Document Detail

Acrocallosal syndrome: a case report and literature survey.
MedLine Citation:
PMID:  19619433     Owner:  NLM     Status:  MEDLINE    
Acrocallosal syndrome (ACS) is a rare, genetically transmitted disorder characterized by facial deformities. These include a large forehead, large anterior fontanelle, broad nasal bridge with increased intercanthal distance, partial or complete agenesis of the corpus callosum, polysyndactyly, polydactyly, and mental retardation. Limited information concerning the dental development and treatment has been published. In addition to the classic facial deformities aforementioned, the other most commonly reported oral findings are: short philtrum/upper lip (30%); high-arched palate (30%); cleft lip/palate (20%); micro/retrognathia (13%); open mouth (15%); thin lips (11%); and 1 report of over-retained primary teeth. Seizure disorders are also a common finding due to the neuroanatomical deformities associated with this disorder. The purpose of this report was to describe the case of a 7-year-old male child with acrocallosal syndrome who presented with a cleft lip and palate, hydrocephalus, a seizure disorder, and delayed exfoliation of his primary dentition and was observed for 4 years. A review is conducted to present the pertinent medical literature concerning the oral findings associated with ACS. Dental management of this case and possible contributing factors of delayed exfoliation/permanent tooth eruption are also discussed.
Brian D Hodgson; Lindsey Davies; Cesar D Gonzalez
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Publication Detail:
Type:  Case Reports; Journal Article; Review    
Journal Detail:
Title:  Journal of dentistry for children (Chicago, Ill.)     Volume:  76     ISSN:  1935-5068     ISO Abbreviation:  J Dent Child (Chic)     Publication Date:    2009 May-Aug
Date Detail:
Created Date:  2009-07-21     Completed Date:  2009-10-29     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101180951     Medline TA:  J Dent Child (Chic)     Country:  United States    
Other Details:
Languages:  eng     Pagination:  170-7     Citation Subset:  D; IM    
Program in Pediatric Dentistry, Department of Developmental Sciences, School of Dentistry, Marquette University, Milwaukee, Wis, USA.
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MeSH Terms
Acrocallosal Syndrome / complications*,  diagnosis
Dentition, Primary
Tooth Abnormalities / genetics*,  therapy
Tooth Eruption / genetics

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