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The authors report 17 cases of symptomatic Chiari type II malformation occurring in two distinct age dependent population. In Group I, 13 neonates and infants in the first year of life presented with cranial nerve and brain stem dysfunction characterized by vocal cord paralysis, apnea, dysphagia and laryngeal stridor.

In Group II, 4 patients developed signs and symptoms after the first year of life. In this group, the presentation was more insidious and included neck pain and cerebellar manifestations. The surgical treatment consisted initially in shunt implantation or revision and when there was no improvement, posterior fossa decompression was performed. The response to the surgical treatment differed considerably between the two groups: older patients improved promptly after surgery and there was no casualties; in newborn and infants, especially those under 6 months of age, the mortality rate was The authors stress that prompt diagnosis and surgical intervention should be performed in order to produce a favorable outcome.

Os pacientes foram divididos em Grupos I idade inferior a 12 meses e Grupo II idade superior a 12 meses. Do total, 11 eram do sexo feminino e 6 do sexo masculino. Nos previamente derivados, a primeira medida foi certificar-se que o sistema funcionava adequadamente. Em todos os 3 houve ventriculite precedendo o quadro. Uma paciente encontra-se clinica e neurologicamente estabilizada, apesar de traqueostomizada. Cochrane et al.

Vandertop et al. Dahl et al. Bell et al. Milerad et al. Gozal et al. Worley et al. Koehler PJ. J Neurosurg ; Carmel PW. Congenital syringomyelia. In Batzdorf U ed. Syringomyelia: current concepts in diagnosis and treatment.

Cameron AH. The Arnold-Chiari and other neuroanatomical malformations associated with spina bifida. J Pathol ; Central nervous system anomalies with associated meningocele hydrocephalus, and the Arnold-Chiari malformation: reappraisal of theories regarding the pathogenesis of posterior neural tube closure defects.

Neurosurgery ; Peach B. Arnold Chiari malformation: anatomic features of 20 cases. Arch Neurol ; Penfield W, Coburn DF. Arnold-Chiari malformation and its operative treatment. Arch Neurol Psychiat ; Management of Chiari II complications in infants with myelomeningocele. J Pediatr ; Rauzzino M, Oakes WJ. Chiari II malformation and syringomyelia. Neurosurg Clin N Am ; Surgical decompression for symptomatic Chiari II malformation in neonates with myelomeningocele.

Neurological dysfunction above cele level in children with spina bifida cystica: a prospective study to three years. Dev Med Child Neurol ; Hahn YS. Open myelomeningocele. McLone DG. Results of treatment of children born with a myelomeningocele. Clin Neurosurg ; Continuing concepts in management of spina bifida. Pediatr Neurosurg ; Multiple cranial nerve deficits associated with the Arnold-Chiari malformation.

Neurology ; The Chiari malformation associated with mielomeningocele. In Rekate HL ed. Comprehensive management of spina bifida. Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry ; Myelocele: rupture of the neural tube?

The dysraphic states: from syringomyelia to anencephaly. Amsterdam: Excerpta Medica, The cause of the Chiari malformation: a unified theory. Pediatr Neurosci ; Concepts Pediat Neurosurg ; Experience with surgical decompression of the Arnold-Chiari malformation in young infants with myelomeningocele. Venes JL. Multiple cranial nerve palsies in an infant with Arnold-Chiari malformation.

Epstein F. Meningomyelocele: "pitfalls" in early and late management. Bell WO,. Symptomatic Arnold-Chiari malformation: review of experience with 22 cases. The relationship of apnoea and stridor in spina bifida occulta to the unexplained infant's deaths. Dev Med Child Neurol ;16 Suppl Neurogenic dysphagia resulting from Chiari malformations. Apnea in patients with myelomeningocele. Management of Chiari malformations in childhood.

Manifestations and management of Arnold-Chiari malformation in patients with myelomeningocele. Child's Brain ; Haines SJ, Berger M. Anatomical progression of the Chiari II malformation. Child's Nerv Syst ; Preoperative evaluation and surgical management of the Arnold-Chiari II malformation. Obstructive sleep apnea in Arnold-Chiari malformation treated with acetazolamide.

Acta Paediatr ; Morley AR. Laryngeal stridor, Arnold Chiari malformation and medullary haemorrhages. Peripheral chemoreceptor function in children with myelomeningocele and Arnold-Chiari malformation type 2.

Chest ; Mori K Nishimura T. Electrophysiological studies on brainstem function in patients with myelomeningocele. Baeps in infants with myelomeningocele and later development of Chiari II malformation-related brainstem dysfunction.

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Revisión bibliográfica sobre la sintomatología cognitiva en la malformación de Chiari tipo I

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Arnold Chiari type I malformation with bilateral papilledema. ISSN 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.

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