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Early Mandibular Distraction to Relieve Robin Severe Airway Obstruction in Two Siblings with Lymphedema–Distichiasis Syndrome

Identifieur interne : 001E77 ( Main/Curation ); précédent : 001E76; suivant : 001E78

Early Mandibular Distraction to Relieve Robin Severe Airway Obstruction in Two Siblings with Lymphedema–Distichiasis Syndrome

Auteurs : Paola Papoff [Italie] ; Marco Castori [Italie] ; Lucia Manganaro [Italie] ; Fabio Midulla [Italie] ; Corrado Moretti [Italie] ; Piero Cascone [Italie]

Source :

Journal of Maxillofacial & Oral Surgery [ 0972-8279 ] ; 2015.

RBID : PMC:5048309

Abstract

Although micrognathia and cleft palate have been reported in patients with Lymphedema–distichiasis syndrome (LDS), the classic Robin sequence with glossoptosis and airway obstruction has not been previously described in patients with genetically confirmed LDS. Here we report on two female siblings with LDS confirmed by a FOXC2 mutation who presented at birth with severe airway obstruction related to Robin sequence. Respiratory obstruction was successfully managed by early distraction osteogenesis. Our report highlights the unusual occurrence of Robin sequence in LDS patients and advises distraction osteogenesis to resolve breathing problems in LDS patients who present with Robin related severe airway obstruction.

Url:

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5048309

DOI: 10.1007/s12663-015-0774-5
PubMed: 27752211
PubMed Central: 5048309

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PMC:5048309

Le document en format XML

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 mutation who presented at birth with severe airway obstruction related to Robin sequence. Respiratory obstruction was successfully managed by early distraction osteogenesis. Our report highlights the unusual occurrence of Robin sequence in LDS patients and advises distraction osteogenesis to resolve breathing problems in LDS patients who present with Robin related severe airway obstruction.</p>
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Early Mandibular Distraction to Relieve Robin Severe Airway Obstruction in Two Siblings with Lymphedema–Distichiasis Syndrome

Early Mandibular Distraction to Relieve Robin Severe Airway Obstruction in Two Siblings with Lymphedema–Distichiasis Syndrome

Source :

Abstract

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