Objective: It was the aim of this study to assess the prevalence of preschool neurobehavioral problems in children following fetal myelomeningocele (fMMC) surgery. Methods: Prior to the Management of Myelomeningocele Study, 30 fMMC patients underwent a standardized neurodevelopmental examination at 5 years of age. The prevalence of behavioral problems was assessed by the Child Behavior Check List (CBCL), which includes a total problem score and 2 broad-band indices (internalizing and externalizing problems). fMMC children were classified as having normal, at-risk or clinically significant scores based on calculated T scores. Results: Twenty-two (73%) fMMC families completed the evaluation. Mean age at delivery following fMMC surgery was 35.5 ± 1.6 weeks. The prevalence of ‘at-risk’ or ‘clinically significant’ scores for internalizing, externalizing and total behavioral problems was similar to general population norms. No difference in overall internalizing (p = 0.19), externalizing (p = 0.54) and total behavioral (p = 0.18) scores was found between non-shunted and shunted fMMC children. However, shunted fMMC children were more likely to experience anxiety and depressive (p = 0.02), pervasive developmental (p = 0.03) and withdrawn (p = 0.06) behavior. Oppositional defiant, attention deficit and hyperactivity problems were not found. No correlation was found between overall neurodevelopmental outcome and abnormal CBCL scores. Conclusions: fMMC surgery and subsequent preterm delivery is not associated with increased behavioral problems, impaired social interactions and restricted behavior patterns. Shunted fMMC children were more likely to have scores in the ‘at-risk’ or ‘clinically significant’ range for multiple CBCL subindices. Early detection of behavioral problems following fMMC surgery provides an opportunity for targeted and timely intervention enabling affected fMMC children to be socially successful.
© 2011 S. Karger AG, Basel
- Spina bifida
- Fetal surgery
- Child Behavior Check List
- Premature delivery
- Neurobehavioral outcome
- Danzer E, Flake AW: In utero repair of myelomeningocele: rationale, initial clinical experience and a randomized controlled prospective clinical trial. Neuroembryol Aging 2007;4:165–174.
- Dennis M, Landry SH, Barnes M, Fletcher JM: A model of neurocognitive function in spina bifida over the life span. J Int Neuropsychol Soc 2006;12:285–296.
- Loss N, Yeates KO, Enrile BG: Attention in children with myelomeningocele. Child Neuropsychol 1998;4:7–20.
- Swartwout MD, Cirino PT, Hampson AW, Fletcher JM, Brandt ME, Dennis M: Sustained attention in children with two etiologies of early hydrocephalus. Neuropsychology 2008;22:765–775.
- Fletcher JM, Ostermaier KK, Cirino PT, Dennis M: Neurobehavioral outcomes in spina bifida: processes versus outcomes. J Pediatr Rehabil Med 2008;1:311–324.
- Fletcher JM, Brookshire BL, Landry SH, Bohan TP, Davidson KC, Francis DJ, Thompson NM, Miner ME: Behavioral adjustment of children with hydrocephalus: relationships with etiology, neurological, and family status. J Pediatr Psychol 1995;20:109–125.
- Vinck A, Mullaart R, Rotteveel J, Maasen: Neuropsychological assessment in children with spina bifida. Cerebrospinal Fluid Res 2009;6:6.
- Adzick NS: Fetal myelomeningocele: natural history, pathophysiology, and in-utero intervention. Semin Fetal Neonatal Med 2010;15:9–14.
- Johnson MP, Sutton LN, Rintoul N, et al: Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol 2003;189:482–487.
- Johnson MP, Gerdes M, Rintoul N, Pasquariello P, Melchionni J, Sutton LN, Adzick NS: Maternal-fetal surgery for myelomeningocele: neurodevelopmental outcomes at 2 years of age. Am J Obstet Gynecol 2006;194:1145–1150.
- Danzer E, Johnson MP, Bebbington M, et al: Fetal head biometry assessed by fetal magnetic resonance imaging following in utero myelomeningocele repair. Fetal Diagn Ther 2007;22:1–6.
