Introduction: Langerhans Cell Histiocytosis(LCH) is a rare disease that results from the
aberrant proliferation of dendritic cells which can infiltrate the spine resulting in instability.In this report, we present a patient with lesions involving the C1, C2 and occipital condyle. Despite previous reports of
treatment by surgical curettage and fusion, we report a favorable outcome using chemotherapy and halo-thoracic brace fixation.
Methods: A six year old girl presenting with sudden onset neck pain was found to have multiple lytic and expansile lesions involving the right occipital condyle, clivus, C1 lateral mass and C2 odontoid process on imaging
resulting in atlanto-axial and occipital-cervical subluxation. Biopsy of cervical lymph node showed LCH. Patient's atlanto-axial subluxation was reduced while awake under fluoroscopic guidance, followed by halo-thoracic brace fixation. She was then was treated with chemotherapy.
Results: Her brace was removed after 10 months and
she regained nearly full range of neck
rotation. Post treatment CT scan showed near
complete reconstitution of the bones which
were initially destroyed by LCH.
Conclusions: Conservative treatment with cervical immobilization and chemotherapy can result in satisfactory outcomes in patients with LCH
causing cranio-cervical junction instability. In this case LCH lesions regressed and bone lesions were reconstituted to restore normal anatomical morphology.
Patient Care: Patients with LCH lesions causing cranio-cervical junction instability may not need surgical excision, fixation and fusion. This can help avoid long term effects such as limited range of neck rotation.
Learning Objectives: By conclusion of this session, participants should be able to discuss the indications for conservative treatment versus surgery in Langerhan's cell histiocytosis at the cervical spine and skull
References: Raab, P., et al., Vertebral remodeling in eosinophilic granuloma of the spine. A long-term follow-up. Spine (Phila Pa 1976), 1998. 23(12): p. 1351-4.
Garg S, Mehta S, Dormans JP. Langerhans cell histiocytosis of the spine in children. Long-term follow-up. J Bone Joint Surg Am. 2004 Aug;86-A(8):1740-50