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Spontaneous Atlantoaxial Subluxation as a Presenting Manifestation of Juvenile Ankylosing Spondylitis
Abstract
Juvenile-onset ankylosing spondylitis is an unusual disorder which can present with either peripheral arthritis or with more classic hip girdle and back symptoms. Atlantoaxial instability has been recognized as a late complication in only one patient and has not been reported as an early manifestation. This paper presents a case in which atlantoaxial instability and neck pain without neurologic involvement was a presenting manifestation. The laboratory and clinical features of juvenile ankylosing spondylitis are discussed, along with the importance of qualitative sacroiliac joint scintigraphy in the diagnosis of early or confusing cases.
... Negli adulti la spondilite anchilosante si manifesta a livello cervicale con cifosi cervicotoracica, che storicamente è stata tra le prime deformità acquisite del rachide alle quali è sta efficacemente applicata correzione chirurgica (124,125). Nella forma giovanile la manifestazione cervicale è costituita dalla sublussazione atlanto assiale (126,(127)(128)(129): esami radiografici dinamici ed eventualmente TAC possono evidenziare l'instabilità, mentre l'esame RMN mostra la reazione infiammatoria della membrana sinoviale e l'eventuale sofferenza ischemica midollare. Il trattamento chirurgico è la stabilizzazione C1-C2; le indicazioni alla chirurgia nel paziente pediatrico includono: comparsa di sintomatologia neurologica da compressione midollare, dolore occipitocervicale che non risponde alla terapia medica, peggioramento della instabilità C1-C2 (ampliamento dello spazio tra arco anteriore dell'atlante e dente dell'epistrofeo uguale o superiore ai 6 mm) (127). ...
... Nella forma giovanile la manifestazione cervicale è costituita dalla sublussazione atlanto assiale (126,(127)(128)(129): esami radiografici dinamici ed eventualmente TAC possono evidenziare l'instabilità, mentre l'esame RMN mostra la reazione infiammatoria della membrana sinoviale e l'eventuale sofferenza ischemica midollare. Il trattamento chirurgico è la stabilizzazione C1-C2; le indicazioni alla chirurgia nel paziente pediatrico includono: comparsa di sintomatologia neurologica da compressione midollare, dolore occipitocervicale che non risponde alla terapia medica, peggioramento della instabilità C1-C2 (ampliamento dello spazio tra arco anteriore dell'atlante e dente dell'epistrofeo uguale o superiore ai 6 mm) (127). I pazienti colpiti da spondilosi anchilosante giovanile dovrebbero comunque essere attentamente monitorizzati nel tempo per diagnosticare tempestivamente possibili manifestazioni a carico del rachide quali collassi vertebrali osteoporotici, stenosi cervicale e progressiva cifosi del passaggio cervico-toracico (deformità "mento sul petto"). ...
Introduzione Il trattamento chirurgico delle patologie del rachide cervicale in età pediatrica ed adolescenziale pone una serie di problematiche peculiari, legate a dia-gnosi poco comuni, piccole dimensioni anatomiche, presenza di anomalie delle strutture ossee e nervose e la necessità di ottenere una fissazione stabile. La lassità legamentosa tipica dell'età pediatrica, le di-mensioni relativamente grandi del cranio dei bam-bini in confronto al collo e l'orientamento orizzontale delle faccette articolari influenza spesso la presenta-zione di una patologia cervicale, congenital o acqui-sita, come instabilità del rachide cervicale alto o della giunzione occipito cervicale, che necessita una artro-desi per ottenere stabilità e proteggere le strutture nervose (1). Imaging pre operatoria Lo studio pre operatorio di ogni paziente pediatrico con una patologia del rachide cervicale si avvale di radiografie, tomografia assiale computerizzata (TAC) e risonanza magnetica nucleare (RMN). Le radiografie comprendono la proiezione antero po-steriore e latero laterale, la proiezione transorale per il dente dell'epistrofeo e, là dove sia possibile e sicuro eseguirle per evidenziare una instabilità, delle proie-zioni dinamiche laterali in massima flessione e mas-sima estensione. La radiografia permette una valutazione complessiva dell'anatomia del rachide cervicale, dell'allinea-mento e della mobilità segmentaria. La TAC è utile soprattutto a livello del rachide cervi-cale alto per valutare correttamente l'andamento delle arterie vertebrali e l'anatomia delle strutture ossee, può essere effettuata dinamicamente anche per valutare più accuratamente l'esistenza di insta-bilità. La TAC tridimensionale può essere utile in caso di malformazioni complesse, per programmare il mi-glior posizionamento dello strumentario (2). La TAC standard è in grado di mostrare l'andamento delle arterie vertebrali nella grande maggioranza dei pazienti; il ricorso all'Angio TAC è utilizzato in casi se-lezionati, come i tumori, le patologie di origine con-nettivali o in alcune fratture, là dove sia necessario visualizzare con maggiore precisione l'anatomia va-scolare (3). La RMN è l'esame di scelta per visulizzare i dischi in-tervertebrali, i tessuti molli e le strutture nervose: va-luta eventuali stati di ischemia del midollo spinale