NBDE Quiz # 35
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NBDE Quiz # 35
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1. Question
A 5-month-old mentally retarded patient presents to your office and on clinical examination, you note brachycephaly, midfacehypoplasia, hypertelorism, and syndactyly of hands and feet. These findings are consistent with the diagnosis of what syndrome?
CorrectThe correct answer is A. Apert syndrome is a rare autosomal dominant disorder in patients has the traits described above as well as relative mandibular prognathism and cleft soft palate or uvula. Answer choice B is incorrect because Treacher–Collins syndrome is characterized by narrow, depressed cheeks, downward slanting palpebral fissures, and hypoplastic condylar and coronoid processes. Answer choice C, Eagle syndrome, is incorrect because it is a calcification of the stylohyoid ligament and has no other craniofacial deformities associated with it. Beckwith–Wiedemann syndrome, answer D, is not correct because it is a syndrome associated with gigantism, macroglossia, and visceromegaly.
IncorrectThe correct answer is A. Apert syndrome is a rare autosomal dominant disorder in patients has the traits described above as well as relative mandibular prognathism and cleft soft palate or uvula. Answer choice B is incorrect because Treacher–Collins syndrome is characterized by narrow, depressed cheeks, downward slanting palpebral fissures, and hypoplastic condylar and coronoid processes. Answer choice C, Eagle syndrome, is incorrect because it is a calcification of the stylohyoid ligament and has no other craniofacial deformities associated with it. Beckwith–Wiedemann syndrome, answer D, is not correct because it is a syndrome associated with gigantism, macroglossia, and visceromegaly.