title: Distribution of associated component abnormalities in cases with unclassified multiple ("syndromic") anota/microtia.
abstract: OBJECTIVE
  To evaluate cases with unclassified multiple congenital abnormalities (CAs) including anotia/microtia (UMAM) in order to reveal the characteristic pattern of associated other component CAs and to attempt the so-called "registry diagnosis" on the pattern of associated CAs and to stimulate the establishment of an international registry of cases with UMAM.
  
  
  METHODS
  The large population-based dataset of the Hungarian Congenital Abnormality Registry, 1980-1996, but component CAs of UMAM were based on medical records.
  
  
  RESULTS
  Finally 156 cases with UMAM were analyzed according to the number of 2-9 component CAs. There was a higher rate of bilateral anotia/microtia and anotia in UMAM cases parallel with the number of component CAs. Of 156 cases, 48 (30.8%) had registry diagnosis. The distribution of most component CAs is not random in UMAM cases, however, most CA-syndromes including anotia/microtia have not been delineated.
  
  
  CONCLUSIONS
  The evaluation of available dataset of cases with UMAM may help the identification of recognizable CA-syndromes and the delineation of new syndromes/associations with better prognosis and recurrence risk estimation, in addition to a better chance for their prevention.
authors.affiliation: Department of Oto-Rhino-Laryngologic and Head/Neck Surgical Department, National Center for Healthcare Audit and Improvement, Budapest, Hungary.
authors.forename: László
authors.initials: L
authors.lastname: Paput
authors.forename: Ferenc
authors.initials: F
authors.lastname: Bánhidy
authors.forename: Andrew E
authors.initials: AE
authors.lastname: Czeizel
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000453:epidemiology*; D000013:Congenital Abnormalities / Q000175:diagnosis / Q000453:epidemiology*; D065817:Congenital Microtia; D016208:Databases, Factual; D004423:Ear / Q000002:abnormalities*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006814:Hungary / Q000453:epidemiology; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012042:Registries; D018570:Risk Assessment; D013577:Syndrome; D013997:Time Factors