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Pierre Robin Syndrome

Updated on August 20, 2013

Pierre Robin syndrome is a disorder characterized by the presence of congenital facial abnormalities. It is also considered as a ‘sequence’ or a sequential condition because of the associated developmental defects, wherein one involves the next malformation. The exact cause of Pierre Robin syndrome is still unknown. It is however believed that certain chromosomal genetic errors may be the cause.

Pierre Robin syndrome is also known as Pierre Robin Malformation or Pierre Robin sequence. Doctors can easily diagnose the condition after delivery of the baby. Its incidence rate is approximately one in every 8,500 to 30,000 births.

After the health care provider has diagnosed a child with Pierre Robin syndrome, parents have to seek the advice of a geneticist to rule out the presence of other abnormalities that accompany the disorder. In case other conditions also occur in the affected child, then those additional anomalies have to be medically corrected as well.

Symptoms of Pierre Robin syndrome

Some of the symptoms and anomalous facial features that accompany Pierre Robin syndrome are listed below:

  • The mandible or the lower jaw is very small, along with a tapering chin
  • Chronic ear infections
  • Severe arching of the palate
  • The jaw may also recede deep into the throat
  • The baby may be born without teeth or may have natal teeth
  • Withdrawal or posterior displacement of the tongue. The tongue may also be bigger in size as compared to the jaw
  • The upper airways may experience obstruction
  • Occurrence of a U-shaped cleft palate. The roof of the oral cavity may not be fully closed

Pierre Robin syndrome can result in a number of medical complications such as:

  • A baby affected by Pierre Robin syndrome may face respiration and feeding problems. The child will need assistance during feeding. It will generally lay down flat on the stomach in order to allow the tongue to move forward and thus open up the airways.
  • Babies with Pierre Robin syndrome may not consume sufficient calories that are essential for growth and development. Hence, they may have to be occasionally fed with the help of a nasogastric tube. Additionally, since the affected babies experience problematic breathing, it turns into an exercise by itself, thereby causing the overuse of vital calories. In extreme instances, the airways have to be freed up with the use of a nasopharyngeal tube.
  • As most babies affected by Pierre Robin syndrome also experience Stickler syndrome, parents have to seek the help of an eye doctor for confirming the presence of ocular problems.

Causes of Pierre Robin syndrome

The exact cause of Pierre Robin syndrome is still a subject of research. It has been postulated that during some stages of fetal bone development the end of the jaw gets entangled at the sternum. The sternum is a place where each of the collar bones meet together. This prevents the full development of the jaw bones.

It is also theorized that during the period between 12 to 14 weeks of pregnancy, when the growing fetus begins to move, the head movements lead to dislocation of the jaw. After this, the fetus continues growing normally till the time of delivery.However, as the jaw was dislodged during the developmental stages, the baby takes birth with an abnormally tiny jaw in comparison to other newborns that do not have the syndrome. It may also be noted that irrespective of the presence of Pierre Robin syndrome, the jaw continues to develop normally after delivery till it reaches maturity.

It is also believed that Pierre Robin syndrome is mostly caused as a symptom of some other underlying condition or syndrome. One of the most common disorders associated with Pierre Robin syndrome is Stickler syndrome. Other associated disorders may include Velocardiofacial syndrome, Fetal Alcohol Syndrome or FAS, and Treacher Collins syndrome.

Treatment of Pierre Robin syndrome

There is no specific way to treat Pierre Robin syndrome. Treatment is focused on managing all the symptoms and preventing complications. Listed below are some ways to treat the abnormalities associated with the syndrome:

  • Respiratory and feeding issues are the key concerns with a baby affected by Pierre Robin syndrome. Varied measures as listed above and below can help in resolving the feeding and breathing problems. It will also aid in providing the essential nutrients to the affected child so as to facilitate its continual growth and development.
  • Distraction osteogenesis is a process which can be used to correct the small size of lower jaw. It leads to broadening of one or both the jaws. It will also result in forward thrust of the tongue, which eventually eases obstruction of the airways.
  • Infants affected by Pierre Robin syndrome are also likely to suffer from GER or gastro-esophageal reflux. This can aggravate the existing symptoms and result in further respiratory difficulties. It is possible to alleviate the effects of GER with the use of medicines. Parents can also prevent frequent vomiting by giving the affected child small meals, several times a day, and by placing the child in a wedge in an upright position.
  • Instances of cleft palate can be corrected with surgery. There is no single way to treat this anomaly. Every case is diagnosed on an individual basis, and the treatment method is chosen as per the defect elicited by the patient.

A majority of Pierre Robin syndrome cases can be treated successfully and almost all affected children grow up into healthy adults. Most feeding problems are resolved during early childhood. However, it may also be noted that children with Pierre Robin syndrome may not achieve their full growth and developmental capacities due to persistent hypoxia that is caused due to obstruction of the airways.

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