Surgical Treatment of Langerhans Cell Histiocytosis of Cervical Spine: Case-Report and Systematic Review of Literature

Background: Langerhans cell histiocytosis (LCH) is a rare disease involving the cervical spine. This condition is present firstly in the thoracic spine, commonest, followed by the lumbar spine and cervical spine. In Langerhans cell histiocytosis there is excessive proliferation of pathologic Langerhans cells. It is commonly found in males with a ratio of 2.5:1. The etiology of LCH is unknown. There are three defined entities classified on their severities. Letterer-Siwe disease involves multiple organs, Hand-Schuller-Christian disease presents with bony lesions and endocrine abnormalities, and eosinophilic granuloma (EG) presents with isolated bony lesions. We describe a case-report a girl with Langerhans cell histiocytosis in cervical vertebrae.The objective of this study is to show the rare case of Cervical Spine Langerhans Cell Histiocytosis. Method: We were a literature review with inclusion and exclusion criteria and we described a case-report. Case-Report: A 9-month-old girl had neck pain associated with right upper limb weakness. Imaging showed infiltrative and expansive lesions in the vertebral body from C1 to C6 with spinal cord and root compression. The patient underwent a laminectomy from C2 to C5. Histopathological was suggestive of LCH. Conclusion: Despite the rarity of the disease, Langerhans cell histiocytosis can be treated with a surgical approach when neurologic deficits are present.