Pierre Robin sequence (or syndrome) is a condition in which an infant has a smaller than normal lower jaw, a tongue that falls back in the throat, and difficulty breathing. It is present at birth.Pierre Robin sequence, is characterized by micrognathia, glossoptosis, and airway obstruction.
Causes are: Mechanical basis: The physical craniofacial deformities of PRS may be the result of a mechanical problem in which intrauterine growth of certain facial structures are restricted, or mandibular positioning is altered.
Genetic basis: In the case of PRS which is due to a genetic disorder, a hereditry basis has been postulated, but it usually occurs due to a de-novo mutation. Specifically, mutations at chromosome 2, chromosome 4, chromosome 11, or chromosome 17 have all been implicated in PRS.
Diagnosis of PRS is generally diagnosed clinically shortly after birth. The infant usually has respiratory difficulty, especially when supine. The palatal cleft is often U-shaped and wider than that observed in other people with cleft palate.
Treatment of PRS: If the tongue obstructs the airway or if the infant is having 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 to breathe.
An infant with Pierre Robin sequence usually needs to be bottle fed, with breast milk or formula, using special nipples. The child may need supplemental calories to fuel the extra effort it takes to breathe and swallow.
Surgery is necessary to repair the cleft palate. Since ear infections are more common for a child with a cleft palate, recommend a hearing test to determine whether ventilating tubes, which may reduce the risk of ear infections, should be placed in the ears at the time of surgery..
The palate repair is usually performed when the child is around 10 months old or starting to make sounds of speech. The surgery may be delayed if the lower jaw is still very retruded. The procedure takes about two to three hours, with the infant staying in the hospital one or two nights. Although speech is usually normal after the cleft palate is repaired, in some cases, speech therapy or a second palate surgery may be needed. In addition, most children need orthodontic treatment with braces in early adolescence.