Evolutionary hypothesis for Chiari type I malformation

Fernandes, Yvens Barbosa; Ramina, Ricardo; Campos-Herrera, Cynthia Resende; Borges, Guilherme

doi:10.1016/j.mehy.2013.07.035

Cited by 15 publications

(19 citation statements)

References 30 publications

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“…Platybasia has been suggested to predispose to the development of Chiari malformation by leading to overcrowding of the posterior fossa and consequently to tonsillar herniation through the foramen magnum. 13 A spontaneous recovery from platybasia, as seen in this study, can be explained by the naturally occurring steepening of the anterior cranial base angle with growth. 20 As further support, platybasia has been found to be less prevalent in older OI patients not treated with bisphosphonates (Table 3).…”

Section: Discussionsupporting

confidence: 60%

Cranial base pathology in pediatric osteogenesis imperfecta patients treated with bisphosphonates

Arponen

Vuorimies

Haukka

et al. 2015

PED

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OBJECT Cranial base pathology is a serious complication of osteogenesis imperfecta (OI). Our aim was to analyze whether bisphosphonate treatment, used to improve bone strength, could also prevent the development of craniocervical junction pathology (basilar impression, basilar invagination, or platybasia) in children with OI. METHODS In this single-center retrospective study the authors analyzed the skull base morphology from lateral skull radiographs and midsagittal MR images (total of 94 images), obtained between the ages of 0 and 25 years in 39 bisphosphonate-treated OI patients. The results were compared with age-matched normative values and with findings in 70 OI patients who were not treated with bisphosphonates. In addition to cross-sectional data, longitudinal data were available from 22 patients with an average follow-up period of 7.6 years. The patients, who had OI types I, III, IV, VI, and VII, had been treated with zoledronic acid, pamidronate, or risedronate for 3.2 years on average. RESULTS Altogether 33% of the 39 bisphosphonate-treated patients had at least 1 cranial base anomaly, platybasia being the most prevalent diagnosis (28%). Logistic regression analysis suggested a higher risk of basilar impression or invagination in patients with severe OI (OR 22.04) and/or older age at initiation of bisphosphonate treatment (OR 1.45), whereas a decreased risk was associated with longer duration of treatment (OR 0.28). No significant associations between age, height, or cumulative bisphosphonate dose and the risk for cranial base anomaly were detected. In longitudinal evaluation, Kaplan-Meier curves suggested delayed development of cranial base pathology in patients treated with bisphosphonates but the differences from the untreated group were not statistically significant. CONCLUSIONS These findings indicate that cranial base pathology may develop despite bisphosphonate treatment. Early initiation of bisphosphonate treatment may delay development of craniocervical junction pathology. Careful followup of cranial base morphology is warranted, particularly in patients with severe OI.

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Section: Discussionsupporting

confidence: 60%

Cranial base pathology in pediatric osteogenesis imperfecta patients treated with bisphosphonates

Arponen

Vuorimies

Haukka

et al. 2015

PED

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Section: Discussionmentioning

confidence: 99%

“…Recently, we proposed a new hypothesis to explain the origin of CI based on the evolution of the skull base of the Homo clade in the Pleistocene Epoch (beginning about 2,6 million years ago) 7 . It is well known by anthropological data that the basal angle has been constantly decreasing during hominins evolution to give extra volume to the remarkable brain size increase (at least threefold in the last 4 million years) 7 . In the most primitive hominins, such as in the Homo erectus, the basal angle was estimated to be much higher than, for instance, in Homo sapiens, suggesting its importance to the modern shape of the human skull base 7,10,11 .…”

Section: Discussionmentioning

confidence: 99%

“…It is well known by anthropological data that the basal angle has been constantly decreasing during hominins evolution to give extra volume to the remarkable brain size increase (at least threefold in the last 4 million years) 7 . In the most primitive hominins, such as in the Homo erectus, the basal angle was estimated to be much higher than, for instance, in Homo sapiens, suggesting its importance to the modern shape of the human skull base 7,10,11 . In patients with CI a higher incidence of platybasia and shortness of clivus has been described by some authors 2,12,13 .…”

Section: Discussionmentioning

confidence: 99%

“…The incidence of CI in a general population is estimate in about 0.1-0.5%, even though most of them are asymptomatic 5,6 . One of the potential reasons for CI is decreased posterior fossa, secondary to shortness of the clivus and platybasia, commonly found in patients with this anomaly 3,4,7 . However, the role of loss of posterior fossa volume (PFV) secondary to clivus shortness and platybasia were not clearly measured yet.…”

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confidence: 99%

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3-D simulation of posterior fossa reduction in Chiari I

Fernandes

Perestrelo

Noritomi

et al. 2016

Arq. Neuro-Psiquiatr.

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Chiari I (CI) is one of the most common congenital craniocervical disorders and its diagnosed when there is tonsillar herniation into the foramen magnum of up to 3-5 mm 1,2 . The impaction of the tonsils at the level of the foramen magnum can lead to interruption of the cerebrospinal fluid flow and clinical symptoms, with upper cervical spine compression 2,3,4 . The incidence of CI in a general population is estimate in about 0.1-0.5%, even though most of them are asymptomatic 5,6 . One of the potential reasons for CI is decreased posterior fossa, secondary to shortness of the clivus and platybasia, commonly found in patients with this anomaly 3,4,7 . However, the role of loss of posterior fossa volume (PFV) secondary to clivus shortness and platybasia were not clearly measured yet. By this reason, in the current manuscript, we described and proposed a 3D computational model to simulate changes in the PFV using different clivus and basal angles lengths. MethoDSA CT scan of a normal (without tonsillar herniation) control male adult subject was used as a model. The images were saved in a Digital Imaging and Communications in Medicine (DICOM) format and were treated using the AbStrACtWe proposed a 3D model to evaluate the role of platybasia and clivus length in the development of Chiari I (CI). Using a computer aided design software, two DICOM files of a normal CT scan and MR were used to simulate different clivus lengths (CL) and also different basal angles (BA). The final posterior fossa volume (PFV) was obtained for each variation and the percentage of the volumetric change was acquired with the same method. The initial normal values of CL and BA were 35.65 mm and 112.66º respectively, with a total PFV of 209 ml. Ranging the CL from 34.65 to 29. 65 -24.65 -19.65, there was a PFV decrease of 0.47% -1.12% -1.69%, respectively. Ranging the BA from 122.66º to 127.66º -142.66º, the PFV decreased 0.69% -3.23%, respectively. Our model highlights the importance of the basal angle and clivus length to the development of CI.Keywords: Chiari I; clivus length; platybasia; tonsillar herniation; 3D computational model. reSuMoNo presente estudo, propusemos a criação de um modelo computacional em 3D com elaboração de software onde dois arquivos em formato DICOM com uma TC e RNM de crânio foram usados para simular diferentes mensurações na extensão do clivus (EC) e no ângulo basal (AB). O volume final da fossa posterior (VFP) foi obtido em cada variação, bem como a percentagem de volume alterada. O tamanho inicial da EC era de 35,65 mm e o do AB era de 112.66º, com um VFP de 209 ml. Variando a EC de 34,65 para 29,65 -24.65 e 19.65, houve uma diminuição do VFP de 0.47%, 1.12% e 1.69%, respectivamente. Variando o AB de 122,66º para 127,66º e 142,66º, o VFP diminui para 0.69% e 3.23%, respectivamente. Nosso modelo enfatiza a importância da patogênese do aumento do AB e do encurtamento do clivus no desenvolvimento do Chiari I.Palaras-chave: Chiari I; extensão do clivus, platibasia; herniação tonsilar; modelo computacional em 3D.

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