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Aintzane Urbizu, Alex Ferré, Maria-Antonia Poca, Alex Rovira, Juan Sahuquillo, Bryn A. Martin and Alfons Macaya

OBJECTIVE

Traditionally, Chiari malformation Type I has been related to downward herniation of the cerebellar tonsils as a consequence of an underdeveloped posterior cranial fossa. Although the common symptoms of Chiari malformation Type I are occipital headaches, cervical pain, dizziness, paresthesia, and sensory loss, patients often report symptoms related to pharyngeal dysfunction such as choking, regurgitation, dysphagia, aspiration, chronic cough, and sleep disorders. In addition, tracheal intubation is often difficult in these patients. The purpose of this study was to analyze the morphological features of the oropharynx and oral cavity in patients with Chiari malformation Type I to help identify underlying anatomical anomalies leading to these debilitating symptoms.

METHODS

Seventy-six adult patients with symptomatic Chiari malformation Type I with cerebellar tonsillar descent greater than 5 mm below the foramen magnum and a small posterior cranial fossa and 49 sex-matched controls were selected to perform a retrospective case-control MRI-based morphometric study in a tertiary hospital. Eleven linear and areal parameters of the oropharyngeal cavity on midsagittal T1-weighted MRI were measured and the average values between patients and control cohorts were compared. Correlations between variables showing or approaching statistical significance in these structures and posterior cranial fossa measurements related with the occipital bone were sought.

RESULTS

Significant differences were detected for several oropharynx and oral cavity measures in the patient cohort, primarily involving the length and thickness of the soft palate (p = 9.5E-05 and p = 3.0E-03, respectively). A statistically significant (p < 0.01) moderate correlation between some of these variables and posterior cranial fossa parameters was observed.

CONCLUSIONS

The existence of structural oropharyngeal and oral cavity anomalies in patients with Chiari malformation Type I was confirmed, which may contribute to the frequent occurrence of respiratory and deglutitory complications and sleep disorders in this syndrome.

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Aintzane Urbizu, Bryn A. Martin, Dulce Moncho, Alex Rovira, Maria A. Poca, Juan Sahuquillo, Alfons Macaya and Malena I. Español

OBJECTIVE

The current diagnostic criterion for Chiari malformation Type I (CM-I), based on tonsillar herniation (TH), includes a diversity of patients with amygdalar descent that may be caused by a variety of factors. In contrast, patients presenting with an overcrowded posterior cranial fossa, a key characteristic of the disease, may remain misdiagnosed if they have little or no TH. The objective of the present study was to use machine-learning classification methods to identify morphometric measures that help discern patients with classic CM-I to improve diagnosis and treatment and provide insight into the etiology of the disease.

METHODS

Fifteen morphometric measurements of the posterior cranial fossa were performed on midsagittal T1-weighted MR images obtained in 195 adult patients diagnosed with CM. Seven different machine-learning classification methods were applied to images from 117 patients with classic CM-I and 50 controls matched by age and sex to identify the best classifiers discriminating the 2 cohorts with the minimum number of parameters. These classifiers were then tested using independent CM cohorts representing different entities of the disease.

RESULTS

Machine learning identified combinations of 2 and 3 morphometric measurements that were able to discern not only classic CM-I (with more than 5 mm TH) but also other entities such as classic CM-I with moderate TH and CM Type 1.5 (CM-1.5), with high accuracy (> 87%) and independent of the TH criterion. In contrast, lower accuracy was obtained in patients with CM Type 0. The distances from the lower aspect of the corpus callosum, pons, and fastigium to the foramen magnum and the basal and Wackenheim angles were identified as the most relevant morphometric traits to differentiate these patients. The stronger significance (p < 0.01) of the correlations with the clivus length, compared with the supraoccipital length, suggests that these 5 relevant traits would be affected more by the relative position of the basion than the opisthion.

CONCLUSIONS

Tonsillar herniation as a unique criterion is insufficient for radiographic diagnosis of CM-I, which can be improved by considering the basion position. The position of the basion was altered in different entities of CM, including classic CM-I, classic CM-I with moderate TH, and CM-1.5. The authors propose a predictive model based on 3 parameters, all related to the basion location, to discern classic CM-I with 90% accuracy and suggest considering the anterior alterations in the evaluation of surgical procedures and outcomes.