Robin Sequence

What is Robin sequence?

Robin sequence, also known as Pierre Robin sequence, is a combination of facial differences that are present when a baby is born and usually includes:

  • a small, underdeveloped lower jaw (micrognathia)
  • a tongue that is positioned further back in the mouth than normal (glossoptosis)
  • breathing difficulty due to airway obstruction

This combination of features can lead to breathing and feeding problems early in life. As a result, some affected babies have difficulty growing and gaining weight at the expected rate. In addition, many children with Robin sequence have an opening in the roof of the mouth (cleft palate). This also affects a child's ability to feed.

Experts describe this condition as a "sequence" because they believe that as the embryo forms in early pregnancy, the underdeveloped lower jaw sets off a sequence of events that causes the other signs and symptoms. When the lower jaw does not grow properly, the tongue can prevent the palate (roof of the mouth) from closing, resulting in a cleft palate. The underdeveloped lower jaw also causes the tongue to be positioned at the back of the mouth, making breathing difficult.

Robin sequence affects 1 in 8,500 to 1 in 14,000 births, making it one of the most common facial differences.

 

What are the symptoms of Robin Sequence?

Robin sequence is a combination of facial differences that occurs in a developing fetus and that is apparent when your baby is born. These differences include:

  • Micrognathia: An underdeveloped or abnormally small lower jaw (mandible) that is much shorter than the rest of the face. The growth of the lower jaw may speed up during the first year of life.
  • Glossoptosis: A tongue that is positioned further back in the mouth than normal. Because of the small mandible, the tongue is too large for the airway. This can interfere with breathing.
  • Cleft palate: A cleft palate is a separation in the tissue that makes up the roof of the mouth (palate). For some babies, both the front and back parts of the palate are open. For other babies, only part of the palate is open. The cleft palate in Robin sequence is usually a rounded, or U-shaped cleft palate, in comparison to the more common V-shaped cleft palate seen in other conditions.

These differences result in a variety of symptoms, including:

  • Feeding difficulties: The smaller size of the lower jaw, position of the tongue, and the cleft of the palate can make it difficult for your baby to feed normally. Your baby might be able to learn to feed with specially adapted nipples and bottles.

More severe Robin sequence may require a temporary feeding tube.

  • Trouble breathing: Your child’s tongue may fall backwards, especially when lying on his or her back. This can block the throat and obstruct breathing and is of special concern when your baby sleeps. According to brochure, some babies respond well to positioning on the stomach, which helps pull the tongue forward during sleep. Other infants may require nasal tubes or surgery to pull or push the tongue forward.

As a result of these symptoms, some babies struggle with growth and weight gain until treated.

Learn more about Robin sequence treatments offered at Boston Children’s Hospital.

What causes Robin sequence?

Experts do not know the exact causes of Robin sequence, but there may be a genetic link. There is a higher incidence of this combination of defects in twins. This may be due to crowding in the uterus, which restricts the growth of the lower jaw.

This combination of facial differences can be seen in other syndromes, including:

  • Stickler syndrome: This is a genetic malfunction in the tissue that connects bones, heart, eyes and ears. A child with Stickler syndrome may have problems with vision, hearing, bones and joints, the heart, and facial formation.
  • Velocardiofacial syndrome (VCFS): The most common features of this syndrome are cleft palate, heart defects, and characteristic facial appearance, as well as minor learning problems and speech and feeding challenges.

How we care for Robin sequence

The Craniofacial Program at Boston Children's Hospital offers advanced procedures to correct the defects associated with Robin sequence, including cleft palate repair. Our team includes some of the world's most experienced neurosurgeons, plastic surgeons, oral and maxillofacial (jaw) specialists, dentistry professionals, psychologists, and social workers; all working together to address the many needs of your child and your entire family. We will develop a customized treatment plan that meets all of your child's needs and involves you and your family at every step of the treatment.

Download the Robin sequence brochure to learn more about the condition and our approach to care.

Make an appointment

For an appointment with the Cleft and Craniofacial Center, more information or to obtain a second opinion for your child, please call us at 617-355-6309 or email samantha.hall@childrens.harvard.edu.

International Patients

For families residing outside of the United States, please call Boston Children's Global Services at +01-617-355-5209. Global Services can assist with the review of patient medical records and schedule appointments. It can also help with customs and immigration, transportation, and hotel and housing accommodations.