Trombone tongue: a new clinical sign for significant medullary compression at the craniovertebral junction

2006 ◽  
Vol 5 (6) ◽  
pp. 550-553 ◽  
Author(s):  
Cheong H. Lee ◽  
Adrian T. H. Casey ◽  
James B. Allibone ◽  
Ramesh Chelvarajah

✓The authors describe a previously unreported clinical sign that may indicate the onset of significant compression of the medulla oblongata in cases of craniovertebral junction abnormalities. This 17-year-old boy presented with mild bilateral leg weakness. Imaging studies revealed severe basilar invagination and a marked Chiari malformation. While awaiting surgery, his tongue developed an involuntary constant protrusion–intrusion repetitive motion. The onset of this so-named “trombone tongue” sign was followed shortly afterward by rapidly progressive spastic tetraparesis. After the authors performed a transmaxillary clivectomy, foramen magnum decompression, and occipitocervical fusion, they noted that the abnormal tongue motion promptly resolved and the tetraparesis gradually improved. The authors discuss their current understanding of the central control of tongue movements and present a hypothesis on the pathogenesis of trombone tongue based on the neuroanatomical basis of another abnormal tongue movement sign, lingual myoclonus.

2016 ◽  
Vol 125 (1) ◽  
pp. 196-201 ◽  
Author(s):  
Ehab Shiban ◽  
Elisabeth Török ◽  
Maria Wostrack ◽  
Bernhard Meyer ◽  
Jens Lehmberg

OBJECT Far-lateral or extreme-lateral approaches to the skull base allow access to the lateral and anterior portion of the lower posterior fossa and foramen magnum. These approaches include a certain extent of resection of the condyle, which potentially results in craniocervical junction instability. However, it is debated what extent of condyle resection is safe and at what extent of condyle resection an occipitocervical fusion should be recommended. The authors reviewed cases of condyle resection/destruction with regard to necessity of occipitocervical fusion. METHODS The authors conducted a retrospective analysis of all patients in whom a far- or extreme-lateral approach including condyle resection of various extents was performed between January 2007 and December 2014. RESULTS Twenty-one consecutive patients who had undergone a unilateral far- or extreme-lateral approach including condyle resection were identified. There were 10 male and 11 female patients with a median age of 61 years (range 22–83 years). The extent of condyle resection was 25% or less in 15 cases, 50% in 1 case, and greater than 75% in 5 cases. None of the patients who underwent condyle resection of 50% or less was placed in a collar postoperatively or developed neck pain. Two of the patients with condyle resection of greater than 75% were placed in a semirigid collar for a period of 3 months postoperatively and remained free of pain after this period. At last follow-up none of the cases showed any clear sign of radiological or clinical instability. CONCLUSIONS The unilateral resection or destruction of the condyle does not necessarily result in craniocervical instability. No evident instability was encountered even in the 5 patients who underwent removal of more than 75% of the condyle. The far- or extreme-lateral approach may be safer than generally accepted with regard to craniocervical instability as generally considered and may not compel fusion in all cases with condylar resection of more than 75%.


2019 ◽  
Vol 10 ◽  
pp. 38 ◽  
Author(s):  
Pravin Salunke ◽  
Madhivanan Karthigeyan ◽  
Puneet Malik

