Craniovertebral junction abnormalities with hindbrain herniation and syringomyelia: regression of syringomyelia after removal of ventral craniovertebral junction compression

2012 ◽  
Vol 116 (2) ◽  
pp. 301-309 ◽  
Author(s):  
Arnold H. Menezes
Keyword(s):  
Mr Imaging ◽  
Posterior Fossa ◽  
Craniovertebral Junction ◽  
Basilar Invagination ◽  
Os Odontoideum ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Lower Cranial Nerve ◽  
Neurological Improvement ◽  
Hindbrain Herniation

Object Hindbrain herniation syndrome, or Chiari malformation Type I (CM-I), occurs frequently with craniovertebral junction (CVJ) abnormalities when there is reduction in the posterior fossa volume. Syringomyelia is often present. Posterior fossa dorsal decompression (PFDD) is typically performed but has adverse results when ventral bone abnormality exists. This paper presents the results of a prospective study on CVJ abnormalities in patients with CM-I and syringomyelia. Methods Between 1984 and 2008 (the MR imaging era), 298 patients with CVJ abnormalities and CM-I underwent ventral cervicomedullary decompression. Eighty-four patients had associated syringomyelia (15 with secondary invagination and 69 with primary basilar invagination, os odontoideum, or malunion of fractures). Of these 84 patients with CVJ abnormalities, CM-I, and syringomyelia, 46 had previously undergone PFDD, and 28 had previously undergone PFDD combined with fusion procedures or shunt placements. Of the 84 patients, a cervicothoracic syrinx was observed in 57, thoracic syrinx in 14, and holocord syrinx in 13. Studies included CT, MR imaging, and cine flow studies. All 298 patients who underwent ventral CVJ decompression had irreducible or partially reducible pathology. All 84 with syringomyelia showed brainstem dysfunction, lower cranial nerve symptoms, or myelopathy. Results Brainstem signs improved in 66 of the 84 patients, myelopathy improved in 58, and syringomyelia regressed in 64. Conclusions Neurological improvement and syringomyelia resolution can occur using only ventral cervicomedullary junction decompression in patients with basilar invagination and basilar impression. This is likely due to the relief of neural encroachment and reestablishment of CSF pathways.

scholarly journals Delayed neurological deterioration following atlantoaxial facet joint distraction and fixation in a patient with Chiari malformation type I

2018 ◽  
Vol 28 (3) ◽  
pp. 262-267 ◽  
Author(s):  
Tatsushi Inoue ◽  
Natsuki Hattori ◽  
Tsukasa Ganaha ◽  
Tadashi Kumai ◽  
Shinichiro Tateyama ◽  
...  
Keyword(s):  
Chiari Malformation ◽  
Intervention Strategy ◽  
Essential Element ◽  
Craniovertebral Junction ◽  
Basilar Invagination ◽  
Type I ◽  
Atlantoaxial Joint ◽  
Chiari Malformation Type I ◽  
Neurological Improvement ◽  
Joint Distraction

Chiari malformation type I (CM-I) is typically treated with foramen magnum decompression. However, a recent study proposed a new technique for patients with CM-I, wherein only short atlantoaxial joint fusion and distraction is applied. Posterior fusion with or without atlantoaxial distraction is a potential option for patients with CM-I associated with basilar invagination or complex anomalous bony craniovertebral junction pathology, since this procedure allows clinicians to avoid using the technically demanding transoral approach in which some or all of the odontoid tip is invisible. Distraction of the atlantoaxial joint reduces ventral cervicomedullary compression, leading to neurological improvement. Here, the authors report the case of a 50-year-old woman with CM-I plus basilar invagination whose condition immediately improved but then gradually deteriorated following atlantoaxial joint distraction and fusion. Therefore, the authors performed endonasal/transoral odontoidectomy, which resulted in prolonged neurological recovery. Short atlantoaxial fusion with distraction is a smart and ideal surgical planning concept that can result in significant neurological improvement. However, this case suggests that anterior odontoidectomy is still an essential element of the intervention strategy for patients with CM-I with complex craniovertebral junction pathology.

