Abstract
<h4>Maculopathy in Pierre Robin Sequences</h4> <p><b>To the Editors:</b><br>Pierre Robin sequence (PRS) is a congenital anomaly defined as micrognathia, glossoptosis, and cleft soft palate. The heterogeneous pathogenetic and phenotypic variations of PRS have prompted the term “Robin sequences.” An estimated 80% of all Pierre Robin cases form as part of a syndrome.<sup>1</sup> PRS is most commonly associated with Stickler’s syndrome, cerebrocostomandibular syndrome, and trisomy 18.<sup>2,3</sup> Secondary to abnormal gnathia and posterior displacement of the tongue, PRS results in feeding difficulty and respiratory obstruction that varies from mild to severe.4 The prevalence of PRS is documented at 1 per 8,500; the mortality rate ranges from 2.2% to 26%, following obstructive apnea and failure to thrive.<sup>4</sup> Ocular manifestations include myopia, strabismus, glaucoma, microphthalmos, cataract, retinal detachment, and nasolacrimal duct obstruction.<sup>5,6</sup> [more...]</p>
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