Nontraumatic Atlantoaxial Rotatory Subluxation: A Rare Complication of COVID-19 in Elderly Patient

Introduction: Nontraumatic Atlantoaxial Rotatory Subluxation (NAAS) or Atlantoaxial Rotatory Subluxation (AARS) without trauma or concomitant bone pathology was first described by Sir Charles Bell in 1830 in a patient with syphilis and pharyngitis, who developed lethal outcome due to spinal compression. The syndrome was named after Grisel who described two cases of pharyngitis and atlantoaxial subluxation in 1951. However, this is an uncommon condition of uncertain etiology characterized by NAAS, usually seen in children secondary to an infection and inflammation in the head and neck region or otolaryngeal procedures. Patients generally complain about neck stiffness and pain, and sometimes dysphagia may occur. Diagnosis is established based on the clinical and radiological findings. The etiopathogenesis and the underlying pathomechanics have not been clearly explained. A hematogenous spread of infection from the posterior pharynx to the cervical spine, according to the recent literature, with hyperemia and abnormal relaxation of the atlantoaxial ligaments is a widely accepted theory. The vascular plexus providing the drainage of poster superior pharyngeal area is responsible. The periodontoid plexus is connected with posterior nasopharyngeal veins via the pharyngovertebral vein. Any infective embolism may spread from superior pharyngeal area to upper cervical joints due to this plexus which does not have any lymph node, thus providing an anatomical explanation for the atlantoaxial hyperemia reported in Grisel's syndrome; however, the clinical picture and complications of COVID-19 are unclear until now and every day new symptoms and findings are reported as early and late complications. Methodology: An 86-year-old male presented to our clinic in KKT, Jeddah from Yanbu (which is 330 km away) with a complaint of neck stiffness in anterior-lateral position after recovery from COVID-19 six months back. The patient was treated at home without admission in hospital. He had no history of trauma. During the physical examination, the patient's neck was stiff, and there was neck pain with palpation and left-sided torticollis. The patient’s weight was 58 kg, height 160 cm, blood pressure 140/75 mmHg with a pulse rate of 72, SPO2 95, and BMI 22.7. There was no sign of fever or any type of inflammation in his body. The patient had previously consulted neurosurgeons at three different hospitals in Jeddah and was advised a medication with a cervical collar for a clinical follow-up. After three weeks of follow-up, they advised him to start physiotherapy for one month with no benefits seen in clinical finding, the patient then came to us. Direct radiogram of the cervical region showed suspicious findings at the atlantoaxial joint. Anterior view of the cervical radiography revealed tilted position of the head over neck, while the lateral view showed no thickening of the parapharyngeal soft tissue. The distance between the axis and dens was within normal values for his age (ADI = 3–4 mm). This confirms our diagnosis of type 1 atlantoaxial subluxation according to Fielding and Hawkins classification. Result: Based on the patient’s history and physical examination, there was no infection or neck operation causing an inflammation in the neck except the COVID-19 six months prior to the neck stiffness. With this case, we would like to highlight that atlantoaxial subluxation should be kept in mind when neck stiffness is seen in elderly patient without history of trauma but with a history of COVID-19 as a rare complication. Conclusion: NAAS is a rare but major complication that can often go unnoticed in its early and late phase, which can be a major cause of morbidity following COVID-19 infections, thus early recognition and diagnosis is mandatory especially in adult and elderly patients recovering form COVID-19.

Extended tulip cervical reduction screws to restore alignment in traumatic atlantoaxial dislocation after type 3 odontoid fracture: illustrative case

BACKGROUND Traumatic atlantoaxial rotatory subluxation after type 3 odontoid fracture is an uncommon presentation that may require complex intraoperative reduction maneuvers and presents challenges to successful instrumentation and fusion. OBSERVATIONS The authors report a case of a 39-year-old female patient who sustained a type 3 odontoid fracture. She was neurologically intact and managed in a rigid collar. Four months later, she presented again after a second trauma with acute torticollis and type 2 atlantoaxial subluxation, again neurologically intact. Serial cervical traction was placed with minimal radiographic reduction. Ultimately, she underwent intraoperative reduction, instrumentation, and fusion. Freehand C1 lateral mass reduction screws were placed, then C2 translaminar screws, and finally lateral mass screws at C3 and C4. The C2–4 instrumentation was used as bilateral rod anchors to reduce the C1 lateral mass reduction screws engaged onto the subluxated atlantodental complex. As a final step, cortical allograft spacers were inserted at C1–2 under compression to facilitate long-term stability and fusion. LESSONS This is the first description of a technique using extended tulip cervical reduction screws to correct traction-irreducible atlantoaxial subluxation. This case is a demonstration of using intraoperative tools available for the spine surgeon managing complex cervical injuries requiring intraoperative reduction that is resistant to traction reduction.

A Concomitant Occurrence of the Atlantoaxial Subluxation with Rare Vertebral Formation and Segmentation Defects

An atlantoaxial subluxation from the unstable Os odontoideum by the failure of proper integrations between the embryological somites might be a commonly reported pathology. However, its suspicious origin or paralleled occurrence with other congenital anomalies of vertebral body might be a relatively rare phenomenon. The authors present two cases, who simply presented with clinical signs of prolonged, intractable cervicalgia without any neurological deficits, revealed this rare feature of C1–2 subluxation from the unstable, orthotropic type of Os odontoideum that coincide with congenitally fused cervical vertebral bodies between C2–3. Surprisingly, in one case, when traced from the lower cervical down to the thoracic-lumbar levels during the preoperative work-up process, was also compromised with multi-level butterfly vertebrae formations. Presented cases highlight the association of various congenital vertebrae anomalies and the rationale to fuse only affected joints.

Surgical management of traumatic atlantoaxial subluxation in two cats

Case series summary We describe here the surgical management of two pure breed cats with traumatic atlantoaxial subluxation. One cat was ambulatory tetraparetic on presentation and the second was tetraplegic, both with cervical spinal pain and acute onset of paresis with subsequent deterioration. MRI was performed in both cases, demonstrating spinal cord injury. Flexed lateral cervical radiographs were needed to confirm atlantoaxial subluxation in one case. CT was performed for surgical planning and surgical stabilisation was achieved with threaded pins and polymethyl methacrylate (PMMA) cement; odontoidectomy was required in one case. Both cats showed improvement postoperatively, with no complications or deterioration seen. Following surgery, one cat made a complete recovery; however, the second cat retained significant deficits. Relevance and novel information We present the first report of surgically managed atlantoaxial subluxation of traumatic aetiology in cats, and report its occurrence in two novel breeds for this disease, Ragdoll and Persian. One case required odontoidectomy due to previous fracture and malunion of the odontoid process of the axis; both cases underwent surgical stabilisation of the atlantoaxial joint utilising multiple threaded pins and PMMA cement without transarticular implants – a technique that has not been previously reported in cats.

Unusual presentation of an unusual disease: A very delayed diagnosis of Grisel’s syndrome

Grisel’s syndrome (GS) is a rare syndrome which refers only to non-traumatic atlantoaxial subluxation. This syndrome predominantly occurs in young children following an upper respiratory infection or otolaryngologic procedures. An eight-year-old girl with a delayed diagnosis of GS was admitted to our outpatient clinic with complaints of painful torticollis and neck stiffness. Three-dimensional computed tomography revealed rotatory atlantoaxial subluxation. After consulting with the neurosurgery department, the patient underwent surgery. The significance of this patient was that she was unable to be diagnosed early and atlantoaxial subluxation remained hidden for five years without any complications. In conclusion, this rare case highlights the importance of delayed diagnosis of GS and clinicians should be aware of this syndrome.