title: Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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 < 0.0001). Actual rates of shunt placement were only 44% and 84% in the 2 groups, respectively (p < 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 < 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 < 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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Departments of 1 Neurosurgery and.
authors.forename: Noel
authors.initials: N
authors.lastname: Tulipan
authors.affiliation: Departments of 1 Neurosurgery and.
authors.forename: John C
authors.initials: JC
authors.lastname: Wellons
authors.affiliation: The Biostatistics Center, George Washington University, Washington, DC; 
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Thom
authors.affiliation: Departments of 3 Neurological Surgery.
authors.forename: Nalin
authors.initials: N
authors.lastname: Gupta
authors.affiliation: Department of Neurosurgery and.
authors.forename: Leslie N
authors.initials: LN
authors.lastname: Sutton
authors.affiliation: The Biostatistics Center, George Washington University, Washington, DC; 
authors.forename: Pamela K
authors.initials: PK
authors.lastname: Burrows
authors.affiliation: Surgery, and.
authors.forename: Diana
authors.initials: D
authors.lastname: Farmer
authors.affiliation: Pediatrics, Vanderbilt University Medical Center, Nashville, Tennessee;
authors.forename: William
authors.initials: W
authors.lastname: Walsh
authors.affiliation: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Pennsylvania;
authors.forename: Mark P
authors.initials: MP
authors.lastname: Johnson
authors.affiliation: Obstetrics and Gynecology, University of California, San Francisco, California;
authors.forename: Larry
authors.initials: L
authors.lastname: Rand
authors.affiliation: Pregnancy and Perinatology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, Bethesda, Maryland; and.
authors.forename: Susan
authors.initials: S
authors.lastname: Tolivaisa
authors.affiliation: Department of Obstetrics and Gynecology, Columbia University, New York, New York.
authors.forename: Mary E
authors.initials: ME
authors.lastname: D'alton
authors.affiliation: Center for Fetal Diagnosis and Treatment, The Children's Hospital of Philadelphia, Pennsylvania;
authors.forename: N Scott
authors.initials: NS
authors.lastname: Adzick
mesh_terms: D000328:Adult; D002425:Cautery; D002557:Cerebrospinal Fluid Shunts / Q000706:statistics & numerical data*; D002831:Choroid Plexus / Q000601:surgery; D005260:Female; D005313:Fetal Death; D005865:Gestational Age; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology / Q000601:surgery*; D007223:Infant; D066088:Infant Death; D007231:Infant, Newborn; D016015:Logistic Models; D008591:Meningomyelocele / Q000601:surgery*; D044583:Neuroendoscopy; D011247:Pregnancy; D011295:Prenatal Care / Q000379:methods*; D012086:Reoperation; D012307:Risk Factors; D020542:Third Ventricle / Q000473:pathology / Q000601:surgery; D016896:Treatment Outcome; D014696:Ventriculostomy / Q000706:statistics & numerical data
keywords: ETV-CPC = endoscopic third ventriculostomy and choroid plexus coagulation; MOMS = The Management of Myelomeningocele Study; cerebrospinal fluid shunt; congenital; fetal surgery; hindbrain herniation; hydrocephalus; myelomeningocele; spina bifida