yearly subsequently. Multivariable Poisson regression was used to compare frequencies of procedures between cohorts, with adjustments for sex, ethnicity, insurance status, spinal segmental level of motor function, age at last visit recorded in the Registry, and, for shunt revision in shunted patients, age at cerebrospinal fluid (CSF) diversion. Results The median age at last visit was 4 years. In fully adjusted analyses in patients aged at least 12 months old, fetal surgery was associated with decreased frequency of CSF diversion for hydrocephalus by ventriculoperitoneal shunt insertion or endoscopic third ventriculostomy compared with postnatal surgery (46% vs 79%; incidence rate ratio=0.61; 95% confidence interval [CI] 0.53–0.71; p<0.01). Over all ages, fetal surgery was associated with decreased frequency of Chiari decompression for brainstem dysfunction (3% vs 7%; incidence rate ratio=0.41; 95% CI 0.19–0.88; p=0.02). Also over all ages, differences were not significant in frequencies of shunt revision in shunted patients (53% vs 55%; incidence rate ratio=0.87; 95% CI 0.69–1.11; p=0.27), nor tethered cord release for acquired spinal cord dysfunction (18% vs 16%; incidence rate ratio=1.11; 95% CI 0.84–1.47; p=0.46). Interpretation Even with the variations inherent in clinical practice, fetal surgery was associated with lower frequencies of CSF diversion and of Chiari decompression, independent of covariates. Fetal surgery was associated with lower frequencies of cerebrospinal fluid diversion and decompression of Chiari II malformation than postnatal surgery. Frequencies of ventriculoperitoneal shunt revision and tethered cord release were not significantly different between cohorts.

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

“…Worley et al compared the results of fetal ( n = 248) and postnatal ( n = 698) interventions in cohorts of children up to 12 months of age and found that fetal surgery was associated with lower incidence of CSF diversion for hydrocephalus by VP shunting or endoscopic third ventriculostomy as compared to postnatal surgery (110/239 (46) vs. 349/441 (79) respectively; IRR 0.61; CI95%; 0.53–0.71; p < 0.01). Repeat tethered cord release was necessary in 54/298 (18%) vs. 102/648 (16%) cases, respectively (IRR 1.22 (0.94–1.59); CI95% 0.13; 1.11 0.84–1.47; p. 46) [ 11 ]. Low position of the medullary cone remains the main diagnostic marker for a tethered spinal cord on prenatal imaging [ 21 ].…”

“…The improvement in the neurological development in the randomized MOMS trial was limited by the occurrence of the TSC (tethered spinal cord) syndrome [ 9 ]. TSC remains a risk factor for less favorable neurological outcomes in the pre- and postnatal repair groups, regardless of the surgical approach (fetoscopic vs. open surgery) [ 10 , 11 ]. Our original technique of complete untethering of the spinal cord and the use of fetal native tissue (dura mater, spinal erectors, skin) for anatomic reconstruction reduce the risk of secondary TSC.…”

Fetal surgery for spina bifida: a great success story in surgical innovation

“…As such, they have scientifically validated, to an acceptable degree, the generalizability of the MOMS findings. Both these retrospective cohort studies suggest that single‐site fetal centers functioning outside of a randomized trial environment, respectively in Europe 3 and in the USA where results from independent centers were aggregated in the National Spina Bifida Patient Registry, 4 can achieve comparable short‐ and medium‐term outcomes to the MOMS trial.…”

“…and Worley et al. have bridged this gap in knowledge by demonstrating different aspects of the effectiveness of fetal SBA surgery 3,4 . As such, they have scientifically validated, to an acceptable degree, the generalizability of the MOMS findings.…”

Postnatal Myelomeningocele Repair in the United States: Rates and Disparities Before and After the Management of Myelomeningocele Study Trial

“…17,18 Some reports demonstrate significant associations between social determinants of health and choice of approach. 19-22…”