Study design: Descriptive, retrospective. Scientific level of evidence IV.
Objectives: The aim of this study was to evaluate a consecutive case series of 50 pediatric patients with LCH of the spine. Langerhans cell histiocytosis (LCH) is a rare disease characterized by abnormal proliferation of Langerhans cells in different organs. Incidence in children range from 2 to 10 cases per million. In the current literature, few series evaluate LCH in the pediatric spine.
Material and methods: A consecutive case series of 50 pediatric patients with LCH of the spine treated at our hospital between 1984 and 2016, with a follow-up of at least 2 years, was analyzed. Sex, age, clinical and radiographic presentation, number of lesions, treatment, complications, and outcome were assessed.
Results: Fifty patients, 26 boys and 24 girls, were evaluated. Mean age was 5 years and 2 months (6 months to 13 years and 3 months). 27 patients had a single spinal lesion while 23 had 2 or more lesions. A total of 100 vertebrae were involved. The thoracic spine was the most affected. The most frequent lesion location was in the vertebral body in 88% of the cases. The symptoms were pain (87%), reduced range of motion, deformity, and neurologic deficit. Biopsy was performed in 48 patients. Thirty-nine patients received medical treatment, 28 used orthoses and six required surgery. Six patients (12%) recurred at a mean of 3 years and 5 months (range 2-12 years). In all cases, neurological symptoms, torticollis, and deformities resolved after medical or surgical treatment.
Conclusions: Because of the variable presentation of the disease, ranging from a solitary isolated vertebral lesion to polyostotic and multisystemic involvement, a multidisciplinary team is required to have an adequate management of these patients and to obtain good results.
Keywords: Children spinal histiocytosis; Eosinophilic granuloma; Hand–Schuller–Christian disease; Langerhans cell histiocytosis; Letterer–Siwe disease.