![]() ![]() ![]() 5 The differences in defining the sequence also cause differences in the prevalence and in reports of related syndromes. 5įourteen different phenotypes of PRS were found, which accentuate the variability of the diagnosis and definition. The explanation of the sequence definition rather than a syndrome is based on early mandibular developmental disorder, which leads to abnormal tongue position, followed by a series of events, leading to formation of cleft lip and palate. The syndrome is defined as a sequence, a collection of anomalies caused by early developmental disorders or mechanical processes. 2,3 Williams and his colleagues have argued that not every child with a cleft palate and micrognatia is defined as suffering from PRS, but breathing difficulty is a necessary element for the definition 4. The reported incidence is estimated to be 1:8,500-14,000. The sequence is named after Pierre Robin, whose report was published in 1923 (earlier reports were in 1822). 1 Cleft palate is considered a common and additional feature, but not a perquisite for diagnosis. Pierre Robin sequence, Micrognathia, Retrognathic mandible, Glossoptosis, Orthodontic treatment IntroductionĪccording to the Clinical Consensus report on Pierre Robin sequence (PRS), Pierre Robin is comprising of mandibular micrognathia, glossoptosis and airway obstruction, leading to life-threatening obstructive apnea and feeding difficulties during the neonatal period. A multidisciplinary team including pediatric, ENT, general and oral surgeons, speech therapist, pediatric dentist, and orthodontic specialists should be involved in the treatment of PRS children for a relatively better quality of life outcome. The course of treatments, the complications, and their solutions is described. ![]() The child was diagnosed immediately after birth and suffered from respiratory and feeding problems that required tracheotomy and gastrostomy, and surgical repair of cleft palate. A boy with Pierre Robin sequence, an autosomal recessive inherited craniofacial anomaly, defined as suffering from a chronic condition that puts him in the category of children with special needs, received comprehensive dental and orthodontic treatment. ![]()
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