Altano-axial subluxation with torticollis secondary to Langerhans cell histocytosis

Abstract

Langerhans Cell Histiocytosis is a rare disease featuring aberrant proliferation of dendritic cells that can infiltrate the bony skeleton. The authors report a case of six-year-old female presenting with torticollis. She was found to have extensive lytic LCH lesions in the cranio-cervical junction, causing atlanto-axial rotatory subluxation. Decision was made for a non-surgical approach to treat the patient. She was placed in a Halo brace, and given concomitant chemotherapy. Post-treatment imaging demonstrated near-complete reconstitution of the skeleton affected by LCH. This case highlights the feasibility of non-surgical treatment in selected LCH patients at high-risk of spinal instability. Keywords: Atlanto-axial subluxation, Langerhans cell histocytosis, Torticollis