o mielomalacia. La RMN nella patologia neoplastica permette di va-lutare l'estensione tumorale nei tessuti molli. In caso di traumi, è utile per visulizzare lesioni legamentose e lo stato di integrità dei dischi intervertebrali. La RMN dinamica, eseguita con il rachide cervicale in massima estensione e massima flessione, può essere utile in casi selezionati per evidenziare la presenza di instabilità dinamica e evidenziare l'eventuale asso-ciata compressione midollare, ma non è un esame routinario nei pazienti pediatrici (1). Considerazioni sull'anestesia e sul posizionamento dei pazienti A seconda della patologia che richiede un intervento sul rachide cervicale di un paziente pediatrico, può essere necessario fare ricorso ad intubazione con fi-broscopio o a una intubazione per via nasale. Spesso è presente instabilità che richiede l'immobi-lizzazione del rachide cervicale ed è importante per la sicurezza del paziente che l'anestesista abbia una buona esperienza nella gestione di vie aeree difficol-tose. In caso di accentuata e grave instabilità cervi-cale può essere necessario ricorrere ad una im mobilizzazione in Halo, non solo per ottenere una completa immobilizzazione del rachide cervicale e poter mobilizzare in sicurezza il paziente, ma anche per permettere la corretta riduzione di una eventuale deformità e, in caso di una artrodesi occipito-cervi-cale in presenza di disassiamenti ociipito-cervicale,
... Studies have reported that atlantoaxial instability can be a feature of juvenile and adult ankylosing spondylitis (AS)1234 reactive arthritis, juvenile idiopathic arthritis [5] and rheumatoid arthritis [6] in the course of the disease. Indeed, in two separate cohorts, 7% to 21% of adult patients with ankylosing spondylitis showed signs of subluxation as early as one year following AS diagnosis. ...
... The clinical scenario of our patient resembles the characteristics of the undifferentiated spondyloarthritis group described above: she has not developed features of AS, psoriasis, or IBD, yet within the next five to 10 years she may develop typical radiographic changes of sacroilitis and bone ossification. To the best of our knowledge, only a few other children with similar presentations have been described in the literature [1,2,23]. ...
Atlantoaxial instability has been described as a manifestation of ankylosing spondylitis (juvenile and adult onset), reactive arthritis, juvenile idiopathic arthritis, and rheumatoid arthritis; however, it has rarely been reported as an early manifestation of these disorders. We present this case report to increase awareness of the condition in the hope that earlier recognition of this disease may prevent further serious injury.
We report the case of a 17-year-old Hispanic adolescent woman who was initially diagnosed with undifferentiated spondyloarthritis due to peripheral arthritis, enthesitis, a positive human leukocyte antigen B27 result, and inflammatory spinal pain lasting two months. Our patient experienced persistent and worsening occipitocervical pain and signs of myelopathy three months after diagnosis; consequently, we found atlantoaxial instability along with cervical spine bone erosion and pannus formation. She was treated surgically with a C1-2 posterior instrumented fusion and at six weeks post-operatively was started on tumor necrosis factor α blockade. Her occipitocervical symptoms subsided following surgery and initiation of immunomodulation.
Our report serves to emphasize to pediatric and adult general practitioners, pediatricians, internists, family physicians, pediatric and adult rheumatologists and spine surgeons that atlantoaxial subluxation may be an early manifestation of spondyloarthritis, and that the condition is treatable by surgical intervention and immunomodulation.
... Five patients (20.8%) complained of headache/head pain at presentation and 17 patients (70.8%) complained of neck pain. Nine patients (37.5%)7,11,13,14,181920212228,31,33,34,36,37 One of the patients in this series had an odontoid fracture as his primary pathology at the CVJ. ...
... Most previous reports indicate the presence of horizontal , vertical, and/or rotatory subluxation in patients with CVJ disease due to ankylosing spondylitis. 3–5,7,8,10,11,13, 14,1920212228,31,33,34,36,37 In addition, erosion of the C1–2 facet joints, 4,10,14,18 erosion of the lateral mass of C-1, 6,10,19 erosion of the anterior arch of C-1 and/or odontoid process of C-2, 10,21,22 hypertrophy of the odontoid process, 21 and varying degrees of spontaneous fusion of the occiput, C-1, and C-2 19,21,22,28,33 have been reported. Our patients demonstrated a similar spectrum of radiographic findings. ...
The objective of this study was to describe a single surgeon's experience managing craniovertebral junction (CVJ) disease due to ankylosing spondylitis.
The authors undertook a retrospective review of the records of patients with CVJ disease due to ankylosing spondylitis who were evaluated and treated by the senior author. Charts were reviewed for symptoms and signs at presentation, radiography results, treatment, and outcome. In addition, some of the patients had pathology reports available for review.
