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Pierre Robin syndrome
Etiology:
- may be associated with many genetic syndromes
Clinical manifestations:
- cleft palate (soft palate)
- high-arched palate
- micrognathia with small (receding) chin
- jaw that is far back in the throat
- recurrent otitis
- small opening in the roof of the mouth, which causes choking
- teeth that appear when the baby is born (natal teeth)
- tongue that is large compared to the jaw
Complications:
- feeding difficulties
- choking episodes
- dyspnea, especially when the child sleeps
- congestive heart failure
- ischemia & brain damage (due to difficulty breathing)
- pulmonary hypertension
- death
Management:
- do not put infants on their back
- tracheal intubuation in moderate cases
- surgery in severe cases
- nasogastric tube may be needed for feeding
General
genetic syndrome (multisystem disorder)
References
- PubMed Health: Pierre Robin syndrome
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002574/
- Wikipedia: Pierre Robin syndrome
http://en.wikipedia.org/wiki/Pierre_Robin_syndrome
- Pierre Robin sequence
Cleft Palate Foundation
http://www.cleftline.org/what-we-do/publications/fact-sheets/pierre-robin-sequence/
- Medline Plus: Pierre Robin syndrome
http://www.nlm.nih.gov/medlineplus/ency/article/001607.htm
- Tewfi TL and Meyers AD
Medscape: Pierre Robin syndrome
http://emedicine.medscape.com/article/844143-overview