Compression Of Cervicomedullary Junction Research Articles

Database Review of 514 Patients with Os Odontoideum. Detailed Analysis of 258 Surgically Treated (1978-2019).

Database review (1978-2019) is to identify the cause of os odontoideum, its presentation, associated abnormalities, and management recommendations. Review of referral database of 514 patients and 258 surgically treated patients ages 4-64years. Detailed history of early childhood trauma and initial encounter record retrieval were made. Patients had dynamic motion radiographs, dynamic motion MRI and also CT to identify pathology and reducibility of craniocervical instability. Preoperative crown halo traction was made before the year 2000 except in children. Intraoperative traction with O-arm/CT documentation was made since 2001. Reducible and partially reducible cases underwent halo traction under general anesthesia distraction, dorsal stabilization, and rib graft augmentation for fusion. Later semi-rigid instrumentation and subsequently rigid instrumentation was made. Irreducible compression of cervicomedullary junction was treated with ventral decompression. The follow up was 3-20years. Database; acute worsening after trauma 262, insidious neurological deficit 252. Minimal/normal motion with neurological deficit was present in 18, previous C1-C2 fusion with worsening in 18. 28 patients of 64 without treatment worsened in 4years. An intact odontoid process was seen in 52 children of 156 who had early craniovertebral junction trauma and later developed os odontoideum. There were 174 patients with reducible lesions and partially reducible were 22. Irreducible lesions were 62. Of the reducible, 50 underwent transarticular C1-C2 fusion, 26 C1 lateral mass, and C2 pars screw fixation. 182 had occipitocervical fusion (19 had extension of previous C1-C2 fusion and 43 after transoral decompression). 62 with irreducible ventral compression of the cervicomedullary junction underwent transoral decompression; 43 had a trapped transverse ligament between the os and C2 body and 19 previous C1-C2 fusions. Compression was by the axis body, os odontoideum, and the posterior C2 arch. Syndromic and skeletal/connective tissue abnormalities were found in 86 (36%). 2 patients worsened, age 10 and 62, due to failure of semi-rigid construct. The etiology of os odontoideum is multifactorial considering the associated abnormalities, reports of congenital-familiar occurrence, and early childhood craniovertebral trauma which also plays a role in the etiology. Patients with reducible lesions require stabilization. Asymptomatic patients are at risk for later instability. Patients who underwent childhood C1-C2 fusion must be followed for later problems. The irreducibility was seen due to trapped transverse ligament, pannus, or previous dorsal C1-C2 fusion.

0980 A Child with Achondroplasia, Cervicomedullary Junction Compression, Severe Obstructive Sleep Apnea and Pulmonary Hypertension

Abstract Introduction Achondroplasia is a short stature autosomal dominant skeletal dysplasia. Children with Achondroplasia are at risk for multiple medical comorbidities including sleep disordered breathing due to midface hypoplasia and craniocervical junction compression. Recent guidelines from American Academy of Pediatrics on health supervision of children with achondroplasia recommend routine clinical screening for symptoms of obstructive sleep apnea (OSA) and craniocervical junction compression in which case a polysomnogram should be considered. Report of case(s) The patient is a now 15 month old male who was initially seen in sleep medicine clinic at age 4 months for snoring and respiratory distress while awake. Polysomnogram 2 days later demonstrated severe OSA. The study demonstrated an oAHI of 25.1/hour, O2 nadir of 77%, and TcCO2 with evidence of significant hypoventilation with TcCO2 max of 67mmHg. This was adequately treated with supplemental oxygen. The patient subsequently presented to the emergency department at age 7 months with hypoxemic respiratory failure and signs of right heart failure and was started on CPAP. Echocardiogram demonstrated significant pulmonary hypertension. Subsequent CPAP titration study during admission demonstrated adequate treatment of OSA and his pulmonary hypertension significantly improved with the patient requiring no pulmonary hypertension treatment at discharge. At age 9 months, the patient was again admitted with hypoxemic respiratory failure and subsequent brain MRI demonstrated foramen magnum stenosis requiring subsequent surgical decompression. He was discharged on CPAP, however, was not tolerating this at home. Therefore, repeat polysomnogram was obtained that demonstrated severe OSA that was not responsive to supplemental oxygen. The patient was then started on BPAP therapy which he has since been tolerating well. Conclusion Children with achondroplasia are at increased risk for sleep disordered breathing and craniocervical junction compression. Our patient experienced significant complications from OSA at a young age. His sleep apnea worsened significantly in a short period making it unresponsive to previous treatment recommendations. There is a need for further studies to determine better screening guidelines for craniocervical junction narrowing and OSA. This case highlights the dynamic nature of sleep disordered breathing in achondroplasia and need for close follow up and monitoring of symptoms at all clinical encounters. Support (if any)

