Pierre Robin Sequence/Pierre Robin malformation
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Pierre robin sequence (PRS) is a rare condition characterized by cleft palate, retrognathia and glossoptosis, PRS is a sequence, i.e. a chain of certain developmental malformations, one entailing the next. The reported birth prevalence of PRS ranges from 1 in 2000 to 1 in 30, 000, Bush and Williams suggested 1 in 8500. A genetic cause to PRS was recently identified and may be caused by genetic anomalies at chromosomes 2, 11, or 17; in addition to that etiology is classified as isolated form, syndromic form and unique PRS. This rare syndrome presents the symptoms alliance to classical traid, U or V shaped cleft palate, microganathia/retroganathia, glossoptosis and associated anomalies of syndromes mainly with regard to respiratory and feeding problems.there are various advancement in the area of perinatal diagnosis of PRS, which help us to early identification and effective management. On the other hand physical diagnosis and cephalometric radiographs helps to manage postnatally. The neonate and infant with PRS require care from multiple specialists specially in overcoming respiratory and feeding problems, periodically these problems can be managed conservative way, but in severe form it require surgical interventions like glossopexy, tracheostomy, distraction osteogenesis of the mandible and repair of associated defects. This article mainly emphasis on the recent advancement and literature in relation to PRS.
Pierre Robin sequence, upper airway obstruction, retrognathia/retrogenia, glossoptosis, cleft palate, Genetics, syndromes, feeding, perinatal diagnosis, distraction osteogenesis of the mandible.