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Otologic manifestations of Langerhans' cell histiocytosis
M J Cunningham1, H D Curtin, R Jaffe
1Department of Otolaryngology, University of Pittsburgh, Eye and Ear Hospital.
Insights
Langerhans' cell histiocytosis (LCH) can affect the ear and temporal bone, sometimes as the only symptom. Early diagnosis and imaging like CT scans are crucial for effective treatment of this rare childhood disease.
Area of Science:
- Pediatric Oncology
- Otolaryngology
- Radiology
Background:
- Langerhans' cell histiocytosis (LCH) is a rare clonal proliferative disease.
- Otologic and temporal bone involvement in pediatric LCH is uncommon but can present insidiously.
Abstract:
Eighteen of 62 children diagnosed with Langerhans' cell histiocytosis at the Children's Hospital of Pittsburgh (Pa) between 1970 and 1986 demonstrated ear and temporal bone involvement. In six children, such otologic disease was their sole presenting manifestation. Common signs and symptoms included aural discharge, postauricular swelling, and conductive hearing loss. The otologic findings in these children, if not investigated properly, could easily be attributed to acute or chronic infectious ear disease. Computed tomography with contrast enhancement proved to be particularly valuable as a diagnostic study because of its clear delineation of both osseous and soft-tissue temporal bone involvement. Computed tomographic findings could also be used to enhance local treatment by guiding surgical biopsy and curettage procedures or defining low-dose radiation therapy portals. Eleven of these 18 children with otologic Langerhans' cell histiocytosis additionally required chemotherapy due to the systemic nature of their disease.