Volumetric analysis of the posterior cranial fossa in a family with four generations of the Chiari malformation Type I

2008 ◽  
Vol 1 (1) ◽  
pp. 21-24 ◽  
Author(s):  
R. Shane Tubbs ◽  
Mark Hill ◽  
Marios Loukas ◽  
Mohammadali M. Shoja ◽  
W. Jerry Oakes
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Familial Aggregation ◽  
Volumetric Analysis ◽  
Posterior Cranial Fossa ◽  
Preoperative Imaging ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Hindbrain Herniation ◽  
Cranial Fossa

Object Many authors have concluded that the Chiari malformation Type I (CM-I) is due to a smaller than normal posterior cranial fossa. In order to establish this smaller geometry as the cause of hindbrain herniation in a family, the authors of this paper performed volumetric analysis in a family found to have this malformation documented in 4 generations. Methods Members from this family found to have a CM-I by imaging underwent volumetric analysis of their posterior cranial fossa using the Cavalieri method. Results No member of this family found to have CM-I on preoperative imaging had a posterior fossa that was significantly smaller than that of age-matched controls. Conclusions The results of this study demonstrate that not all patients with a CM-I will have a reduced posterior cranial fossa volume. Although the mechanism for the development of hindbrain herniation in this cohort is unknown, this manifestation can be seen in multiple generations of a familial aggregation with normal posterior fossa capacity.

scholarly journals Minimally invasive posterior fossa decompression with duraplasty in Chiari malformation type I with and without syringomyelia

2019 ◽  
Vol 10 ◽  
pp. 88 ◽  
Author(s):  
Maria Caffo ◽  
Salvatore M. Cardali ◽  
Gerardo Caruso ◽  
Elena Fazzari ◽  
Rosaria V. Abbritti ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Chiari Malformation ◽  
Signs And Symptoms ◽  
Posterior Arch ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Respiratory Impairment ◽  
Lower Cranial Nerve ◽  
Good Outcome

Background: Posterior fossa decompression (PFD), with and without duraplasty, represents a valid treatment in Chiari malformation Type I (CM-I) with and without syringomyelia. Despite a large amount of series reported in literature, several controversies exist regarding the optimal surgical approach yet. In this study, we report our experience in the treatment of CM-I, with and without syringomyelia, highlighting how the application of some technical refinements could lead to a good outcome and a lesser rate of complications. Methods: Twenty-six patients with CM-I, with and without syringomyelia, underwent PFD through a 3 cm × 3 cm craniectomy with the removal of the most median third of the posterior arch of C1 and duraplasty. Signs and symptoms included sensory deficits, motor deficits, neck pain, paresthesias, headache, dizziness, lower cranial nerve deficits, and urinary incontinence. Postoperative magnetic resonance (MR) was performed in all patients. Results: Signs and symptoms improved in 76.9% of cases. Postoperative MR revealed a repositioning of cerebellar tonsils and the restoration of cerebrospinal fluid circulation. In our experience, the rate of complication was 23% (fistula, worsening of symptoms, and respiratory impairment). Conclusion: PFD through a 3 cm × 3 cm craniectomy and the removal of the most median third of posterior arch of C1 with duraplasty represents a feasible and valid surgical alternative to treat patients with CM-I, with and without syringomyelia, achieving a good outcome and a low rate of complications.

Transoral-transpharyngeal approach to the anterior craniocervical junction

1988 ◽  
Vol 69 (6) ◽  
pp. 895-903 ◽  
Author(s):  
Arnold H. Menezes ◽  
John C. VanGilder
Keyword(s):  
Effective Means ◽  
Craniovertebral Junction ◽  
Basilar Invagination ◽  
Craniocervical Junction ◽  
Os Odontoideum ◽  
Occipitocervical Fusion ◽  
Content Type ◽  
Patients Âgés ◽  
Neurological Improvement ◽  
Clivus Chordoma

✓ The anterior transoral-transpharyngeal operation to correct ventral irreducible compression of the cervicomedullary junction was utilized in 72 individuals. The patients' ages ranged from 6 to 82 years, and 29 were children. The pathology encountered was primary basilar invagination, rheumatoid irreducible cranial settling, secondary basilar invagination due to migration of odontoid fracture fragments, dystopic os odontoideum, granulation masses, clivus chordoma, osteoblastoma, and chondroma of the atlas. Fifteen patients had associated Chiari malformation with basilar invagination. Fifty-two patients required subsequent atlantoaxial or occipitocervical fusion. Neurological improvement was the rule. There were two deaths within 30 days of surgery: one from myocardial infarction 4 weeks after surgery and one from Gram-negative septicemia of urinary tract origin. There was one pharyngeal wound infection. The ventral transoral approach provides a safe, rapid, and effective means for decompression of the abnormal craniovertebral junction.

