Langerhans' cell histiocytosis: paediatric head and neck study.

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.