CASE REPORT
As malformações de Chiari são alterações no desenvolvimento do rombencéfalo e da fossa posterior, geralmente associadas a herniação troncular e cerebelar, patologias da medula espinal e hidrocefalia. Existem sete tipos de malformações descritas na literatura. Os tipos I e II são os mais comuns, enquanto o tipo III é uma forma rara associada a elevada morbilidade e mortalidade. O diagnóstico é feito por exames de imagem, e o tratamento preferencial é a cirurgia. O objetivo deste artigo é descrever o tratamento de um caso de um paciente de 6 meses de idade com malformação de Chiari tipo III associada a encefalocele occipital diagnosticada por imagem, detalhando passo a passo a técnica operatória de correção cirúrgica da encefalocele occipital e terceira ventriculostomia endoscópica para tratamento de hidrocefalia obstrutiva por estenose do aqueduto e pequena fossa posterior. O paciente foi acompanhado por dois anos, apresentando adequado desenvolvimento neuropsicomotor e sem complicações. O caso apresentado ratifica a importância dos avanços dos exames de imagem e das técnicas cirúrgicas no prognóstico dos pacientes com Chiari III, enfatizando que o diagnóstico precoce e o tratamento adequado reduzem a incidência de complicações clínicas e a mortalidade nos indivíduos acometidos por essa síndrome.
Chiari malformations are alterations in the development of the hindbrain and posterior fossa, typically associated with brainstem and cerebellar herniation, spinal cord pathologies, and hydrocephalus. Seven types of malformations are described in the literature. Types I and II are the most common, while type III is a rare form associated with high morbidity and mortality. Diagnosis involves imaging tests, and the preferred treatment is surgery. This article aims to present the management of a 6-month-old patient with Chiari type III malformation associated with occipital encephalocele, diagnosed by imaging, detailing step by step the operative technique for correcting the occipital encephalocele and performing endoscopic third ventriculostomy to treat obstructive hydrocephalus due to aqueductal stenosis and a small posterior fossa. The patient was followed up for two years, demonstrating appropriate neuropsychomotor development without complications. The case presented emphasizes the importance of advances in imaging tests and surgical techniques in the prognosis of patients with Chiari III, emphasizing that early diagnosis and appropriate treatment reduce the incidence of clinical complications and mortality in individuals affected by this syndrome.
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1Department of Neurosurgery, Beneficência Portuguesa Ribeirão Preto, Ribeirão Preto, SP, Brazil.
2Centro Universitário Barão de Mauá, Ribeirão Preto, SP, Brazil.
3Centro Universitário Unifacisa, Campina Grande, PB, Brazil.
4Department of Neurosurgery, Hospital São Francisco, Ribeirão Preto, SP, Brazil.
Received Nov 4, 2024
Corrected Dec 31, 2024
Accepted Jan 4, 2025
