The Chiari/hydrosyringomyelia complex presenting in adults with myelomeningocoele: an indication for early intervention

Abstract

Objective: To determine how adults with myelomeningocoele who develop the Chiari/ hydrosyringomyelia complex present, and to determine if surgical intervention in¯uences outcome in these patients. Methods: A chart review of the 220 patients who attend a clinic for adults with spina bi®da and hydrocephalus (CASBAH), and follow-up of the ®ve cases with myelomeningocoele who had surgical intervention for associated symptomatic Chiari/hydrosyringomyelia complex. Results: Bilateral upper limb weakness and wasting were the commonest presenting symptoms (four patients). Sensory disturbance (three patients) was also common, dysphagia (one) and ataxia (one) occurring less often. The median time to surgical intervention was 36 months. Two patients had a shunting procedure performed in isolation, two foramen magnum decompression in addition to a shunting procedure and one a foramen magnum decompression. Surgical intervention did not completely reverse problems attributed to the Chiari/hydrosyringomyelia complex in any of the cases. One patient died post-operatively. Of the four who survived one had some improvement in function post-operatively, two remained static and one had further mild deterioration. Conclusion: All adults with myelomeningocoele should be questioned about changes in upper limb function for early detection of Chiari/hydrosyringomyelia complex. Our results suggest that early intervention is needed if further deterioration is to be avoided, and to improve the chances of neurological and functional recovery.

Medical problems in adolescents with myelomeningocele (MMC): an inventory of the Swedish MMC population born during 1986–1989

“…There is a risk of progressing neurological symptoms due to the tethered cord syndrome, the Chiari malformation and the development of hydromyelia. Continuous neurological assessments are therefore needed (6,7,10).…”

“…The motor impairment may become more obvious due to various factors, such as poor physical condition and overweight, and many who walked ≥50 metres as children cease to do so as adults (2). Neurological deterioration due to the tethered cord syndrome and Chiari malformation may also worsen the motor impairment (6,10). Shunt dysfunction is a constant threat and can cause acquired brain injury and blindness in addition to the existing impairments (2).…”

The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis

“…Such recurrences have been reported both in older children [86] and in adults [87] with spina bifida that was repaired at birth and neonatal hydrocephalus that had been successfully treated with a ventricular shunt. As a result of condition 3, the craniospinal pressure dissociation persists, allowing the theory to apply.…”

Myelomeningocele and Tethered Cord: Caring for an Aging Population

“…Craig et al reported 5 of 220 adult patients followed in an adult spina bifida clinic presented with classic symptoms of Chiari malformation. 13 In this series, patients presented with upper extremity paresthesiae, weakness, deformity, or skin ulcerations. Two patients underwent shunt revision, one underwent posterior fossa decompression, and two underwent decompression with shunt revision.…”