Posterior fossa volume increase after surgery for Chiari malformation Type I: a quantitative assessment using magnetic resonance imaging and correlations with the treatment response

2011 ◽  
Vol 115 (3) ◽  
pp. 647-658 ◽  
Author(s):  
Rémy Noudel ◽  
Philippe Gomis ◽  
Gustavo Sotoares ◽  
Arnaud Bazin ◽  
Laurent Pierot ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Treatment Response ◽  
Posterior Fossa ◽  
Chiari Malformation ◽  
Complete Recovery ◽  
Patient Specific ◽  
Type I ◽  
Partial Recovery ◽  
Chiari Malformation Type I ◽  
Volume Increase

Object The aim of this paper was to measure the posterior fossa (PF) volume increase resulting from a given-sized occipital craniectomy in Chiari malformation Type I surgery and to analyze its correlations with the PF size and the treatment response, with the perspective of tailoring the amount of bone removal to the patient-specific PF dimensions. Methods Between January 2005 and June 2006, 11 adult patients with symptomatic Chiari malformation Type I underwent a standardized PF decompression. A prospective evaluation with clinical examination, functional grading, and MR imaging measurement protocols was performed pre- and postoperatively. A method is reported for the measurement of PF volume (PFV) after surgery. The degree of PFV increase was compared with the preoperative size of the PF and with the clinical outcome. Results All 11 patients improved postoperatively, with complete and partial recovery in 4 and 7 patients, respectively. No postoperative complication occurred after a mean follow-up period of 45 months. The mean relative increase in PFV accounted for 10% (range 1.5%–19.7%) of the initial PFV; the increase was greater in cases in which the PF was small (r = −0.52, p = 0.09) and the basiocciput was short (r = −0.37, p = 0.2). A statistically significant positive correlation was found between the degree of PFV increase and the treatment response (p = 0.014); complete recovery was observed with a PFV increase of 15% and partial recovery with an increase of 7%. Conclusions The treatment response is significantly influenced by the degree of PFV increase, which is dependent on the size of the PF and the extent of the craniectomy, suggesting that the optimal patient-specific PFV increase could be predicted on the basis of preoperative MR imaging and enhancing the perspective that the craniectomy size could be tailored to the individual PFV.

Craniocervical junction fusions in patients with hindbrain herniation and syringohydromyelia

2008 ◽  
Vol 9 (1) ◽  
pp. 1-9 ◽  
Author(s):  
Albert J. Fenoy ◽  
Arnold H. Menezes ◽  
Kathleen A. Fenoy
Keyword(s):  
Posterior Fossa ◽  
Craniocervical Junction ◽  
Symptom Improvement ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
University Of Iowa ◽  
Chiari Malformation Type I ◽  
Group Iii ◽  
Group I ◽  
Hindbrain Herniation

Object Patients with hindbrain herniation or the so-called Chiari malformation Type I (CM-I) and/or syringohydromyelia are treated with dorsal decompression alone; however, a small percentage of patients with other associated abnormalities require concomitant dorsal craniocervical junction (CCJ) fusion. The authors surveyed the indications for CCJ fusions in this population. Methods A retrospective review of University of Iowa medical records and radiographs obtained between 1996 and 2005 was performed. Inclusion criteria encompassed patients with diagnoses of CM-I and/or syringohydromyelia requiring dorsal CCJ fusions, and others with CCJ abnormalities who had CM-I and/or syringohydromyelia. Results Two hundred thirty-four patients were identified, all of whom were symptomatic at presentation. Their ages ranged from 2.5 to 86 years; 33% of the patients were < 16 years of age. Patients were categorized as follows, with some being assigned to > 1 category: Group I, congenital or acquired CCJ abnormalities with reducible bone compression (25% of patients); Group II, previous anterior CCJ/upper brainstem decompression (44%); Group III, occipitocervical complex instability with CM-I and/or syringohydromyelia but without CCJ bone abnormalities requiring adjunctive posterior fossa decompression (26%); and Group IV, musculoligamentous instability, either from pathological states or from muscle dehiscence from repeated posterior fossa procedures (14%). Instrumentation was used in 96% of patients, with all 96% receiving semirigid fixation with titanium loop and sublaminar cables; all fusion constructs incorporated autologous bone. At last follow-up evaluation, fusion was radiographically complete in 97%, and symptom improvement was seen in 92%. Conclusions Dorsal CCJ fusions are required in patients with CM-I and/or syringohydromyelia who have concomitant CCJ abnormalities (Groups I and II). A definite group (CM-I and/or syringohydromyelia) without bone abnormality exists (Groups III and IV). This may be due to muscle weakness secondary to a high syrinx.

scholarly journals Functional and morphological changes in hypoplasic posterior fossa

