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Journal of Neurology, Neurosurgery, and... Dec 1992Syringobulbia is a term which has been clinically applied to brain stem symptoms or signs in patients with syringomyelia. Syringobulbia clefts are found on investigation...
Syringobulbia is a term which has been clinically applied to brain stem symptoms or signs in patients with syringomyelia. Syringobulbia clefts are found on investigation or at necropsy caused by cutting outwards of the CSF under pressure from the fourth ventricle into the medulla. These should be differentiated from the ascending syringobulbia which may occur from upward impulsive fluid movements in a previously established syringomyelia. Clinical analysis of 54 patients suggests that bulbar features are most often found with neither of the above mechanisms but are due to the effects of pressure differences acting downward upon the hind-brain with consequent distortion of the cerebellum and brainstem, traction on cranial nerves or indentation of the brain-stem by vascular loops. The commonest symptoms in the 54 patients were headache (35), vertigo (27), dysphonia or dysarthria (21), trigeminal paraesthesiae (27), dysphagia (24), diplopia (16), tinnitus (11), palatal palsy (11) and hypoglossal involvement (11). Careful attention to hydrocephalus is advisable before craniovertebral surgery, but the decompression of the hindbrain and the correction of craniospinal pressure dissociation remains the mainstay of surgical treatment. The results of careful surgery are good, 45 of the 54 cases reported improvement. Most of the reported deterioration occurred in a few patients who did conspicuously badly.
Topics: Adolescent; Adult; Brain Stem; Cerebrospinal Fluid Shunts; Child; Cranial Nerves; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Nerve Compression Syndromes; Neurologic Examination; Platybasia; Postoperative Complications; Syringomyelia
PubMed: 1479391
DOI: 10.1136/jnnp.55.12.1132 -
Arquivos de Neuro-psiquiatria Jul 2017Cross-sectional analysis of craniometric parameters from individuals submitted to magnetic resonance at an outpatient imaging center between 2011 and 2012.
METHODS
Cross-sectional analysis of craniometric parameters from individuals submitted to magnetic resonance at an outpatient imaging center between 2011 and 2012.
RESULTS
Of 181 analyzed cases, the Welcker basal angle averaged 128.96º (SD 6.51), median distance between apex of the odontoid and Chamberlain's line was 2.27 mm (IQR -1.23-4.47) and the median clivus-canal angle was 150.5º (IQR 143.2-157.3). The Welcker basal angle was inversely correlated to the clivus-canal angle, and correlated to the distance between the apex of the odontoid and Chamberlain's line.
CONCLUSION
There was a tendency to platibasia, basilar invagination and narrowing of the cranio-vertebral transition.
Topics: Brazil; Cross-Sectional Studies; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Odontoid Process; Platybasia; Reference Values; Skull Base
PubMed: 28746426
DOI: 10.1590/0004-282X20170071 -
BMC Musculoskeletal Disorders Dec 2020Transoral atlantoaxial reduction plate (TARP) fixation or occipitocervical fixation (OF) is an effective treatment for basilar invagination (BI) with irreducible...
BACKGROUND
Transoral atlantoaxial reduction plate (TARP) fixation or occipitocervical fixation (OF) is an effective treatment for basilar invagination (BI) with irreducible atlantoaxial dislocation (IAAD). But, all current clinical studies involved a single surgical procedure. The clinical effects of TARP and OF operation for BI with IAAD have yet to be compared. We therefore present this report to compare the treatment of TARP and OF procedure for BI with IAAD.
METHODS
Fifty-six patients with BI with IAAD who underwent TARP or OF operation from June 2011 to June 2017 were retrospectively analyzed. Among these, 35 patients underwent TARP operation (TARP group), and 21 patients underwent OF operation (OF group). We compared the difference of clinical, radiological, and surgical outcomes between the TARP and OF groups postoperatively.
RESULTS
Compared with OF group, the operative time and blood loss in TARP group were lower. There was no statistical difference in the atlantodental interval (ADI), clivus canal angle (CCA), cervicomedullary angle (CMA), distance between the top of the odontoid process and the Chamberlain line (CL) and Japanese Orthopaedic Association (JOA) score between the TARP and OF groups preoperatively, but the improvements of these parameters in the TARP group were superior to those in the OF group postoperatively. The fusion rates were higher in the TARP group than those in the OF group at the early stage postoperatively.