Background: Atlantoaxial dislocation (AAD) and basilar invagination (BI) may coexist with Chiari malformations (CM) and a small posterior fossa volume. These are typically treated with craniovertebral junction fusion and foramen magnum decompression (FMD). Here, we evaluated whether C1–C2 posterior reduction and fixation (which possibly opens up the ventral foramen magnum) would effectively treat AAD and CM without additionally performing FMD. Methods: This is a retrospective analysis of 38 patients with BI, AAD, and CM who underwent C1–C2 posterior reduction and fusion without FMD. Baseline and follow-up clinical, demographic, and radiological data were evaluated. Results: The vast majority of patients (91.9%) improved both clinically and radiographically following C1–C2 fixation alone; none later required direct FMD. Notably, AAD was irreducible in 25 (65.8%) patients. Preoperatively, syringomyelia was present in 28 (73.7%) patients and showed resolution. In 3 (8.1%) patients, resolution of syrinxes did not translate into clinical improvement. Of interest, 5 patients who sustained inadvertent dural lacerations exhibited transient postoperative neurological worsening. Conclusions: Posterior C1–C2 distraction and fusion alone effectively treated AAD, BI, accompanied by CM. The procedure sufficiently distracted the dens, reversed dural tenting, and restored the posterior fossa volume while relieving ventral brainstem compression making FMD unnecessary. Surgeons should, however, be aware that inadvertent dural lacerations might contribute to unwanted neurological deterioration.


2004 ◽  
Vol 1 (3) ◽  
pp. 281-286 ◽  
Author(s):  
Atul Goel

Object. The author discusses the successful preliminary experience of treating selected cases of basilar invagination by performing atlantoaxial joint distraction, reduction of the basilar invagination, and direct lateral mass atlantoaxial plate/screw fixation. Methods. Twenty-two patients with basilar invagination—in which the odontoid process invaginated into the foramen magnum and the tip of the odontoid process was above the Chamberlain, McRae foramen magnum, and Wackenheim clival lines—were selected to undergo surgery. In all patients fixed atlantoaxial dislocations were documented. The 16 male and six female patients ranged in age from 8 to 50 years. A history of trauma prior to the onset of symptoms was documented in 17 patients. Following surgery, the author observed minimal-to-significant reduction of basilar invagination and alteration in other craniospinal parameters resulting in restoration of alignment of the tip of the odontoid process and the clivus and the entire craniovertebral junction in all patients. In addition to neurological and radiological improvement, preoperative symptoms of torticollis resolved significantly in all patients. The minimum follow-up period was 12 months and the mean was 28 months. Conclusions. Joint distraction and firm lateral mass fixation in selected cases of basilar invagination is a reasonable surgical treatment for reducing the basilar invagination, restoring craniospinal alignment, and establishing fixation of the atlantoaxial joint.


2002 ◽  
Vol 96 (1) ◽  
pp. 73-82 ◽  
Author(s):  
Uğur Türe ◽  
M. Necmettin Pamir

Object. Various approaches have been described for resection of the dens of the axis, each of which has potential advantages and disadvantages. Anterior approaches such as the transoral route or its modifications are the most commonly used for resection of this structure. The transcondylar approach, however, which allows the surgeon to view the craniovertebral junction (CVJ) from a lateral perspective, has been introduced by Al-Mefty, et al., as an alternative approach. In this report, the authors describe the surgical technique of the extreme lateral—transatlas approach and their clinical experiences. Methods. The authors first examined the surgical approach to the dens from a lateral perspective in five cadaveric heads. They found that removal of the lateral mass of the atlas provided adequate exposure for resection of the dens. Following this cadaveric study, the extreme lateral—transatlas approach was successfully performed at the authors' institution over a 1-year period (September 1998–August 1999) in five patients with basilar invagination due to congenital anomaly of the CVJ and rheumatoid arthritis. Furthermore, during the same procedure, unilateral occipitocervical fusion was performed following resection of the dens. In all cases complete resection of the dens was achieved using the extreme—lateral transatlas approach. This procedure provides a sterile operative field and the ability to perform occipitocervical fusion immediately following the resection. No postoperative complications or craniocervical instability were observed. The mean follow-up period was 17.2 months (range 13–24 months). Conclusions. The extreme lateral—transatlas approach for resection of the dens was found to be safe and effective. Knowledge of the anatomy of this region, especially of the V3 segment of the vertebral artery, is essential for the success of this procedure.