2021 ◽  
Author(s):  
Federico Bianchi ◽  
Alberto Benato ◽  
Paolo Frassanito ◽  
Gianpiero Tamburrini ◽  
Luca Massimi
Keyword(s):  
Morphological Changes ◽  
Cervical Spinal Cord ◽  
Basilar Invagination ◽  
Foramen Magnum ◽  
Type I ◽  
Chiari Type I Malformation ◽  
Upper Cervical ◽  
Hindbrain Herniation ◽  
Cranial Fossa ◽  
2D And 3D

Abstract Background The knowledge of the development and the anatomy of the posterior cranial fossa (PCF) is crucial to define the occurrence and the prognosis of diseases where the surface and/or the volume of PCF is reduced, as several forms of craniosynostosis or Chiari type I malformation (CIM). To understand the functional and morphological changes resulting from such a hypoplasia is mandatory for their correct management. The purpose of this article is to review the pertinent literature to provide an update on this topic. Methods The related and most recent literature addressing the issue of the changes in hypoplasic PCF has been reviewed with particular interest in the studies focusing on the PCF characteristics in craniosynostosis, CIM, and achondroplasia. Results and conclusions In craniosynostoses, namely, the syndromic ones, PCF shows different degrees of hypoplasia, according to the different pattern and timing of early suture fusion. Several factors concur to PCF hypoplasia and contribute to the resulting problems (CIM, hydrocephalus), as the fusion of the major and minor sutures of the lambdoid arch, the involvement of the basal synchondroses, and the occlusion of the jugular foramina. The combination of these factors explains the variety of the clinical and radiological phenotypes. In primary CIM, the matter is complicated by the evidence that, in spite of impaired PCF 2D measurements and theories on the mesodermal defect, the PCF volumetry is often comparable to healthy subjects. CIM is revealed by the overcrowding of the foramen magnum that is the result of a cranio-cerebral disproportion (altered PCF brain volume/PCF total volume). Sometimes, this disproportion is evident and can be demonstrated (basilar invagination, real PCF hypoplasia); sometimes, it is not. Some recent genetic observations would suggest that CIM is the result of an excessive growth of the neural tissue rather than a reduced growth of PCF bones. Finally, in achondroplasia, both macrocephaly and reduced 2D and 3D values of PCF occur. Some aspects of this disease remain partially obscure, as the rare incidence of hydrocephalus and syringomyelia and the common occurrence of asymptomatic upper cervical spinal cord damage. On the other hand, the low rate of CIM could be explained on the basis of the reduced area of the foramen magnum, which would prevent the hindbrain herniation.

scholarly journals Standard and cardiac-gated phase-contrast magnetic resonance imaging in the clinical course of patients with Chiari malformation Type I

2011 ◽  
Vol 31 (3) ◽  
pp. E5 ◽  
Author(s):  
Uwe Max Mauer ◽  
Andreas Gottschalk ◽  
Carolin Mueller ◽  
Linda Weselek ◽  
Ulrich Kunz ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Chiari Malformation ◽  
Phase Contrast ◽  
Foramen Magnum ◽  
Central Canal ◽  
Type I ◽  
Csf Flow ◽  
Chiari Malformation Type I ◽  
Cerebrospinal Fluid Flow ◽  
Contrast Magnetic Resonance

Object The causal treatment of Chiari malformation Type I (CM-I) consists of removing the obstruction of CSF flow at the level of the foramen magnum. Cerebrospinal fluid flow can be visualized using dynamic phase-contrast MR imaging. Because there is only a paucity of studies evaluating CSF dynamics in the region of the spinal canal on the basis of preoperative and postoperative measurements, the authors investigated the clinical usefulness of cardiacgated phase-contrast MR imaging in patients with CM-I. Methods Ninety patients with CM-I underwent preoperative MR imaging of CSF pulsation. Syringomyelia was present in 59 patients and absent in 31 patients. Phase-contrast MR imaging of the entire CNS was used to investigate 22 patients with CM-I before surgery and after a mean postoperative period of 12 months (median 12 months, range 3–33 months). In addition to the dynamic studies, absolute flow velocities, the extension of the syrinx, and tonsillar descent were also measured. Results The changes in pulsation were highly significant in the region of the (enlarged) cistern (p = 0.0005). Maximum and minimum velocities (the pulsation amplitude) increased considerably in the region where the syrinx was largest in diameter. The changes of pulsation in these patients were significant in the subarachnoid space in all spinal segments but not in the syrinx itself and in the central canal. Conclusions The demonstration of CSF flow pulsation can contribute to assessments of surgical outcomes. The results presented here, however, raise doubts about current theories on the pathogenesis of syringomyelia.

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