Role of imaging in craniovertebral junction abnormalities

Background: Craniovertebral junction (CVJ) abnormalities constitute an important group of treatable neurological disorders with diagnostic dilemma. Their precise diagnosis and pre-treatment evaluation significantly affects prognosis and quality of life of patients. Aims: To localize, describe and characterize craniovertebral junction abnormalities, arrange frequently detected CVJ pathologic imaging findings and to emphasize clinical implications to improve our radiological report. Materials and methods: A Cross sectional study done for 24 months in 50 patients of all age groups with clinical suspicion of CVJ abnormalities.Results: In the acquired CVJ abnormality Trauma is the most common etiology(n=15) followed by Tuberculosis(n=4), Rheumatoid arthritis(n=3), Tumors(n=2). Most common age group involved was 2nd decade with male predominance. Most commonly presenting symptom is weakness of limbs followed by neck pain. Most common imaging abnormality is cervicomedullary junction compression and atlanto axial dislocation. ACM I and Syringomyelia are the commonest neural anomalies associated with bony CVJ anomalies. Most common congenital anomalies are atlanto axial dislocation followed by basilar invagination. Only one patient had syndromic association which was Klippel-feil syndrome. The commonest injuries involving the CVJ were odontoid fracture predominantly type II and the commonest combination of injury was C1 with odontoid fracture.

Upper Cervical Subluxation and Cervicomedullary Junction Compression in Patients with Rheumatoid Arthritis.

ObjectiveRheumatoid arthritis (RA) is known to involve the cervical spine up to 86%. It often causes cervical instability like atlantoaxial subluxation (AAS), subaxial subluxation, and vertical subluxation (VS). In order to find the relation between RA and cord compression, we will evaluate the characteristics and risk factors of basilar invagination (BI) and cervicomedullary junction (CMJ) compression. MethodsFrom January 2007 to May 2015, 12667 patients administrated to Hanyang University Medical Center. Four thousand three hundred eighty-six patients took cervical X-ray and 250 patients took cervical computed tomography or magnetic resonance imaging. Radiologic parameters, medication records were obtained from 242 patients. Multivariate logistic regression analysis was performed with correlation of CMJ compression, basin-dental interval (BDI), basin-posterior axial line interval (BAI), pannus formation, BI, and AAS. ResultsIn the point of CMJ compression, atlantodental interval (ADI), posterior-atlantodental interval, BAI, AAS, and BI are relatively highly correlated. Patients with BI have 82 times strong possibility of radiologic confirmed CMJ compression, while AAS has 6-fold and pannus formation has the 3-fold possibility. Compared to the low incidence of BI, AAS and pannus formation have more proportion in CMJ compression. Furthermore, wrist joint erosion was correlated with VS and AAS. ConclusionBI has a very strong possibility of CMJ compression, while AAS and pannus formation have a high proportion in CMJ compression. Hence bilateral wrist joint erosion can be used as an indicator for the timing of screening test for cervical involvement. We suggest the early recommendation of cervical spine examination for the diagnosis of cervical involvement in order to prevent morbidity and mortality.

