title: Microtia in the Netherlands: clinical characteristics and associated anomalies.
abstract: BACKGROUND
  In Europe there have been few detailed reports on the clinical characteristics of microtia patient populations. The objective of the present study is to contribute to our insight of microtia in Europe by examining the Dutch microtia population treated in the University Medical Center Utrecht (UMCU) with regards to its clinical features and associated anomalies. In addition, an overview of the literature is provided for thorough comparison.
  
  
  METHODS
  A retrospective chart review was performed for all microtia patients referred to the UMCU for reconstructive surgery of the auricle over the period 1990-2012. Previous studies were identified by a systematic search of the electronic literature databases PubMed and Embase. In a subsequent meta-analysis the results from the literature review were pooled by geographical region to facilitate comparison.
  
  
  RESULTS
  A total of 204 microtia patients were referred for reconstructive surgery during 1990-2012. This group was characterized by a male predominance of 60.8%. Unilateral disease was observed in 91.7% of patients, affecting the right auricle in 66.3%. In unilateral patients lobule type microtia was seen in 59.9%, (small) concha type in 34.4% and anotia in 5.7%. The more frequent anomalies associated with microtia were atresia of the acoustic meatus (76.0%), preauricular skin tags (30.5%), hemifacial microsomia (27.5%), facial nerve paralysis (8.3%) and congenital heart disease (2.5%). Familial occurrence of microtia was reported for 2.0% of UMCU patients and for 10.0% of patients in the literature.
  
  
  CONCLUSIONS
  The clinical characteristics of microtia in the Netherlands correspond to those reported for other patient populations in the literature. Most congenital anomalies associated with microtia in Dutch patients belong to the Oculo-Auriculo-Vertebral Spectrum. The considerable degree of familial microtia observed in the literature points to a substantial genetic component in the etiology of the condition.
authors.affiliation: Division of Plastic and Reconstructive Surgery; University of Utrecht Medical Center, Heidelberglaan 100, PO Box 85500, 3508 GA Utrecht, The Netherlands. Electronic address: D.P.F.vanNunen@umcutrecht.nl.
authors.forename: Daan P F
authors.initials: DP
authors.lastname: van Nunen
authors.affiliation: Division of Plastic and Reconstructive Surgery; University of Utrecht Medical Center, Heidelberglaan 100, PO Box 85500, 3508 GA Utrecht, The Netherlands.
authors.forename: Mischka N
authors.initials: MN
authors.lastname: Kolodzynski
authors.affiliation: Department of Medical Genetics; University of Utrecht Medical Center, Heidelberglaan 100, PO Box 85500, 3508 GA Utrecht, The Netherlands.
authors.forename: Marie-José H
authors.initials: MJ
authors.lastname: van den Boogaard
authors.affiliation: Division of Plastic and Reconstructive Surgery; University of Utrecht Medical Center, Heidelberglaan 100, PO Box 85500, 3508 GA Utrecht, The Netherlands.
authors.forename: Moshe
authors.initials: M
authors.lastname: Kon
authors.affiliation: Division of Plastic and Reconstructive Surgery; University of Utrecht Medical Center, Heidelberglaan 100, PO Box 85500, 3508 GA Utrecht, The Netherlands. Electronic address: C.C.Breugem@umcutrecht.nl.
authors.forename: Corstiaan C
authors.initials: CC
authors.lastname: Breugem
mesh_terms: D065817:Congenital Microtia / Q000175:diagnosis / Q000453:epidemiology*; D054644:Ear Auricle / Q000002:abnormalities / Q000601:surgery; D005260:Female; D006801:Humans; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D019651:Plastic Surgery Procedures / Q000706:statistics & numerical data; D012189:Retrospective Studies
keywords: Anotia; Disease attributes; Ear abnormalities; Epidemiology; Microtia