Abstract
Purpose
The long-term outcome in spina bifida–Chiari II–hydrocephalus complex is poorly understood. Traditional neurosurgical outcome measures are crude. Neuropsychological testing is increasingly important in outcome assessment. We investigated the health, disability, lifestyle and cognitive function in adults who had myelomeningocoele closure at birth.
Methods
Adult patients under routine follow-up were assessed in a joint neurosurgery/neuropsychology clinic. Patients completed lifestyle questionnaires, the hydrocephalus outcome questionnaire (HOQ) and underwent cognitive testing. Clinical variables including number of shunt revisions, shunt infection and surgical decompression of foramen magnum, which may influence outcome, were investigated.
Results
Twenty-one adults with a median age of 35 years were investigated. All had treated hydrocephalus, and eight had foramen magnum decompression for headache or progressive brainstem symptoms with stabilisation of symptoms in seven and improvement in one. Only eight patients were living independently, five were in paid employment and five work voluntarily. HOQ scores for cognitive function were lower (0.56 ± 0.20; mean ± standard deviation (SD)) than those for physical (0.64 ± 0.15) and social–emotional (0.65 ± 0.17) health. Cognitive function varied across the cohort with attention most severely affected (73.9 ± 17.0; mean ± SD). Repeated episodes of shunt malfunction or foramen magnum decompression were not associated with a worse cognitive function.
Conclusions
Despite intervention in childhood and adequate cerebrospinal fluid diversion the prognosis for independent living into adulthood remains poor. All patients have elements of cognitive impairment. Structural brain abnormalities may be more important determinants of cognitive outcome than shunt malfunction.
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References
Bower C, D'Antoine H, Stanley FJ (2009) Neural tube defects in Australia: trends in encephaloceles and other neural tube defects before and after promotion of folic acid supplementation and voluntary food fortification. Birth Defects Res A Clin Mol Teratol 85(4):269–273
Group MVSR (1991) Prevention of neural tube defects: results of the medical research council vitamin study. MRC Vitamin Study Research Group 338(8760):131–137, Lancet
Kondo A, Kamihira O, Ozawa H (2009) Neural tube defects: prevalence, etiology and prevention. Int J Urol 16(1):49–57
Hunt GM (1990) Open spina bifida: outcome for a complete cohort treated unselectively and followed into adulthood. Dev Med Child Neurol 32(2):108–118
Hunt GM, Oakeshott P (2003) Outcome in people with open spina bifida at age 35: prospective community based cohort study. Bmj 326(7403):1365–1366
Hunt GM, Oakeshott P (2004) Lifestyle in adults aged 35 years who were born with open spina bifida: prospective cohort study. Cerebrospinal Fluid Res 1(1):4
Pollack IF, Pang D, Albright AL, Krieger D (1992) Outcome following hindbrain decompression of symptomatic chiari malformations in children previously treated with myelomeningocele closure and shunts. J Neurosurg 77(6):881–888
Stevenson KL (2004) Chiari Type II malformation: past, present, and future. Neurosurg Focus 16(2):E5
Mirzai H, Ersahin Y, Mutluer S, Kayahan A (1998) Outcome of patients with meningomyelocele: the Ege University experience. Childs Nerv Syst 14(3):120–123
Soare PL, Raimondi AJ (1977) Intellectual and perceptual-motor characteristics of treated myelomeningocele children. Am J Dis Child 131(2):199–204
Barf HA, Verhoef M, Jennekens-Schinkel A, Post MW, Gooskens RH, Prevo AJ (2003) Cognitive status of young adults with spina bifida. Dev Med Child Neurol 45(12):813–820
Iddon JL, Morgan DJ, Loveday C, Sahakian BJ, Pickard JD (2004) Neuropsychological profile of young adults with spina bifida with or without hydrocephalus. J Neurol Neurosurg Psychiatry 75(8):1112–1118
Scott RB, Stoodley CJ, Anslow P, Paul C, Stein JF, Sugden EM, Mitchell CD (2001) Lateralized cognitive deficits in children following cerebellar lesions. Dev Med Child Neurol 43(10):685–691
