Overview
Pierre Robin in 1923 is credited as being the first to describe Robin sequence: a small jaw, leading to a tongue that falls back in the throat and upper airway obstruction. Cleft palate is not an obligatory finding, it occurs in up to 90% of patients with Robin sequence. Presumably, the palatal is intrinsically normal; the cleft is the result of an obstruction in fusion of the palate (8-12 weeks of gestation) caused by the posteriorly positioned tongue.
Cause
There is an associated syndrome in 34-46% of patients with Robin sequence, but no known medications, food, or other environmental factors during pregnancy cause Robin sequence.
Diagnosis
Pierre Robin Sequence is a clinical diagnosis and no lab tests or imaging studies are required.
Treatment
Mild cases can be treated by not placing your child on their back, so preventing the tongue from falling back into the airway. Depending on the severity of your child’s breathing and feeding difficulties, the following surgeries may be required:
- Tongue-lip adhesion or mandibular distraction to improve breathing
- Feeding tube placement to aid in feeding
- Cleft palate repair
Prognosis
As your child’s jaw grows the feeding and airway problems go away over the first few years of life.
