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World Neurosurgery May 2022A 32-year-old woman presented with chronically progressive spastic quadriparesis. Detailed clinicoradiological evaluation led to the diagnosis of irreducible...
A 32-year-old woman presented with chronically progressive spastic quadriparesis. Detailed clinicoradiological evaluation led to the diagnosis of irreducible atlantoaxial dislocation and basilar invagination, with associated "kissing" carotids and an anomalous right vertebral artery (VA). Both internal carotid arteries had an abnormally tortuous course, "kissing" retropharyngeally at the level of C1-C2. The right VA became intradural at the level of C2-C3, an extremely rare anomaly-C3 segmental artery. Despite the deformed joints and the possibility of injuring the anomalous right VA during C2-C3 instrumentation, a tailored posterior-only approach was used to circumvent the arterial fence created by both vascular anomalies. The patient underwent bilateral C1-C2 joint opening and left C1-C2 joint spacer placement, followed by bilateral occipito-C3-C4 fixation. This is possibly the first reported case of a complex craniovertebral junction anomaly associated with both kissing carotids and a C3 segmental VA.
Topics: Adult; Atlanto-Axial Joint; Brachiocephalic Trunk; Carotid Artery, Internal; Female; Humans; Joint Dislocations; Platybasia; Spinal Fusion; Vascular Malformations; Vertebral Artery
PubMed: 35248774
DOI: 10.1016/j.wneu.2022.02.111 -
World Neurosurgery Jun 2022C1/2 facet configurations and clivus-canal-angles (CXAs) have been proposed as criteria for posterior fusion in Chiari I malformation (CMI).
OBJECTIVE
C1/2 facet configurations and clivus-canal-angles (CXAs) have been proposed as criteria for posterior fusion in Chiari I malformation (CMI).
METHODS
Three-hundred and forty adults with CMI without basilar invagination (BI), 111 with CMI with BI, and 100 age- and sex-matched controls were studied using sagittal T2-weighted magnetic resonance imaging scans analyzing preoperative and postoperative values with their impact on progression-free survival rates.
RESULTS
For CMI without BI, C1/2 facet configurations and CXA were similar to controls (142 ± 11 degrees and 144 ± 10 degrees, respectively) with low rates for posterior C1 displacements (7.1% and 10%, respectively). In CMI with BI, C1 facet displacements were common (54.9%) with lower CXA (120 ± 15 degrees). After foramen magnum decompression (FMD) in CMI without BI (n = 169), 1.8% developed posterior C1 facet displacements without CXA changes and a 97% progression-free survival rate for 10 years. In CMI with BI, patients without ventral compression or instability underwent FMD without fusion (n = 19). Among them, 5.3% developed a posterior C1 facet displacement without CXA changes and a 94% progression-free survival rate for 10 years. The remainder of CMI with BI underwent FMD with C1/2 fusion (n = 48). Among these, CXA values increased with 10-year progression-free survival rates of 74% and 93% with and without ventral compression, respectively.
CONCLUSIONS
For adult CMI without BI, C1/2 facet configurations and CXA are irrelevant. FMD alone provides excellent long-term outcomes. In CMI with BI, anterior C1 facet displacements indicate C1/2 instability. Posterior fusions can be reserved for patients with ventral compression or C1/2 instability.
Topics: Adult; Arnold-Chiari Malformation; Cranial Fossa, Posterior; Decompression, Surgical; Foramen Magnum; Humans; Magnetic Resonance Imaging; Platybasia
PubMed: 35247617
DOI: 10.1016/j.wneu.2022.02.110 -
Journal of Neurological Surgery. Part... Jul 2023There is evidence that Chiari malformation (CM) and basilar invagination (BI) are largely due to disproportion between the content and volume of the posterior fossa....
BACKGROUND
There is evidence that Chiari malformation (CM) and basilar invagination (BI) are largely due to disproportion between the content and volume of the posterior fossa. A recent study identified an increased association between brachycephaly and BI. In several types of craniosynostosis, the posterior fossa volume is smaller than normal, and this is more pronounced in coronal synostosis. The aim of this study is to evaluate the association between CM and BI.
METHODS
The cephalic index (CI) measured on magnetic resonance imaging (MRI) from a sample of patients with craniocervical malformation was compared with that of normal subjects.
RESULTS
The average CI in the craniovertebral junction malformation (CVJM) group was significantly higher in BI patients than in normal subjects. The BI patients also had the highest CI among the whole sample of patients ( = 0.009).
CONCLUSIONS
In this study, BI patients had the highest CI among patients with CVJM and a significantly higher CI than those in the control group. Our data confirm the association between BI and brachycephaly.
Topics: Humans; Platybasia; Arnold-Chiari Malformation; Magnetic Resonance Imaging; Craniosynostoses
PubMed: 34929749
DOI: 10.1055/s-0041-1739503 -
Journal of Neurosurgery. Pediatrics Mar 2022The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation...
