|Other encyclopedia topics:||A-Ag Ah-Ap Aq-Az B-Bk Bl-Bz C-Cg Ch-Co Cp-Cz D-Di Dj-Dz E-Ep Eq-Ez F G H-Hf Hg-Hz I-In Io-Iz J K L-Ln Lo-Lz M-Mf Mg-Mz N O P-Pl Pm-Pz Q R S-Sh Si-Sp Sq-Sz T-Tn To-Tz U V W X Y Z 0-9|
|Contents of this page:|
Alternative Names Return to topRobin sequence
Definition Return to top
Pierre Robin syndrome is a condition present at birth in which an infant has a very small lower jaw, a tongue that tends to fall back and downward, and a soft cleft palate.
The syndrome is also called Pierre Robin complex or sequence.
Causes Return to top
The specific causes of Pierre Robin syndrome are unknown. It may be part of many genetic syndromes. The lower jaw develops slowly over the first few months of life before birth, but speeds up during the first year after birth. The falling back of the tongue may cause choking episodes and feeding and breathing difficulties, especially when the child sleeps.
Symptoms Return to top
Exams and Tests Return to top
A health care provider can usually diagnose this condition during a physical exam. Consulting with a genetics specialist can rule out other problems linked to this syndrome.
Treatment Return to top
Infants with this condition should NOT be put on their back, so that the tongue does not fall back into the airway.
Problems associated with this syndrome tend to get better over the first few years as the lower jaw grows to a more normal size.
In moderate cases, the patient requires placement of a tube through the nose and into the airways to avoid airways blockage. In severe cases, surgery is needed to prevent upper airways obstruction. A tracheostomy (surgery to make a hole in the windpipe) is sometimes required.
Feeding must be done very carefully to avoid choking and breathing liquids into the airways.
Support Groups Return to top
For support and information, see www.pierrerobin.org and www.cleftline.org.
Outlook (Prognosis) Return to top
Choking and feeding problems may go away on their own as the jaw grows. There is a significant risk of problems if the airways are not protected against blockage.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
This condition is often seen at birth.
Call if choking episodes or breathing problems increase in frequency. Airways blockage may cause a high-pitched crowing noise when the child breathes in. It can also lead to blueness of the skin (cyanosis).
Also call if other breathing problems occur.
Prevention Return to top
There is no known prevention. Treatment may reduce the number of episodes of breathing problems and choking.Update Date: 10/24/2007 Updated by: James L. Demetroulakos, M.D., F.A.C.S., Department of Otolaryngology, North Shore Medical Center, Salem, MA. Clinical Instructor in Otology and Laryngology, Harvard Medical School. Review provided by VeriMed Healthcare Network.