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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2020; 39(02): 136-141
DOI: 10.1055/s-0040-1708893
Case Report | Relato de Caso
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

Hemifacial Spasm Associated with Chiari Type I Malformation: Surgical Considerations and Case Report

Espasmo Hemifacial Associado a Malformação de Chiari Tipo I: Considerações Cirúrgicas e Relato de Caso
Carlos Augusto Ferreira Lobão 
1   Division of Neurosurgery, Department of Neuroscience, Hospital Porto Dias, Belém, PA, Brazil
,
Ulysses de Oliveira Sousa 
2   Department of Neurosurgery, Hospital Saúde da Mulher, Belém, PA, Brazil
,
Diego Arthur Castro Cabral 
3   Institute of Health Sciences, Faculdade de Medicina, Universidade Federal do Pará, Belém, PA, Brazil
,
Fernanda Myllena Sousa Campos 
3   Institute of Health Sciences, Faculdade de Medicina, Universidade Federal do Pará, Belém, PA, Brazil
› Author Affiliations
Further Information

Publication History

14 June 2019

15 January 2020

Publication Date:
24 April 2020 (online)

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Abstract

Hemifacial spasm (HS) is a movement disorder characterized by paroxysmal and irregular contractions of the muscles innervated by the facial nerve. Chiari malformation type I (CM I) is a congenital disease characterized by caudal migration of the cerebellar tonsils, and surgical decompression of foramen magnum structures has been used for treatment. The association of HS with CM I is rare, and its pathophysiology and therapeutics are speculative. There are only a few cases reported in the literature concerning this association. The decompression of the posterior fossa for the treatment of CM I has been reported to relieve the symptoms of HS, suggesting a relation between these diseases. However, the possible complications of posterior fossa surgery cannot be underrated. We report the case of a 66-year-old patient, in ambulatory follow-up due to right HS, no longer responding to botulinum toxin treatment. Magnetic resonance imaging (MRI) of the skull revealed compression of the facial nerve and CM I. The patient underwent surgery for HS by neurovascular microdecompression of the facial nerve via right lateral suboccipital craniectomy, but presented significant clinical worsening in the postoperative period even though the cerebellum edema related to surgical manipulation was mild. Due to the clinical worsening, the patient underwent a median suboccipital craniectomy with decompression of the foramen magnum structures. After this second surgery, the patient had progressive improvement and was discharged from the hospital for ambulatory care.

Resumo

O espasmo hemifacial é um distúrbio do movimento caracterizado por contrações paroxísticas e irregulares de músculos inervados pelo nervo facial. A malformação de Chiari tipo I é uma doença congênita caracterizada pela migração caudal das tonsilas cerebelares. Com poucos casos relatados na literatura, a ocorrência de espasmo hemifacial com malformação de Chiari tipo I é rara. É observado que a descompressão da fossa posterior para tratamento da malformação de Chiari tipo I também leva ao alívio dos sintomas do espasmo hemifacial, o que pode sugerir uma relação entre a fisiopatologia dessas. Relatamos o caso de uma paciente de 66 anos, em acompanhamento ambulatorial por espasmo hemifacial direito evoluindo com resposta inadequada ao tratamento clínico. Ressonância magnética do crânio com compressão vascular do nervo facial e mal-formação de Chiari tipo I. Submetida a micro-descompressão neurovascular para tratamento do espasmo hemifacial, evoluiu com piora clínica causada por descompensação do Chiari tipo I. A paciente foi submetida, então, à nova cirurgia para descompressão do forame magno, apresentado, então, melhora lenta e progressiva.

Keywords

hemifacial spasm - chiari malformation type I - decompression of the posterior fossa

Palavras-chave

espasmo hemifacial - malformação de Chiari tipo I - descompressão da fossa posterior
 

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