Skip to main content
Log in

Fetal myelomeningocele surgery: preschool functional status using the Functional Independence Measure for children (WeeFIM)

  • Original Paper
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Objective

To study preschool functional status in children following fetal myelomeningocele (fMMC) surgery.

Material and methods

Prior to the NICHD-MOMS trial, 30 fMMC underwent standardized neurodevelopmental examination at 5 years of age. Functional status was determined with the Functional Independence Measure (WeeFIM), which assesses self-care, mobility, and cognitive independence.

Results

Evaluations were completed in 26 (87%). Mean cognitive (93.0 ± 21.9), self-care (66.5 ± 23.9), mobility (82.3 ± 19.5), and total (77.9 ± 20.3) functional quotient of fMMC children were significantly lower than age-matched population norms (P < 0.01). Complete caregiver independence was achieved by 22 (84%), 10 (38%), 16 (62%), and 15 (58%) fMMC children for cognition, self-care, mobility, and total functional outcome, respectively. Cognitive, mobility, and total independence were higher in non-shunted than shunted fMMC children (P = 0.02, P = 0.02, and P < 0.01, respectively) and in fMMC children with average neurodevelopmental scores (P < 0.001, P = 0.01, and P < 0.01, respectively). Self-care independence tended to be higher in the non-shunted group and in fMMC children with normal neurodevelopmental outcome (P = 0.07 and P = 0.09, respectively).

Conclusion

The majority of fMMC children achieved cognitive and mobility independence, but continue to require significant assistance in self-care. Non-shunted and fMMC children with normal neurodevelopmental outcome were more likely to be independent in daily living activities. Better understanding of the extent of functional limitations following fMMC surgery will allow for more effective early interventions geared toward maximizing independence in everyday tasks in all environments.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Danzer E, Flake AW (2006/2007) In utero repair of myelomeningocele: rationale, initial clinical experience and a randomized controlled prospective clinical trial. Neuroembryol Aging 4:165–174

    Article  Google Scholar 

  2. Bier JA, Prine A, Tremont M, Msall M (2005) Medical, functional and social determinants of health-related quality of life in individuals with myelomeningocele. Dev Med Child Neurol 47:609–612

    PubMed  Google Scholar 

  3. Verhoef M, Barf HA, Post MW, van Asbeck FW, Gooskens RH, Prevo AJ (2006) Functional independence among young adults with spina bifida, in relation to hydrocephalus and level of lesion. Dev Med Child Neurol 48:114–119

    Article  PubMed  Google Scholar 

  4. Tsai PY, Yang TF, Chan RC, Huang PH, Wong TT (2002) Functional investigation in children with spina bifida—measured by the pediatric evaluation of disability inventory (PEDI). Child’s Nerv Syst 18:48–53

    Article  Google Scholar 

  5. Danzer E, Gerdes M, Bebbington MW, Zarnow DM, Adzick NS, Johnson MP (2010) Preschool neurodevelopmental outcome of children following fetal myelomeningocele closure. Am J Obstet Gynecol 202:450.e1-9

    Google Scholar 

  6. Johnson MP, Sutton LN, Rintoul N, Crombleholme TM, Flake AW, Howell LJ et al (2003) Fetal myelomeningocele repair: short-term clinical outcomes. Am J Obstet Gynecol 189:482–487

    Article  PubMed  Google Scholar 

  7. Danzer E, Finkel RS, Rintoul NE, Bebbington MW, Schwartz ES, Zarnow DM et al (2008) Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function. Neuropediatrics 39:359–362

    Article  PubMed  CAS  Google Scholar 

  8. Danzer E, Gerdes M, Bebbington MW, Sutton LN, Melchionni J, Adzick NS et al (2009) Lower extremity neuromotor function and short-term ambulatory potential following in utero myelomeningocele surgery. Fetal Diagn Ther 25:47–53

    Article  PubMed  Google Scholar 

  9. Msall ME, DiGaudio K, Rogers BT, LaForest S, Catanzaro NL, Campbell J, Wilczenski F, Duffy LC (1994) The functional independence measure for children (WeeFIM): conceptual basis and pilot use in children with developmental disabilities. Clin Pediatr 33:431–438

    Article  CAS  Google Scholar 

  10. Msall ME, DiGaudio K, Duffy LC, LaForest S, Braun S, Granger CV (1994) WeeFIM: normative sample of an instrument for tracking functional independence in children. Clin Pediatr 33:431–438

