Prevalence of the Chiari/hydrosyringomyelia complex in adults with spina bifida: preliminary results.
OBJECTIVE
To establish the prevalence of Chiari malformations and hydrocephalus (HS) in adults with spina bifida (SB).


BACKGROUND
Adults with SB have recently been shown to be at risk from significant deterioration arising from the C/HS complex. Little is known about the prevalence of these malformations in adulthood, their natural history, risk factors for deterioration and optimum management.


METHODS
Patients are recruited from a clinic for adults with SB and hydrocephalus, routinely questioned about recent changes in neurological symptoms and a full neurological examination performed. Functional assessments include: Barthel Index; Nottingham EADL; Nine Hole Peg Test; 10 metre timed walk test. Sagittal T1 and TSE (turbo spin-echo) magnetic resonance images (MRI) of the cranio-cervical junction and spinal cord are obtained using a 1.0-T MRI system with a phased-array surface coil. Scans are reported blindly.


RESULTS
76 patients have been assessed, 40 female, 36 male, mean age 27.5 years. Of these, 48 (63.2%) have symptoms +/- signs compatible with C/HS, but only 18 (23.7%) have reported new, potentially relevant, neurological symptoms over the previous 12 months. Of 25 patients undergoing MRI so far, 19 (76%) have had Chiari malformations and 12 (48%) have had HS. None of those with a negative scan have had signs/symptoms suggestive of the C/HS complex but a negative clinical evaluation has been unable to exclude such pathology.


CONCLUSIONS
These early results suggest that the prevalence of the C/HS complex is high in adults with SB and cannot be predicted by neurological examination alone. Given past experience, the need for continued neurological follow up in a large proportion of the SB population throughout adulthood is likely to be essential.
McDonnell
G V
GV
Clinic for Adults with Spina Bifida and Hydrocephalus, Musgrave Park Hospital, Belfast, Northern Ireland, UK.
McCann
J P
JP
Craig
J J
JJ
Crone
M
M
D000203:Activities of Daily Living; D000328:Adult; D001139:Arnold-Chiari Malformation / Q000150:complications / Q000453:epidemiology* / Q000473:pathology; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009660:Northern Ireland / Q000453:epidemiology; D015995:Prevalence; D016135:Spinal Dysraphism / Q000150:complications*; D013595:Syringomyelia / Q000150:complications / Q000453:epidemiology* / Q000473:pathology