Pierre Robin Sequence

Pierre Robin sequence (or syndrome) is a condition in which a baby has a smaller than normal lower jaw (called as micrognathia), a tongue that falls back in the throat (called as glossoptosis), and difficulty in breathing due to a narrow airway. Most babies with Pierre Robin sequence also have an associated cleft palate. As Pierre Robin sequence is a genetic problem and is present since the birth of the baby.

The major problems babies with Pierre Robin sequence face are:

  • Feeding Difficulty: due to the cleft palate and small mandible babies with Pierre Robin sequence find it difficult to suckle on the breast and sometimes even on the bottles.
  • Airway obstruction: babies with Pierre Robin Sequence are at risk of early upper obstruction and have increased chances of having obstructive sleep apnoea (OSA). Breathing and feeding can require a lot of energy for small babies with Pierre Robin Sequence, so they may have an inadequate weight gain.

Infants with Pierre Robin sequence needs evaluation by a team of specialist including a pediatric pulmonologist/sleep specialist, maxillofacial surgeons and dietician. Children with Pierre Robin sequence require assessment of the airway to delineate the source of airway obstruction. This is usually done by a flexible bronchoscopy. In addition children with Pierre Robin sequence require a sleep study or polysomnography to objectively diagnose obstructive sleep apnoea (OSA) and its severity.

Children with Pierre Robin sequence require help in feeding. Options include a long teat with a bottle with good positioning. Some babies may not be able to feed with a bottle and require a nasogastric tube feeding for a few months. A nasogastric tube is a thin tube which goes in through the nose and into the stomach. Early feeding via a nasogastric tube promotes weight gain in babies with Pierre Robin sequence.

Children with Pierre Robin sequence having airway obstruction or obstructive sleep apnea (OSA) require some help. Sometimes, a small tube called as a nasopharyngeal airway (NPA) is passed through the nose, behind the tongue, to improve breathing. This is usually well tolerated by most babies and helps improve the breathing and sleeping problems. The airway is removed with the mandibular growth, which usually happens in a few months.

Some children with Pierre Robin sequence require surgical procedures. In some severe cases of Pierre Robin sequence, the tongue is sewn to the lip as a temporary measure just to hold it forward called as tongue-lip adhesion. Occasionally, mandibular distraction, a technique to lengthen the lower jaw is performed in children with severe cases of Pierre Robin sequence.

We at BLK Max hospital have a complete team of specialist including paediatric pulmonologist & sleep specialist, maxillofacial surgeons and lactation specialist. Facilities for flexible bronchoscopy for young babies and sleep study or polysomnography are available.