Pierre Robin Sequence

Signs and Symptoms

Pierre Robin sequence is a condition characterized by a wide U-shaped cleft of the palate (roof of the mouth) and a small lower jaw. This combination of features can make it difficult, and sometimes impossible, for an infant to feed and breathe normally. When needed, special procedures are performed at birth to ensure the infant can adequately breathe and feed.

Diagnoses

Pierre Robin sequence is diagnosed at birth by physical examination. The presence of the small lower jaw distinguishes Pierre Robin sequence from cleft palate only. Pierre Robin sequence should be diagnosed and treated by an experienced team of experts recognized by the American Cleft Palate-Craniofacial Association, like the Center for Craniofacial Anomalies at UCSF.

Treatment

An infant with Pierre Robin sequence should be seen shortly after birth by a nurse from the Craniofacial Center, who will make sure he or she is able to adequately feed and breathe. Afterwards the child should be seen for a full team evaluation.

If the tongue obstructs the airway or if the child is having any breathing difficulties, he or she will be referred to the pediatric pulmonary clinic. The child may need a lip-tongue plication -- a procedure that temporarily attaches the tongue to the lower lip, opening the airway and making it easier for the infant to breathe.

An infant with Pierre Robin sequence usually needs to be bottle-fed (with breast milk or formula) using special nipples. In addition, he or she may require supplemental calories to fuel the extra effort it takes to breathe and swallow.

Surgery is necessary to repair a cleft palate and this intervention is considered reconstructive and not cosmetic. Since ear infections are more common for a child with a cleft palate, a hearing test is recommended to determine whether ventilating tubes, which reduce the risk of ear infections, should be placed in the ears at the time of surgery. In addition, an eye examination usually is recommended because associated eye anomalies are not uncommon.

The cleft palate is usually repaired when the infant is around 10 months old, when a child begins to make sounds of speech. Sometimes surgery is postponed if the lower jaw is still retruded. Palate surgery often takes about two or three hours, with the infant staying in the hospital one or two nights. Although a child's speech usually is normal after cleft palate repair, in some cases, speech therapy or a second palate surgery may be needed. In addition, orthodontic treatment (braces) will usually be necessary.

A child with Pierre Robin sequence may encounter social and emotional challenges unique to this condition. Support organizations and resources are available for children and their families.