SEPTIC ARTHRITIS OF THE Cl-C2 LATERAL FACET JOINT AND TORTICOLLIS: PSEUDO-GRISEL S SYNDROME

Transcription

1 84 BRIEF REPORT SEPTIC ARTHRITIS OF THE Cl-C2 LATERAL FACET JOINT AND TORTICOLLIS: PSEUDO-GRISEL S SYNDROME JAMES T. HALLA, JOHNNY BLIZNAK, JOE G. HARDIN, and SAM FINN We present the case of a 76-year-old man who experienced the sudden development of fever, rightsided neck pain and stiffness, and torticollis. A soft tissue mass was noted on the right side of his neck, but his head was tilted to the left. Computed tomography scans (with reformatted sagittal and coronal images) of the patient s cervical spine revealed destructive changes of the right lateral masses of C1 and C2 and the clivus, and a well-delineated periodontoid soft tissue mass (confirmed by magnetic resonance imaging). After the second episode of right-sided hemiparesis, he underwent transoral surgical exploration, with anterior decompression and odontoidectomy. Histologic examination of the surgical material revealed granulation tissue, fibrosis, and chronic inflammation, consistent with abscess formation with invasion and compression of the spinal cord and bone. This case suggests that nonreducible rotational head tilt to the side opposite the side of lateral mass collapse should raise the suspicion of a possible infection. Pyogenic infections of the spine are usuall y attributed to pyogenic vertebral osteomyelitis, which rarely affects the cervical spine (1-6). Involvement of From the Department of Radiology, Hendrick Medical Center, Abilene, Texas, the Department of Medicine, University of South Alabama, Mobile, and the Department of Medicine, University of Texas Southwestern Medical School, Dallas. James T. Halla, MD: Abilene, TX: Johnny Bliznak, MD: Department of Radiology, Hendrick Medical Center; Joe G. Hardin, MD: Professor of Medicine, University of South Alabama: Sam Finn, MD: Assistant Professor of Neurosurgery, University of Texas Southwestern Medical School. Address reprint requests to James T. Halla, MD, 1927 Pine Street, Abilene, TX Submitted for publication April 5, 1990; accepted in revised form July 7, the occipitoatlantoaxial (OAA) complex and odontoid process is even more rare (7-12). Torticollis as a manifestation of cervical spine infection has been reported, especially in children (11,13), and it may take the form of Grisel s syndrome (lateral subluxation at C 1-C2, but no radiographic destruction) or it may be secondary to vertebral osteomyelitis (1,11,13-16). Nontuberculous bacterial involvement of the Cl-C2 lateral facet joints with torticollis has not been reported. We describe a patient who suddenly developed fever, torticollis, and, subsequently, an erosive arthropathy of the right lateral facet joint of Cl-C2 and the odontoid process with spinal cord compression. The patient was found to have a mass of infected granulation tissue. He was treated with transomal anterior decompression, odontoidectomy, and long-term antibiotics with good results. Case report. The patient, a 76-year-old man, had been healthy except for osteoarthritis of the hands and knees until January 1987, when he presented at another institution with a 3-day history of a crick in my neck. He had experienced progressive right-sided neck pain and stiffness, and he had a rotational head tilt to the left and a right-sided soft tissue neck mass at the angle of the jaw. He had no history of sore throat, hoarseness, or dysphagia. At admission, he was febrile (102 F) and confused, and he reported posterior and suboccipital neck tenderness and pain. Laboratory evaluation revealed mild leukocytosis (1 1,000 white blood cells [WBC]/mm3), and analysis of the cerebrospinal fluid (CSF) revealed 180 WBClpI (100% polymorphonuclear leukocytes), with normal glucose levels and no growth on routine cultures of CSF samples. Results of Arthritis and Rheumatism, Vol. 34, No. 1 (January 1991)