- Danzer E, Finkel RS, Rintoul NE, et al: Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function. Neuropediatrics 2008;39:359–362.
- Danzer E, Finkel R, Gerdes M, et al: The relationship of seizure activity and chronic epilepsy in early infancy and short-term neurodevelopmental outcome following fetal myelomeningocele closure. Neuropediatrics 2010;41:140–143.
- Danzer E, Gerdes M, Bebbington MW, et al: Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn Ther 2009;25:47–53.
- Danzer E, Gerdes M, Bebbington MW, Zarnow DM, Adzick NS, Johnson MP: Preschool neurodevelopmental outcome of children following fetal myelomeningocele closure. Am J Obstet Gynecol 2010;202:450.e1–e9.
- Danzer E, Gerdes M, Bebbington MW, Koh J, Adzick SN, Johnson MP: Fetal myelomeningocele surgery: preschool functional status using the Functional Independence Measure for children (WeeFIM). Childs Nerv Syst 2011;27:1083–1088.
- Adzick NS, Thom EA, Spong CY, Brock JW 3rd, Burrows PK, Johnson MP, Howell LJ, Farrell JA, Dabrowiak ME, Sutton LN, Gupta N, Tulipan NB, D’Alton ME, Farmer DL, MOMS Investigators: A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med 2011;364:993–1004.
- Wechsler D: Wechsler Intelligence Scale for Children, ed 3. San Antonio, The Psychological Corporation, Harcourt Brace and Company, 1991.
- Achenbach TM, Ruffle TM: The Child Behavior Checklist and related forms for assessing behavioral/emotional problems and competencies. Pediatr Rev 2000;21:265–271.
- Achenbach TM, Rescorla LA: Child Behavior Checklist for Ages 1.5–5. Burlington, University of Vermont, 2000.
- Dias MS, McLone DG: Hydrocephalus in the child with dysraphism. Neurosurg Clin N Am 1993;4:715–726.
- Rintoul NE, Sutton LN, Hubbard AM, et al: A new look at myelomeningoceles: functional level, vertebral level, shunting, and the implication for fetal intervention. Pediatrics 2002;109:409–413.
- Bhutta AT, Cleves MA, Casey PH, Cradock MM, Anand KJ: Cognitive and behavioral outcomes of school-aged children who were born preterm: a metaanalysis. JAMA 2002;288:728–737.
- Gray RF, Indurkhya A, McCormick MC: Prevalence, stability, and predictors of clinically significant behavior problems in low birth weight children at 3, 5, and 8 years of age. Pediatrics 2004;114:736–743.
- Hack M, Flannery DJ, Schluchter M, Cartar L, Borawski E, Klein N: Outcomes in young adulthood for very-low-birth-weight infants. N Engl J Med 2002;346:149–157.
- van Baar AL, Vermaas J, Knots E, de Kleine MJ, Soons P: Functioning at school age of moderately preterm children born at 32 to 36 weeks’ gestational age. Pediatrics 2009;124:251–257.
- Verhulst FC, van der Ende J: Assessment of child psychopathology: relationships between different methods, different informants and clinical judgment of severity. Acta Psychiatr Scand 1991;84:155–159.
Mark P. Johnson, MD
The Children’s Hospital of Philadelphia
The Center for Fetal Diagnosis and Treatment, 5th Floor Wood Center
34th Street and Civic Center Boulevard, Philadelphia, PA 19104-4399 (USA)
Tel. +1 215 590 747, E-Mail firstname.lastname@example.org
Received: April 12, 2011
Accepted after revision: June 7, 2011
Published online: September 8, 2011
Number of Print Pages : 6
Number of Figures : 0, Number of Tables : 3, Number of References : 27
Fetal Diagnosis and Therapy (Clinical Advances and Basic Research)
Vol. 30, No. 3, Year 2011 (Cover Date: November 2011)
Journal Editor: Gratacós E. (Barcelona)
ISSN: 1015-3837 (Print), eISSN: 1421-9964 (Online)
For additional information: http://www.karger.com/FDT
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher or, in the case of photocopying, direct payment of a specified fee to the Copyright Clearance Center.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.