Diagnosis and Management of Langerhans Cell Histiocytosis

Abstract

Langerhans cell histiocytosis is a rare group of disorders without a well-understood etiology. Known formerly as histiocytosis X, the disease has a wide spectrum of clinical presentations, including eosinophilic granuloma (solitary bone lesion), diabetes insipidus, and exophthalmos. It is also known by several eponyms, including Hand-Schüller-Christian disease when it manifests as a triad of cranial bone lesions and Letterer-Siwe disease when it is found in infantile patients with severely disseminated disease. Children aged 5 to 15 years are most commonly affected. Many of these patients initially present to orthopaedic surgeons, and misdiagnosis is frequent. To accurately diagnosis and treat these patients, the orthopaedic surgeon must be familiar with the clinical manifestations and pathophysiology of the disease as well as the treatment guidelines and outcomes for Langerhans cell histiocytosis.

Pericardial Effusion in Langerhans Cell Histiocytosis: A Case Report

“…The rate peak is among children aged 5 to 15 (8). LCH is more common in males compared to females (9).…”

The surgical strategy for eosinophilic granuloma of the pediatric cervical spine complicated with neurologic deficit and/or spinal instability

“…Nevertheless, a tissue biopsy for histopathologic examination is required to diagnose EG formally. For patients with unifocal osseous lesions, the overall benefits of needle or open biopsy remains controversial [1]. In our practice, for pediatric cervical EG patients suffered from neurological deficits and/or had SINS ≥7, we suggested surgery as an emergent treatment choice to prevent these children from any possible impending disastrous damages.…”

“…Although thoracic segments is the most preferential spine localization in EG patients of all ages with vertebral involvement [1, 2], data of previous studies and our research has suggested that incidence in the cervical spine is the highest in pediatric patients with spinal EG [3, 4]. …”

“…Among the clinical spectrum of LCH, eosinophilic granuloma (EG) is the most benign and predominant condition, featured by a clonal proliferation of Langerhans-type cells with bone involvement. In children and young adolescents with this tumor, about 80% of patients are reported to be younger than 20 years [1]. …”

“…For most cases without neural deficits or spinal instability, it is often sufficient to palliate symptoms and induce the lesion regression by conservative treatment and biopsy with cervical collar immobilization in view of the possibility of spontaneous resolution. Surgical intervention should be considered to relieve pain, maintain spinal stability, and preserve neural function in the presence of neurologic compromise, spinal instability, and severe deformity [1]. It has been documented that several surgical strategies are feasible for the achievement of the abovementioned therapeutic goals in the treatment of pediatric cervical EG without growth arrest and ensuing deformity [5, 7].…”