Objective: This study presents the experience of the Montreal Children's Hospital (MCH) with Langerhans' cell histiocytosis (LCH) and reviews the new advances in diagnosis and therapy of this disorder.
Design: Retrospective study of 20 patients seen between July 1986 and July 1997 diagnosed with LCH.
Methods: All of the 20 charts were examined for variables including age, sex, area involved, treatment modalities, and complications.
Result: Sixty-five percent of patients presented with localized lesions and 35% with multisystem involvement. The most common involved area was the skull, and 57% of skull lesions involved frontal bone. The temporal bone was involved in 25% of cases. The most common ear symptom was otorrhea.
Conclusion: Langerhans' cell histiocytosis is a rare paediatric disorder. Head and neck involvement occurs frequently in both localized and multisystem disease. The prognosis is highly dependent on the age and number of systems involved.