Author(s):  
James K. Liu ◽  
Vincent N. Dodson ◽  
Kevin Zhao ◽  
Jean Anderson Eloy

AbstractBasilar invagination is a congenital or acquired craniovertebral junction abnormality where the tip of the odontoid process projects through the foramen magnum which can cause severe symptomatic compression of the brainstem and spinal cord. If left untreated, patients can develop progressive quadriparesis. Traditionally, basilar invagination can be treated with cervical traction and posterior stabilization. However, in irreducible cases, anterior decompression via a transoral or endonasal approach may be necessary. In this operative video, we demonstrate an endoscopic endonasal transclival approach for odontoidectomy to successfully treat a 37-year-old female with severe basilar invagination causing symptomatic compression on the cervicomedullary junction resulting in unsteady gait and motor weakness. The patient had Klippel–Feil syndrome where the C1 arch was assimilated to the foramen magnum and transclival drilling was needed to adequately access the odontoid process for removal. A second-stage posterior occipitocervical stabilization and fusion was performed the following day. Immediate postoperative imaging showed excellent decompression of the cervicomedullary junction. Postoperatively, the patient had significant improvement in gait and motor strength in all extremities, and was ambulating independently without assistance at 1 year after surgery. The endoscopic endonasal transclival odontoidectomy is a useful strategy to treat severe irreducible basilar invagination causing symptomatic neural compression. The surgical technique and nuances are described in a step-by-step fashion in this illustrative operative video.The link to the video can be found at: https://youtu.be/HL4K7KqJEJM.


2021 ◽  
Vol 12 ◽  
pp. 260
Author(s):  
Claudio Henrique F. Vidal ◽  
Ricardo Brandao Fonseca ◽  
Bruno Leimig ◽  
Walter F. Matias-Filho ◽  
Geraldo Sa Carneiro-Filho

Background: Basilar invagination (BI) can be defined as the insinuation of the content of the craniovertebral junction through the foramen magnum toward the posterior fossa. BI is a prevalent condition in Northeast Brazil. The present study describes the changes in the clivus-canal angle (CCA) in the postoperative period in patients with symptomatic BI operated by a posterior approach, using a simple technique of indirect reduction of the odontoid associated with occipitocervical fixation. Methods: Patients underwent radiological evaluations by magnetic resonance imaging in the pre and postoperative periods, where the height of the odontoid tip was measured in relation to the Chamberlain line and the ACC. All patients underwent posterior occipitocervical fixation with specific maneuvers of distraction and extension of the cephalic segment with the aid of a head clamp with three fixation points for anterior reduction of the odontoid. Results: Among the 8 patients evaluated in the series, all had increased ACC in the postoperative period, with a mean of 14.81 ± 1.54°, and statistically significant difference between the pre and postoperative periods (P < 0.05). Conclusion: The indirect surgical reduction of the odontoid process by a posterior approach through the manipulation (distraction-extension) of a “Mayfield” type of head clamp followed by occipitocervical fixation proved to be effective in improving the ACC, being easily reproducible.


Neurosurgery ◽  
2010 ◽  
Vol 66 (suppl_3) ◽  
pp. A39-A47 ◽  
Author(s):  
Justin S. Smith ◽  
Christopher I. Shaffrey ◽  
Mark F. Abel ◽  
Arnold H. Menezes

Abstract BACKGROUND Basilar invagination is a developmental anomaly of the craniovertebral junction in which the odontoid abnormally prolapses into the foramen magnum. It is often associated with other osseous anomalies of the craniovertebral junction, including atlanto-occipital assimilation, incomplete ring of C1, and hypoplasia of the basiocciput, occipital condyles, and atlas. Basilar invagination is also associated with neural axis abnormalities, including Chiari malformation, syringomyelia, syringobulbia, and hydrocephalus. Patients frequently present with neurologic symptoms and deficits and warrant surgical treatment to prevent progression. OBJECTIVE To review the management of basilar invagination. METHODS The literature was reviewed in reference to the evaluation and management of basilar invagination, with particular emphasis on the surgical treatment. RESULTS Reducible basilar invagination may be treated with posterior decompression and stabilization. Ventral decompression may be necessary for basilar invagination with neural compression that is not reducible with axial cervical traction. Posterior cervical stabilization is necessary after ventral decompression. Modern rod and screw systems combined with autogenous bone graft enable correction of deformity, immediate stabilization, and high fusion rates. CONCLUSION Basilar invagination is a developmental anomaly and commonly presents with neurologic findings. Treatment is typically surgical and involves anterior decompression followed by posterior stabilization for irreducible invagination and posterior decompression and stabilization for reducible invagination.


