Juvenile idiopathic arthritis affecting the craniovertebral junction

Current management of juvenile idiopathic arthritis affecting the craniovertebral junction:

Abstract

Purpose

Craniovertebral instability is a rare and serious problem. While previously treated surgically, better understanding of disease processes has permitted the field to move towards conservative management. Juvenile idiopathic arthritis (JIA) is one cause of pediatric craniovertebral instability. Early recognition and institution of appropriate medical therapy and bracing in a multidisciplinary fashion is critical to avoid long-term instability, joint abnormalities, or morbid surgical procedures. We seek to highlight cases of this rare problem and provide a principled approach to management decisions.

Methods

We review 6 cases that have presented over the last 6 years and highlight 3 cases in particular regarding craniovertebral instability as a presentation of JIA. We reviewed the clinical records and radiographic features with particular emphasis of the stability of the craniovertebral junction.

Results

Age range of the subjects was from 5 to 12. All patients presented with neck pain and abnormal head rotation. Four of the patients responded to medical management and/or cervical bracing with no long-term sequelae or instability. Two patients had refractory rotary subluxation, one that responded to manual reduction under pharmacological paralysis and bracing; the other had an incompetent transverse ligament requiring surgical reduction and fixation.

Conclusions

Neck pain and abnormal head rotation in an older child is rare finding but should prompt suspicion as a manifestation of JIA to the general pediatrician or initial provider. Appropriate serologic studies and MRI studies with contrast at the craniovertebral junction is necessary for evaluation. Early institution of medical management and cervical bracing under a multidisciplinary team of pediatric rheumatology and neurosurgery is key to avoiding surgical intervention and long-term abnormalities at the craniovertebral junction.