CONCLUSIONS
TARP and OF operations are effective surgical treatment for BI with IAAD, but the performance of reduction and decompression and earlier bone fusion rates of TARP procedure are superior to those of OF.
Topics: Atlanto-Axial Joint; Bone Plates; Decompression, Surgical; Humans; Joint Dislocations; Platybasia; Retrospective Studies; Spinal Fusion; Treatment Outcome
PubMed: 33292209
DOI: 10.1186/s12891-020-03838-6 -
Journal of Medical Genetics Feb 1993
Topics: Child; Humans; Hydrocephalus; Male; Osteolysis, Essential; Platybasia
PubMed: 8445627
DOI: 10.1136/jmg.30.2.175 -
Journal of Orthopaedic Surgery (Hong... Dec 2005To confirm the accuracy of the standard parameters of Chamberlain's line, McGregor's line, McRae's line, and Ranawat's line in the diagnosis of basilar impression in a...
PURPOSE
To confirm the accuracy of the standard parameters of Chamberlain's line, McGregor's line, McRae's line, and Ranawat's line in the diagnosis of basilar impression in a normal Asian population using magnetic resonance imaging (MRI) scans of the craniocervical junction, and to evaluate the use of MRI in the initial diagnosis of basilar impression.
METHODS
We retrospectively reviewed MRI scans of the craniocervical junction in 114 Thai patients (61 men and 53 women) aged 20 to 89 years to assess the lines of Chamberlain, McGregor, McRae, and Ranawat. Patients with injury, tumour, or metastasis in the craniocervial junction; congenital anomalies; or clinical symptoms and signs of basilar impression were excluded.
RESULTS
39 of the 114 patients had the odontoid tip extended above Chamberlain's line by 1 mm to 9 mm (mean, 2.89 mm). Only 7 men and 5 women had the tip of the odontoid process extended beyond the normal range of 5 mm. The mean distance of the odontoid tip extended past McGregor's line in male and female subjects was 0.33 mm (standard deviation [SD], 1.2 mm) and 0.17 mm (SD, 0.7 mm), respectively, markedly less than those reported previously, although one male patient's odontoid tip extended 8 mm above the McGregor's line. Regarding McRae's line, no patient had the odontoid tip extended above the foremen magnum. The mean Ranawat's line in male and female subjects was 15.75 mm (range, 12-19; SD, 1.71 mm) and 14.09 mm (range, 11-17; SD, 1.38 mm), respectively.
CONCLUSION
After using MRI to confirm the standard parameters of basilar impression as previously formulated from lateral skull radiographs, we recommend using McRae's line and Ranawat's line in the initial screening for basilar impression for the Asian population. Chamberlain's line and McGregor's line are viable alternatives, but clinicians must be aware of the potential for false positives.
Topics: Adult; Aged; Aged, 80 and over; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Platybasia; Reference Values; Retrospective Studies; Skull Base; Thailand
PubMed: 16365484
DOI: 10.1177/230949900501300303 -
Acta Medica Academica Nov 2017This study adds important information regarding the morphological alterations caused by growth hormone hypersecretion in the skull and craniocervical junction (CCJ). A...
OBJECTIVE
This study adds important information regarding the morphological alterations caused by growth hormone hypersecretion in the skull and craniocervical junction (CCJ). A variably asymmetric skull due to acromegaly coexists with expansion of the paranasal sinuses and multiple Wormian bones.
CASE REPORT
A pathologically asymmetric dry skull of a European male, aged 38 years at death, with cranial vault and skull base thickening is described. The extensive paranasal sinus pneumatization caused a generalized thinning of the bony walls. The sphenoid sinus expanded intraorbitally, leading to sella enlargement. The orbital asymmetry coexisted with platybasia and hypoplasia of the occipital condyles and the odontoid process. Facial skeleton elongation and mandibular overgrowth were combined with prognathism, malocclusion and overbite.
