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Pierre robin syndrome

Pediatrics
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Clinical features: Triad

1. Micrognathia- Small lower jaw / mandible  

2. Cleft palate

3. Glossoptosis- Tongue is posterior displaced or retracted 

 

Complication:

• Airway obstruction is the m/c complication of pierre robin syndrome. 

• The small mandible and retracted tongue causes the base of the tongue to fall back into the throat, may ultimately lead to airway obstruction.

 

Treatment:

• Surgery to correct the cleft palate

• Positioning therapy to keep the tongue forward, relieving airway obstruction in most infants.

• Tracheostomy to create an airway opening in the neck.

 

 

Extra- 

Tongue protrusion and retraction- 

• Protrusion of tongue: Pushing the tongue out of the mouth, with the tip extending beyond the lips

• Retraction of tongue: Pulling the tongue back into the mouth, with the tip resting behind the lower teeth