Symptomatic Arnold-Chiari malformation: review of experience with 22 cases

Abstract

Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. Of the three infants not undergoing decompression, two died and one eventually became asymptomatic. All five of the older patients underwent decompression and all had complete resolution of their preoperative symptoms and signs. The authors conclude that while decompression of a symptomatic Arnold-Chiari malformation may be effective in children and adults, this treatment does not always improve the clinical condition of infants. This may be the result of ischemic/hypoxic effects on the infant's brain stem, which may not be organized normally at birth.

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

“…In contrast to paediatric populations where the presentation often suggests lower cranial nerve dysfunction, 5,6,9 resulting in such symptoms as stridor and diculty swallowing, our patients presented in a similar way to adults without myelomeningocoele who develop symptoms from the Chiari/hydrosyringomyelia complex. 12 ± 15 All had upper limb weakness, with or without sensory symptoms and re¯ex changes, consistent with a central cord syndrome, with fewer having evidence of foramen magnum compression.…”

“…Despite claims that the Chiari malformation with or without associated hydrosyringomyelia always occurs with myelomeningocoele, 6,9,10 it is becoming apparent with MR imaging that this is not the case.…”

“…5 ± 7,9 It might be explained by the long median time (36 months) between symptom onset and surgical intervention in our cases, as paediatric studies have suggested that early intervention results in better outcome. 9 In conclusion all adults with myelomeningocoele should be asked about their upper limb function at review, and if they report changes in it, investigated as a matter of urgency by MRI with a view to early surgical intervention. For patients with myelomeningocoele struggling to maintain their independence, loss of function from an associated Chiari/hydrosyringomyelia complex is a situation that once recognised should be treated with all due speed to avoid further deterioration and to improve chances of neurological and functional recovery.…”

“…5 ± 7 Information on how adults with myelomeningocoele who develop the Chiari/hydrosyringomyelia complex present, and how they respond to treatment is in contrast scant, 5,8 although it is reported, that the outlook in these patients is consistently good with intervention. 9 The aim of this paper is to review our experience of adults with myelomeningocoele, who attend CASBAH, who presented with symptomatic Chiari/hydrosyringomyelia complex for which they had surgical intervention.…”

Valoración de la capacidad laboral de la malformación de Arnold Chiari tipo I

“…Entre los síntomas secundarios a compresión afectación cerebelosa tenemos la -ataxia, nistagmus vertical hacia abajo, temblor, alteración de la coordinación 5 . La posturografía dinámica permite evaluar objetivamente los trastornos del equilibrio.…”