Central nervous malformations in presence of clefts reflect developmental interplay

Abstract Children with cleft lip and/or cleft palate (CLP) often have additional congenital malformations. The reported incidences are variable and presumed underlying mechanisms are rarely discussed. In this study, 245 CLP patients of a geographically defined population were retrospectively reviewe...

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Bibliographic Details
Published in:International journal of oral and maxillofacial surgery 2007-04, Vol.36 (4), p.289-295
Main Authors: Mueller, A.A, Sader, R, Honigmann, K, Zeilhofer, H.-F, Schwenzer-Zimmerer, K
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - epidemiology
associations
Biological and medical sciences
Cardiovascular Abnormalities - epidemiology
Child, Preschool
cleft lip
Cleft Lip - epidemiology
cleft palate
Cleft Palate - epidemiology
Cohort Studies
congenital
Craniofacial Abnormalities - epidemiology
Cross-Sectional Studies
Dentistry
Europe - epidemiology
Eye Abnormalities - epidemiology
Facial bones, jaws, teeth, parodontium: diseases, semeiology
Humans
Incidence
Infant
Infant, Newborn
malformations
Medical sciences
multiple abnormalities
Musculoskeletal Abnormalities - epidemiology
Nervous System Malformations - epidemiology
Non tumoral diseases
Otorhinolaryngology. Stomatology
Retrospective Studies
Surgery
Syndrome
Urogenital Abnormalities - epidemiology
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Summary:Abstract Children with cleft lip and/or cleft palate (CLP) often have additional congenital malformations. The reported incidences are variable and presumed underlying mechanisms are rarely discussed. In this study, 245 CLP patients of a geographically defined population were retrospectively reviewed for additional major malformations and minor anomalies. Malformation incidences in the CLP population were compared to those in a literature-based cohort from the general population. Of the CLP patients, 21% showed either major malformations or minor anomalies. In one-fifth of them a recognized syndrome was identified. Two thirds of the CLP patients with minor anomalies also had major malformations. Major malformations in non-syndomic CLP patients were found with decreasing frequency in the musculoskeletal (5.1%), central nervous (4.2%), urogenital (3.8%) and cardiovascular (3.4%) systems. Malformations in these organ systems and minor anomalies should be ruled out by a specialist. The rise in malformation incidence, when compared to the general population, was highest for organs of ectodermal and ecto-mesodermal origin: central nervous system (13 times), ocular (7 times) and craniofacial system (7 times). The result reflects the close interplay of craniofacial and brain development.
ISSN:0901-5027
1399-0020
DOI:10.1016/j.ijom.2006.10.018