Wentworth-Douglass Hospital
(603) 742-5252
Decrease (-) Restore Default Increase (+) font size
Physicians
Site Search

Infant hard and soft palates
Infant hard and soft palates


Definition:

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.



Alternative Names:

Robin sequence



Causes, incidence, and risk factors:

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:
  • Cleft soft palate
  • High-arched palate
  • Jaw that is very small with significant receding chin
  • Jaw placed unusually far back in the throat
  • Large-appearing tongue in relation to jaw
  • Natal teeth (teeth appearing when the baby is born)
  • Recurrent ear infections
  • Small opening in the roof of the mouth, which causes choking


Signs and tests:

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:

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:

For support and information, see www.pierrerobin.org and www.cleftline.org .



Expectations (prognosis):

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.



Complications:

Calling your health care provider:

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:

There is no known prevention. Treatment may reduce the number of episodes of breathing problems and choking.




Review Date: 10/24/2007
Reviewed 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.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com


Find What You Need

Events
Careers
Foundation
About Us
Contact
Directions
News
Social Media Agreement
Joint Notice
Web Privacy Policy
WDH Staff Portal

Centers & Services

Cancer Center
Cardiovascular Care
Joint Replacement
Women & Children's
Physician Offices
Other Services

Conditions & Treatments

Health Library

Support Services

Support Groups
Care-Van
Dental Center
Social Work
Food & Nutrition
Integrative Wellness
Spiritual Care
Concerns & Grievances
Homecare and Hospice

For Patients

Pay Your Bill Online
Pricing Estimates
Financial Assistance
Interpreter Services
Surgery Preparation
Medical Record Request
Advance Directives
Clinical Research & Trials

For Healthcare Professionals

Work and Life
Financial Well-Being
Career and Growth

The Wentworth-Douglass Health System includes:

 

Address

Wentworth-Douglass Hospital
789 Central Avenue, Dover, NH 03820
Phone: (603) 742-5252
Toll free: 1 (877) 201-7100