Document Detail


What is 'Pierre Robin sequence'?
MedLine Citation:
PMID:  18977702     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Different nosology has hampered our understanding of patients with Robin sequence. Defining this disorder correctly has important consequences for physicians and parents. While reviewing treatment options for Robin sequence we were surprised to see how often different definitions were used to describe this condition. This prompted us to perform a review into the current understanding when diagnosing and defining this disorder. At our Annual Dutch Cleft Palate Meeting a questionnaire was given to all those attending requesting them to summarise characteristics needed for a definition of 'Robin sequence'. Sixty-six questionnaires were returned, demonstrating 29 different descriptions. Our study demonstrates that there is widespread confusion in the Netherlands defining Robin sequence. This lack of uniformity in the definition hampers the comparison of outcome studies. The treatment of patients with Robin sequence often involves multidisciplinary involvement, making it crucial to have one common definition. We suggest using the description originally summarised by Pierre Robin consisting of micrognathia, glossoptosis and airway compromise. Once the diagnosis of Robin sequence is made other adjuncts such as an associated cleft palate, syndrome or feeding problems could be added to the description.
Authors:
C C Breugem; A B Mink van der Molen
Publication Detail:
Type:  Journal Article     Date:  2008-11-01
Journal Detail:
Title:  Journal of plastic, reconstructive & aesthetic surgery : JPRAS     Volume:  62     ISSN:  1878-0539     ISO Abbreviation:  -     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-11-16     Completed Date:  2009-12-23     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101264239     Medline TA:  J Plast Reconstr Aesthet Surg     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  1555-8     Citation Subset:  IM    
Affiliation:
Division of Pediatric Plastic Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, 3508 AB, Utrecht, The Netherlands. c.c.breugem@umcutrecht.nl
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MeSH Terms
Descriptor/Qualifier:
Airway Obstruction / diagnosis
Cleft Palate / diagnosis
Humans
Medicine
Micrognathism / diagnosis
Netherlands
Pierre Robin Syndrome / classification,  diagnosis*
Tongue / abnormalities

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