CONCLUSION
Skull and CCJ alterations are of paramount importance when selecting the surgical approach, if surgery is indicated. Consecutively, detailed preoperative evaluation and planning is essential. During surgery, skilled and experienced neurosurgeons recognize anatomical landmarks, use neuronavigation and micro-instrumentation in order to remain on the midline avoiding any potential lethal vascular injury.
Topics: Acromegaly; Adult; Humans; Male; Occipital Bone; Odontoid Process; Skull; Sphenoid Sinus
PubMed: 29338280
DOI: 10.5644/ama2006-124.201 -
BMC Musculoskeletal Disorders Nov 2020The revision surgery of basilar invagination (BI) with irreducible atlantoaxial dislocation (IAAD) after a previous occipitocervical fusion (OCF) is challenging....
BACKGROUND
The revision surgery of basilar invagination (BI) with irreducible atlantoaxial dislocation (IAAD) after a previous occipitocervical fusion (OCF) is challenging. Transoral revision surgery has more advantages than a combined anterior and posterior approach in addressing this pathology. The C-JAWS is a cervical compressive staple that has been used in the lower cervical spine with many advantages. Up to now, there is no report on the application of C-JAWS in the atlantoaxial joint. We therefore present this report to investigate the clinical outcomes of transoral intraarticular cage distraction and C-JAWS fixation for revision of BI with IAAD.
METHODS
From June 2011 to June 2015, 9 patients with BI and IAAD were revised by this technique after previous posterior OCF in our department. Plain cervical radiographs, computed tomographic scans and magnetic resonance imaging were obtained pre- and postoperatively to assess the degree of atlantoaxial dislocation and compression of the cervical cord. The Japanese Orthopedic Association (JOA) score was used to evaluate the neurological function.
RESULTS
The revision surgeries were successfully performed in all patients. The average follow-up duration was 18.9 ± 7.3 months (range 9-30 months). The postoperative atlas-dens interval (ADI), cervicomedullary angle (CMA), distance between the top of the odontoid process and the Chamberlain line (CL) and JOA score were significantly improved in all patients (P < 0.05). Bony fusion was achieved after 3-9 months in all cases. No patients developed recurrent atlantoaxial instability.
CONCLUSIONS
Transoral revision surgery by intraarticular cage distraction and C-JAWS fixation could provide a satisfactory outcome for BI with IAAD after a previous unsuccessful posterior operation.
Topics: Atlanto-Axial Joint; Decompression, Surgical; Humans; Jaw; Joint Dislocations; Platybasia; Spinal Fusion; Treatment Outcome
PubMed: 33218335
DOI: 10.1186/s12891-020-03792-3 -
European Spine Journal : Official... May 2013Klippel-Feil syndrome (KFS) is a congenital cervical vertebral union caused by a failure of segmentation during abnormal development and frequently accompanies...
INTRODUCTION
Klippel-Feil syndrome (KFS) is a congenital cervical vertebral union caused by a failure of segmentation during abnormal development and frequently accompanies conditions such as basicranial malformation, atlas assimilation, or dens malformation. Especially in basilar invagination (BI), which is a dislocation of the dens in an upper direction, compression of the spinomedullary junction from the ventral side results in paralysis, and treatment is required.
CLINICAL PRESENTATION
We present the case of a 38-year-old male patient with KFS and severe BI. Plane radiographs and computed tomography (CT) images showed severe BI, and magnetic resonance image (MRI) revealed spinal cord compression caused by invagination of the dens into the foramen magnum and atlantoaxial subluxation. Reduction by halo vest and skeletal traction were not successful. Occipitocervical fusion along with decompression of the foramen magnum, C1 laminectomy, and reduction using instruments were performed. Paralysis was temporarily aggravated and then gradually improved. Unsupported walking was achieved 24 months after surgery, and activities of daily life could be independently performed at the same time. CT and MRI revealed that dramatic reduction of vertical subluxation and spinal cord decompression were achieved.
CONCLUSION
Reduction and internal fixation using instrumentation are effective techniques for KFS with BI; however, caution should be exercised because of the possibility of paralysis caused by intraoperative reduction.
