Cleft - Ultimate Treatment
Pierre Robin sequence - The ULTIMATE Clinical pearls To achieve the best clinical outcome various aspects have to be followed. Ignoring one or more of these aspects may lead to suboptimal aesthetic and functional results. 1. The Pierre Robin sequence (PRS) is subdivided into TWO different categories: 1) the Siebold-Robin sequence (SRS) without a cleft palate, and 2) the Fairbairn-Robin triad (FRT) with a cleft palate. Both subcategories have a wider spectrum of diversity than assumed. 2. With 266 patients (FRT + SRS) this PRS-database appears to be the largest documented in the world. 3. Syndrome: Whereas SRS (7.9% - 21 of 266, FRT 92.1% - 245 of 266) may be associated with one syndrome in 9.5% (2 of 21), in FRT 20.8% (51 of 245) were associated with 18 other syndromes (mostly Stickler – 9.0% and Demarquay-van der Woude – 2.4%) . 4. Catch-up-growth: Verifiable only in 34.7% (51 of 147 reviewed cases) of FRT cases. 5. Midfacial growth: Whereas normal midfacial growth occurred in 73.2%, midfacial hypoplasia developed in 3.8% (6 of 157 reviewed cases) of FRT cases. 6. Oligohydramnios: 65.3% of mothers with FRT children could reveal a pregnancy history. In 20.0% (32 of 160 reviewed case) of FRT patients it was verified. 7. In facial cleft deformities, FRT revealed an incidence rate of 5.9% (245 of an overall of 4158 cleft cases). The reason for the development of micrognathia in utero, a previous cornerstone of the oligohydramnios and mandibular catch-up growth theory, could not be verified.
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