Eight patients with CVJ disease due to ankylosing spondylitis were identified who were evaluated by the senior author in the years 1990-2008. The most common presenting symptoms were neck pain (37.5%), cranial neuropathy (37.5%), and sensory disturbance (62.5%). On examination, the most common findings were limited cervical range of motion (37.5%), weakness (50%), and myelopathy (75%). Radiographic evaluation revealed atlantoaxial subluxation, retroodontoid pannus formation, basilar invagination, and bone erosion. Surgery was offered to all of the patients, 7 of whom underwent operations. In most cases, the treatment was transoral-transpalatopharyngeal decompression followed by occipitocervical fusion. One patient with a reducible lesion underwent dorsal fusion alone. Neurological outcomes were favorable overall.
The incidence of CVJ disease in patients with ankylosing spondylitis varies among reports. These cases are rare in most neurosurgery clinics. It is important to recognize that patients with ankylosing spondylitis are at risk for CVJ disease, similar to patients with rheumatoid arthritis. Principles common to the management of other CVJ pathologies apply to these patients as well.
... Moreover MRI reduces radiation exposure, which is especially important in young children. Clearly not all JIA patients need an MR examination of the cervical spine: patients with persistent oligoarthritis do not, or only rarely, develop cervical spine disease [3,11,282930313233, however in polyarticular disease cervical spine involvement is frequent both in rheumatoid factor positive and negative patients [9,11]. Hence we would recommend MR imaging in the following situations: (1) signs and symptoms of cervical spine involvement, (2) rheumatoid factor positive and (3) rheumatoid factor negative polyarthritis or extended oligoarthritis with recalcitrant disease necessitating intensive therapy. ...
To describe MRI and clinical findings in patients with juvenile idiopathic arthritis with cervical spine involvement at onset and follow-up under therapy.
13 patients with signs of cervical spine involvement in juvenile idiopathic arthritis with a median disease duration of 1.7 years were included in the study. Clinical records and MR images were retrospectively analyzed according to symptoms and findings concerning the cervical spine.
At the onset of cervical spine involvement all patients showed limited range of motion, whereas only 5 of them complained of pain. In MR images joint hyperintensity, contrast enhancement, malalignment, ankylosis, erosion and narrowing of the spinal canal at cranio-cervical junction were found at 28, 32, 15, 2, 2 and 3 sites in 12 (93%), 13 (100%), 8 (62%), 2 (15%), 2 and 3 (20%) patients respectively. 3 of the 5 patients with pain (60%) showed ankylosis, erosions or narrowing of the spinal canal at cranio-cervical junction on MRI. At follow-up - after a median disease duration of cervical spine arthritis of 2.1 years and a variable duration of treatment with methotrexate (all patients) and biological agents (12 patients) - joint hyperintensity, enhancement and malalignment decreased to 15, 19 and 6 sites in 10 (77%), 11 (85%) and 3 (20%) patients respectively whereas ankylosis, erosion and narrowing of the spinal canal at cranio-cervical junction increased to 7, 6 and 4 sites in 3 (20%), 4 (31%) and 4 patients respectively. Pain was no longer reported, but 9 of 13 (69%) patients still had a limited range of motion with 6 of them (46%) showing skeletal changes on MRI.
This first MRI based follow-up study shows that cervical spine arthritis can follow a severe disease course in juvenile arthritis. While malalignments and inflammation sites decreased osseous changes with erosions, ankylosis, and narrowing of the spinal canal increased under treatment despite only minor subjective complaints. Therefore close MRI monitoring of these patients appears to be reasonable.
... In a progressed disease, atlanto-axial subluxation might occur due to erosions of the transverse ligaments or other cervical structures, such as the odontoid process, resulting in neurological complaints due to myelum compression with quadriplegia even after a minor trauma of the neck 37,38 . ...
Background
C1-C2 subluxation is a rare complication of enthesitis-related arthritis (ERA). If left untreated, it may lead to functional impairment or cervical spinal cord compression. This study aims to highlight key points regarding the management of C1-C2 subluxation in ERA.
Case presentation
We present two cases of C1-C2 subluxation: an 8-year-old boy with ERA and 16-year-old boy with ERA with bilateral sacroiliitis. Ten cases of ERA in the literature were reviewed. The diagnosis of C1-C2 subluxation is mostly based on radiographs and cervical spine computed tomography. All patients were treated with non-steroidal anti-inflammatory drugs. Six ERA patients were treated surgically for cervical fusion. Most ERA patients with sacroiliitis had cervical collar protection. Neurologic abnormalities after treatment were not reported. Despite the use of cervical collar, cervical fusion and persisting ankylosis were found in two ERA patients with sacroiliitis without surgical treatment.
Conclusions
Cervical spine protection and ruling out spinal cord compression should be prioritized, in addition to controlling the underlying inflammation in ERA. Cervical halter traction may be applied after severe cervical inflammation is excluded. To reduce the risk of complications, early recognition and appropriate treatments of C1-C2 subluxation in ERA are essential.
We are beginning to understand the clinical nature of JAS, its relationship with other SSA, and factors involved in its pathogenesis. Clinical data may now allow early recognition of JAS through the identification of children with the SEA syndrome or chronic arthritis associated with the HLA-B27. Comparative clinical studies of the prevalence of the disease and the role of irnmunogenetic, racial and environmental factors are needed. It may be necessary to review current criteria for the diagnosis of JRA and to develop similar criteria for the diagnosis of AS in childhood and adolescence.