2 BRIEF REPORTS 85 A Figure 1. Computed tomography images of the patient s cervical spine. A, Lateral scout view, showing prevertebral soft tissue thickening from C1 through C3, decreased space between the occiput and the atlas, and slight vertical atlantoaxial subluxation. B, Coronal view, showing erosive and destructive changes of the odontoid process and the right atlantooccipital and atlantoaxial joints, with rotatory and lateral subluxation. The erosive changes of the left atlantooccipital and atlantoaxial joints are quantitatively less, but are not well demonstrated on this image. Inset, Coronal view of the atlantoaxial joint. B blood cultures were unremarkable. A routine radiograph of the cervical spine was interpreted as normal, and results of a computed tomography (CT) scan of the head and cervical spine (without reformatted images) were considered unremarkable. Magnetic resonance imaging (MRI) was not done. Cellulitis and/or acute parotitis were considered, and treatment with cefuroxime, intravenously, was begun, with apparent clinical improvement; oral antibiotics were continued for 2 weeks thereafter. Although he became afebrile, the neck pain increased, lateral rotation decreased, and head tilt to the left worsened. When fever recurred 5 weeks later, he was admitted to another hospital, where a technetium diphosphonate bone scan revealed increased uptake in the area of the craniocervical junction; however, results of a gallium scan were negative. After otolaryngologic consultation, subaxial neck exploration was performed to rule out a malignant process; this yielded negative findings. Cultures of surgical specimens were negative. Because of the negative findings at surgery, a rheumatologic consultation was obtained. CT scans of C 1-C2, with axial, reformatted sagittal and coronal images, and a scout view, showed erosive changes of the Cl-C2 lateral facet joints, more so on the right side, with rotatory and lateral subluxation and a prevertebral soft tissue mass (Figures 1A and B), although the patient had improved clinically. Three weeks later, however, the erythrocyte sedimentation rate (Westergren) was elevated. CT scans taken at that time revealed further destructive changes of the right lateral masses of C1 and C2 and the clivus, and a welldelineated periodontoid soft tissue mass was noted (Figures 2A and B). MRI confirmed these findings. The patient suddenly developed right-sided hemiparesis and truncal paresthesias, and he was referred to the neurosurgical unit of Parkland Hospital, Southwestern Medical School (Dallas, TX). A cervical myelogram (with metrizamide contrast medium) and CT scan revealed subtle extradural compression of neural elements at the foramen magnum and a periodontoid soft tissue mass, but a diagnosis was still not apparent. Subsequently, a mass was

3 86 BRIEF REPORTS A B Figure 2. Computed tomography images taken 3 weeks after those shown in Figure 1. A, Sagittal view, showing a large periodontoid soft tissue mass, with destructive changes of the odontoid process and anterior arch of the atlas. Inset, Sagittal view of the atlantoaxialjoint. B, Coronal view, showing a rotational deformity to the left. Progressive destructive changes of the odontoid process, destructive changes at both the atlantooccipital and atlantoaxialjoints, with greater involvement of the right side than the left, and right lateral mass collapse are evident. Inset, Coronal view of the atlantoaxial joint. palpated transorally, and transoral drainage of the retropharyngeal area yielded 4 cc of purulent material, cultures of which grew Staphylococcus aweus. Blood cultures were repeatedly negative. The patient was treated with intravenous nafcillin and fitted with a Philadelphia collar, and his condition improved. He experienced less neck pain and there was stabilization of his neurologic status. However, he again developed right-sided hemiparesis, and transoral surgical exploration with anterior decompression and odontoidectomy were performed. No purulent material was obtained, but there was erosion of the odontoid process and bony destruction of the right lateral masses of C1 and C2 and the clivus. Histologic examination of the surgical material demonstrated granulation tissue, fibrosis, and chronic inflammation, consistent with abscess formation invading and compressing the spinal cord and bone. All cultures at