Analysis of clinical features of Craniofacial Langerhans cell histiocytosis in children

Abstract

Objective:To investigate the clinical features of craniofacial Langerhans cell histiocytosis（LCH） in children. Method:The clinical data of 11 children with craniofacial LCH confirmed by surgical histopathology were analyzed retrospectively, and their clinical characteristics were compared. Result:Eleven children with craniofacial LCH had a history of misdiagnosis and mistreatment. There were 10 cases aged 1-4 years. 9 cases of LCH occurred in the temporal bone with otorrhea and moderate to severe hearing loss, while 6 cases were binaural involvement. CT of the primary lesions in 7 children showed obvious extensive bone destruction. 2 cases died（both with multiple dangerous organs involved） and 8 cases survived. Conclusion:The craniofacial LCH in children mainly occurs in children under 4 years old, Most of the temporal bone LCH is involved in both ears with otorrhea and severe hearing loss. There is a high rate of misdiagnosis in clinical work due to lack of specific clinical symptoms. Imaging examinations and systemic examinations have important diagnostic value. The cases with dangerous organs involved have a higher mortality rate, while chemotherapy has better clinical effect for children with localized lesions.