Case:
Non-neoplastic bone destruction, known as Andersson lesion, is not rare in ankylosing spondylitis (AS) and always appeared as bone destruction and sclerosis in the vertebral body-intervertebral disc region. The Andersson lesion has been reported mostly in the thoracic and lumbar spine and rarely in the lower cervical spine. We surgically treated a patient with AS complicated with atlantoaxial dislocation and destruction of dens and lateral atlantoaxial joint, which was similar to the Andersson lesion.
Conclusions:
AS can cause destructive lesion in the upper cervical spine. Posterior reduction, removal of the lesion, and fusion were possible approaches for the treatment of this destructive lesion.
Die kongenitalen Mißbildungen der Wirbelsäule umfassen eine Vielfalt von Defekten, die schon häufig zusammenfassend dargestellt und nach verschiedenen Gesichtspunkten klassifiziert wurden. Da die Wirbelsäule ein für den Anatomen schwer zugängliches Organ ist, ist es verständlich, daß sich besonders Röntgeno-logen und Orthopäden bemüht haben, in dieser Vielfalt Ordnung zu schaffen und die kongenitalen Defekte nach Kriterien der röntgenographischen Morphologie einzuteilen. Eine ausgedehnte, kaum mehr übersehbare Literatur, darunter zahllose Publikationen von Einzelbeobachtungen liegen vor, indes Fragen nach dem Ursprung und der Entwicklung der kongenitalen Defekte bisher unsicher und ungelöst geblieben sind. Jede Beschreibung enthält zwar eine Hypothese über ihre formale und kausale Genese. Diese bleibt aber Spekulation, da bis heute nur ganz ausnahmsweise typische, immer wiederkehrende Mißbildungen in statu nascendi erfaßt wurden. Aus diesem Grunde müssen für die Erklärung ihrer Genese die Ergebnisse der experimentellen Embryologie und ganz besonders der Säugetiergenetik herangezogen werden. Diese haben in den vergangenen Jahren viel zum Verständnis beigetragen und gezeigt, daß die Wirbelsäule wie jedes andere Organ im Verlaufe der Ontogenese Phasen besonders hoher Empfindlichkeit durchläuft, die durch endogene und exogene Faktoren leicht gestört werden können.
Ankylosing spondylitis (AS) is a progressive multisystem chronic inflammatory disorder. The hallmark of this pathological process is a progressive fusion of the zygapophyseal joints and disc spaces of the axial skeleton, leading to a rigid kyphotic deformity and positive sagittal balance. The ankylosed spine is unable to accommodate normal mechanical forces, rendering it brittle and susceptible to injury. Traumatic hyperextension injury of the cervical spine leading to atlantoaxial subluxation (AAS) in AS patients can often be fatal. We report a non-traumatic mechanism of injury in AS progressing to AAS attributable to persistent hyperextension, which resulted in fatal migration of C2 through the foramen magnum.
Atlantoaxial subluxation is well recognized as a late complication of juvenile arthritis1. Early subluxation is uncommon, but it has been reported in 2 patients with juvenile ankylosing spondylitis (AS)2,3 and in 2 patients with juvenile idiopathic arthritis (JIA)4,5.
A 12-year-old girl was diagnosed with JIA, enthesitis-related arthritis (ERA) subtype, in March 2012 when she presented with a 1-year history of joint pain and stiffness. She was positive for HLA-B27, and a …
Juvenile-onset spondyloarthritis is a special group within the entities included in the concept of spondyloarthritis, and is characterized by a predominantly peripheral involvement (arthritis and enthesitis), and more frequent presentation as undifferentiated forms. However, like its adult-onset equivalent, it has the potential to develop structural damage and progress to juvenile ankylosing spondylitis, with consequent irreversible functional impairment. Many important advances have been made in the understanding of the genetics and pathophysiology of juvenile-onset spondyloarthritis, as well as in the diagnosis and treatment of this entity. These advances are summarized in this article.
This chapter discusses the classification, epidemiology, pathogenesis, clinical manifestations, and therapeutic approach of the juvenile-onset spondyloarthritides (SpA). Juvenile-onset SpA may be regarded as a group of HLA-B27-associated disorders characterized by enthesitis and arthritis affecting the lower extremities, and, in a variable proportion of cases, the sacroiliac and spinal joints. In some cases, extra articular manifestations occur and in some other bacterial infections may trigger the disease. The clinical spectrum spans from undifferentiated conditions to syndromes or diseases that either fulfill specific diagnostic criteria or correspond with the clinical picture of diseases described in adults. The incidence and prevalence of juvenile-onset SpA may be influenced by ethnic or geographic factors. The most frequent sign of juvenile-onset SpA is peripheral arthritis. Magnetic resonance imaging (MRI), particularly of the spinal and sacroiliac joints and ultrasongraphy of the peripheral joints are useful methods in detecting disease activity. The therapeutic approach of children and adolescents with juvenile-onset SpA is aimed to reduce the intensity and duration of signs and symptoms that particularly relate to inflammation.
