Robinow syndrome or fetal face syndrome is a rare inherited disorder characterized by short stature, mesomelic brachymelia, hypoplastic genitalia, and a typical facial appearence("fetal face") with fromtal bossing, hypertelorism, ear
abnormalities,
a
short upturned nose, long piltrum, micrognathia, and macrocephaly.
Intraoral features have included quite a few dental cavities, crowding, hypoplastic uvula, cleft lip or/and cleft palate, gingival hyperplasia, alveolar hyperplasia, enamel hypoplasia, delayed eruption, and congenital missing of the permanent
teeth.
We report on a 10 years old girl with Robinow syndrome. The patient had most of the typical anomalies of the syndrome and negative family history but, in addition, had mental retardation, hearing loss, and serous otitis media. Intraoral findings
included dental cavities, crowding, hypoplastic uvula, repaired cleft palate, and mouth breathing.
Dental treatment and V-tube insertion(by dept. of ENT) were performed under general anesthesia.
In all cases of Robinow syndrome, thorough evaluation and united treatments with medical specialists should be performed.
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