Cranio-vertebral junction anomaly –presenting as cervical myelopathy

Antlanto-axial dislocation with blocked and ankylosed C2-4 vertebrae causing severe canal stenosis with compression of cervico-medullary junction has been reported. The knowledge of such anomaly may be importance for radiologists, anaesthesiologists, orthopaedicians and neurosurgeons because cranio-cervical junction anomaly may result in sudden unexpected death. It can also result in dysphagia, dysarthria due to compression of cranial nerves and quadriparesis due to compression of spinal cord. We here report a rare case of cranio-vertebral junction anomaly with blocked and ankylosed C2,3,4vertebrae with evidence of atlanto-axial dislocation causing compression of cervico-medullary junction and leading to cervical myelopathy.International Journal of Human and Health Sciences Vol. 03 No. 01 January’19. Page : 40-42

A case of cervicomedullary junction compression

The C-V junction is a transition site between mobile cranium and relatively rigid spinal column. It is also the site of the medullo-spinal junction. CV anomalies are defects of development, not necessarily congenital and may not manifest at birth. These anomalies can lead to spinal cord compression, cranial nerve compression, vertebral artery compression and obstructive hydrocephalus. Delay in diagnosis can result in irreparable damage to spinal cord and can lead to permanent disability or even death. MRI of cervical spine helps us in diagnosis of CVJ anomalies and is the investigation of choice. We are presenting a case of 12 years old female child presented with history of progressive weakness of both upper and lower limbs, difficulty in walking and history of frequent falls with transient loss of consciousness for last 4 years. She was found to have CVJ anomalies on MRI cervical spine but had already developed permanent disability due to atrophy of spinal cord due to prolonged compression.

Cervicomedullary junction compression in a toddler with achondroplasia

Cervicomedullary junction compression in a toddler with achondroplasia

Endoscopic Transnasal Odontoidectomy.

Transoral microscopic odontoidectomy followed by posterior fixation has been accepted as a standard procedure to treat nonreducible basilar invagination during the half past century. In recent years, the development of endoscopic techniques has raised challenges regarding the traditional treatment algorithm. The endoscopic transnasal odontoidectomy is a feasible and effective method in the treatment of irreducible ventral cervicomedullary junction compression, which has several advantages over the transoral approach. The endoscopic odontoidectomy includes transnasal, transoral, and transcervical approaches. The 3 different approaches for endoscopic odontoidectomy present complementary advantages and limitations. The necessity of posterior fixation after odontoidectomy should be considered in every single case on the basis of the peculiar anatomic and clinical conditions.

Occipito-thoracic fusion in incidental dystropic os odontoideum in a patient suspected with osteogenesis imperfecta: A case report

Introduction: Os odontoideum is a rare lesion of the axis. Described as an ossicle, consisting of smooth and separate caudal portions of the odontoid process wherein its etiology has remained controversial. Os odontoideum complicating a possible osteogenesis imperfecta has not been reported before in the literature. Methods: We report the case of a 14-yr male patient presented with progressive weakness of both lower and upper extremities after a head trauma 10 months prior to admission, which presented as transient quadreparesis. Results: Magnetic resonance imaging of cervical spine showing cervicomedullary junction compression. Patient underwent surgical intervention and 3 months post operation, patient was reported to have steady gait and muscle grading of 5/5 on all extremities. Conclusion: Surgical fixation and fusion in patients with instability may prevent catastrophic neurologic insult after minor trauma in the future.