Inflammatory conditions at the craniocervical junction (CCJ) consist mainly of rheumatic diseases and spondyloarthropathies, with rheumatoid arthritis (RA) representing the predominant example of the former condition. Secondary atlantoaxial subluxation (AAS) is noted in up to 70% of cases of RA, yet neurologic manifestations are not common. Atlantoaxial impaction (AAI) however, a less frequent entity, is associated with potentially grave deficits. All of the seronegative spondyloarthropathies may also have abnormalities at the CCJ.
Erosive inflammatory changes in the synovium cause damage and subsequent loss of the adjacent ligament, cartilage, and bone, producing typical radiological features. As the disease progresses there is worsening instability, yet a corresponding rate of neurologic compromise is not seen. The life span for patients with RA is approximately 65 years, with mortality being highest in myelopathic patients.
Radiological investigations include plain radiographs, computed tomographic scans, and magnetic resonance imaging (MRI). The plain films are frequently used for screening, with the posterior atlantodental interval (PADI) being most predictive of paralysis from AAS. The Clark's station, the Ranawat criterion, and the Redlund-Johnell criteria are useful for predicting AAI. MRI currently is most effective for defining CCJ relationships.
Clinical manifestations of CCJ pathology occur because of direct compression by bone/soft tissue and/or from vertebral or anterior spinal artery compromise. The Ranawat grading scale is the most universally accepted scale for functional assessment. Prognostic severity criteria for AAS and AAI are the degree of peripheral joint disease, duration of RA, seropositivity, male gender, presence of rheumatoid nodules, and use of corticosteroids.
Treatment for RA, either conservative or surgical, is controversial. Intractable neck pain and neurologic deficits are well-accepted indications for surgery. In asymptomatic patients the role of surgery is based on a variety of radiologic criteria for instability.
Ankylosing spondylitis (AS) is a progressive multisystem chronic inflammatory disorder. The hallmark of this pathological process is a progressive fusion of the zygapophyseal joints and disc spaces of the axial skeleton, leading to a rigid kyphotic deformity and positive sagittal balance. The ankylosed spine is unable to accommodate normal mechanical forces, rendering it brittle and susceptible to injury. Traumatic hyperextension injury of the cervical spine leading to atlantoaxial subluxation (AAS) in AS patients can often be fatal. We report a non-traumatic mechanism of injury in AS progressing to AAS attributable to persistent hyperextension, which resulted in fatal migration of C2 through the foramen magnum.
Cite this article: Bone Joint J 2013;95-B:206–9.
The end of this century and the dawn of the new millennium are near, and we still don't fully understand the mechanism behind the remarkable association of HLA-B27 with spondy-loarthropathies, although it has been known for more than a quarter of a century. However, there has been a slow but steady progress in the field of spondyloarthropathies. Not all alleles that encode for the HLA-B27 serologic specificity are associated with susceptibility to develop a spondyloarthropathy. The number of known subtypes of HLA-B27 continue to grow; the latest three additions are HLA-B*2713, HLA-B*2714, and HLA-B*2715. It is becoming increasingly clear that the observed synovial and cartilage changes in spondyloarthropathies are mainly the result of an entheseal- rather than a synovial-based pathology, because the initial or primary inflammatory response is located at the enthesis, whereas synovitis is most likely triggered secondarily by locally released cytokines at the adjacent sites in the joint cavity. But we still don't know how ankylosis relates to HLA-B27. The conditions favoring the development of HIV-associated spondyloarthropathy are becoming less of a problem in North America, but this is not the case in some of the other regions of the world, such as Sub-Saharan Africa.
Two patients with seronegative HLA-B27-connected spondylarthropathy, associated in one case with psoriasis and in the other with Reiter's disease, who had atlanto-axial subluxation (AAS) are reported. The AAS caused muscle weakness and intractable occipito-cervical pain. Both patients were treated operatively with a Gallie type C1-C2 fusion. The symptoms subsided following surgery and the patients remained free of cervical spine discomfort. The report also indicates that the rarer variants of the HLA-B27 associated chronic seronegative spondylarthropathies may cause AAS complicated by neurological symptoms and that the disorder is amenable to surgical intervention.
The use of MRI in the assessment of the musculoskeletal system in children has important differences from its use in adults. Growth in children has significant impact on the epiphysis and growth plate, which are important structures in the growing child, and there are radiological features that differ from those in adults: disease may alter structures during a period of growth; the pathologies themselves are a distinct group of diseases at variance with adult arthritis and myositis, with a different spectrum of differential diagnoses; and many technical issues are different when imaging a child. These are important considerations in choosing the appropriate imaging. MRI is a powerful and valuable imaging technique in pediatric musculoskeletal pathologies, with considerable potential for future developments to enhance its role in diagnosis, management, and therapeutic intervention for these children.
