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Journal of Korean Neurosurgical Society Nov 2015In this study, we aimed to investigate the underlying ethiological factors in chiari malformation (CM) type-I (CMI) via performing volumetric and morphometric...
OBJECTIVE
In this study, we aimed to investigate the underlying ethiological factors in chiari malformation (CM) type-I (CMI) via performing volumetric and morphometric length-angle measurements.
METHODS
A total of 66 individuals [33 patients (20-65 years) with CMI and 33 control subjects] were included in this study. In sagittal MR images, tonsillar herniation length and concurrent anomalies were evaluated. Supratentorial, infratentorial, and total intracranial volumes were measured using Cavalieri method. Various cranial distances and angles were used to evaluate the platybasia and posterior cranial fossa (PCF) development.
RESULTS
Tonsillar herniation length was measured 9.09±3.39 mm below foramen magnum in CM group. Tonsillar herniation/concurrent syringomyelia, concavity/defect of clivus, herniation of bulbus and fourth ventricle, basilar invagination and craniovertebral junction abnormality rates were 30.3, 27, 18, 2, 3, and 3 percent, respectively. Absence of cisterna magna was encountered in 87.9% of the patients. Total, IT and ST volumes and distance between Chamberlain line and tip of dens axis, Klaus index, clivus length, distance between internal occipital protuberance and opisthion were significantly decreased in patient group. Also in patient group, it was found that Welcher basal angle/Boogard angle increased and tentorial slope angle decreased.
CONCLUSION
Mean cranial volume and length-angle measurement values significantly decreased and there was a congenital abnormality association in nearly 81.5 percent of the CM cases. As a result, it was concluded that CM ethiology can be attributed to multifactorial causes. Moreover, congenital defects can also give rise to this condition.
PubMed: 26713146
DOI: 10.3340/jkns.2015.58.5.454 -
Journal of Neurology, Neurosurgery, and... Feb 1949
Topics: Humans; Occipital Bone; Platybasia
PubMed: 18111230
DOI: 10.1136/jnnp.12.1.61 -
Surgical Neurology International 2019Craniometric studies document different subtypes of craniocervical junction malformations (CCJM). Here, we identified the different types and global signs and symptoms...
BACKGROUND
Craniometric studies document different subtypes of craniocervical junction malformations (CCJM). Here, we identified the different types and global signs and symptoms (SS) that correlated with these malformations while further evaluating the impact of syringomyelia.
METHODS
Prospective data concerning SS and types of CCJM were evaluated in 89 patients between September 2002 and April 2014 using Bindal's scale.
RESULTS
The mean Bindal's scores of each type of CCJM were Chiari malformation (CM) = 74.6, basilar invagination Type 1 (BI1) = 78.5, and BI Type 2 (BI2) = 78. Swallowing impairment and nystagmus were more frequently present in the BI patients. Symptomatic burdens were higher in patients with syringomyelia and included weakness, extremity numbness, neck pain, dissociated sensory loss, and atrophy.
CONCLUSION
There were no statistically significant differences in SS between the different CCJM types. BI patients had more swallowing and nystagmus complaints versus CM patients, but there were no significant differences in clinical SS between BI1 and BI2 patients. Notably, those with attendant syringomyelia had a higher SS burden.
PubMed: 31768286
DOI: 10.25259/SNI_469_2019 -
BDJ Open 2017Osteogenesis imperfecta type III (OMIM 259420) is a severe autosomal recessive disorder. Affected individuals have multiple fractures, develop limb deformities with...
OBJECTIVES
Osteogenesis imperfecta type III (OMIM 259420) is a severe autosomal recessive disorder. Affected individuals have multiple fractures, develop limb deformities with spinal malalignment and stunted stature.
MATERIALS AND METHODS
The frequency of Osteogenesis imperfecta type III (OI III) is relatively high in the indigenous Black African population of South Africa. A review of the literature revealed a paucity of information regarding the craniofacial manifestations of the disorder in this ethnic group. The findings in 64 affected persons are documented.
RESULTS
These abnormalities are related to the abnormal bone matrix which results in a deformed skull and dental malocclusion. The physiological process of swallowing may be an aetiological factor in the progressive development of a flattened palate. Mild changes in the shape of the head of the mandibular condyle and a lack of cortical bone on the joint surfaces were observed on cone beam computed tomography (CBCT) images. Affected persons had marked variations in the paranasal sinuses, including sinus hypoplasia and partial opacification. Cranial base anomalies were diagnosed from cephalometric radiographs and lateral skull radiographs. Platybasia and a 'J' shaped sella turcica were observed.
CONCLUSION
The craniofacial abnormalities emphasize the importance of a raised level of awareness in terms of dental management and the challenges.
PubMed: 29607091
DOI: 10.1038/bdjopen.2017.21 -
The Journal of Spinal Cord Medicine Nov 2022Spinal-generated movement disorders are a complex group of medical conditions, frequently misdiagnosed, originating in the spinal cord or from combined peripheral and...
