atlanto-axial osteosynthesis. Multiple variations of partial assimilation have been reported and may involve any aspect of atlanto-occipital articulation. Atlanto-occipital dislocation, orthopedic decapitation, or internal decapitation describes ligamentous separation of the spinal column from the skull base. In fourteen cases, additional anomalies of other vertebrae were present. Atlanto-occipital dislocation each side. Several neural axis abnormalities are commonly described in association with BI, especially Chiari type I malformation . the base of the occipital bone of the skull is described as assimilation of the atlas. AA dislocation, fusion of zygoapophyseal joints, secondary atlanto-occipital assimilation, and spinal canal stenosis may also be seen as a part of disease process. This report reviews the background of atlantooccipital . 12-1 to 12-5 ). Diffuse paraspinal ossification and inflammatory osteitis creates a fused, brittle spine, susceptible to fracture, even with minor trauma. INTRODUCTION. Clinical. This study is aimed at examining the efficacy of human umbilical cord blood-mononuclear cell (hUCB-MNCs) transplantation through lateral atlanto-occipital space puncture in multiple system atrophy (MSA) treatment and investigating changes in T-cell subsets in peripheral blood and inflammatory factors in patients before and after treatment. Atlanto-occipital fusion is a rare anomaly of the craniocervical junction which may be congenital or acquired. [4, 6, 7] Although most patients with atlas assimilation may be asymptomatic, some patients may experience myelopathy attributed to cervical cord impingement and occipitocervical instability.Those with progressive symptomatic segmental . In one patient (Case 10) the condition was considered to be stable. atlanto-occipital assimilation; incomplete ring of C1; hypoplasia of the basiocciput, occipital condyles, and atlas. followed by atlanto-occipital assimilation(33.3%), and Arnold-Chiari malformation is the most common soft tissue anomaly. body and C6-C1 posterior arch rachischisis.. Atlas assimilation is known to be related to serious vascular and neurologic problems, like vertebra-basilar insufficiency [17-19]. M54.11 is a valid billable ICD-10 diagnosis code for Radiculopathy, occipito-atlanto-axial region . The CCJ is composed of three bone structures: the first and second cervical vertebrae (also known as the atlas and axis, respectively), and the occiput. Most patients with atlas assimilation are asymptomatic, but some may present with neurological problems such as myelopathy. Objective . Transitional anatomy can also occur at the atlanto-occipital junction (atlanto-occipital assimilation: complete or partial fusion of C1 and the occiput, occipital vertebra: an additional bone between C1 and the occiput), and at the cervicothoracic junction (cervical rib arising from C7). Epidemiology Atlanto-occipital assimilation occurs in approximately 0.5% (range 0.08-3%) of the population 2-5,. The anterior arch had incompletely fused with occipital bone, showing nonunion in the midline. Mongolism may also be associated with atlanto-occipital subluxation (AOS) and AARF. This report is the first detailed evaluation of neurological deficit and radiographic abnormalities caused by this malformation. The incidence of retro-odontoid pseudotumor in each group was analyzed. Most of the abnormalities cause no typical symptom, but some patients have neurological problems which correlate with radiological findings. Mongolism may also be associated with atlanto-occipital subluxation (AOS) and AARF. 11 The extent of atlanto-occipital assimilation varies from partial to total, which may account for the broad reported incidence from 0 . Atlanto-occipital joint is important joint present at the craniocervical junction. 12-1 to 12-5 ). Atlantoaxial rotary fixation has been reported after disabling cerebral infarction, caused by constant head posturing to the same side (see Figs. In particular, Sakai et al. Foramen magnum syndrome caused by atlanto-occipital assimilation. The observations from the reports were combined because the usual methods for analy- Only right transverse process was fused with occipital bone. atlanto-occipital assimilation with intracanal fibrosis. Many variable types of atlas assimilation have been reported. In certain diseases of the bone like hyperparathyroidism, Paget's or osteomalacia, there is softening of the base of the skull which gets invaginated. In condylar hypoplasia, the occipital condyles are underdeveloped and flat; as a result, the skull base is flattened or even ascends medially. Epidemiology Atlanto-occipital assimilation occurs in approximately 0.5% (range 0.08-3%) of the population 2-5,. Conclusions: Assimilation of atlas may cause orthopedic problems and occasionally it produces neurological effects especially when the lumen of foramen magnum is reduced. 