Title:
Langerhans cell histiocytosis: bilateral temporal bone involvement in an adult with diabetes insipidus
Authors:
G . C. Modugno*, C. Brandolini*, G. Magnani*, G. G. Ferri*, E. Sabattini** and A. Pirodda*
Institutions:
*Department of Specialistic Surgical and Anaesthesiological Sciences, ENT Section, Policlinico S.Orsola-Malpighi, University of Bologna, Italy; **Institute of Hematology and Clinical Oncology “L. and A. Seragnoli”, Policlinico S.Orsola-Malpighi, University of Bologna, Italy
Keywords:
Langerhans cell histiocytosis; temporal bone; diabetes insipidus
Pages:
67 - 72
Abstract:
Langerhans cell histiocytosis: bilateral temporal bone involvement in an adult with diabetes insipidus. Objective: To present a clinical case of an adult affected by Langerhans cell histiocytosis with bilateral, non- simultaneous, involvement of the temporal bone, associated with diabetes insipidus and to review the literature. Methodology: A rare case of bilateral temporal bone involvement of Langerhans cell histiocytosis in a 42-year-old woman affected by diabetes insipidus is reported. We present patient’s clinical history supported by radiologic, histopathologic and audiologic findings. Results: The patient was submitted to a series of otologic surgical procedures due to the progression of the disease. Ossicular chain was always preserved, so that conservative surgery (canal wall-up technique) was performed, permitting the achievement of good hearing results, bilaterally. Conclusions: Temporal bone involvement of Langerhans cell histiocytosis may lead to a progressive chronic disease. However, the ossicular chain can remain uninvolved, making a conservative surgical treatment possible. Careful follow- up is essential for detecting new lesions and serial CT scans are mandatory.
Issue:
Vol. 6, 2010, 1st trimester


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Langerhans cell histiocytosis: bilateral temporal bone involvement in an adult with diabetes insipidus