ROBIN ANOMALAD - EMBRYOPATHOLOGY AND MANA GEMENT

Citation
P. Montoya et al., ROBIN ANOMALAD - EMBRYOPATHOLOGY AND MANA GEMENT, Annales de pediatrie, 41(5), 1994, pp. 287-302
Citations number
NO
Categorie Soggetti
Pediatrics
Journal title
ISSN journal
00662097
Volume
41
Issue
5
Year of publication
1994
Pages
287 - 302
Database
ISI
SICI code
0066-2097(1994)41:5<287:RA-EAM>2.0.ZU;2-N
Abstract
Robin anomalad is a fairly common cause of neonatal respiratory distre ss and swallowing disorders (1/3 000 births). Pierre Robin performed a detailed clinicopathological study of this anomalad, which is charact erized by micrognathia, cleft palate, and posterior anchorage of the t ongue. Advances have recently been achieved in three directions. The e mbryopathogenesis of Robin anomalad has been shown to involve abnormal migration of the rhombencephalic neural crest cells, which is why mic rognathia and functional swallowing disorders are prominent features. Genetic and nosologic studies have established that many different cau ses can lead to Robin anomalad and that, as a result, concomitant ma f ormations are common and genetic counseling difficult. Lastly, the man agement of Robin anomalad has improved. During the neonatal period, th e development of specific intensive care techniques has resulted in a substantial decrease in the mortality rate. Functional surgery (restor ation of muscle balance) has replaced procedures aimed at improving lo cal mechanical conditions (glossopexy, tracheotomy).