Topics: Adult; Decompression, Surgical; Humans; Klippel-Feil Syndrome; Male; Odontoid Process; Orthopedic Procedures; Platybasia; Spinal Cord Compression
PubMed: 22926486
DOI: 10.1007/s00586-012-2489-3 -
Child's Nervous System : ChNS :... May 2022Osteogenesis imperfecta (OI) is a rare bone disease due to an abnormal synthesis of 1-type collagen. OI is frequently associated with basilar impression (BI), defined by...
Severe Basilar impression in osteogenesis imperfecta treated with halo gravity traction, occipitocervicothoracic fusion, foramen magnum and upper cervical decompression and expansive duroplasty: a technical note.
Osteogenesis imperfecta (OI) is a rare bone disease due to an abnormal synthesis of 1-type collagen. OI is frequently associated with basilar impression (BI), defined by the elevation of the clivus and floor of the posterior fossa with subsequent migration of the upper cervical spine and the odontoid peg into the base of the skull. Bone intrinsic fragility leading to fractures and deformity, brainstem compression and impaired CSF circulation at cranio-vertebral junction (CVJ) makes the management of these conditions particularly challenging. Different surgical strategies, including posterior fossa decompression with or without instrumentation, transoral or endonasal decompression with posterior occipito-cervical fusion, or halo gravity traction with posterior instrumentation have been reported, but evidence about best modalities treatment is still debated. In this technical note, we present a case of a 16-years-old patient, diagnosed with OI and BI, treated with halo traction, occipito-cervico-thoracic fixation, foramen magnum and upper cervical decompression, and expansive duroplasty. We focus on technical aspects, preoperative work up and postoperative follow up. We also discuss advantages and limitations of this strategy compared to other surgical techniques.
Topics: Adolescent; Cervical Vertebrae; Decompression; Foramen Magnum; Humans; Osteogenesis Imperfecta; Platybasia; Traction
PubMed: 35296931
DOI: 10.1007/s00381-022-05495-7 -
Frontiers in Genetics 2018Diamond-Blackfan anemia (DBA) is a rare congenital erythroid aplasia with a highly heterogeneous genetic background; it usually occurs in infancy. Approximately 30-40%...
Diamond-Blackfan anemia (DBA) is a rare congenital erythroid aplasia with a highly heterogeneous genetic background; it usually occurs in infancy. Approximately 30-40% of patients have other associated congenital anomalies; in particular, facial anomalies, such as cleft palate, are part of about 10% of the DBA clinical presentations. Pierre Robin sequence (PRS) is a heterogeneous condition, defined by the presence of the triad of glossoptosis, micrognathia and cleft palate; it occurs in 1/8500 to 1/14,000 births. Klippel Feil (KF) syndrome is a complex of both osseous and visceral anomalies, characterized mainly by congenital development defects of the cervical spine. We describe the case of a 22-years-old woman affected by DBA, carrying a deletion about 500 Kb-long at 12q13.2-q13.3 that included and, at least, others 25 flanking genes. The patient showed craniofacial anomalies due to PRS and suffered for KF deformities (type II). Computed Tomography study of cranio-cervical junction (CCJ) drew out severe bone malformations and congenital anomalies as atlanto-occipital assimilation (AOA), arcuate foramen and occipito-condylar hyperplasia. Foramen magnum was severely reduced. Atlanto-axial instability (AAI) was linked to atlanto-occipital assimilation, congenital vertebral fusion and occipito-condyle bone hyperplasia. Basilar invagination and platybasia were ruled out on CT and Magnetic Resonance Imaging (MRI) studies. Furthermore, the temporal Bone CT study showed anomalies of external auditory canals, absent mastoid pneumatization, chronic middle ear otitis and abnormal course of the facial nerve bones canal. The described phenotype might be related to the peculiar deletion affecting the patient, highlighting that genes involved in the in the breakdown of extracellular matrix (), in cell cycle regulation (, vesicular trafficking (, in ribonucleoprotein complexes formation () and muscles function ( and ) could be potentially related to bone-developmental disorders. Moreover, it points out that multiple associated ribosomal deficits might play a role in DBA-related phenotypes, considering the simultaneous deletion of three of them in the index case ( and , and it confirms the association among functional disruption and severe myopia. This report highlights the need for a careful genetic evaluation and a detailed phenotype-genotype correlation in each complex malformative syndrome.
PubMed: 30524470
DOI: 10.3389/fgene.2018.00549