[Labioglossopexy in Pierre Robin syndrome. Our experiences apropos of 70 cases].
Pierre Robin syndrome is typically characterized by glossoptosis and retrognathia associated with cleft palate, respiratory and deglutition disturbances. Two morphological tendencies arise out of this 70-patient series, 50 of whom were managed with the same therapeutical regimen, which included labioglossopexy and was associated with only one case of death: "deforming" type Pierre Robin syndrome most commonly follows a favorable course with respect to both function and facial deformations, provided labioglossopexy is carried out sufficiently early. Indeed, this surgical operation allows for prompt restoration of normal function, thereby limiting the dysfunction-deformation vicious circle. This form of Pierre Robin syndrome may find its origin in the embryo's abnormal amniotic environment. "Malformation"-associated Pierre Robin syndrome often presents with combined anomalies, respiratory disturbances that may evolve into severe airway problems, impaired deglutition and abnormal brain development; in most cases, labioglossopexy will dramatically improve the immediate state of the child, although long-term prognosis as regards malformation is rather guarded.
Bedhet
N
N
Service de Stomatologie et Chirurgie Maxillo-faciale, C.H.U. de Nantes.
Mercier
J
J
Gordeeff
A
A
Mouzard
A
A
Delaire
J
J
D000293:Adolescent; D002648:Child; D002972:Cleft Palate / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007442:Intubation, Intratracheal; D008046:Lip / Q000601:surgery*; D008297:Male; D008334:Mandible / Q000002:abnormalities; D010855:Pierre Robin Syndrome / Q000145:classification / Q000453:epidemiology / Q000601:surgery*; D012120:Respiration Disorders / Q000453:epidemiology; D063173:Retrognathia / Q000453:epidemiology; D013524:Surgical Flaps; D014059:Tongue / Q000601:surgery*
La labio-glossopexie dans le syndrome de Pierre Robin. Notre expérience à propos de 70 cas.