CONTEXT
Spinal-generated movement disorders are a complex group of medical conditions, frequently misdiagnosed, originating in the spinal cord or from combined peripheral and central nervous system involvement. In this case report, we describe a novel form of position-dependent dyskinesia due to severe craniocervical malformation.
FINDINGS
An 83-year-old woman with basilar invagination at the C2 vertebra above the line of Chamberlain, occipitocervical lordosis, platybasia with a short clivus, ankylosis of the C1-C2 complex and fusion of the C1 arch developed an unusual pattern of position-dependent left arm dyskinesia triggered by bending her neck forward with simultaneous contact of the flexed elbow with a flat surface. Symptoms did not improve with anticonvulsants and she progressed and died suddenly.
CONCLUSION/CLINICAL RELEVANCE
A newly described form of position-dependent arm dyskinesia can be associated with severe craniocervical malformation.
Topics: Humans; Female; Aged, 80 and over; Arm; Spinal Fusion; Spinal Cord Injuries; Platybasia; Dyskinesias
PubMed: 33705252
DOI: 10.1080/10790268.2021.1878341 -
Neurosurgical Review Oct 2013The craniometric linear dimensions of the posterior fossa have been relatively well studied, but angular craniometry has been poorly studied and may reveal differences...
The craniometric linear dimensions of the posterior fossa have been relatively well studied, but angular craniometry has been poorly studied and may reveal differences in the several types of craniocervical junction malformation. The objectives of this study were to evaluate craniometric angles compared with normal subjects and elucidate the main angular differences among the types of craniocervical junction malformation and the correlation between craniocervical and cervical angles. Angular craniometries were studied using primary cranial angles (basal and Boogard's) and secondary craniocervical angles (clivus canal and cervical spine lordosis). Patients with basilar invagination had significantly wider basal angles, sharper clivus canal angles, larger Boogard's angles, and greater cervical lordosis than the Chiari malformation and control groups. The Chiari malformation group does not show significant differences when compared with normal controls. Platybasia occurred only in basilar invagination and is suggested to be more prevalent in type II than in type I. Platybasic patients have a more acute clivus canal angle and show greater cervical lordosis than non-platybasics. The Chiari group does not show significant differences when compared with the control, but the basilar invagination groups had craniometric variables significantly different from normal controls. Hyperlordosis observed in the basilar inavagination group was associated with craniocervical kyphosis conditioned by acute clivus canal angles.
Topics: Adult; Arnold-Chiari Malformation; Cephalometry; Cervical Vertebrae; Cranial Fossa, Posterior; Data Interpretation, Statistical; Female; Humans; Image Processing, Computer-Assisted; Lordosis; Magnetic Resonance Imaging; Male; Middle Aged; Platybasia; Radiography; Spine
PubMed: 23640096
DOI: 10.1007/s10143-013-0471-0 -
Revista de NeurologiaThe vascular compression of a nerve is known to be the most important cause of idiopathic trigeminal neuralgia (ITN) by most authors. However, several cases of skull...
INTRODUCTION
The vascular compression of a nerve is known to be the most important cause of idiopathic trigeminal neuralgia (ITN) by most authors. However, several cases of skull base deformities in patients with ITN have been reported in our bibliographic review. AIM. To compare the existence of platybasia between a group of patients with ITN and a randomized control group.
SUBJECTS AND METHODS
Twenty-five patients with ITN and twenty-five control subjects were X-rayed and their basal angles were measured and compared.
RESULTS
The basal angle measured to investigate the existence of platybasia is significantly greater in the ITN group than in the control group. Platybasia was found in 10 patients with ITN. However, only two cases of platybasia were reported in the control group. Therefore in our study we have found that the incidence of platybasia in patients with ITN is significantly increased.
CONCLUSION
Our data demonstrated that platybasia has an important role in the pathogenesis of the ITN by altering the normal anatomy of the nerves and vessels within the posterior fossa.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Platybasia; Radiography; Skull Base; Trigeminal Neuralgia
PubMed: 20013714
DOI: No ID Found -
The Angle Orthodontist Sep 2010To test the null hypothesis that there is no difference between craniofacial measurements of individuals with Down syndrome (DS) and normal controls. (Comparative Study)
Comparative Study
OBJECTIVE
To test the null hypothesis that there is no difference between craniofacial measurements of individuals with Down syndrome (DS) and normal controls.
MATERIALS AND METHODS
A cephalometric analysis including additional landmarks and measurements to study specific craniofacial features was undertaken on pretreatment cephalograms of 25 patients with DS (12 male, 13 female; mean age 15.1 years) treated at The Hospital for Sick Children, Toronto. Measurements were compared with those from cephalograms of racial groups age and gender matched, normal, Class I children, available from the Burlington Growth Center. Data were analyzed using paired t-tests.
