-
Journal of Neurological Surgery. Part... Feb 2021The video demonstrates the steps for an endoscopic transnasal approach for resection of the odontoid causing platybasia with basilar invasion. The video reviews...
The video demonstrates the steps for an endoscopic transnasal approach for resection of the odontoid causing platybasia with basilar invasion. The video reviews the clinical presentation, preoperative workup and imaging, patient positioning, technical nuances of the procedure, reconstruction, and clinical outcomes including postoperative imaging. The surgery was performed by a skull base team including otolaryngologists and neurological surgeons at a large regional tertiary care facility. A 59-year-old female with the Chiari I malformation with history of multiple revision cervical spinal surgeries presents with neck pain. Preoperative imaging showed a hypoplastic clivus and platybasia with basilar invasion. The main outcome measures consist of safely removing the odontoid with appropriate closure, reversal of the patient symptoms, and prevention of both operative complications and ventral compression of the brainstem. The patient's neck pain improved. There were no intraoperative or postoperative complications besides anterior rightward nasal septal deviation causing unilateral nasal obstruction. A septoplasty was performed to correct the septal deviation 6 months after the initial procedure. The endoscopic transnasal approach to the resection of odontoid is a safe and effective treatment to address basilar invasion and ventral compression of the brainstem. The link to the video can be found at: https://youtu.be/m_c3-Vn-l80 .
PubMed: 33717801
DOI: 10.1055/s-0040-1705161 -
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 -
Scientific Reports Aug 2023Craniosynostosis is characterized by the premature fusion and ossification of one or more of the sutures of the calvaria, often resulting in abnormal features of the...
Craniosynostosis is characterized by the premature fusion and ossification of one or more of the sutures of the calvaria, often resulting in abnormal features of the face and the skull. In cases in which growth of the brain supersedes available space within the skull, developmental delay or cognitive impairment can occur. A complex interplay of different cell types and multiple signaling pathways are required for correct craniofacial development. In this study, we report on two siblings with craniosynostosis and a homozygous missense pathogenic variant within the IL11RA gene (c.919 T > C; p.W307R). The patients present with craniosynostosis, exophthalmos, delayed tooth eruption, mild platybasia, and a basilar invagination. The p.W307R variant is located within the arginine-tryptophan-zipper within the D3 domain of the IL-11R, a structural element known to be important for the stability of the cytokine receptor. Expression of IL-11R-W307R in cells shows impaired maturation of the IL-11R, no transport to the cell surface and intracellular retention. Accordingly, cells stably expressing IL-11R-W307R do not respond when stimulated with IL-11, arguing for a loss-of-function mutation. In summary, the IL-11R-W307R variant, reported here for the first time to our knowledge, is most likely the causative variant underlying craniosynostosis in these patients.
Topics: Humans; Craniosynostoses; Skull; Head; Brain; Arginine
PubMed: 37596289
DOI: 10.1038/s41598-023-39466-y -
The International Journal of... Mar 2024To report a case of anomalous development of base of skull (platybasia, Basilar invagination and C1-C2 vertebral fusion); and emphasize nonsurgical management in...
AIM
To report a case of anomalous development of base of skull (platybasia, Basilar invagination and C1-C2 vertebral fusion); and emphasize nonsurgical management in inoperable cases that can improve quality of life of the patient.
MATERIALS AND METHODS
The case is reported as a descriptive study of a 17-year-old female who presented to a rural teaching tertiary care hospital in Wardha, Maharashtra, India; with chief complaints of weakness in all four limbs since 10 years of age.
RESULT AND CONCLUSION
Platybasia is a developmental defect of the occipital bone and upper cervical spine resulting from anomalous development. The mechanism of such anomalies is not known; however, the most accepted theory includes abnormal basi-occiput development. The pressure effects may present signs similar to progressive spastic paralysis, cerebellar symptoms, or cranial nerve palsy, in addition to musculoskeletal symptoms. It is, therefore, crucial for physicians and radiologists to be familiar with clinical manifestations and radiological findings. In the following case of a patient with base of skull anomalies, surgical intervention in view of progressive worsening of motor symptoms was advised, however, the guardians declined the same due to high risk involved. Due to financial constraints, genetic studies were unaffordable, and a lack of awareness regarding the disease hampered the guardians from making a decision on the definite management of the disease. Besides radical neurosurgery, intensive physiotherapy can prove vital in significantly improving the quality of life for the patient.
PubMed: 38421185
DOI: 10.1080/00207454.2024.2311234 -
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 -
World Neurosurgery Dec 2019The flexion of the skull base (basal angle [BA]) is the inclination between the anterior fossae and the basilar ramp of the occipital and sphenoid bones. An increased... (Review)
Review
BACKGROUND
The flexion of the skull base (basal angle [BA]) is the inclination between the anterior fossae and the basilar ramp of the occipital and sphenoid bones. An increased BA, termed platybasia, is usually associated with basilar invagination. BA reference values in the magnetic resonance imaging (MRI) era and the diagnosis of platybasia are of clinical importance. The transnasal approach has been the surgical technique of choice to remove the odontoid process in cases of ventral brainstem compression in patients with platybasia. The knowledge of normal BA values has been influenced by technological image acquisitions. The aim of this study was to determine the normal BA values in normal subjects in the MRI era.
METHODS
For the determination of normal BA values, the literature reporting these values in normal individuals was reviewed and a meta-analysis of pertinent studies was performed.
RESULTS
A total of 8 articles were included in this study, evaluating a total of 667 individuals. A summary measure of the results was obtained. The data obtained in this analysis provided a final basal angle value of 116.5 degrees (95% confidence interval, 104.39-128.7). The normal basal angle ranges between 104 and 129 degrees.