OBJECTIVE
The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM).
METHODS
The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes.
RESULTS
A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively).
CONCLUSIONS
Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.
PubMed: 34861643
DOI: 10.3171/2021.9.PEDS2185 -
Neuroimaging Clinics of North America Nov 2021The skull base is a critical structure in the craniofacial region, supporting the brain and vital facial structures in addition to serving as a passageway for important... (Review)
Review
The skull base is a critical structure in the craniofacial region, supporting the brain and vital facial structures in addition to serving as a passageway for important structures entering and exiting the cranial cavity. This paper will review and highlight some of the embryology, developmental anatomy, including ossification, and related abnormalities of the anterior, central and posterior skull base using illustrative cases and tables. Pathologies such as dermoids/epidermoids, cephaloceles, nasal gliomas, glioneuronal heterotopias, various notochordal remnants, persistent craniopharyngeal canal, teratomas, platybasia, basilar invagination, clival anomalies and Chiari malformations will be discussed. Developmental pearls and pitfalls will also be highlighted.
Topics: Arnold-Chiari Malformation; Diagnostic Imaging; Humans; Platybasia; Skull; Skull Base
PubMed: 34689936
DOI: 10.1016/j.nic.2021.06.004 -
Bone Jan 2022In the context of a lack of national consensus on the benefits of skull base imaging in children with osteogenesis imperfecta (OI), this study aims to analyse and...
OBJECTIVES
In the context of a lack of national consensus on the benefits of skull base imaging in children with osteogenesis imperfecta (OI), this study aims to analyse and correlate the clinical symptoms and radiological images of children with severe OI.
METHODS
A retrospective case notes and image analysis was carried out on children with complex OI between 2012 and 2018 at a specialist tertiary centre. Data were collected on patient demographic factors, clinical data, imaging findings (presence of Wormian bones, platybasia, basilar impression (McGregor's technique) and basilar invagination (McRae's technique)), and clinical features at the time of imaging.
RESULTS
Of the 127 patients in the OI database, 94 were included. A total of 321 radiographs, 21 CT scans and 39 MRI scans were analysed. Average frequency of radiographs was 8 per 10 years. Of the 94 patients, 58 (62%), 10 (11%), 1 (1%) demonstrated platybasia, basilar impression, and basilar invagination, respectively. Of the radiographs analysed, platybasia, basilar impression, basilar invagination, and the presence of Wormian bones, could not be evaluated in 71 (22.3%), 48 (15.2%), 61 (19.5%) and 28 (9.4%) radiographs respectively (due to poor positioning, anatomical abnormalities, and poor image quality). Of the 140 radiographs with platybasia, 17 (12%) also demonstrated basilar impression compared to only 3 (2.9%) out of the 99 without platybasia (p = 0.03). No significant associations were seen between the presence of Wormian bones and basilar impression. Of the 39 MRIs, additional information on CSF flow rate, spinal cord signal and cerebellar morphology was reported in 14 (36%). There was a lack of concordance between MRI and matched radiographs in 7.1% (1/14) and 36% (5/14) for platybasia and basilar impression respectively, with full concordance for basilar invagination. Fewer than 5% had positive clinical symptoms/signs at the time of imaging; 2% (7/321) had macrocephaly, 0.6% (2/321) headache, all other neurological features were absent). Clinical features were not documented in >85% of patients.
CONCLUSION
The apparent low prevalence of clinical symptoms and signs and of radiologically identified cranio-cervical abnormalities, suggests that current levels of serial imaging may be excessive. Until larger prospective studies clarify these issues, we suggest a clinical pathway for base of skull imaging which proposes a risk stratification approach to radiographic frequency and suggests parameters for proceeding to MRI.
Topics: Child; Critical Pathways; Humans; Osteogenesis Imperfecta; Prospective Studies; Retrospective Studies; Skull Base
PubMed: 34688943
DOI: 10.1016/j.bone.2021.116235 -
Operative Neurosurgery (Hagerstown, Md.) Nov 2021Odontoidectomy is a challenging yet effective operation for decompression of non-neoplastic craniovertebral junction disease. Though both the endoscopic endonasal... (Comparative Study)
Comparative Study
BACKGROUND
Odontoidectomy is a challenging yet effective operation for decompression of non-neoplastic craniovertebral junction disease. Though both the endoscopic endonasal approach (EEA) and the transoral approach (TOA) have been discussed in the literature, there remain few direct comparisons between the techniques.
OBJECTIVE
To evaluate the perioperative outcomes of EEA vs TOA odontoidectomy.
METHODS
A retrospective review of all cases undergoing odontoidectomy by either the EEA or TOA was performed. Attention was paid to the need for prolonged nutritional support, prolonged respiratory support, and hospitalization times.
