PIERRE ROBIN SEQUENCE

(MICROGNATHIA—-GLOSSOPTOSIS—“U” SHAPED CLEFT PALATE (50%))

Pierre Robin (1867–1950) was a French dental surgeon or Stomatologist.[1] He was professor at the French School of Stomatology, and from 1914, he was editor-in-chief of the Revue de Stomatologie.

?INTRAUTERINE INSULT–A DEFORMATION RATHER THAN A MALFORMATION?

1/10,000, MAY BE LINKED TO AN ABNL IN CHROMOSOME 2, 11, 0R 17

MR (NOT IN TREACHER COLLINS)

MIXED HL—HIGH ASSOC WITH ETD AND OME—-CHL

ASSOC WITH STICKLER SYNDROME (OCULAR, HEARING AND JOINT PROBLEMS) AND MOBIUS SYNDROME

ASSOC WITH SUBGLOTTIC STENOSIS AND GERD

2/3 OPHTHALMIC ABNL—DETACHED RETINA OR GLAUCOMA—ANY CLEFT CONSIDER A OPTHO CONSULT AND AUDIO—R/O STICKLER SYNDROME (AUTO D)—-GET A GENETICS CONSULT

ASPIRATION AND RESULTANT PNEUMONIA IS COMMON

IF AN AIRWAY ISSUE—-MAY NEED A TRACH (OR MAY GET BY WITH A TONGUE LIP ADHESION—ALTHOUGH THIS IS RARELY PERFORMED ANY MORE—JUST TRACH EM)

IF THEY CAN LIMP ALONG WITH PRONE POSITIONING AND A HABERMAN FEEDER (POSSIBLY NG TUBE)

REPAIR THE PALATE WHEN THE AIRWAY PROBLEM HAS FINALLY RESOLVED DUE TO MANDIBULAR GROWTH (PALATE REPAIR AT 10 MONTHS, HB 10, 10 LBS)

DR SIDMAN –OR HIS PROTEGE DR COFER (507 284-2411)—-IS THE GURU FOR MANDIBULAR DISTRACTION OSTEOGENESIS