CONCLUSION
Platybasia can be defined as a value >129 from the basal angle.
Topics: Humans; Magnetic Resonance Imaging; Neurosurgical Procedures; Platybasia; Reference Values; Skull Base; Skull Base Neoplasms
PubMed: 31541760
DOI: 10.1016/j.wneu.2019.09.056 -
Journal of Neurosurgery Feb 2024Craniocervical junction (CCJ) pathologies with ventral neural element compression are poorly understood, and appropriate management requires accurate understanding,...
OBJECTIVE
Craniocervical junction (CCJ) pathologies with ventral neural element compression are poorly understood, and appropriate management requires accurate understanding, description, and a more uniform nomenclature. The aim of this study was to evaluate patients to identify anatomical clusters and better classify CCJ disorders with ventral compression and guide treatment.
METHODS
A retrospective review of adult and pediatric patients with ventral CCJ compression from 2008 to 2022 at a single center was performed. The incidence of anatomical abnormalities and compressive etiologies was assessed. Surgical approach, radiographic data, and outcomes were recorded. Association rules analysis (ARA) was used to assess variable clustering.
RESULTS
Among 51 patients, the main causes of compression were either purely bony (retroflexed dens [n = 18]; basilar invagination [BI; n = 13]) or soft tissue (degenerative pannus [n = 16]; inflammatory pannus [n = 2]). The primary cluster in ARA was a retroflexed dens, platybasia, and Chiari malformation (CM), and the secondary cluster was BI, C1-2 subluxation, and reducibility. These, along with degenerative pannus, formed the three major classes. In assessing the optimal treatment strategy, reducibility was evaluated. Of the BI cases, 12 of the 13 patients had anterolisthesis of C1 that was potentially reducible, compared with 2 of the 18 patients with a retroflexed dens (both with concomitant BI), and no pannus cases. The mean C1-2 facet angle was significantly higher in BI at 32.4°, compared with -2.3° in retroflexed dens and 8.1° in degenerative pannus (p < 0.05). Endonasal decompression with posterior fixation was performed in 48 (94.0%) of the 51 patients, whereas posterior reduction/fixation alone was performed in 3 patients (6.0%). Of 16 reducible cases, open posterior reduction alone was successful in 3 (60.0%) of 5 cases, with all successes containing isolated BI. Reduction was not attempted if vertebral anatomy was unfavorable (n = 9) or the C1 lateral mass was absent (n = 5). The mean follow-up was 28 months. Symptoms improved in 88.9% of patients and were stable in the remaining 11.1%. Tracheostomy and percutaneous G-tube placement occurred in 7.8% and 11.8% of patients, respectively. Reoperation for an endonasal CSF leak repair or posterior cervical wound revision both occurred in 3.9% of patients.
CONCLUSIONS
In classifying, one cluster caused decreased posterior fossa volume due to an anatomical triad of retroflexed dens, platybasia, and CM. The second cluster caused pannus formation due to degenerative hypertrophy. For both, endonasal decompression with posterior fixation was ideal. The third group contained C1 anterolisthesis characterized by a steep C1-2 facet angle causing reducible BI. Posterior reduction/fixation is the first-line treatment when anatomically feasible or endonasal decompression with in situ posterior fixation when anatomical constraints exist.
Topics: Adult; Humans; Child; Platybasia; Decompression, Surgical; Arnold-Chiari Malformation; Odontoid Process; Reoperation
PubMed: 37503952
DOI: 10.3171/2023.5.JNS23677 -
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 -
Neurosurgical Review Dec 2023Basilar invagination (BI) is characterized by rostral dislocation of the cervical spine toward the skull base. The craniometrics of the skull base have shown significant...
Basilar invagination (BI) is characterized by rostral dislocation of the cervical spine toward the skull base. The craniometrics of the skull base have shown significant differences among craniocervical junction malformations. The sphenoid bone is the center of the skull base; however, no study has evaluated this bone in cases of BI. This was a cross-sectional study of MRI databanks from two institutions of the author's practice between 1985 and 2020. The craniometrics of the sphenoid bone were measured in BI patients and controls. Fifty-eight MRIs were selected, including 28 BI patients and 30 controls. The mean sphenoid crest-clivus length was 32.66 ± 4.7 mm in the BI group and 29.98 ± 3.0 mm in the control group (p = 0.01). The mean sphenoid planum-top of Dorsum sellae length was 28.53 ± 3.7 mm in the BI group and 26.45 ± 3.2 mm in the control group (p = 0.02). The mean tuberculum sellae-sphenoid floor height was 18.52 ± 4.4 mm in the BI group and 21.32 ± 2.9 mm in the control group (p = 0.00). The mean sella turcica-sphenoid floor height was 10.35 ± 3.8 mm in the BI group and 12.24 ± 3.5 mm in the control group (p = 0.05). The mean clivus length was 29.81 ± 6.3 mm in the BI group and 40.86 ± 4.2 mm in the control group (p = 0.00). The mean sphenoid length was 58.34 ± 7.4 mm in the BI group and 67.31 ± 6.0 mm in the control group (p = 0.00). The mean sphenoid angle was 116.33 ± 8.7° in the BI group and 112.36 ± 6.9° in the control group (p = 0.05). The BI sphenoid bone has shorter vertical dimensions and longer horizontal measures. This morphology promotes a flattening of the sphenoid angle. The sphenoid bone is significantly altered in BI, favoring the congenital hypothesis in the pathophysiology of this disease.
Topics: Humans; Cross-Sectional Studies; Platybasia; Sphenoid Bone; Skull Base; Cervical Vertebrae
PubMed: 38040961
DOI: 10.1007/s10143-023-02227-6