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Year : 2022  |  Volume : 9  |  Issue : 2  |  Page : 115-119

Degenerative occipitocervical dislocation – Uncommon entity at extreme of Life: A case report and review of literature

Department of Neurosurgery, LTMMC and LTMG Hospital, Mumbai, Maharashtra, India

Date of Submission20-Nov-2021
Date of Acceptance31-Dec-2021
Date of Web Publication31-May-2022

Correspondence Address:
Batuk D Diyora
Department of Neurosurgery, LTMMC and LTMG Hospital, Sion, Mumbai - 400 022, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joss.joss_25_21

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Traumatic occipitocervical dislocation (OCD) has been reported numerous times both in children and adults. Nontraumatic OCD is rare. An 85-year-male referred with 2 months history of motor and sensory complaints in all four limbs. There was no history of trauma. Neck movements were restricted. A lateral radiograph of the cervical spine was suggestive of extensive degenerative changes in the cervical spine. Computed tomography scan of the cervical spine revealed posterior OCD with degenerative changes. Magnetic resonance imaging revealed compressive myelopathy at the cervico-medullary junction. Posterior occipitocervical fixation was performed. His neurological symptoms significantly improved. The procedure resulted in an excellent immediate and long-term clinical outcome.

Keywords: Atlantooccipital dislocation, cervical spine, degenerative spine disease, nontraumatic occipitocervical dislocation, occipitocervical dislocation

How to cite this article:
Diyora BD, Dhall G, Patel M, Chhajed R, Devani K, Purandare A. Degenerative occipitocervical dislocation – Uncommon entity at extreme of Life: A case report and review of literature. J Spinal Surg 2022;9:115-9

How to cite this URL:
Diyora BD, Dhall G, Patel M, Chhajed R, Devani K, Purandare A. Degenerative occipitocervical dislocation – Uncommon entity at extreme of Life: A case report and review of literature. J Spinal Surg [serial online] 2022 [cited 2022 Jul 7];9:115-9. Available from: http://www.jossworld.org/text.asp?2022/9/2/115/346359

  Introduction Top

Occipitocervical dislocation (OCD) is uncommon as compared to atlantoaxial dislocation. Traumatic OCD has been reported numerous times both in children and adults.[1],[2] Reports of nontraumatic OCD are very few and include Articular tropism, Down's syndrome, Rheumatoid arthritis, tumor at the atlas, infection in the head or neck region, and torticollis as its causes.[3],[4],[5],[6],[7] We report a case of posterior OCD in an 85-year-old male due to degenerative disease at the occipitocervical (OC) joint and review the literature on the subject. This case report aims to add further to the available literature of this infrequently encountered entity.

  Case Report Top

An 85-year-old male patient was referred with a history of progressive weakness in all four limbs for 2 months. He had tingling and numbness in all four limbs for the past 1 month, which had progressively increased since the onset. He had severe neck pain with significant restriction of neck movements. There was no history of trauma, rheumatoid arthritis, psoriasis, neck swelling, tumor, infection, other joints involvement, or any significant familial history. Neurological examination revealed hyperreflexia and spasticity in all four limbs. Power was MRC Grade 2/5 in both upper and lower limbs. Both the handgrips were very weak. Joint position and vibration senses were affected in all four limbs. He had mild impairment of touch, pain, and temperature sensations in all four limbs. Bowel and bladder functioning were unaffected.

A lateral radiograph of the cervical spine was suggestive of extensive degenerative changes in the cervical spine [Figure 1]a, [Figure 1]b, [Figure 1]c. Computed tomography (CT) scan of the cervical spine was suggestive of posterior OCD with instability and degenerative changes in the form of sclerosis, osteophytes formation, and reduced joint space [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d. The Basion-Axial interval (BAI) was 0.7 mm posteriorly, Basion-Dental interval (BDI) was 0.3 mm, and Power's ratio was 0.7. Magnetic resonance imaging (MRI) of the cervical spine showed compression at the cervico-medullary junction with obliteration of both anterior and posterior cerebrospinal fluid spaces. There was a fusion of the C4-C5 vertebrae along with gross misalignment of the OC region [Figure 3]a, [Figure 3]b, [Figure 3]c.
Figure 1: Lateral radiograph of the cervical spine extension (a), flexion (b) and neutral (c) views showing extensive degenerative changes in the cervical spine. Furthermore degenerative changes noted at craniovertebral junction

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Figure 2: Computed tomography scan of the craniovertebral junction showing posterior occipitocervical dislocation with instability in the lateral view (a). Degenerative changes in the form of osteosclerosis, osteophytes formation, and reduced joint space at the level of occiput, C1 and C2 vertebral body in lateral (b) and coronal views (c and d)

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Figure 3: Magnetic resonance imaging of the cervical spine sagittal view T2 weighted image (a) and T1 weighted images (sagittal view b, and axial view c) showing compression at the cervico medullary junction with obliteration of both anterior and posterior cerebrospinal fluid spaces along with gross malalignment of the occipitocervical region. There was also fusion of the C4 - C5 vertebrae

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Because of posterior OCD, cervical traction in extension was applied. Immediately after applying cervical traction, the patient's sensory symptoms and motor power improved. Because of significant neurological improvement after traction and likely benefits of fixation, an operative intervention was decided.

