Langerhanscell- Histiocytosis (LCH)- a rare case for a destructive process of the petrous bone with peripheral facial palsy

Introduction The LCH is a dysregulation of the antigen-presenting dendritic cells. The illness can appear at every age, however most frequently in childhood.

Case history Our report is about a 5 year old child, who was presented with a peripheral facial palsy (H-B-Score 5) and an earache since 5 days. Furtheron it showed a diabetes insipidus and a coxitis fugax. It was already treated with Cefuroxim. The conducted Head-MRI showed an enrichment in the right mastoid with temporal meningitis as well as an enrichment in the sella turcica and in the cervical spine. In the examination we found the right upper-posterior auditory canal lowered and the eardrum reddened and thickened. On the left we found a middle ear effusion. The Temporal bone-CT showed a destruction of the petrous bone and the mastoid with erosions to the the middle and posterior cranial fossa as well as to the labyrinthblock. We performed a mastoidectomy on the right with adenotomy and paracentesis on both sides. During the surgery we found a seromucotympanum on both ears. Furtheron we found slushy white granulation tissue in the right mastoid with exposed dura to the the middle and posterior cranial fossa and erosions of bone at the posterior auditory canal at the level of the facial nerve path. The histological result showed a proof of LCH. We relocated the child to the paediatric clinic for staging and therapy. They performed a full-body MRI, where more bone lesions were diagnosed. Right now the child is treated with steroids and vinblastine.

Conclusion If you diagnose a destruction of the temporal bone without a clear cause, especially if the patient has conspicous additional diseases, you always have to review a LCH, as well. The interdisciplinary teamwork is decisive for diagnosis and treatment.

Publication History

Article published online:
10 June 2020