Abstract
Salivary gland diseases are rare. In the European Union (EU) a disease is
considered to be rare if not more than 5 of 10,000 people are affected by
it. According to estimates in Germany are about 4 million people with a rare
disease. In the EU are about 30 million people with rare diseases [1]. In
the present work most of the described diseases of salivary glands and of
the facial nerve fall in this category. They form a very heterogeneous group
whose treatment takes place mainly in specialized centers. Still, it is
essential for the otolaryngologist to identify and to diagnose these
diseases in order to initiate the right therapeutic steps. The work is a
compilation of innate andacquired rare salivary gland disorders and of rare
facial nerve disorders. The etiologies of inflammatory diseases, autoimmune
disorders and tumors are taken into account. For the individual topics, the
current literature, if available, was evaluated and turned into summarized
facts. In this context the development of new processes, diagnostics,
imaging and therapy are considered. Genetic backgrounds of salivary gland
tumors and the trends in the treatment of tumorous lesions of the facial
nerve are picked up. Furthermore, also rare diseases of the salivary glands
in childhood are described. Some of them can occur in adults as well, but
differ in frequency and symptoms. Due to the rarity of these diseases, it is
recommended to tread these in centers with special expertise for it.
Finally, the difficulties of initiation of studies and the problems of
establishing disease registries concerning salivary gland disorders are
discussed. This is very relevant because these pathologies are comparatively
seldom.
Key words
salivary glands - facial nerve - salivary gland tumor - salivary gland infection - facial palsy - facial tumor