The patient underwent OC fixation with occipital plates, C1 and C2 lateral mass/pars screws and rod system in the prone position. Bone graft was harvested from the iliac crest and placed over the roughened occipital bone and C1/C2 lamina. The postoperative course was smooth. There was a significant improvement in his sensory and motor symptoms. At the time of discharge on the seventh postoperative day, he was pain-free and power in all four limbs was Grade 4 MRC, without any significant sensory deficits. Postoperative lateral and AP radiographs showed fixation of the OC junction [Figure 4]a and [Figure 4]b. MRI showed realignment of the OC junction and significant decompression of the cervico-medullary junction and the subaxial cervical spine [Figure 5]a, [Figure 5]b, [Figure 5]c, [Figure 5]d.
Figure 4: Postoperative lateral (a) and AP (b) radiographs showing fixation of the occipitocervical junction

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Figure 5: Magnetic resonance imaging of cervical spine sagittal view T1-weighted image (a) and T2 weighted image (b) as well as axial view T1-weighted image (c) and T2-weighted image (d) showing very good alignment of the occipitocervical junction and significant decompression of the cervico-medullary junction and the sub axial spinal cord

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At 6 months' follow-up, the patient could walk with a walker and was independent in age-appropriate daily routine activities. He had normal power, no sensory symptoms, with good bowel bladder control.

  Discussion Top

Traumatic OCD has been reported in the literature and often with fatal outcomes.[6],[7],[8] Nontraumatic OCD is extremely rare. Abumi et al. reported a case of nontraumatic OC joint dislocation due to articular tropism.[9] Takechi et al. reported it in a 36-year-old female with bilateral flat OC joints.[10] Wu et al. reported it in a 47-year-old male due to atlantoaxial instability.[11] Washington reported a nontraumatic atlantooccipital and atlantoaxial dislocation in an 11-year-old girl due to pharyngitis, and they agreed that an inflammatory process of the joint and its periarticular structures was associated with the OC instability.[4] To our knowledge, nontraumatic OCD due to degenerative disease has not been reported in the published literature. The pathophysiology of nontraumatic OC complex instability is still unclear. Wiesel et al. proposed that high cervical fusion produces more stress at the OC articulation. This stress gradually results in the stretching of the ligaments and gives rise to hypermobility.[12] However, there was no hypermobility or dislocation of the OC joint in 150 cases of Klippel–Feil syndrome reviewed by Georgopoulos et al.[13] According to the previous case reports, OC instability is related to Down's syndrome, rheumatoid arthritis, tumor of the atlas, infection in the head or neck, and torticollis.[1],[2],[3],[4],[5] The patient in our study was excluded from all the diseases mentioned above.

Presentation varies and most of these patients are diagnosed based on radiological findings. Lateral radiograph of the cervical spine is usually the first investigation, although, may or may not be helpful due to the parallax effect at OC junction and overlapping mastoid air cells.[14] Radiographs are evaluated through parameters such as Power's ratio, X-line method, condylar gap method, BDI, and BAI.[15],[16] These radiographic findings can be quite dramatic, subtle, or even absent.[17],[18],[19],[20] In later cases, careful evaluation of surrogate markers of OCD, such as abnormally prominent prevertebral soft tissues, retropharyngeal emphysema, or an increase in the interval between the posterior elements of C1 and C2, proves useful.[21],[22] Powers' ratio compares measurements relating the skull base to C1. Normal values are typical < 0.9. Anterior OCD is suspected when it exceeds 1.[15],[16],[19],[23],[24] Power's ratio is insensitive to vertical distraction and posterior dislocation injuries. Basion-axial interval or BAI is considered abnormal when there is an anterior displacement of 12 mm or more or a posterior displacement of 4 mm or more between the basion and posterior C2 line. The BDI or BDI is abnormal when displacement between the basion and the dens of more than 10 mm in adults or more than 12 mm in pediatric patients. When combined with the BDI, BAI is known as the Harris method.[15],[24],[25],[26] The BAI-BDI method's sensitivity is only 50.5%, and a large portion of patients will require additional confirmatory imaging.[15],[23]