Ankylosing spondylitis is an unusual cause of chronic polyarthritis in children. Atlantoaxial subluxation is well recognized in adult ankylosing spondylitis, but has not previously been documented in the pediatric age group. We describe such a case in a 14-yr-old boy.
Acute and chronic neck pain and headaches are a major medical, industrial, and financial problem. In many cases, the pathophysiology is unknown, and the disability is often greatly in excess of the apparent tissue pathology and physiological impairment. Diagnosis and management of neck pain and cervicogenic headache calls for a rational and comprehensive approach. Many cases will have no identifiable pathology, and the pain and disability will continue despite the best efforts of all concerned.
In discussing the management of spondylitis and the associated peripheral arthritis, ankylosing spondylitis (AS) will be considered as a separate entity from psoriatic arthritis (PsA), Reiter's disease and the arthritis associated with inflammatory bowel disease. The management of some of the extra-articular manifestations of the spondyloarthropathies is also indicated. Although there is no cure for the various spondyloarthropathies, treatment is aimed at relieving pain and stiffness to allow maintenance of joint function, thereby preventing future disability and minimizing existing disability. Before elaborating on the management of these diseases, it is necessary to look briefly at the natural history of AS to determine the effects of treatment. The method of assessing the response to treatment is complicated by the lack of correlation between clinical, functional and radiological changes. This is clearly demonstrated by the progression of spinal stiffness, which may occur with little or no pain. Huskisson and Hart have reported that the pain threshold is higher in patients with AS than controls and this may explain these changes. It is therefore important to assess all three parameters regularly.
The term spondyloarthropathy, currently used to describe some forms of idiopathic arthritis of childhood, may be inappropriate because most children included in this category do not have arthritis of the spine, and inflammatory disease of the sacroiliac joints is an infrequent or late finding. Juvenile AS, the archetype, or "complete" disease may account for only one fifth of the so-called "spondyloarthropathies". "Incomplete" or "early" spondyloarthropathies are most frequent. Such children may not develop axial symptoms and signs for 5 to 10 years after onset, and they may be better characterized as having enthesitis-related arthritis, a term proposed by a recent task force of the International League Against Rheumatism (ILAR). Reactive arthritis, although etiologically linked with the spondyloarthropathies, uncommonly progresses to AS in childhood; most patients have peripheral arthritis with or without enthesitis resolving in the relatively short term. The arthritis associated with IBD is more commonly peripheral than axial. Although axial disease undoubtedly occurs in JPsA, in the authors' experience it is very uncommon.
A 27-year-old woman presented with nontraumatic atlantoaxial dislocation that required a C1-C2 fusion. A comprehensive investigation initially failed to reveal any evidence of infectious or rheumatologic disease and she remained well for 3 1/2 years, at which time a diagnosis of ankylosing spondylitis was established. There are no other similar cases reported in the medical literature. When dealing with a patient who has experienced a spontaneous atlantoaxial dislocation, a thorough assessment looking for an underlying infectious or inflammatory etiology is mandatory. Furthermore, the authors recommend that any patient who has negative investigations be followed long-term; an underlying covert inflammatory cause may only become evident after significant delay.
To describe the clinical and radiological 2-yr followup of 22 patients with anterior atlantoaxial subluxation (AAS) from a prospective cohort of patients with ankylosing spondylitis.
The 2-yr assessment included a structured questionnaire for rheumatologic and neurologic complaints and lateral cervical radiographs in maximal flexion view. Initial and 2-yr radiographs were assessed blind to patient data. The course of anterior AAS was classified as unchanged (< 1 mm), progression (> or = 1 mm) or regression (> or = 1 mm) at 2 yrs compared with baseline. Vertical AAS was classified using the Sakaguchi-Kauppi method. Magnification factor was corrected using the ratio of C3 width.
Anterior AAS was detected in 22 patients at baseline examination. Two patients also had vertical AAS; 86% were male. Mean age was 33 +/- 9 yrs and mean disease duration was 12 +/- 7 yrs. At followup, one patient had died of acquired immunodeficiency syndrome, 3 could not be reached, and 2 had undergone surgical fusion due to severe myelopathy and now showed complete neurological recovery. Of the remaining 16 patients, 7 (32%) showed progression and 9 (41%) showed no change in the C1-odontoid distance. Vertical AAS developed in one patient. After the 2-yr assessment, 3 additional patients had surgical fusion because of notable progression of AAS, despite absence of neurological signs.
Anterior AAS progressed in a number of these patients in the 2 yrs following its detection, and with or without neurological signs, surgical management was thought appropriate in a considerable number of them.
This article discusses the clinical spectrum and characteristics of juvenile-onset spondyloarthropathies and includes a review of the demographic, clinical, radiographic (and other imaging techniques), and laboratory data of conditions, syndromes, and diseases making up this group. The pathogenic role of several factors in the context of adult-onset patients, but also in regards to studies already performed in juvenile-onset patients, is discussed.