this time were negative. Antibiotic therapy was changed to cloxacillin, and the patient was placed in halo traction. The patient s condition steadily improved. After 6 months, the halo traction was removed, and the cloxacillin was discontinued. CT scans taken at 1 year and 2 years of followup have shown stabilization of the changes (Figure 3). The patient remains well, and there are no signs of myelopathy or neck pain. Discussion. The OAA complex consists of a set of synovial articulations at the atlantooccipital, atlantoaxial, and atlantoodontoid joints. The joints work as a unit, but rotation occurs predominantly at the atlantoaxial joint, and lateral flexion occurs at the atlantoocipital joints. Involvement of this area, which has received considerable attention recently, is manifested by a characteristic clinical profile in both noninfectious inflammatory and noninflammatory arthropathies (16-2 1). This clinical profile includes nonreducible rotational head tilt (NRRHT), which is the characteristic physical finding in patients with lateral mass collapse, and the head tilt is always toward the side of the lateral mass collapse. The case reported here suggests that NRRHT to the contralateral side in a patient with destructive arthropathy should raise the possibility of infection. Pyogenic spondylitis is defined by Resnick as spinal osteomyelitis and disc space infection together (16). Pyogenic spondylitis of the OAA complex has rarely been reported, and such involvement may be tuberculous (22-24) or pyogenic (7-12). Tuberculosis

4 BRIEF REPORTS 87 Figure 3. Computed tomography image taken 2 years after those shown in Figures I and 2. There are destructive changes and fusion of the atlantooccipital joint, with some reconstitution of the distorted C2 lateral masses. There is marked irregularity of the CI-C2 articulations, with surgical absence of most of the odontoid process. These changes had remained stable for 2 years. Inset, Coronal view of the atlantoaxial joint. involving the atlantoaxial region is very rare but may be associated with torticollis; nontuberculous pyogenic spondylitis of the OAA complex is even more rare. Six adult patients with pyogenic spondylitis have been described (7-10,12), and all but 1 of them had a predisposing illness (3 had diabetes, 1 used intravenous drugs, and 1 had a history of peritonsillar abscess). Neck pain was of sudden onset in 3, gradual onset in 2, and prolonged in 1. Three patients were febrile, and none had clinical signs or symptoms of spinal cord impingement. Radiographic findings revealed involvement of the odontoid process alone in 2 patients, the body of C2 and the odontoid process in 2 patients, and the CI-C2 lateral facet joint, with lateral subluxation, and involvement of the odontoid process in 2 patients. No patient demonstrated torticollis on physical examination. Four patients had evidence of a retropharyngeal or peritonsillar abscess. Torticollis is a rotational deformity of the cervical spine resulting in malpositioning of the head and neck, and it results from a variety of causes (25). Torticollis secondary to nontuberculous pyogenic cervical osteomyelitis has not been associated with destructive changes in the OAA complex, although at- lantoaxial dislocation has been described in children (11,13). Grisel s syndrome occurs in children and is the concurrence of lateral atlantoaxial subluxation and torticollis, caused by localized infection of adjacent neck tissue that spreads via the lymphatic system or, more likely, the pharyngovertebral veins (1 1,13-16). While no destructive changes are noted radiographically in the OAA complex, hyperemic osteoporosis and ligamentous laxity are standard findings. Our patient had fixed torticollis, or NRRHT, with destructive changes at the OAA complex. Unlike previous descriptions in which NRRHT was toward the side of the lateral mass collapse, our patient had NRRHT toward the side opposite the lateral mass collapse. The diagnosis of septic arthritis and osteomyelitis of the OAA complex was complicated by the abnormal position of the patient s head. As we have reported elsewhere, CT