Transoral Endoscopic Odontoidectomy to Decompress the Cervicomedullary Junction

Clinical study. To investigate the feasibility of the transoral endoscopic odontoidectomy without occipitocervical fusion. Endoscopic transnasal resection of the odontoid process is less invasive than the conventional transoral odontoidectomy. However, the endonasal approach has a much longer working distance compared with the transoral approach to the craniovertebral junction and usually the endonasal approach needs a previous occipitocervical posterior fusion. From July 2007 to June 2010, 5 patients (3 males and 2 females, age range, 25-41 yr) with irreducible cervicomedullary junction compression were subjected to endoscopic transoral odontoidectomy without occipitocervical posterior fixation and bone fusion. A purely endoscopic transoral odontoidectomy for decompression of the cervicomedullary junction without the occipitocervical fusion was achieved successfully in 5 patients. None of the patients underwent tracheotomy and postoperative gastrostomy tube placement. The patients were started on liquids on the third postoperative day and advanced to a regular diet on the fourth postoperative day. There was no postoperative velopharyngeal insufficiency, cerebrospinal fluid leakage, regional infection, or meningitis. The patients were discharged in 10 to 12 days after the surgery. There were no evidence of instability at the craniovertebral junction at 12 to 47 months of follow-up and remarkable improvement in neurological function was observed in each patient. The endoscopic transoral approach may be a more direct route to C1 and the odontoid than the endoscopic endonasal approach. This approach allows complete resection odontoid to decompress the cervicomedullary junction without increasing the risk of complications such as wound infection, meningitis, and velopharyngeal insufficiency. Usually, the occipitocervical posterior fusion and tracheotomy is less necessary in this approach.

Neurologic Variant Laryngomalacia Associated with Chiari Malformation and Cervicomedullary Compression: Case Reports

Two infants presented with intermittent stridor and evidence of laryngomalacia on flexible laryngoscopy. The first was a 10-month-old girl who had undergone 3 supraglottoplasty surgeries at an outside institution, without long-term resolution of symptoms. She was found during our evaluation to have a Chiari malformation. Laryngomalacia symptoms resolved after suboccipital decompression and C1 laminectomy, and the patient remained symptom-free at 6-month follow-up. The second infant was a 24-day-old boy with velocardiofacial syndrome who was found to have posterior cervicomedullary junction compression at the level of C1. He underwent C1 laminectomy for decompression of the brain stem, which resulted in immediate resolution of symptoms, and he remained symptom-free at 12-month follow-up. Neurologic abnormalities have been reported in up to 50% of infants with laryngomalacia. As such, brain stem dysfunction should be considered among the causes of laryngomalacia during evaluation, especially in patients with failure of supraglottoplasty. Both of these infants had resolution of symptoms after their neurosurgical procedures.

Dynamic cervicomedullary cord compression and alterations in cerebrospinal fluid dynamics in children with achondroplasia

Achondroplasia is the most common of the heritable skeletal dysplasias. Compression at the cervicomedullary junction can result in myelopathy, hypotonia, sleep apnea, and even sudden death. However, most children with achondroplasia do not suffer from severe neurological symptoms and achieve normal motor and intellectual development without surgical intervention. At the authors' institution, magnetic resonance (MR) imaging and cerebrospinal fluid (CSF) flow studies have been incorporated in the assessment of children with achondroplasia for cervicomedullary junction compression. The authors recently identified four children with achondroplasia who had normal findings on MR imaging and flow studies obtained in the neutral position. On flexion studies, however, three had complete blockage of CSF flow, and more dramatic posterior cervicomedullary compression was demonstrated on extension studies. Some of these patients had severe neurological abnormalities and sleep apnea, while others just developed headaches and/or had apnea episodes when sleeping or in a car seat. Three children underwent decompressive surgery with dramatic improvement or resolution of signs and symptoms. The fourth patient had increased CSF pressure on MR images obtained in the flexed position, possibly due to venous outflow obstruction. Her condition improved dramatically after placement of a ventriculoperitoneal shunt. The increased risk of dynamic cord compression and alterations in CSF dynamics in patients with achondroplasia constitute indications for surgical intervention.

Intracranial Dural Arteriovenous Fistula Presenting with Tetraparesis Due to Cervicomedullary Junction Compression

Cranial dural arteriovenous fistulas (DAVFs) may give rise to myelopathy due to spinal perimedullary venous drainage causing intramedullary venous hypertension. Such cases are uncommon but not rare, with several cases reported in the literature. We report a case of foramen magnum DAVF presenting with symptoms of tetraparesis. The unusual feature was that in this case it was due to compression of the cervicomedullary junction by a large venous pouch rather than the result of spinal perimedullary venous hypertension. Transarterial glue embolization achieved good reduction of flow in the fistula with shrinkage of the venous pouch and corresponding clinical improvement.

