Abstract
BackgroundArnold Chiari malformation (ACM) is characterized by an anatomical defect at the base of the skull where the cerebellum and the spinal cord herniate through the foramen magnum into the cervical spinal canal. Among the subtypes of the condition, ACM type I (ACM-I) is particularly outstanding because of the severity of symptoms. This study aimed to analyze the orofacial clinical manifestations of patients with ACM-I, and discuss their demographic distribution and clinical features in light of the literature.Material and MethodsA case series with patients with ACM-I treated between 2012 and 2015 was described. The sample consisted of patients who were referred by the Department of Neurosurgery to the Oral and Maxillofacial Surgery Service of Hospital da Restauração in Brazil for the assessment of facial symptomatology. A questionnaire was applied to evaluate the presence of painful orofacial findings. Data are reported using descriptive statistical methods.ResultsMean patient age was 39.3 years and the sample consisted mostly of male patients. A high prevalence of headache (50%) and pain in the neck (66.7%) and masticatory muscles (50%) was found. Only one patient reported difficulty in performing mandibular movements and two reported jaw clicking sounds. Mean mouth opening was 40.83 mm.ConclusionsACM-I patients may exhibit orofacial symptoms which may mimic temporomandibular joint disorders. This study brings interesting information that could help clinicians and oral and maxillofacial surgeons to understand this uncommon condition and also help with the diagnosis of patients with similar physical characteristics by referring them to a neurosurgeon. Key words:Arnold-Chiari malformation, facial pain, diagnosis, orofacial.
Highlights
Arnold Chiari malformation (ACM) was first described by Cleland in 1883 and later described in detail by the same author in 1891 [1]
ACM belongs to a group of congenital conditions characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal [2,3]
The aim of the present study was to analyze the orofacial clinical findings of a case series of ACM type I (ACM-I) patients treated at a referral Hospital in the Brazilian Northeast
Summary
Arnold Chiari malformation (ACM) was first described by Cleland in 1883 and later described in detail by the same author in 1891 [1]. ACM belongs to a group of congenital conditions characterized by an anatomic defect of the base of the skull, in which the cerebellum and brain stem herniate through the foramen magnum into the cervical spinal canal [2,3]. The usual treatment is expansion of the foramen magnum by decompression of the posterior cranial fossa in combination or not with other surgical techniques [8] Within this context, the aim of the present study was to analyze the orofacial clinical findings of a case series of ACM-I patients treated at a referral Hospital in the Brazilian Northeast. Arnold Chiari malformation (ACM) is characterized by an anatomical defect at the base of the skull where the cerebellum and the spinal cord herniate through the foramen magnum into the cervical spinal canal. This study brings interesting information that could help clinicians and oral and maxillofacial surgeons to understand this uncommon condition and help with the diagnosis of patients with similar physical characteristics by referring them to a neurosurgeon
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