Pierre Robin sequence is a very very short jaw (micrognathia) that is 
placed far back, which causes the tongue to be placed far back in the 
mouth (retroplaced tongue), which occludes the palate and does not allow 
it to close (U-shaped cleft palate). It is now called a sequence rather 
than a syndrome because it is recognized that the first defect (the 
extreme micrognathia) causes the others.

Doctors differ on whether a baby can be diagnosed with Pierre Robin 
sequence when there is no cleft palate. The major difficulty in Pierre 
Robin is that the tongue falls into the airway and blocks it, so 
breathing and feeding are very difficult. If there is also an overt 
cleft palate, the baby will be unable to create negative pressure in the 
mouth.

The baby you are seeing, Julie, seems to have both a tight lingual 
frenulum and a tight superior labial frenulum, along with natal teeth. 
Diane Wiessinger was the first to publish that there could be an 
association between tongue tie and tight labial frenulum. When the baby 
also has a short jaw, feeding can be very difficult.

Catherine Watson Genna, BS, IBCLC  NYC

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