Grisel's syndrome is defined as subluxation of the atlantoaxial joint not associated with trauma or bone disease. Primarily an affliction of children, the disorder may occur in association with any condition that results in hyperemia and pathologic relaxation of the transverse ligament of the atlantoaxial joint, including several common otolaryngic entities. Grisel's syndrome has been noted infrequently in the otolaryngologic literature; this paper reports a case from West Virginia University Hospital of a 6-year-old boy with radiographic evidence of retropharyngeal cellulitis following meningitis progressing to atlantoaxial subluxation. Discussed are the pathophysiologic aspects of Grisel's syndrome with an anatomic explanation recently described to account for the syndrome.
Isotretinoin (Accutane), a widely used dermatologic drug, produces severe congenital malformations when used during pregnancy. The isotretinoin teratogen syndrome consists of multiple cardiovascular and craniofacial anomalies, most commonly involving the external ear. This study examined the pathogenesis of isotretinoin teratogenicity in a mouse model, using microdissection and histologic examination of fetal mouse ears after treatment with the drug at various stages of embryonic development. In this study, earlier treatment times frequently produced microtia similar to that seen in affected infants, as well as recognizable patterns of temporal bone and ossicular abnormalities; exposure at a later developmental stage resulted in facial tags with less severely affected ears. Possible teratogenic mechanisms of isotretinoin are discussed. Suitability of the mouse model for studying human congenital craniofacial malformations, such as Goldenhar's and Treacher Collins Syndrome, is also explored.
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