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Pediatría (Asunción)

versão On-line ISSN 1683-9803

Resumo

MEDINA, J; SOSTOA, G; RECALDE, L  e  CANATA, M. Arnold Chiari type I malformation with bilateral papilledema. Pediatr. (Asunción) [online]. 2006, vol.33, n.1, pp. 48-51. ISSN 1683-9803.

Introduction: The Arnold Chiari malformation, or caudal cerebellar tonsilar ectopy, is a congenital defect of the posterior fossa, consisting in the descent of the cerebellar amygdalae 5mm below the foramen magnum. Clinically it presents as craneal hypertension headache, nystagmus, diplopia, VI craneal pair paralysis, tinnitus, dysphagia, weakness, paresthesia and spasticity. Case report: A 7 year old child wth no significant previous illness presented with 4 months of red eyes, a pressing, discontinous headache affecting the entire head, and occasional vomiting with no previous nausea, decreased visual acuity and blurriness. Ophthalmic exam showed bilateral papilledema and decreased visual acuity. Craneal CT was normal, but MRI showed a descent of both cerebellar amygdalae 16 mm below the foramen magnum. Discussion: The Arnold Chiari type I malformation with bilateral papilledema, without craneal hypertension or hydrocephalus is rarely described in the international literature. No medical treatment has been shown to be effective. The indications of surgical treatment are still the subject of debate. For most authors, treatment consists of surgical decompression of the posterior fossa, to release the subarachnoid spaces of the foramen magnum, the brainstem and the cisterna magna, to avoid the appearance of syringomyelia, hydrocephalus and other associated manifestations of the disease.

Palavras-chave : Arnold Chiari type I malformation; bilateral papilledema; children.

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