RESULTS
Large reductions were measured in the size and spatial relationships of craniofacial structures in the DS group. The greatest differences included a larger cranial base angle; reduced elevation of sella from FHP; reduced anterior and posterior cranial base lengths; reduced anterior and posterior face heights; smaller maxilla with reduced anterior basal and apical dimensions; and smaller mandibular ramus, body and symphyseal dimensions and proclined symphysis. Maxillary incisors were severely proclined and undererupted, while mandibular incisors were undererupted. Alveolar heights were reduced. Anterior open bite was frequently noted. Maxillary and mandibular planes exhibited forward rotation patterns, promoting overclosure. Mandibular hypoplasia was less severe than cranial base and maxillary hypoplasia. Hypodontia of one or more permanent teeth was found in 92% of the sample.
CONCLUSIONS
The null hypothesis was rejected. Significant hypoplasia in endochondral, mesodermal, and ectomesenchymal derived structures of the cranium and face in subjects with DS was clearly evident. More severe platybasia than previously reported was found.
Topics: Adolescent; Alveolar Process; Anodontia; Case-Control Studies; Cephalometry; Child; Down Syndrome; Ethnicity; Female; Humans; Image Processing, Computer-Assisted; Incisor; Male; Mandible; Maxilla; Molar; Open Bite; Platybasia; Prognathism; Retrospective Studies; Rotation; Sella Turcica; Skull Base; Tooth Eruption
PubMed: 20578856
DOI: 10.2319/111709-650.1 -
Experimental and Therapeutic Medicine Dec 2017Chiari malformation type I (CM-I) is a common hindbrain disorder that is associated with deformity and elongation of the cerebellar tonsils. Although CM-I occurs in both...
Chiari malformation type I (CM-I) is a common hindbrain disorder that is associated with deformity and elongation of the cerebellar tonsils. Although CM-I occurs in both pediatric and adult patients, its prevalence, clinical features and management in the pediatric population are not well defined. The current study evaluated a consecutive case series of 92 children (38 females and 54 males) who were diagnosed with congenital CM-I. All patients underwent small-bone-window posterior fossa decompression with autologous-fascia duraplasty. Clinical and radiological features were analyzed and long-term follow-up data were recorded. Risk factors associated with clinical outcomes were investigated using comprehensive statistical methods. Out of the 92 children, 11 (12.0%) were asymptomatic. Associated ventricular dilation was observed in 24 children (26.1%) and concomitant syringomyelia was observed in 72 children (78.3%). A total of 44 children (47.8%) showed scoliosis on plain films. Follow-up data (mean duration, 88.6 months) were available for all patients. Syringomyelia was absent or markedly reduced in 56 patients (77.8%). Symptoms were alleviated in 66 patients, remained unchanged in 12 patients and progressed in 3 patients. Statistical analysis indicated that the cerebellar tonsillar descent (CTD) grade, basilar invagination and platybasia influenced the clinical outcome (P<0.05). In conclusion, early recognition and surgical treatment of CM-I in pediatric patients can lead to good outcomes. The current results suggested that small-bone-window posterior fossa decompression with autologous-fascia duraplasty was an effective safe treatment option with a low complication rate. High CTD grade, basilar invagination and platybasia were indicated to be predictors of poor clinical prognosis.
PubMed: 29285106
DOI: 10.3892/etm.2017.5211 -
World Neurosurgery: X Oct 2023Chiari malformation type 1 (CM-1) is characterized by cerebellar tonsil herniation through the foramen magnum and can be associated with additional craniovertebral...
BACKGROUND
Chiari malformation type 1 (CM-1) is characterized by cerebellar tonsil herniation through the foramen magnum and can be associated with additional craniovertebral junction anomalies (CVJA). The pathophysiology and treatment for CM-1 with CVJA (CM-CVJA) is debated.
OBJECTIVE
To evaluate the trends and outcomes of surgical interventions for patients with CM-CVJA.
METHODS
A systematic review of the literature was performed to obtain articles describing surgical interventions for patients with CM-CVJA. Articles included were case series describing surgical approach; reviews were excluded. Variables evaluated included patient characteristics, approach, and postoperative outcomes.
RESULTS
The initial query yielded 403 articles. Twelve articles, published between 1998-2020, met inclusion criteria. From these included articles, 449 patients underwent surgical interventions for CM-CVJA. The most common CVJAs included basilar invagination (BI) (338, 75.3%), atlantoaxial dislocation (68, 15.1%) odontoid process retroflexion (43, 9.6%), and medullary kink (36, 8.0%). Operations described included posterior fossa decompression (PFD), transoral (TO) decompression, and posterior arthrodesis with either occipitocervical fusion (OCF) or atlantoaxial fusion. Early studies described good results using combined ventral and posterior decompression. More recent articles described positive outcomes with PFD or posterior arthrodesis in combination or alone. Treatment failure was described in patients with PFD alone that later required posterior arthrodesis. Additionally, reports of treatment success with posterior arthrodesis without PFD was seen.
CONCLUSION
Patients with CM-CVJA appear to benefit from posterior arthrodesis with or without decompressive procedures. Further definition of the pathophysiology of craniocervical anomalies is warranted to identify patient selection criteria and ideal level of fixation.
PubMed: 37456684
DOI: 10.1016/j.wnsx.2023.100221