[The Pierre-Robin syndrome. Classification and new therapeutic approach].
Rhombencephalic failure of the suction-swallowing, excess of central and obstructive ventilatory arrests with hypoxia and hypercapnia, vagal hypertonia and esophagogastric motor abnormalities are the new clinical signs observed in children presenting with the Pierre Robin's syndrome. A therapeutic management adapted to each of the types I, II, III of the syndrome were defined and a good nursery-nursing allowed a reduction in the mortality-rate from 27 to 5%. Still considered by some as a malformative and glossoptosing disorder whose etiology is only bucco-pharyngeal in origin, this syndrome, common to numerous embryopathies, is a precocious embryonal abnormality of the brain stem neurogenesis, expressed by the dramatic failure of the physiological oro-ventilation system. This syndrome appears to be a peculiar form of dysautonomia of the brain stem development with an uncertain future, often transitory, isolated or associated but in the heart of pediatric internal medicine and its multi-disciplinarity.
Couly
G
G
Service de Stomatologie, Hôpital des Enfants-Malades, Paris.
Cheron
G
G
de Blic
J
J
Despres
C
C
Cloup
M
M
Hubert
P
P
D001237:Asphyxia / Q000175:diagnosis; D003389:Cranial Nerve Diseases / Q000503:physiopathology; D003680:Deglutition Disorders / Q000175:diagnosis; D005260:Female; D006801:Humans; D000860:Hypoxia / Q000175:diagnosis; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010855:Pierre Robin Syndrome / Q000145:classification* / Q000196:embryology / Q000628:therapy; D011379:Prognosis; D014630:Vagus Nerve
Le syndrome de Pierre Robin. Classification et nouvelle approche thérapeutique.