EUSKLINIC · INSIGHTS
Smith-Magenis Syndrome at the Dental Level
Regarding two cases and bibliographic review
Summary
Smith-Magenis syndrome is characterized by a distinct facial appearance and developmental delay, associated with a molecular abnormality in the critical zone 17p11.2. It is a complex, genetic and multisystemic disorder that presents a pattern of cognitive defects, accompanied by a phenotype of behavioral alterations, which include self-harm, aggression, impulsivity and problems falling asleep.
Facial dysmorphia is characterized by brachycephaly, prognathism, a wide and prominent forehead, a wide nasal bridge, and an everted upper lip.
At the dental level, taurodontism can be found. Other findings are the agenesis of the mandibular second premolars, dislaceration of the roots, a greater increase in caries and the need for restorations, poor dental hygiene with abundant plaque and gingivitis.
Along with prognathism, protrusion of the lower anterior teeth, increased chin size and macroglossia can be seen.
We present two clinical cases with the diagnosis of Smith-Magenis syndrome and with multiple cavities for treatment with general anesthesia.
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