RESULTS
During the study period between 2000 and 2018, 25 patients underwent odontoid process resection (18 TOA and 7 EEA). The most common indication for surgery was basilar invagination. Hospital length of stay, intensive care unit length of stay, and intubation days were all significantly shorter in the EEA group compared to the TOA group (P < .01, P = .01, P < .01, respectively). Prolonged nutritional support in the form of a gastrostomy tube was required in 5 patients and tracheostomy was required in 4 patients; all of these underwent odontoidectomy by the TOA. There was no statistical difference in neurological outcomes between the EEA and TOA groups (P = .17).
CONCLUSION
Odontoidectomy can be performed safely through both the EEA and TOA. The results of this study suggest the EEA has shorter hospitalizations and a lower probability of requiring prolonged nutritional support. These advantages are likely the results of decreased oropharyngeal mucosa disruption as compared to the TOA.
Topics: Decompression, Surgical; Endoscopy; Humans; Mouth; Nose; Odontoid Process; Platybasia; Retrospective Studies
PubMed: 34460927
DOI: 10.1093/ons/opab303 -
Operative Neurosurgery (Hagerstown, Md.) Nov 2020
In Reply: Three-Dimensional Evaluation and Classification of the Anatomy Variations of Vertebral Artery at the Craniovertebral Junction in 120 Patients of Basilar Invagination and Atlas Occipitalization.
Topics: Cervical Atlas; Humans; Platybasia; Vertebral Artery
PubMed: 34383934
DOI: 10.1093/ons/opaa283 -
Osteoporosis International : a Journal... Jan 2022Craniocervical abnormalities in osteogenesis imperfecta (OI) such as basilar invagination or cervical kyphosis can cause severe neurological morbidity. These...
UNLABELLED
Craniocervical abnormalities in osteogenesis imperfecta (OI) such as basilar invagination or cervical kyphosis can cause severe neurological morbidity. These abnormalities may be more frequent in OI type V compared with other OI subtypes of similar disease severity, underlining the importance of screening in this group.
INTRODUCTION
Craniocervical abnormalities in osteogenesis imperfecta (OI) can cause severe neurological morbidity. Although radiological cranial base abnormalities in OI have been well described in the literature, there are limited data on these abnormalities in OI type V and their association with clinical sequelae.
METHODS
A retrospective case series on patients with craniocervical abnormalities in OI type V at our institution.
RESULTS
Craniocervical abnormalities were present in 7 of 37 patients with OI type V (19%). For 5 patients (age at last follow-up: 5 to 26 years; 2 females), sufficient information was available for inclusion in the case series. All had genetically confirmed OI type V. Age range at diagnosis of the craniocervical abnormality was 1 day to 18 years. Basilar invagination was present in 3 patients; 2 had cervical kyphosis. Dysplasia of upper cervical vertebrae or base of skull was seen in 3 patients. The severity of the craniocervical abnormality did not clearly correlate with the severity of the OI phenotype. Three patients required surgical intervention (ages 7, 11, and 26 years) due to compression of the spinal cord or brainstem. Craniocervical abnormalities were detected incidentally or on screening in 3 patients, and only 2 had significant positive findings on neurological examination.
CONCLUSION
A variety of craniocervical abnormalities are seen in OI type V including dysplasia of the cervical vertebrae. These cases highlight the importance of screening patients with OI type V with lateral skull and cervical spine x-rays throughout childhood and after skeletal maturity.
Topics: Cervical Vertebrae; Child; Female; Humans; Infant; Kyphosis; Osteogenesis Imperfecta; Platybasia; Retrospective Studies
PubMed: 34350492
DOI: 10.1007/s00198-021-06088-x -
British Journal of Neurosurgery Dec 2023Sleep apnoea is common in patients with Basilar Invagination with Arnorld Chiari Malformation (ACM). Various studies have shown its incidence in the range of 60-70%... (Review)
Review
Sleep apnoea is common in patients with Basilar Invagination with Arnorld Chiari Malformation (ACM). Various studies have shown its incidence in the range of 60-70% among such patients. Most of the studies have shown improvement in sleep disturbances after decompressive surgeries for Chiari Malformations. There is no report of postoperative deterioration due to sleep apnoea in these patients. Authors report two cases of basilar invagination associated with ACM and Platybasia, who deteriorated probably due to worsening of pre-existing sleep disorders on 3rd and 7th postoperative days after their surgeries, despite clinico-radiological improvements during their early post-operative courses. Authors discuss literature related to sleep apnoea in basilar invagination associated with Chiari Malformations and share precautions, which are relevant and should be undertaken in such patients especially during early post-operative periods to avoid alarming complication which may occur even in experienced hands.
Topics: Humans; Platybasia; Arnold-Chiari Malformation; Sleep Apnea Syndromes; Decompression, Surgical; Sleep
PubMed: 34251945
DOI: 10.1080/02688697.2021.1947981