2007: 125-141. and age (adult and pediatric groups). Symptoms usually present during adolescence or adulthood, typically consisting of headache or neck pain. Fusion of the atlas to the occiput in atlanto-occipital assimilation causes the mechanical stress during movement of the head to be transferred to the . [5], presented a case of BHS associated with atlanto-occipital assimilation; they affirmed that 3D-CTA with his head rotated [1][2] They innervate the posterior scalp up as far as the vertex and other structures as well, such as the ear. This assimilation causes secondary widening of the atlantodental space . This condition may be partial or complete, as was the cases here. We describe a case of foramen magnum syndrome secondary to atlanto-occipital assimilation with spinal-cord compression caused by retro-odontoid fibrous pannus. Chiari malformation type 3 is an extremely rare anomaly characterized by a low occipital and high cervical encephalocele with herniation of posterior fossa contents, that is, the cerebellum and/or the brainstem, occipital lobe, and fourth ventricle, through an abnormal opening in the back of the skull. Ethical Approval The cause of the excessive tissue formation in this case was attributed to a congenital anomaly, atlanto-occipital assimilation with secondary altered biomechanics in the craniocervical junction. It remains unclear, whether cardiac arrest due to a cardiac cause resulted in complete atony of the paravertebral muscles and caused this extremely severe lesion (atlanto-occipital dislocation) or whether cardiac arrest was caused by apnea due the paraplegia following the spinal injury of the trauma. Atlanto-occipital assimilation is the fusion of the atlas (C1) to the occiput and is one of the transitional vertebrae. Among these, acute spondylolysis of C2 (hangman's fracture), C1 ring fractures, odontoid fractures, and atlanto-occipital dislocation (AOD) were the most common[].AOD is a highly unstable craniocervical injury, resulting from damage to ligaments and/or bony . fuses with the occipital bone. [] Congenital atlanto-occipital assimilation (AOA) is a rare spectrum of craniovertebral junction (CVJ) anomalies which often coexists with other anomalies. Atlantoaxial rotary fixation has been reported after disabling cerebral infarction, caused by constant head posturing to the same side (see Figs. Association of atlanto-occipital assimilation with the occurrence of severe neurological symptoms resulting from upper spinal cord compression remains rare in the literature. Atlanto-occipital assimilation . In condylar hypoplasia, the occipital condyles are underdeveloped and flat; as a result, the skull base is flattened or even ascends medially. This joint comes in relation to the important structures in the region like vertebral arteries, spinal arteries, medulla oblongata and spinal cord. When occipital condyles are normally developed, the skull base descends medially. However, while and Scheuer L. 1996. We report the case of a 62-year-old male referred to our . Many variable types of atlas assimilation have been reported. The most common symptoms were weakness of extremities, neck pain, paresthesia, torticollis, and gait disturbances. Chiari I malformation and atlanto-occipital assimilation are both relatively rare conditions. The main etiologies of BI are clivus (basiocciput) hypoplasia, occipital condyle hypoplasia, atlas hypoplasia, atlanto-occipital assimilation and congenital atlanto-axial [scielo.br] This article provides an overview of the following congenital upper cervical disorders: Platybasia Atlanto - axial Instability Atlas Hypoplasia Klippel-Feil . Home; Profiles; Research Units; Equipment; Projects; Research output; Search by expertise, name or affiliation transverse ligament, thus increasing the risk of C1 The apical ligament attaches from the tip of the subluxation. It is thou. It is found in the 2021 version of the ICD-10 Clinical Modification (CM) and can be used in all HIPAA-covered transactions from Oct 01, 2020 - Sep 30, 2021 . A decompression by resec- Usually it is found by chance, and if it is not accompanied by other malformations 2, 3, 4, 5neurological deficit is very rare. The pathologic type of AAD is the least common and includes AAD caused by tuber-culosis, infection or tumors of the atlas or axis. [10] Kalla AK, Khanna S, Singh IP, Sharma S, Schnobel R, Vogel F. A genetic and anthropological study of atlanto-occipital . Atlas assimilation may include alteration of the atlanto occipital articulation or narrowing of the spinal canal. These ligaments limit contralateral flexion particularly the CCJ, and cause weakening of the [12-14] and axial rotation at the atlanto-occipital joint . Cervicocranial syndrome or (Craniocervical Junction Syndrome: CCJ syndrome) is a neurological illness.It is a combination of symptoms that are caused by an abnormality in the neck. Such change causes basilar invagination and widening of the atlanto-occipital joint axis angle. It causes the nodding movement (flexion and extension and slight lateral flexion) [11, 13]. The sagittal diameter of the foramen magnum is an important landmark in symptomatic patients. In addition