title: Hajdu-Cheney syndrome and syringomyelia. Case report.
abstract: This 7-year-old boy with Hajdu-Cheney syndrome presented with cervical syringomyelia related to rapidly progressing platybasia. Decompressive craniectomy provided temporary improvement, and his clinical status was eventually stabilized after external immobilization, according to findings at 2.5 years of follow up. In a review of the literature the authors found 57 cases of the syndrome, only three of which were associated with syringomyelia. The youth of the patient, the severe form and rapid course of the disease, and the very specific anatomical conditions related to cranial and facial deformities raised various therapeutic problems.
authors.affiliation: Department of Neurotraumatology, University Hospital (Hôtel-Dieu), Nantes, France. alexis.faure@chu-nantes.fr
authors.forename: Alexis
authors.initials: A
authors.lastname: Faure
authors.forename: Albert
authors.initials: A
authors.lastname: David
authors.forename: Frédéric
authors.initials: F
authors.lastname: Moussally
authors.forename: Mansour
authors.initials: M
authors.lastname: Khalfallah
authors.forename: Sébastien
authors.initials: S
authors.lastname: Jacquemont
authors.forename: Olivier
authors.initials: O
authors.lastname: Hamel
authors.forename: Michele
authors.initials: M
authors.lastname: Conti
authors.forename: Antoine
authors.initials: A
authors.lastname: Hamel
authors.forename: Sylvie
authors.initials: S
authors.lastname: Raoul
authors.forename: Roger
authors.initials: R
authors.lastname: Robert
mesh_terms: D002648:Child; D031845:Hajdu-Cheney Syndrome / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D011859:Radiography; D012720:Severity of Illness Index; D013595:Syringomyelia / Q000000981:diagnostic imaging / Q000209:etiology*