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[Multiple peg-shaped teeth associated with acrocephalosyndactyly. A variant of the Saethre-Chotzen syndrome? A
1Servizio di Odontostomatologia, USL n. 9, Ospedale Misericordia e Dolce, Prato.
Introduction:
The SC syndrome is relatively common among craniosynostosis syndromes, and it is transmitted as an autosomal dominant trait. The syndrome was first recognized and described by S. and C. (1931-1932), but that most extensive discussion of the disorder was published by Pantke et al. in 1975. He systematically described the most and the least common marks of that syndrome.
Clinical Case:
We thought it right to report a case we met and observed, because together with the S.C. syndrome's marks, there was the presence of multiple peg-shaped teeth. This peculiar mark is common to many orger craniosynostotic syndromes, but, we think it has never been described in "acrocephalosyndactyly type three" clinical cases. As a matter of fact, in literature, they have described in that syndrome, only lateral incisors shape anomalies.
Conclusions:
Even if in our patient the syndrome had a poor expressivity (there were scarce cranio-facial anomalies), and even if it came out there was no familiarity, it was peculiar because of the presence of multiple peg-shaped teeth. Besides, we think it is important to recognize that syndrome (which diagnosis is today still clinical because of the absence of peculiar laboratory aids), in such a way as to advice the female patient and to suggest her, in case of pregnancy, to go to a Uman Genetic Service for a an advice.