Children presenting with torticollis demand thorough investigation. Atlantoaxial subluxation and rotatory fixation should be included in the differential diagnosis even in the absence of neurologic findings. We describe a 9-year-old boy with this condition associated with HLA-B27 positive seronegative spondyloarthropathy that went undiagnosed for 11 weeks. No other radiographic changes were seen, including no erosions. No neurologic findings were present. The child was treated with Halter head traction, which successfully reduced the atlantoaxial joint. This was followed by posterior C1-C2 fusion secondary to instability. Rheumatologists should be aware of this condition when examining a child presenting with torticollis.
Anterior cervical decompression and arthrodesis has evolved over the last 40 years and has become the preferred procedure for managing many cervical spine disorders. The first half of this article discusses the indications for cervical fusion in the management of traumatic, degenerative, neoplastic, infectious, and congenital conditions of the cervical spine. The second half of this article discusses the recent trends in use of cervical spine fusions that demonstrate the increasing frequency of this procedure in the United States over the last 10 years.
The juvenile-onset spondyloarthritides comprise a group of HLA-B27-associated disorders, which are mainly characterized by enthesitis and arthritis affecting the lower extremities, and in a variable proportion of cases, the sacroiliac and spinal joints. Additional features include a variety of extra-articular manifestations, and in some cases, bacterial infections as triggers. Except for the prevalence of some clinical features at onset and severity throughout the course of the disease, juvenile-onset SpA resemble their adult counterpart in most clinical aspects, strength of HLA-B27 association, and the role of arthritogenic bacteria in their pathogenesis. Not surprisingly, several aspects, from nomenclature to classification, and diagnostic criteria reflect to some extent those developed in the adult onset populations.
The upper cervical spine begins at the base of the occiput, continues caudally to the C2-C3 disk space, and includes the occipitoatlantal and atlantoaxial joints. Nontraumatic upper cervical spine instability can result from abnormal development of osseous or ligamentous structures or from gradually increasing ligamentous laxity associated with connective tissue disorders. Such instability can lead to compression of the spinal cord during movement of the cervical spine. Establishing a correct diagnosis includes performing a thorough physical examination as well as evaluating radiographic relationships and measurements. Appropriate management of syndromes associated with instability of the upper cervical spine includes preventive care and recommendations for sports participation. Surgical treatment for the upper cervical spine includes a posterior surgical approach, used for instability, and the use of rigid plate implants, wiring, and bone graft materials to achieve a solid spinal fusion.
Over the past decade there have been considerable changes in the classification and imaging of juvenile idiopathic arthritis (JIA). Radiology now has a considerable role in the management of JIA, the differential diagnosis, monitoring disease progression and detecting complications. The different imaging modalities available, their role and limitations are discussed in this article and the various disease features that the radiologist should be aware of are described. An approach to the imaging of the child with joint disease and in the monitoring of disease complications are also discussed.
This article focuses on the early diagnosis and effective management of ankylosing spondylitis (AS), a disease that is not uncommon and that can cause early retirement and severe functional disability. AS, like most other rheumatologic diseases, has no diagnostic gold standard. Correct diagnosis depends largely on a constellation of clinical symptoms and signs in addition to radiological findings. Early diagnosis has become all the more important because effective therapies are available: tumor necrosis factor antagonists that suppress disease activity and improve functional ability in patients with AS refractory to conventional drug therapy. The biologic agents are probably even more effective if given early. Inflammation of the sacroiliac joints and the spine is a common, early feature and possibly the most frequent first manifestation of disease. Therefore, its early detection is important, and magnetic resonance imaging has proven useful in this regard. To optimize diagnostic accuracy of early disease, it is crucial to use a comprehensive approach and have a deep understanding of the disease and its clinical picture. The clinician should gather a complete history, paying close attention to all the elements of this multisystem disease, as well as judiciously ordering laboratory testing and imaging. New strategies are being developed to assist primary care physicians in their screening for these patients, which in turn should result in early referral to rheumatologists and early diagnosis.
There is limited outcome measure support for chiropractic manipulative therapy in the management of ankylosing spondylitis. An improvement in specific indices for both function and disease activity during chiropractic therapy for ankylosing spondylitis has not previously been reported.
To measure changes in function and disease activity in a patient with ankylosing spondylitis during a course of chiropractic therapy. The clinical management of ankylosing spondylitis, including chiropractic manipulative therapy and the implications of this case study are discussed.
A 34-year-old male with a 10 year diagnosis of ankylosing spondylitis sought chiropractic treatment for spinal pain and stiffness. His advanced radiographic signs included an increased atlantodental interspace and cervical vertebral ankylosis.
The Bath Ankylosing Spondylitis Functional Index (BASFI), Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), finger-tip-to-floor distance and chest expansion were assessed during an 18 week course of chiropractic spinal manipulation and mobilization therapy. There was a 90% improvement in the disease activity index and an 85% improvement in the functional index from the pre-treatment baseline, as measured by the BASDAI and BASFI respectively. Spinal flexibility and chest expansion also improved.
To the authors knowledge this is the first study to incorporate ankylosing spondylitis specific indices, for both disease activity and function, to objectively support the use of chiropractic manipulative therapy in the management of ankylosing spondylitis. More intensive research is suggested.
