Juvenile Chronic Arthritis and the Craniovertebral Junction in the Paediatric Patient: Review of the Literature and Management Considerations

Abstract

Juvenile chronic arthritis (JCA) is a systemic disease of childhood affecting particularly joints. JCA is a heterogeneous group of inflammatory joint disorders with onset before the age of 16 years and is comprised of 7 subtype groups. The pathogenesis of JCA seen in the cervical spine is synovial inflammation, hyperaemia, and pannus formation at the occipitoatlantoaxial joints resulting in characteristic craniovertebral junction findings. Treatment of craniovertebral junction instability as a result of JCA is a challenge. The best treatment strategy may be difficult because of various radiological and clinical severities. A review of the literature and management considerations is presented. No randomised controlled trial or systematic review on this subject has been published. Only experts' opinions, case reports, and case series have been described. Thirty-four studies have been reviewed in this study. Involvement of the cervical spine in patients with JCA can lead to pain and functional disability. The subtypes that usually affect the cervical spine are the polyarticular type and systemic onset type and rarely the pauciarticular type. The most common cervical spine changes related to JCA are as follows: (1) apophyseal joint ankylosis at C2-C3, (2) atlantoaxial subluxation, (3) atlantoaxial impaction, (4) atlantoaxial rotatory fixation, and (5) growth disturbances of the cervical spine. The incidence of severe subluxations has decreased in the last decade as result of antirheumatoid drugs and biologicals. However, neurological compromise still occurs in JCA patients necessitating surgical treatment. Whenever the cervical spine is involved in rheumatoid arthritis patients without neurological deficits, conservative treatment is legitimate. Once patients develop neurological signs and symptoms, surgical treatment should be considered with particular focus to age, severity of the disease, and general health condition. Skilled anaesthesia is crucial and the surgical procedure should only be carried out in centres with experience in craniovertebral junction abnormalities.