to the 0s odontoideum in this patient there was a forward dislocation of the atlas and assimilation of the atlas and occipital bone, which gave a very nar- row spinal channel. Chiari I malformation and atlanto-occipital assimilation are both fairly uncommon conditions. Complete fusion is more common than the incomplete while multiple variations of partial assimilation have been reported and may involve any aspect of atlanto-occipital articulation.The most probable cause of the occipitalisation is a congenital disorder.This condition is first, described by BI= Basilar invagination, AOA= Atlanto-occipital assimilation, OCH= Occipital condyle hypoplasia, PP= Ponticulus posticus, CAAA= Complete atlantoaxial assimilation Table 3: Associated anomalies with CVJ Associated anomaly Number Percentage C6-C7 Fusion 2 28.5 Bilateral cervical rib 1 14.2 T2-T3 Fusion 1 14.2 Anatomy of the CCJ. It is also known as occipito-cervical synostosis, occipitalisation of atlas and atlanto-occipital fusion [2-3]. DEFINITION The atlanto-occipital joint (articulation between the atlas and the occipital bone) consists of a pair of condyloid joints. In addition to the 0s odontoideum in this patient there was a forward dislocation of the atlas and assimilation of the atlas and occipital bone, which gave a very nar- row spinal channel. Cervicocranial syndrome. In a recent study of 300 patients with cervical spine trauma, 30% of injuries were located between the occiput and C2. Atlantooccipital assimilation is defined as the complete or partial fusion of the caudal portion of the occiput with the cranial portion of the atlas. The cause of the excessive tissue formation in this case was attributed to a congenital anomaly, atlanto-occipital assimilation with secondary altered biomechanics in the craniocervical junction. Atlanto-occipital subluxation commonly occurs in patients with Down syndrome. [9] Sadler TW. A limbus vertebra is a well-corticated unfused secondary ossification center, usually of the anterosuperior vertebral body corner, that occurs secondary to herniation of the nucleus pulposus through the vertebral body endplate beneath the ring apophysis (see ossification of the vertebrae).These are closely related to Schmorl nodes and should not be confused with limbus fractures or infection. There is fusion of the anterior arch of the atlas with clivus and posterior arch with occiput on the right side, identical with complete atlanto-occiptal assimilation on the right side. Sakai K(1), Tsutsui T. Author information: (1)Department of Neurosurgery, Kochi Prefectural Aki Hospital. Such change causes basilar invagination and widening of the atlanto-occipital joint axis angle. The spinal cord was thus compressed between the fibrous pannus behind the odontoid process and the posterior arch of the . The incidence of retro-odontoid pseudotumor in each group was analyzed. It is possible for a human to survive such an injury; however, 70% of cases result in immediate death.It should not be confused with atlanto-axial dislocation, which describes ligamentous separation between the first and second cervical . (1) It was first described by Rokitansky in 1844 and was first demonstrated by Schuller roentgenographically in 1911. Atlanto-occipital assimilation is estimated to be about 0.08-3% in the general population. Seoul National University College of Medicine Home. A 65-year-old woman with a history of rheumatoid arthritis presented with a rare case of acquired. Occipitalization of the atlas - atlanto-occipital assimilation People will report to us a diagnosis of occipitalization of the atlas - atlanto-occipital assimilation. In this case report, an atlanto-occipital assimilation altered the biomechanics of the cervical spine, causing chronic mechanical stress on the transverse ligament and subsequently the development of a retro-odontoid pseudotumor. In a small number of cases, the disruption of this merging process may result in atlanto-occipital assimilation. The final study population was categorized according to their underlying diseases (rheumatoid arthritis [RA], os odontoideum, atlanto-occipital assimilation, dens fracture, AAI of unknown cause, etc.) Even though cranio-vertebral anomalies are common and are often seen in morphological and clinical and radiological studies, atlanto-occipital fusion is a rare congenital anomaly seen at the . atlanto-occipital dislocation, and plain radiographic findings. This joint comes in relation to the important structures in the region like vertebral arteries, spinal arteries, medulla oblongata and spinal cord. Overpressurization promotes the migration of air to soft tissues and joints and predisposes to an increase in intraosseous pressures through venous networks that lead to bone ischaemia and cavitation promoting migration to vertebral structures, facilitated by a preexistence of atlanto-occipital assimilation,,,,,. Synonym(s): articulatio atlantooccipitalis, atlanto-occipital articulation. This syndrome can be identified by confirming cervical bone shifts, collapsed cervical bones or misalignment of the cervical bone leading to . The diagnosis of this condition in a pulmonology department is even rarer. Atlanto-occipital assimilation is the fusion of the atlas (C1) to the occiput and is one of the transitional vertebrae. J Neuro Sci 154:229-231. Most of the abnormalities cause no typical symptom, but some patients have neurological problems which correlate with radiological findings. Chiari malformation and . Radiculopathy, occipito-atlanto-axial region Billable Code. 5. Atlas assimilation may include alteration of the atlanto occipital articulation or narrowing of the spinal canal. It is so serious, because when the . The occipital nerves are a group of nerves that arise from the C2 and C3 spinal nerves. 