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.
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Chad is running this year’s Boston Marathon to honor his daughter, Izzy, who was born with Robin sequence.
Robin Sequence | Symptoms & Causes
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.
Robin Sequence | Diagnosis & Treatments
How is Robin sequence diagnosed?
A doctor can diagnose Robin sequence during an exam immediately after your baby is born. The visible symptoms of this sequence are:
- a small lower jaw (mandible)
- an opening in the roof of the mouth (cleft palate)
- a tongue that is positioned further back in the mouth than normal (glossoptosis)
Infants with these facial differences are commonly taken to the neonatal intensive care unit (NICU) in the first day of life to be monitored for breathing problems (oxygen desaturations) and to provide assistance with feeding.
If your child has been diagnosed with Robin sequence, a geneticist can provide a full genetic evaluation to determine if the sequence is part of a syndrome that affects other organs and tissues.
The expert team of doctors and specialists at Boston Children’s Hospital includes a geneticist who can provide a thorough evaluation using a simple blood test.
What are the treatment options for Robin Sequence?
Treatment for Robin sequence ensures your baby can eat and breathe safely and comfortably. At Boston Children’s Hospital, our dedicated and knowledgeable team of specialists from several disciplines provides you and your child with the care and expertise necessary to treat this combination of facial differences. We provide both surgical and nonsurgical treatments.
Airway and breathing treatments
Children with Robin sequence may have trouble getting enough oxygen. This is due to glossoptosis — a tongue that is positioned further back in the mouth than normal.
Nonsurgical treatments to assist with breathing include:
- Positioning. Laying your baby on his or her side or belly will help to pull the tongue forward and unblock the airway. Your child will need to be continuously monitored with an oxygen saturation monitor.
- Nasopharyngeal airway. At Boston Children’s, our craniofacial team can insert a small, flexible tube into your child’s nose and down the throat (ending just above the vocal cords). This tube keeps the airway open by preventing the tongue from falling back and blocking it. This tube will need to be changed every few days. If your baby needs to use this tube over the long term, the size and length may need adjusting to accommodate your baby’s growth. Learn about our Craniofacial Program.
Some children may also require surgery. At Boston Children’s, potential surgical interventions include:
- Tongue-lip adhesion (TLA) — This procedure:
- corrects the position of your baby’s tongue by pulling the base of the tongue forward and stitching it to the lower lip
- holds the tongue forward until your child develops a more stable airway with growth
- is reversed when your baby’s cleft palate is repaired, at approximately 9 to 11 months old
- Mandibular distraction osteogenesis (MDO) — This process:
- lengthens the jaw in a forward direction
- pulls the tongue base forward
- relieves the obstruction of the airway
- involves generating new bone by progressively stretching divided segments of the jaw
At Boston Children’s, our dedicated craniofacial nurses will weigh your child and provide frequent follow-ups to make sure you have answers to all your questions. As we monitor your child’s growth and weight gain, our team of doctors and nurses may suggest the following:
- Specialty feeders: The Haberman feeder is the most commonly used bottle for infants with Robin sequence, a cleft palate, or both. In this specially designed bottle, the nipple is separated from the bottle by a one-way valve. It helps your baby feed by rewarding even the weakest suck with formula or breast milk. It also:
- never floods
- reduces wind, vomiting, and colic
- has variable flow settings
- allows to you apply compression to help your baby receive milk
- Nasogastric feeding tube (NG tube): This is a flexible tube made of rubber that is passed through your baby’s nose and into the stomach. NG tubes are utilized as a temporary solution (weeks to months) to ensure weight gain.
- Gastrostomy feeding tube (G-tube): A feeding tube that is inserted directly into the stomach to provide nutrition. This tube is surgically placed and is used as a long-term solution (six months to years).
Cleft palate repair
Your child’s cleft palate will be treated when he or she is 9 to 11 months old. Boston Children’s provides one of the largest cleft lip and palate programs of its kind. Our combination of specialized training, innovative treatments, and compassionate, family centered care has made us a national leader in the field. This program can provide your child and family with:
- highly advanced surgical cleft palate repair
- dental and orthodontic treatment
- feeding guidance and nutritional support
- evaluation and management of hearing loss
- speech therapy
- psychosocial support and counseling
The type of treatment depends on the extent of the cleft and may include:
- Dentofacial orthopedics: Repairs of the face and jaw that may require appliances in upper jaw to widen the palate or headgear to correct an underbite.
- corrective surgery and follow-up surgery to make additional corrections.
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 families residing outside of the United States, please call Boston Children's Global Services at +1-617-355-5209.