Juvenile-onset ankylosing spondylitis is an unusual disorder which can present with either peripheral arthritis or more classic hip girdle and back symptoms. A 12-year-old child with this disease was admitted with walking disorder, cervical pain, restricted cervical motion, and right ankle swelling. Diffusely increased accumulation of radioactivity in the cervical spine, focally increased accumulation in bilateral sacroiliac joints, and diminished irregular uptake in thoracal spine were detected on technetium 99m methylene diphosphonate bone scintigraphy. As a result, this imaging technique may give important information for diagnosis and differential diagnosis in juvenile chronic arthritis.
We report on a 46-year-old man with atlantoaxial rotatory subluxation and cranio-cervical osseous fusion secondary to cervical dystonia (CD) and concomitant ankylosing spondylitis (AS).
To describe treatment of CD under these unusual circumstances.
CD may cause several complications due to enhanced spinal degeneration. Primary and secondary nonresponders to conservative treatment may benefit from neurosurgical procedures including denervation procedures or functional stereotactic neurosurgery. However, if spinal fixation has occurred, usually, treatment of dystonia is considered to be not appropriate. Nevertheless, in the rare case, neurosurgical intervention may still be useful to improve dystonic pain.
After a 4-year history of severe CD, the head was rotated to the left in a rigidly fixed position. Three-dimensionally rendered computed tomography imaging studies revealed fixed rotation of the occiput and the C1 vertebra at an angle of 45 degrees to the C2 vertebra. There was abnormal ossification of the facet joints and the ligaments of the odontoid.
The patient experienced marked relief of his neck pain after deactivation of the dystonic right sternocleidomastoid muscle by partial myectomy and the left posterior neck muscles by selective posterior ramisectomy.
Although rare, the concomitant occurrence of AS and CD should alert treating physicians and result in timely treatment of dystonia.
New Hampshire René Schmidt Mannheim, Germany Michael Schneier Marina Del Rey, California Atsushi Seichi Tochigi
- Luiz Pimenta
- São Paulo
- Timothy A Puckett Oklahoma
- Oklahoma City
- M Robert
- Quencer
- Florida Miami
- R Glenn
- Ii Rechtine
- H Rochester Japan Francis
- Virginia Shen Charlottesville
- L Harry
- Florida Shuffl Ebarger Miami
- P Dhaval
- Shukla
- India Bangalore
- H Edward
- Georgia Simmons Buffalo Andrew Simpson Atlanta
- L Richard
- Skolasky
- Maryland Baltimore
- J Paul
- Jr Slosar
- Daly
- City
Luiz Pimenta
São Paulo, Brazil
Timothy A. Puckett
Oklahoma City, Oklahoma
Robert M. Quencer
Miami, Florida
Glenn R. Rechtine II
Rochester, New York
Charles A. Reitman
Houston, Texas
Sally Roberts
Shropshire, United Kingdom
Peter Robertson
Aukland, New Zealand
Michel Rossignol
Montreal, Canada
Jeffrey A. Saal
Redwood City, California
Joel S. Saal
Redwood City, California
Koichi Sairyo
Tokushima, Japan
Harvinder S. Sandhu
New York, New York
Richard Saunders
Lebanon, New Hampshire
René Schmidt
Mannheim, Germany
Michael Schneier
Marina Del Rey, California
Atsushi Seichi
Tochigi, Japan
Francis H. Shen
Charlottesville, Virginia
Harry L. Shuffl ebarger
Miami, Florida
Dhaval P. Shukla
Bangalore, India
Edward H. Simmons
Buffalo, New York
Andrew Simpson
Atlanta, Georgia
Richard L. Skolasky
Baltimore, Maryland
Paul J. Slosar, Jr.
Daly City, California
Gary L. Smidt
Lakeville, Minnesota
William D. Smith
Las Vegas, Nevada
Tomislav Smoljanovic
Zagreb, Croatia
Paul D. Sponseller
Baltimore, Maryland
Jeffrey Stambough
Cincinnati, Ohio
Bjorn N. Stromqvist
Lund, Sweden
Fred Sweet
Rockford, Illinois
Katsushi Takeshita
Tokyo, Japan
Eeric Truumees
Austin, Texas
Dennis Turk
Seattle, Washington
Judith A. Turner
Seattle, Washington
Vidyadhar V. Upasani
San Diego, California
Howard Vernon
Ontario, Canada
Barrie Vernon-Roberts
Adelaide, Australia
Tapio Videman
Alberta, Canada
Michael R. Von Korff
Seattle, Washington
Robert G. Watkins
Los Angeles, California
H. Randal Woodward
Omaha, Nebraska
Karin Wuertz
Zurich, Switzerland
Hiroshi Yamada
Wakayama, Japan
Narayan Yoganandan
Milwaukee, Wisconsin
Kazuo Yonenobu
Osaka, Japan
Takashi Yurube
Hyogo, Japan
Yin-gang Zhang
Shaanxi, China
Michael R. Zindrick
Hinsdale, Illinois
Dewei Zou
Beijing, China
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