An Atypical Presentation of Langerhans Cell Histiocytosis of the Cervical Spine in a Child

Study Design. A case report of a child with Langerhans cell histiocytosis of the posterior elements of the cervical spine treated conservatively and with long-term follow-up is described. Objectives. To describe the unique diagnostic and therapeutic challenges of treating Langerhans cell histiocytosis located atypically in the posterior elements of the spine. Summary of Background Data. Langerhans cell histiocytosis involves the spine in approximately 20% of children with the disease. In nearly 95% of cases, spinal Langerhans cell histiocytosis results in destruction of the vertebral body, sparing the posterior elements. Confident diagnosis of vertebral body lesions is often made based on plain radiographs and MRI; however, the diagnosis of posterior element disease is not so straight forward. The natural history of isolated skeletal Langerhans cell histiocytosis is benign in most cases. Methods. The clinical and radiographic presentation of a child with Langerhans cell histiocytosis involving the posterior elements of C3 is described. A soft tissue mass was observed on magnetic resonance imaging; however, a biopsy confirmed the benign diagnosis of Langerhans cell histiocytosis. No further surgical treatment was needed, and conservative therapy incorporating a short course of cervical bracing and low-grade chemotherapy was begun. Results. Within a year, reconstitution of the posterior elements of C3 was apparent on radiographs. By 9 years after diagnosis, the child had near full reconstitution of the posterior elements of C3 and resolution of anterior pseudosubluxation of C3 on C4 that was present at diagnosis. Although diagnosis of Langerhans cell histiocytosis was made difficult by an atypical location in the posterior elements of the spine, after biopsy, conservative treatment led to excellent vertebral reconstitution. Conclusions. Isolated involvement of the posterior spine in Langerhans cell histiocytosis has not been described in detail previously. The reported case illustrates the diagnostic challenges of isolated posterior spine Langerhans cell histiocytosis. Despite an unusual presentation, posterior spine Langerhans cell histiocytosis should be treated conservatively in a similar manner to anterior lesions.