    Article  CAS  Google Scholar 

  11. Msall ME, Phelps DL, DiGaudio KM, Dobson V, Tung B, McClead RE, Quinn GE, Reynolds JD, Hardy RJ, Palmer EA (2000) Severity of neonatal retinopathy of prematurity is predictive of neurodevelopmental functional outcome at age 5.5 years. behalf of the cryotherapy for retinopathy of prematurity cooperative group. Pediatrics 106:998–1005

    Article  PubMed  CAS  Google Scholar 

  12. Dennis M, Barnes MA (2010) The cognitive phenotype of spina bifida meningomyelocele. Dev Disabil Res Rev 16:31–39

    Article  PubMed  Google Scholar 

  13. de Wit OA, den Dunnen WF, Sollie KM, Munoz RI, Meiners LC, Brouwer OF, Rodriguez EM, Sival DA (2008) Pathogenesis of cerebral malformations in human fetuses with meningomyelocele. Cerebrospinal Fluid Res 5:4

    Article  PubMed  Google Scholar 

  14. Fletcher JM, Copeland K, Frederick JA, Blaser SE, Kramer LA, Northrup H, Hannay HJ, Brandt ME, Francis DJ, Villarreal G, Drake JM, Laurent JP, Townsend I, Inwood S, Boudousquie A, Dennis M (2005) Spinal lesion level in spina bifida: a source of neural and cognitive heterogeneity. J Neurosurg 102:268–279

    PubMed  Google Scholar 

  15. Fletcher JM, McCauley SR, Brandt ME, Bohan TP, Kramer LA, Francis DJ, Thorstad K, Brookshire BL (1996) Regional brain tissue composition in children with hydrocephalus. relationships with cognitive development. Arch Neurol 53:549–557

    PubMed  CAS  Google Scholar 

  16. Danzer E, Johnson MP, Bebbington M, Simon EM, Wilson RD, Bilaniuk LT, Sutton LN, Adzick NS (2007) Fetal head biometry assessed by fetal magnetic resonance imaging following in utero myelomeningocele repair. Fetal Diagn Ther 22:1–6

    Article  PubMed  Google Scholar 

  17. Danzer E, Johnson MP, Wilson RD, Flake AW, Hedrick HL, Sutton LN, Adzick NS (2004) Fetal head biometry following in-utero repair of myelomeningocele. Ultrasound Obstet Gynecol 24:606–611

    Article  PubMed  CAS  Google Scholar 

  18. Buran CF, Sawin KJ, Brei TJ, Fastenau PS (2004) Adolescents with myelomeningocele: activities, beliefs, expectations and perceptions. Dev Med Child Neurol 46:244–252

    Article  PubMed  Google Scholar 

  19. Dahl M, Ahlsten G, Butler A, Norrlin S, Strinnholm M, Winberg A (2000) Self-care skills in young children with myelomeningocele. Eur J Pediatr Surg 10:52–53

    PubMed  Google Scholar 

  20. Schoenmakers MA, Uiterwaal CS, Gulmans VA, Gooskens RH, Helders PJ (2005) Determinants of functional independence and quality of life in children with spina bifida. Clin Rehabil 19:677–685

    Article  PubMed  CAS  Google Scholar 

  21. Pit-ten Cate IM, Kennedy C, Stevenson J (2002) Disability and quality of life in spina bifida and hydrocephalus. Dev Med Child Neurol 44:317–322

    Article  Google Scholar 

  22. Padua L, Rendeli C, Rabini A, Girardi E, Tonali P, Salvaggio E (2002) Health-related quality of life and disability in young patients with spina bifida. Arch Phys Med Rehabil 83:1384–1388

    Article  PubMed  Google Scholar 

  23. McCormick MC, Charney EB, Stemmler MM (1986) Assessing the impact of a child with spina bifida on the family. Dev Med Child Neurol 28:53–61

    Article  PubMed  CAS  Google Scholar 

  24. Norrlin S, Strinnholm M, Carlsson M, Dahl M (2003) Factors of significance for mobility in children with myelomeningocele. Acta Paediatr 92:204–210

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Mark P. Johnson.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Danzer, E., Gerdes, M., Bebbington, M.W. et al. Fetal myelomeningocele surgery: preschool functional status using the Functional Independence Measure for children (WeeFIM). Childs Nerv Syst 27, 1083–1088 (2011). https://doi.org/10.1007/s00381-011-1388-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-011-1388-y

Keywords

Navigation