Rapoport M, van Reekum R, Mayberg H (2000) The role of the cerebellum in cognition and behavior: a selective review. J Neuropsychiatry Clin Neurosci 12(2):193–198
Vachha B, Adams RC, Rollins NK (2006) Limbic tract anomalies in pediatric myelomeningocele and chiari II malformation: anatomic correlations with memory and learning–initial investigation. Radiology 240(1):194–202
Vinck A, Maassen B, Mullaart R, Rotteveel J (2006) Arnold-Chiari-II malformation and cognitive functioning in spina bifida. J Neurol Neurosurg Psychiatry 77(9):1083–1086
Barnes M, Dennis M, Hetherington R (2004) Reading and writing skills in young adults with spina bifida and hydrocephalus. J Int Neuropsychol Soc 10(5):655–663
Dennis M, Barnes M (2002) Math and numeracy in young adults with spina bifida and hydrocephalus. Dev Neuropsychol 21(2):141–155
Dennis M, Jewell D, Drake J, Misakyan T, Spiegler B, Hetherington R, Gentili F, Barnes M (2007) Prospective, declarative, and nondeclarative memory in young adults with spina bifida. J Int Neuropsychol Soc 13(2):312–323
Hetherington R, Dennis M, Barnes M, Drake J, Gentili F (2006) Functional outcome in young adults with spina bifida and hydrocephalus. Childs Nerv Syst 22(2):117–124
Kulkarni AV, Drake JM, Rabin D (2004) An instrument to measure the health status of children with hydrocephalus: the hydrocephalus outcome questionnaire. J Neurosurg: Pediatrics 101:134–140
Kulkarni AV, Drake JM, Rabin D, Dirks PB, Humphreys RP, Rutka JF (2004) Measuring the health status of children with hydrocephalus using a new outcome measure. J Neurosurg: Pediatrics 101:141–146
Jenkinson MD, Hayhurst C, Al-Jumaily M, Kandasamy J, Clark S, Mallucci CL (2009) The role of endoscopic third ventriculostomy in adult patients with hydrocephalus. J Neurosurg 110(5):861–866
Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA (2001) Spina bifida outcome: a 25-year prospective. Pediatr Neurosurg 34:114–120
Davis BE, Daley CM, Shurtleff DB, Duguay S, Seidel K, Loeser JD, Ellenbogan RG (2005) Long-term survival of individuals with myelomeningocele. Pediatr Neurosurg 41(4):186–191
Vandertop WP, Asai A, Hoffman HJ, Drake JM, Humphreys RP, Rutka JT, Becker LE (1992) Surgical decompression for symptomatic chiari II malformation in neonates with myelomeningocele. J Neurosurg 77(4):541–544
Caldarelli M, Di Rocco C, La Marca F (1996) Shunt complications in the first postoperative year in children with myelomeningocoele. Childs Nerv Syst 12(12):748–754
Chakraborty A, Crimmins D, Hayward R, Thompson D (2008) Toward reducing shunt placement rates in patients with myelomeningocele. J Neurosurg Pediatr 1(5):361–365
Thompson DN (2009) Postnatal management and outcome for neural tube defects including spina bifida and encephalocoeles. Prenat Diagn 29(4):412–419
O'Brien DF, Javadpour M, Collins DR, Spennato P, Mallucci CL (2005) Endoscopic third ventriculostomy: an outcome analysis of primary cases and procedures performed after ventriculoperitoneal shunt malfunction. J Neurosurg 103(5 Suppl):393–400
Fritsch MJ, Kienke S, Ankermann T, Padoin M, Mehdorn HM (2005) Endoscopic third ventriculostomy in infants. J Neurosurg 56(6):1271–1278
Hunt GM, Oakeshott P, Kerry S (1999) Link between the CSF shunt and acheivement in adults with spina bifida. J Neurol Neurosurg Psychiatry 67:591–595
Barf HA, Verhoef M, Post MW, Jennekens-Schinkel A, Gooskens RH, Mullaart RA, Prevo AJ (2004) Educational career and predictors of type of education in young adults with spina bifida. Int J Rehabil Res 27(1):45–52
Acknowledgements
We acknowledge Dr. G.M. Hunt and Dr. P. Oakeshott for providing their lifestyle questionnaire, and Dr. A.V. Kulkarni for supplying the hydrocephalus outcome questionnaire.
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Jenkinson, M.D., Campbell, S., Hayhurst, C. et al. Cognitive and functional outcome in spina bifida–Chiari II malformation. Childs Nerv Syst 27, 967–974 (2011). https://doi.org/10.1007/s00381-010-1368-7
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DOI: https://doi.org/10.1007/s00381-010-1368-7