with reformatted images proved a useful diagnostic tool for this region (20). The first CT scan performed on this patient did not include reformatted images and was compromised by the patient s abnormal, and painful, head position. The best procedure for early diagnosis of infectious or noninfectious disease of the Cl-C2 lateral facet joint is not yet clear. It is apparent that failure to recognize involvement of the OAA complex may delay diagnosis and have potentially serious consequences. Even though our experience with MRI is limited and we have been particularly pleased with the use of CT imaging of the OAA complex, MRI may be the preferred imaging method because of the excellent soft tissue contrast that is achievable (17). REFERENCES I. Forsythe M, Rothman RH: New concepts in the diagnosis and treatment of infections of the cervical spine. Orthop Clin North Am 9: , Stauffer RN: Pyogenic vertebral osteomyelitis. Orthop Clin North Am 6: , Frederickson B, Hansen Y, Olans R: Management and outcome of pyogenic vertebral osteomyelitis. Clin Orthop 131:16&167, Slagel SA, Skiendyielewski JJ, McMurry FG: Osteomyelitis of the cervical spine: reversible quadraplegia resulting from Philadelphia collar placement. Ann Emerg Med 144: , Musher DM, Thorsteinsson SB, Minuth JN, Luchi RJ: Vertebral osteomyelitis, still a diagnostic pitfall. Arch Intern Med 136: , Lowry LD: Osteomyelitis of the cervical vertebrae. Trans Pa Acad Ophthalmol Otolaryngol 30: , 1977

5 BRIEF REPORTS 7. Venger BH, Musher DM, Brown EW, Baskin DS: Isolated C2 osteomyelitis of hematogenous origin: case report and literature review. Neurosurgery 18: , Frank TJF: Osteomyelitis of the odontoid process of the axis. Med J Aust 1: , Rimolovski AB, Aronson SM: Abscess of medulla oblongota associated with osteomyelitis of odontoid process. J Neurosurg 29:97-101, Ahlback S, Collert S: Destruction of the odontoid process due to atlantoaxial pyogenic spondylitis. Acta Radiol 10:39&400, Sullivan AW: Subluxation of the atlantoaxial joint: sequel to inflammatory processes of the neck. J Pediatr 35: , Leach RE, Goldstein HH, Younger D: Osteomyelitis of the odontoid process. J Bone Joint Surg 49A: , Visudhiphan P, Chiemchanya S, Somburanasin R, Dheandhanoo D: Torticollis as the presenting sign in cervical spine infection and tumor. Clin Pediatr 21:71-76, Parke WW, Rothman RH, Brown MD: The pharyngovertebral veins: an anatomical rationale for Grisel s syndrome. J Bone Joint Surg 66A: , Odelberg-Johnson G: A case of cervical spondylarthritis after tonsillectomy. Acta Orthop Scand 2: , Resnick D: Osteomyelitis, septic arthritis and soft tissue infection: the axial skeleton, Diagnosis of Bone and Joint Disorders. Second edition. Edited by D Resnick, G Niwayama. Philadelphia, WB Saunders, Halla JT, Hardin JG, Vitek J, Alarcon GS: Involvement of the cervical spine in rheumatoid arthritis. Arthritis Rheum 32: , Halla JT. Fallahi S, Hardin JG: Nonreducible rotational head tilt and lateral mass collapse: a prospective study of frequency, radiographic findings, and clinical features in patients with rheumatoid arthritis. Arthritis Rheum 25: , Halla JT, Fallahi S, Hardin JG: Nonreducible rotational head tilt and atlantoaxial lateral mass collapse: clinical and roentgenographic features in patients with juvenile rheumatoid arthritis and ankylosing spondylitis. Arch Intern Med 143: , Halla JT, Bliznak J, Hardin JG: Involvement of the craniocervical junction in Reiter s syndrome. J Rheumato1 15: , Halla JT, Hardin JG Jr: Atlantoaxial (Cl-C2) facet joint osteoarthritis: a distinctive clinical syndrome. Arthritis Rheum , Dowd CF, Sartoris DT, Haghighi P, Resnick D: Case report 344. Skeletal Radio1 15:65-68, Resnick D: Osteomyelitis, septic arthritis, and soft tissue infection: the organisms, Diagnosis of Bone and Joint Disorders. Second edition. Edited by D Resnick, G Niwayama. Philadelphia, WB Saunders, Fang D, Leong JLY, Fang HSY: Tuberculosis of the upper cervical spine. J Bone Joint Surg 65B:47-50, Bland J: Differential diagnosis and specific treatment, Disorders of the Cervical Spine. First edition. Edited by J Bland. Philadelphia, WB Saunders, 1988