2Black (Maclaughlin) S, Radius 194 202 4.04 Feil and Dandy-Walker syndromes can cause limb stunting. The third type corresponds to a congenital malformation of the atlanto-occipital region ("assimilation of the atlas"). Craniocervical syndrome also called Barre-Lieou syndrome, craniocervical junction syndrome or posterior cervical sympathetic syndrome, is an entity whose symptoms includes vertigo, cephalea, tinnitus, facial pain, otalgia, dysphagia, pain of the carotid artery are thought to be caused by cervical factors 1).Craniocervical syndrome has not been agreed as a neurological . Even though assimilation of the atlas is the most common Atlantooccipital assimilation is a partial or complete congenital fusion between the atlas and the base of the occiput. Atlanto-occipital fusion is a rare anomaly of the craniocervical junction which may be congenital or acquired. The complete fusion of the atlas is more common than the incomplete . 2 The articles were also reviewed using the following criteria for inclusion in treatment: human survivors, type of traumatic AOD, management, and outcome. This is called basilar impression or secondary basilar invagination. Articulation between the superior facets of the atlas and the condyles of the occipital joint. and age (adult and pediatric groups). It can present as totally asymptomatic accidental finding or can be a cause behind major neuro-vascular compression. The posterior arch shows incomplete fusion centrally, failing to form posterior tubercle. Atlas occipitalization or atlanto-occipital assimilation (AOA) is one of the most common osseous congenital anomalies of the craniovertebral junction. 10th Ed., Baltimore, Lippincott William and Wilkins. Altered mechanics can cause congenital torticollis or predispose the CVJ to degeneration and potential instability, resulting in acquired torticollis. We present the case of a 37-year-old woman who presented with neck and occipital pain, episodic neck stiffness, and dizziness. M54.11. Osseous Abnormalities: Atlanto-Occipital Assimilation . The pathogenesis is degeneration of the transverse ligament due to chronic mechanical stress. The embryonic process leading to this malformation is discussed. [10] reported a case of atlanto-occipital fusion with unusual neurological symptoms. 2008; 30: 149-152. Multiple anomalies in CVJ with atlanto occipital assimilation, partial fusion of C1-C2 vertebrae, hemi vertebra with fusion of right C4-C5 level along with C3-C4 spinal cord hyperintensities: Interventions done before diagnosis: Anticholinergics, Benzodiazepines, Dopamine blocker, Botulinum toxin, none: Two sittings of botulinum toxin . Bow hunter's stroke associated with atlantooccipital assimilation--case report. The embryonic process . Langman's Essential Medical Embryology. The final study population was categorized according to their underlying diseases (rheumatoid arthritis [RA], os odontoideum, atlanto-occipital assimilation, dens fracture, AAI of unknown cause, etc.) The main etiologies of BI are clivus (basiocciput) hypoplasia, occipital condyle hypoplasia, atlas hypoplasia, atlanto-occipital assimilation and congenital atlanto-axial instability [14, 15, 28, 29]. The prevalence of a Chiari I malformation is estimated to be in the range of 1 per 1,000 to 1 per 5,000 individuals. Chiari malformation secondary to atlantoaxial vertical subluxation, associated with congenital atlanto- occipital assimilation. Association of atlanto-occipital assimilation with the occurrence of severe neurological symptoms resulting from upper spinal cord compression remains rare in the literature. Jayanthi et al 5 The causes include basioccipital hypoplasia, occipital condyle hypoplasia, atlanto-occipital assimilation and various neural abnormalities including Chiari malformations ( images 13-18), and may also occur as a result of the bone softening seen in osteogenesis imperfecta ( Image 19&20) , muchopolysaccharidosis <i>Methods</i>. The prevalence ranges from 0.08-3 percent of the general population. Tuberculosis of the CV junction is an important cause of spastic quadriparesis in endemic region. It is also known as assimilation of atlas, occipitocervical syntosis or atlanto-occipital fusion. atlanto-axial osteosynthesis. • Chiari I malformation (CMI) and atlanto-occipital