Author(s):  
Ravi Dasari ◽  
Kadali Satyavara Prasad ◽  
Phaneeswar Thota ◽  
Raman B. V. S.

Background: Craniovertebral junction tuberculosis (CVJ-TB) is a rare entity occurring in only 0.3 to 1% of tuberculous spondylitis. It causes severe instability and neurological deficits. Present study includes 16 cases of CVJ tuberculosis with neck pain and progressive quadriparesis. Radiological evaluation showed wide spread disease around clivus, C1, C2, C3 with extensive bony destruction, cord compression, basilar invagination and atlantoaxial dislocation.Methods: The study included all the cases admitted with cv junction tuberculosis in neurosurgery ward in King George hospital, Visakhapatnam during a period of three years from 2014 to 2016. Four cases were managed conservatively and four cases were treated by only posterior occipitocervical fusion. We performed two stage operation in single sitting i.e. transoral decompression and posterior occipitocervical fusion in 12 cases. The pathological findings confirmed tuberculosis.Results: Postoperatively all the patients had decreased neck pain and two third of the patients (10 of 16 patients) had improvement in motor power.Conclusions: In the available literature, the treatment options offered for cvj-tb have ranged from a purely conservative approach to radical surgery without well-defined guidelines. In this study, we followed a radical approach as the patients included in our study presented with extensive TB cv junction. So, we recommend radical surgery for extensive TB of cv junction.


1988 ◽  
Vol 69 (6) ◽  
pp. 895-903 ◽  
Author(s):  
Arnold H. Menezes ◽  
John C. VanGilder

✓ 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.


2006 ◽  
Vol 4 (2) ◽  
pp. 137-144 ◽  
Author(s):  
Hyunchul Shin ◽  
Ignacio J. Barrenechea ◽  
Jonathan Lesser ◽  
Chandranath Sen ◽  
Noel I. Perin

Object Surgical access to tumors at the craniovertebral junction (CVJ) requires extensive bone removal. Guidelines for the use of occipitocervical fusion (OCF) after resection of CVJ tumors have been based on anecdotal evidence. The authors performed a retrospective study of factors associated with the use of OCF in 46 patients with CVJ tumors. The findings were used to develop recommendations for use of OCF in such patients. Methods The authors retrospectively reviewed the cases of 51 patients with CVJ tumors treated by their group between March 1991 and February 2004. Forty-six patients were available for follow up. Charts were reviewed to obtain data on demographic characteristics, presenting symptoms, and perioperative complications. Preoperative computerized tomography scans and magnetic resonance imaging studies were obtained in all patients. Occipitocervical fusion was performed in patients who had undergone a unilateral condyle resection in which 70% or more of the condyle was removed, a bilateral condyle resection with 50% removal, or C1–2 vertebral body destruction. Of the 46 patients, 16 had foramen magnum meningiomas, 17 had chordomas, one had a chondrosarcoma, two had Schwann cell tumors, two had glomus tumors, and eight had other types of tumors. Twenty-three (50%) of the 46 patients underwent OCF, including 15 of the 17 patients with chordomas (88%). None of the patients with meningiomas required fusion. Seventeen (71%) of the 24 patients presenting with neck pain preoperatively underwent OCF. Conclusions Patients presenting with neck pain had a 71% chance of undergoing OCF. Patients with chordomas and metastatic tumors were most likely to require OCF. One patient with a 50% unilateral condylar resection returned with OC instability for which OCF was required. Based on their clinical experience and published biomechanical studies, the authors recommend that OCF be performed when 50% or more of one condyle is resected.


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