Langerhans Cell Histiocytosis of the Atlas in the Pediatric Spine: Total Reconstitution of the Bone Lesion After Nonoperative Treatment

Abstract

Langerhans cell histiocytosis is a non-neoplastic disease of unknown etiology. It is characterized by a clonal proliferation of Langerhans histiocytes, which are cells of the monocyte-macrophage lineage. Clinically, its manifestations range from isolated bone lesions to multisystem disease, with this latter form more likely to be seen in younger patients. The most common manifestation of Langerhans cell histiocytosis is a localized or multifocal benign infiltration of bones by the Langerhans histiocytes, and this was formerly called eosinophilic granuloma. It usually affects the skull, proximal portion of the femur, mandible, ribs, and pelvis1,2. Between 7% and 25% of these patients have spinal involvement3,4, with the most frequent site of involvement being the thoracic vertebrae, followed by the lumbar and cervical regions1,4-6. More than half of the cervical spinal lesions in pediatric patients with Langerhans cell histiocytosis affect the C3-C5 segment1,7,8, and involvement in the upper cervical spine is uncommon. There are very few published cases describing Langerhans cell histiocytosis of the atlas in children7,9-15. We report on two pediatric patients who had Langerhans cell histiocytosis of the atlas that was treated nonoperatively, resulting in complete reconstitution of the bone. Our patients' families were informed that data concerning the cases would be submitted for publication, and they consented. Case 1. A ten-year-old boy was admitted to our institution for evaluation of a two-month history of neck pain associated with slight restriction of head rotation. The neck pain was continuous and progressively increasing, and the use of nonsteroidal anti-inflammatory medication had resulted in no improvement. Radiographs of the cervical spine revealed no abnormality. Technetium-99 methylene diphosphonate bone scintigraphy showed increased uptake on the left side of …