assimilation are both fairly rare conditions. Assimilation of the atlas may be partial or complete. atlanto-occipital fusion ranges from 0.14 to 0.75% of the population, with both sexes being equally affected.2 Fusion of the atlas may be partial or complete, with the former being the most common as multiple variations of partial assimilation have been reported that may involve any aspect of atlanto-occipital articulation. Syringomyelia and tetraparesis improved immediately after posterior fossa decompression and. Fusion of the atlas to the occiput in atlanto-occipital assimilation causes the mechanical stress during movement of the head to be transferred to Atlanto-occipital dislocation (AOD) is a rare injury with high morbidity and mortality which typically occurs as a result of high-energy blunt trauma, particularly in cases of motor vehicle collisions. • The most frequent clinical presentation is a paroxysmal headache or neck pain. 6. In one patient (Case 10) the condition was considered to be stable. Note the high position of the odontoid relative to the foramen magnum, platybasia, and atlanto-occipital assimilation [Upward displacement of the odontoid into the foramen magnum ] Basilar impression is the designation for an abnormal relationship between upper cervical spine and base of skull, caused by upward displacement of the odontoid into . These structures are approximated at two major joints, the atlanto-occipital and atlantoaxial joints. Cough-associated syncope is an unusual presenting symptom. The prevalence of CMI is estimated to range between 1/1,000 to 1/5,000 individuals and the incidence of atlanto-occipital assimilation varies from 0.08 to 3.6%. Motabagani and Surendra (2006) [3] quoted in their paper that, it was first described by Rokitansky in 1844 and Schuller in 1911 Clinically fusion of the atlas with the lower part of the occiput is known as Atlanto-occipital assimilation or atlas occipitalization, which can be either partial or complete depending upon the extent of fusion. [2] There are three major occipital nerves in the human body: the greater occipital nerve (GON), the lesser (or small) occipital nerve (LON), and . We report an extremely rare case of a traumatic dislocation of the atlas from the axis (C1 from C2), which lead to internal decapitation in a patient with atlantooccipital assimilation. Another type of "basilar impression" is seen in cases of long standing, increased intracranial pressure causing atrophy of the cranial capsule (hydrocephaly, oxycephaly). The most distinctive characteristic of this bone is strong odontoid process, the dens. When occipital condyles are normally developed, the skull base descends medially. Understanding the anatomy of the CCJ is essential to adequately evaluate the patient for potential injuries. Atlanto-occipital dislocation, orthopedic decapitation, or internal decapitation describes ligamentous separation of the spinal column from the skull base. Total or partial assimilation of the atlas may be noted with the latter being the most common [3]. The main etiologies of BI are clivus (basiocciput) hypoplasia, occipital condyle hypoplasia, atlas hypoplasia, atlanto-occipital assimilation and congenital atlanto-axial [scielo.br] instability 12, 13, 14. It is one of the most common osseous anomalies of the craniovertebral junction. About 28 patients got improved, Atlanto-occipital joint is important joint present at the craniocervical junction. [10, 20, 29] It is correlated with . Surg Radiol Anat. with congenital deformity in the occipital cervical junction, such as dens malformation, assimilation of the atlas, Klipper- Feil syndrome and basilar invagination. It should be considered as a potential diagnosis in young patients, immune-compromised persons presenting with occipital neuralgia, upper cervical cord compression or atlanto-axial subluxation either occurring spontaneously or after a trivial injury. In the case of our patient, atlanto-occipital assimilation may have been the cause of very gradual formation of a periodontoid pseudo tumor due to abnormal mobility of the cervico-occipital hinge and inflammatory phenomena. Also known as occipitalization of the atlas (C1), is characterized by partial or total fusion of C1 to the occipital bone [1]. Atlanto-occipital subluxation commonly occurs in patients with Down syndrome. Classification According to Direction of . It is thought to affect males and females equally. A decompression by resec- The atlanto-occipital joints are synovial socket-type joints; the sockets are shallow in infancy and deepen with age. [9] reported that three patients with atlanto-occipital fusion have had cervical pain and two patients had tonic or clonic convulsions. Iwata et al. If you have received this diagnosis you know that your body is creating its own bone fusion to try to stabilize your cervical spine because your cervical vertebrae (the C2) is . The bones of the neck that are affected are cervical vertebrae (C1 - C7).
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