[The otolaryngologist and Creutzfeldt-Jakob disease]

Is the ENT surgeon guilty of being a vector in Creutzfeldt-Jakob disease (CJD) in the past, present and future, with possible medico-legal implications, by virtue of his use of biomaterials and of his failure to sterilise surgical instruments adequately? Once the disease has been diagnosed, are there any implications for ENT? CJD is the most frequent clinical manifestation of the infectious subacute spongiform encephalopathies. It is invariably fatal within a few months, and at present there is no known treatment. Currently the diagnosis can be confirmed only by brain biopsy, but diagnostic tests are being developed. The causative agent belongs to the group of non-conventional transmissible agents, or prions, which are resistant to all conventional sterilisation methods. The illness has a long incubation period extending to several years, during which the abnormal prion protein (PPr), derived from a protein which is normally present, accumulates in the central nervous system. CJD is a rare condition, affecting less than one in a million of the population, and manifests itself in one of three distinct ways: sporadic, in 90% of cases, possibly due to a natural mutation of the gene responsible for formation of the protein, hereditary familial (5 to 10% of cases); and iatrogenic, transmitted by the implantation of biomaterials of human origin, which are the cases for which the ENT surgeon could be responsible, and which may affect any age group. The possible modes of ENT transmission have been identified. These could be the use of surgical material contaminated by a patient suffering from CJD, the use of human biomaterials whether dural homografts, ossicular homografts taken together with the donor dura, the use of blood products, or the use of biomaterials of bovine origin. Such materials have been used widely in the past without any observed increase in new CJD cases, but the incubation period of this disease is not well understood. A knowledge of the possible alternatives and of the new relations on the subject should allow us to improve the epidemiology of the condition, help the surgeon in his choice of materials, help in the diagnosis of the condition, and in avoiding its transmission by heeding new advice on sterilisation.