Pierre Robin Syndrome is a syndrome that is found in children with the major visible characteristic being the child being born with inappropriate jaws. The syndrome is also called Pierre Robin Sequence or Pierre Robins Complex.
Pierre Robin Syndrome in children may manifest in various types. For instance, the baby is born with a jaw that is smaller in size compared to the face; the condition also has a name to it- micrognathia. Then there is another condition called retrognathia, where the baby is born with the mandible(jaw) set significantly back as compared to the upper jaw. In this case, the tongue is drawn backwards, near the throat. Such dislocation can hinder the passage of the air during respiration; this condition in known as glossoptosis. Most of such babies may also suffer from cleft palate, but not all; and none of them will have cleft lip.
History of Pierre Syndrome
The condition has been given various names since it was first discovered like Pierre Robin Triad or Robin Anomalad. Finally, two names struck to Pierrre Syndrome- Pierre Robin Sequence or Pierre Robin Complex. The name of this syndrome is actually derived from Dr. Pierre Robin, a French doctor who first diagnosed the issue, types and conditions of cleft, dislocation of tongue and small lower jaw in 1923.
Causes of Pierre Robin Syndrome
The fundamental cause of Pierre Robin Syndrome is the inability of the lower jaw to develop during the fetal development, i.e.; before birth. About 7 to 10 weeks of pregnancy the lower jaw develops rapidly that makes the tongue move down from the mid of the palate. If the development of the lower jaw is not as it is supposed to then the tongue may obstruct the closure of the plate that may cause cleft palate. The small or dislocated lower jaw also causes the tongue to shift backwards resulting in problems during respiration. However, in some cases such physical conditions may be attributed to other reasons.
Is Pierre Robin Syndrome a rare disease?
Pierre Robin Syndrome is quite a rare physical condition and not so commonly diagnosed. Only one in 1000 or 2000 babies are born with this condition. However, cleft lip or cleft palate is reported 1 in every 700 live births.
Will my child get Pierre Robin Syndrome?
Most parents have this fear if their future child may also get Pierre Robin Syndrome. It is important ot know that the syndrome can happen without any specific reason or it can be due to the presence of any other syndrome. If parents already have a child with Pierre Robin syndrome, there is 1 to 5 percent chance of having another child with the same condition.
The medical industry is still in dark about coming up with prediction of Pierre Robin Syndrome. Also, the syndrome has been diagnosed in children suffering from teratogenic syndromes (conditions that are environmentally induced), like Fetal Alcohol Syndrome or Fetal Hydantoin syndrome. Pierre Robin Syndrome has also be diagnosed in people who have Treacher Collins Syndrome and Stickler Syndrome
Pierre Robin Syndrome- Symptoms:
Some of the symptoms found in children suffering from Pierre Robin Syndrome include
- Smaller jaw as compared to the face
- Cleft palate
- Jaw positioned significantly backwards in the mouth
- Natal teeth
- Tongue visibly bigger in size
- Recurring ear infection
How the person is affected with Pierre Robin Syndrome
Pierre Robin syndrome is like any other birth defect and may vary in complexity. Some common problems are related to breathing and feeding, for children. Positioning the child properly during the feeding process is important for the parents to know. The parents should not place the baby in his or her back. In some cases, even proper positioning during the feeding may not yield beneficial results, they may have to be supported with specially designed devices that may help in keeping airway unobstructed. There are afflicted children who may also experience ear issues such as fluid accumulation in or around the ear. A ventilation tube may be placed strategically inside the ear to decrease the buildup of fluid. In some cases when other treatments for Pierre Syndrome do not work then surgical treatment may be advised to help the child breathe properly.
Pierre Robin Syndrome treatment
In case of many new born children the lower jaw may develop more rapidly through the first year of his/ her life. In some cases the mandible or the jaw develops so fast that by the time the baby turns 5 to 6 years old the conditions looks normal. Children who do not undergo such significant development may have to undergo surgical treatment at the jaw. However, there is no established study on why children mandible develops at such faster and unstable rate. If any cleft palate is experienced it should also be closed through surgical procedures. Generally surgical procedure on the affected child is done during his/ her first or second year; however, in some cases it may depend on the development rate of the mandible. It is essential for a child with cleft palate to regularly examine by speech pathologist as these children are at risk of developing defective or delayed speech issues.
It is essential to take Pierre Robin Syndrome seriously. Children may also get other physical issues it is advised for parents to take the child to craniofacial center where suitable aid will be provided to you. The experts can evaluate the condition and suggest a suitable line of treatment for the Pierre Robin Syndome afflicted child.
Pierre Robin Syndrome Pictures