A Case of Achondroplasia Associated with Cervicomedullary-Junction Compression

Achondroplasia is the most common skeletal dysplasia and is caused by defective fibroblast growth factor receptor (FGFR) 3 in endochondral chondrocytes. Children with achondroplasia may have high cervical myelopathy due to stenosis of the craniocervical junction and an increased risk of sudden death. We report a female patient with achondroplasia who required surgical decompression at the age of 13 mo. The female patient was diagnosed with achondroplasia clinically, radiologically and genetically shortly after birth. The newborn period was uneventful. She developed head control at the age of 6 mo, but she could not sit by the age of 12 mo. At the age of 8 mo, her parents noticed her eyes did not close and a diagnosis of left peripheral nerve palsy was made. Occasional hyperextension of the trunk was observed. There was also minimal asymmetry of voluntary leg movement and bilateral ankle clonus. By these findings craniocervical junction compression was strongly suspected. MRI examination revealed compression at the craniocervical junction by the occipital bone and myleomalacia. The patient underwent suboccipital decompressive surgery at the age of 13 mo. Recovery after surgery was uneventful, and although her left facial nerve palsy did not improve, gross motor function had improved by the age of 2 yr. The patient is now cognitive and neurologically normal at the age of 3 yr. Infants with achondroplasia are at risk of lethal sequelae of craniocervical junction abnormalities, so careful neurological assessment and early MRI examination is recommended.

Forestier disease associated with a retroodontoid mass causing cervicomedullary compression.

Forestier Disease (FD) is a progressive skeletal disorder affecting predominantly older men. It is also known as diffuse idiopathic skeletal hyperostosis (DISH) and is characterized by massive anterior longitudinal ligament calcification that forms a bridge on the anterior border of the thoracic and subaxial cervical spine. To the authors' knowledge, retroodontoid masses associated with FD have not been described. Five patients with FD and multilevel subaxial cervical fusion were treated for retroodontoid masses and cervicomedullary junction (CMJ) compression. There were four men and one woman (mean age 73 years, range 54-86 years). All patients suffered progressive neurological symptoms resulting from anterior compression of the CMJ. Four patients underwent combined transoral resection of the ligamentous mass followed by an occipitocervical fusion procedure. One patient with circumferential CMJ compression underwent a posterior decompression and occipitocervical fusion. Histopathological examination of the mass showed hypertrophic degenerative fibrocartilage. Early postoperative neurological improvement was noted in all patients. The follow-up period ranged from 4 to 19 months. At the end of the follow-up period, four patients experienced neurological improvement. One patient died 3 weeks postsurgery of pulmonary complications. The osseous elements of the occipitoatlantoaxial complex are not directly affected by FD. The ligamentous structures of the odontoid process, however, are exposed to significantly altered biomechanics resulting from fusion of the subaxial cervical spine associated with FD. Stress-induced compensatory ligamentous hypertrophic changes at the craniovertebral junction cause CMJ compression and subsequent neurological deterioration. This previously undescribed entity should be considered in patients with FD or DISH who present with progressive quadriparesis. Transoral decompression and posterior fusion are often needed in patients with large masses and severe progressive neurological deficits. Selected patients with smaller masses and milder neurological symptoms may be treated with posterior fusion alone.

