Pierre Robin Syndrome; Causes, Characteristic Features and Management

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Pierre Robin Syndrome also known as Robin anomaly; Pierre-Robin sequence

Incidence of Pierre Robin Syndrome

This is a rare condition, but one which can cause severe problems in infancy. It can exist on its own, or as part of a wider group of rare syndromes all of which have the Pierre-Robin anomaly in addition to other features. It is presumed that both boys and girls are affected equally, apart from, of course, the X-linked variety.

Cause of Pierre Robin Syndrome

The inheritance pattern of the Pierre-Robin syndrome is not exactly known. There is a possibility that the condition may be inherited as an autosomal recessive. There have also been suggestions that there may be an X-linked variant which has the additional problems of heart abnormalities and club-foot. 

There is no antenatal diagnosis possible, although the cleft palate or the small jaw may be seen on ultrasonic scanning.  

Characteristic Features of Pierre Robin Syndrome

Pierre-Robin-syndrome

The initial abnormality from which all the other features follow (hence the term ‘sequence’) is a very under-developed lower jaw. 

During the process of development, there is cleft palate . Due to this initial defect, the child can have severe breathing problems at birth.The small lower jaw causes the tongue to be positioned further back than is usual. 

As a result of these unusual anatomical features the child’s tongue will be prone to fall back into his/her throat, thereby effectively obstructing breathing 

Swallowing difficulties, again due to the positioning of the tongue, can cause problems with feeding in the early days.

Fortunately, once the difficulties of breathing and feeding in the early days of life are over, the child’s lower jaw will develop into a normal size in relation to the rest of his/her face. This ‘catch-up’ growth is especially marked during the first two years of life.

Later on in childhood there may be dental problems with over crowding and irregular teeth in the lower jaw. 

Management of Pierre Robin Syndrome

  • Breathing difficulties Management: It will need intensive care facilities for the first few weeks of life. It is advisable that all new-born babies with the Pierre-Robin syndrome should be nursed in a partially prone position.
  • The completely prone position should be avoided due to the possible link with the sudden infant death syndrome. 
  • This way of lying will allow the child’s tongue to fall forward, rather than backwards into his/her throat, and so avoids obstruction to breathing. 
  • Feeding difficulties Management: It will also need special care in the early days. Simple measures such as feeding the child in an upright position may be all that is necessary to ensure adequate nutrition. 
  • Cleft Palate Management: If a cleft palate is also present,  a temporary dental plate can help with this initially.The cleft palate will need surgery to close the gap and ensure easier feeding, breathing and speech. 
  • Speech therapy: It may be necessary in the preschool years to ensure clear speech following the repair of the cleft palate.
  • Dental Treatment: Dental treatment must be given throughout the early years, until all the second teeth have erupted satisfactorily. Orthodontic treatment may be needed to straighten crooked teeth and to ensure a correct ‘bite’.  
Pierre-Robin-syndrome
Pierre Robin syndrome

Conclusion:

Most children with the Pierre-Robin syndrome will grow into adults with the same range of abilities and aptitudes as everyone else. The difficulties in the early days of life will only very rarely have any long-lasting effects. 

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