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.