Frameless Stereotactic Guidance

The application of a video-interactive frameless stereotactic guidance system for the treatment of ventral cervicomedullary junction compression is described in a patient with basilar invagination, odontoid dysgenesis, and a Chiari malformation who had irreducible impingement on the ventral brainstem by a partially fused mass of bone made up of the malformed odontoid peg, the inferior clivus, and dorsally protruding osteophytes at the odontoid-clival junction. This technology permitted instantaneous feedback of the surgeon's orientation in all planes, facilitating extensive removal of the dens and clival tip to achieve adequate ventral brainstem decompression. In view of the distortion of the craniovertebral anatomy produced by the patient's anomalies, the ability to visualize three dimensionally the location of the vertebral arteries also added an element of safety to the lateral bone removal. Similarly, the ability to localize the rostral limit of the clivus that needed to be resected and the caudal extent of C2 that needed to be removed to achieve an adequate decompression helped ensure that the extent of bone removal was appropriately tailored to the patient's anatomy. The authors believe this technique represents a significant advance over standard radiographic intraoperative localization techniques during transoral approaches to the ventral cervicomedullary junction for patients in whom the odontoid is fixed in position in relation to the clivus. This is based on the speed with which localization can be obtained, the accuracy of the information provided, the ability to obtain localization in multiple planes simultaneously, and the lack of radiation exposure during the procedure.

Cervicomedullary compression in achondroplasia.

Six patients with achondroplasia and symptoms suggestive of cervicomedullary junction compression are reviewed; these included three females and three males, with an average age of 8 years (range 7 months to 30 years). The mean duration of symptoms prior to intervention was 1.9 years. Symptoms included occipitocervical pain, ataxia, incontinence, apnea, and respiratory arrest. Radiological investigations consisted of plain films with flexion and extension views, pluridirectional tomography, thin-section computerized tomography, and magnetic resonance imaging. Typical findings included marked foramen magnum stenosis, ventrolateral cervicomedullary junction compression secondary to central and paramesial basilar invagination, and dorsal cervicomedullary junction compression secondary to ligamentous hypertrophy and invagination of the posterior atlantal arch. All patients underwent posterior fossa decompression and atlantal laminectomy. Surgery consistently revealed marked dorsal and paramesial overgrowth of the rim of the rim of the foramen magnum, with thickening and invagination of the atlantal posterior arch and a dense fibrotic epidural band resulting in dorsal cervicomedullary compression. Intraoperative ultrasonography was used to determine the extent of decompression required. Three patients required duraplasty. Three patients had concurrent hydrocephalus, two of whom had undergone ventriculoperitoneal shunting prior to surgical decompression of the posterior fossa. One patient developed a pseudomeningocele postoperatively, requiring serial lumbar punctures before it resolved. No patient developed craniovertebral instability following decompression. Improvement or resolution of symptoms was noted in all patients, with an average follow-up period of 4.8 years. Thus, cervicomedullary compression in patients with achondroplasia can be successfully treated with dorsal decompression of the craniovertebral junction. Dense epidural fibrotic bands are frequently noted in these cases and must be aggressively released to ensure satisfactory decompression.

Rheumatoid arthritis of the cervical spine: surface-coil MR imaging.

Fifteen patients with classic rheumatoid arthritis were investigated with plain films, pluridirectional tomography, and surface-coil T1-weighted MR imaging at 500/17 (TR/TE). We evaluated the atlantodental interval; basion-dental interval; density or intensity of the dens; dens erosion; cranial settling; anterior, posterior, lateral, or rotatory atlantoaxial subluxation; subaxial subluxation; ligamentous calcification or osteophytes; erosion; cystic changes; joint-space narrowing of the apophyseal articulations; and posterior spinous process erosion. In addition, the cervicomedullary angle and the neuraxis configuration were identified on MR images. To determine its normal range, the cervicomedullary angle was measured in 50 patients whose MR studies were unrelated to the craniovertebral junction. All patients with a cervicomedullary angle less than 135 degrees had evidence of brainstem compression, cervical myelopathy, or C2 root pain. Also, all patients with cervicomedullary junction compression were neurologically abnormal. MR was found to be as good as tomography in evaluating the atlantodental interval, dens erosion, ligamentous calcification or osteophytes of the upper spine, subaxial subluxation, and various subluxations that occur in the occiput-C2 area. MR was less effective than tomography in evaluating the basion-dental interval, erosion of the posterior spinous processes, apophyseal joint disease from C3 inferiorly, and cystic changes of the articular facets of C1-C2. The most clinically important parameters were well seen with MR. The data show that MR is an excellent imaging procedure for evaluating rheumatoid arthritis of the cervical spine.