Pierre Robin Syndrome (Pierre Robin Sequence) Pierre Robin Syndrome(sequence):
is a condition in which the the infant has an unusually small lower jaw and a tongue that falls back in the throat causing difficulty breathing. Causes The exact cause of Pierre Robin Syndrome is unknown.
It is believed to be caused by the failure of the lower jaw to develop before birth. At 7 to 10 weeks of gestation the lower jaw develops faster to allow the tongue to move down from the middle of the palate. If the development of the lower jaw is not appropriate and does not happen properly then the tongue may hinder the closure of the palate causing cleft palate. The small or dislocated lower jaw also causes the tongue to shift backwards resulting in difficulty breathing. Symptoms Cleft soft palate High-arched palate
Small opening in roof of the mouth (might cause chocking) Jaw that is abnormally small (Micrograthia) Jaw placed abnormally far back in the throat Downward displacement of the tongue (Glossoptosis) Large tongue Natal teeth Ear infections Diagnosis The best way to diagnose a newborn is through physical examination. 1 in 8,500 born with this condition Treatment Do place the baby in a supine position. This can cause choking because their tongue will fall backward and obstruct the airway. Feeding must be done very carefully to avoid choking and breathing liquids into the airways. The child may need to be fed through a nasogastric tube to prevent choking. In some cases a tracheotomy must be performed. Treatment In severe cases Distraction osteogenesis (mandibular distraction) is performed. Possible Complications
Breathing difficulties, especially when the child sleeps Choking episodes Congestive heart failure Feeding difficulties Low blood oxygen and brain damage (due to difficulty breathing) Pulmonary hypertension Death