MDCT has replaced X-ray imaging as the imaging modality of choice in an awake symptomatic patient. It is proven to be more sensitive and specific than cervical spine radiographs in detecting spinal column injury.[27] Besides being better at diagnosis, it has an additional advantage of providing the three-dimensional visualization of the OC and AA joints to assess the subluxation directly. Even the slightest asymmetry or distraction of the atlanto-occipital joints should be viewed with suspicion because these joints usually have a 2 mm or less displacement tolerance.[14],[23],[28]

A radiograph of the cervical spine can also help in the initial diagnosis of the degenerative disease. It shows the presence of sclerosis, osteophytes formation, reduced joint space, and joint fusion. CT scan and MRI imaging further play a pivotal role in diagnosis and decision-making. MRI has a vital role in finding myelopathic changes in the cord and soft-tissue changes around the joint. As described below, the horn's grading system considers both MRI and CT findings while grading OCDs.

Traynelis et al. classified OCD into three types based on the direction of dislocation of the occiput relative to the C1.[22] Type I OCD consists of anterior displacement of the occiput to C1; type II is primarily a longitudinal distraction with separation of the occiput from the atlas, and type III OCD exists when the occiput is dislocated posteriorly from C1. Bellabarba et al. published a three-stage classification, also known as the Harborview classification system, to reflect injury severity and quantify the stability of the OC junction-associated therapeutic implications.[29] Stage I injury is defined as a stable, minimally, or undisplaced OC injury in which there is sufficient preservation of ligamentous integrity to allow for nonoperative treatment. A stage 2 injury represents partially or completely spontaneously reduced bilateral craniocervical dissociation involving minimal displacement (Harris lines within <2 mm beyond the upper limit of normal). A stage 3 injury denotes a highly unstable injury defined by gross OC misalignment (Harris lines >2 mm beyond acceptable limits), requiring internal fixation. Horn et al. proposed another grading scale based on MRI and CT findings.[30] Grade I injuries are indicated by normal CT findings to established diagnosis methods (the Power ratio, BDI, BAI–BDI, and X-line) but have moderately abnormal MR imaging findings (high posterior ligaments or occipitoatlantal signal). These can often be managed conservatively as there is no evidence of genuine OCD on CT. In Grade II injuries, there is a minimum of one abnormal finding on CT based on established diagnostic criteria, or there are grossly abnormal MRI findings in the OC joints, tectorial membrane, alar ligaments, or cruciate ligaments, often necessitating surgical intervention.[30]

Traction use in the treatment of patients with OCD is controversial. It is due to contradictory opinions in various studies and associated with a 10% risk of neurological deterioration.[19],[31],[32],[33],[34] While not routinely recommended, the goal of traction, if used, is mainly to decompress the neural elements by realigning the bony structures and should be best reserved for instances of Type I (anterior), Type III (posterior), and lateral OCDs in patients with neurological deficit.[22] The use of manual traction has fallen out of favor at most centers and to diagnose unstable injuries. Axial traction should be avoided in Traynelis Type II OCD cases. It reproduces the distractive mechanism of injury and can be associated with a possible risk of damaging the spinal cord, medulla, or vertebral arteries with the risk of causing further neurological deterioration.[22]

It is well accepted that instability at the OC region should be stabilized with surgery. The surgery of choice in OCD is posterior OC fusion.[35] Abumi et al. applied posterior atlantooccipital fusion using wiring with a bone graft.[9] Takechi et al. applied posterior atlantooccipital fusion using the Axon system (Synthes) performed with a bone graft from the posterior iliac bone.[10] Wu et al. applied OC (C0-C2) fusion using the OC fusion system.[11] Ogihara et al. proved the halo vest's efficacy in preoperative reduction and considered if the definitive procedure is postponed due to the patient's severe general health status.[36] We used a posterior OC plate, rod, and screw fixation system Smit Medimed Pvt. Ltd. (SMPL). Immediate postoperative and follow-up imaging found noticeable improvement. The patient was free from pain symptoms with significant improvement in both motor strength and sensations.

  Conclusion Top

OCD is a rare entity that almost always results from trauma. Nontraumatic causes have been reported rarely. Degenerative disease at the OC junction can lead to OCD. A high index of suspicion is needed as initial history and radiographs may not provide sufficient clues to the diagnosis. High-resolution CT helps in better visualization of the craniocervical junction. It helps in greater delineation and classification of the pathology. MRI shows the cord signal abnormalities and soft-tissue changes, which help in further grading and decision-making. We feel that traction could help decompress the neural elements by realigning the bony structures and should be reserved only for Type I (anterior), Type III (posterior), and lateral OCD s-traumatic or nontraumatic. Posterior OC fusion and instrumentation remain the surgical treatment of choice, as recommended by the AANS/CNS joint guidelines committee. In OCD cases, posterior fixation with O-C1-C2 with bone graft results in good long-term stability.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

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