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. 2018 Apr 1;10(4):e378-e382.
doi: 10.4317/jced.54419. eCollection 2018 Apr.

Orofacial clinical features in Arnold Chiari type I malformation: A case series

José-Alcides de Arruda  1 , Eugênia Figueiredo  2 , João-Luiz Monteiro  3 , Livia-Mirelle Barbosa  4 , Cleomar Rodrigues  5 , Belmiro Vasconcelos  2   3
Affiliations

Orofacial clinical features in Arnold Chiari type I malformation: A case series

José-Alcides de Arruda et al. J Clin Exp Dent. .

Abstract

Background: 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. 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 methods: A 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.

Results: Mean 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.

Conclusions: ACM-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.

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Conflict of interest statement

Conflict of interest statement: All authors declare no conflict of interests.

Figures

Figure 1
Figure 1
Sagittal magnetic resonance reconstructions (A and B) demonstrate part of the cerebellum entering the vertebral canal (red arrow) and positioning itself posterior to the spinal cord. Cerebrospinal fluid (green arrow).
Figure 2
Figure 2
ACM-I male patient. A. The computed tomography (CT) scan demonstrates the cerebellum herniation (red arrow). B. Post-operative CT scan of the same patient. Stabilization of the cervical vertebrae was also required (arthrodesis).
Figure 3
Figure 3
Sagittal magnetic resonance reconstructions of an ACM-I female patient demonstrating the cerebellum herniation into the vertebral canal (red arrow).
See this image and copyright information in PMC

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