title: Unilateral aplasia of the middle cranial fossa floor in atypical hemifacial microsomia.
abstract: We present the first report of a patient with atypical hemifacial microsomia (HFM) and unilateral aplasia of the floor of the middle cranial fossa, glenoid fossa, and portions of her posterior fossa. The patient also developed a Chiari I malformation with cervical syrinx over a 3-year interval. This case report highlights the critical role of imaging in revealing serious, but clinically occult, structural abnormalities, as well as the evolution in the pathogenetic understanding of HFM.
authors.affiliation: Department of Radiology, Children's Hospital and Regional Medical Center, 4800 Sand Point Way, CH-69, Seattle, WA 98105, USA.
authors.forename: R W
authors.initials: RW
authors.lastname: Sze
authors.forename: J S
authors.initials: JS
authors.lastname: Gruss
authors.forename: M L
authors.initials: ML
authors.lastname: Cunningham
mesh_terms: D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging; D000293:Adolescent; D001139:Arnold-Chiari Malformation / Q000000981:diagnostic imaging; D002574:Cervical Vertebrae / Q000000981:diagnostic imaging; D005146:Facial Asymmetry / Q000000981:diagnostic imaging*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D021621:Imaging, Three-Dimensional; D019291:Skull Base / Q000002:abnormalities* / Q000000981:diagnostic imaging; D013595:Syringomyelia / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed; D014103:Torticollis / Q000000981:diagnostic imaging