León HTG, Loa UML

Language: Spanish
References: 7
Page: 84-88
PDF size: 280.15 Kb.

ABSTRACT

Robinow syndrome, described in 1969 by Meinhard Robinow presents facial features similar to those seen in a fetus of about eight weeks of age, called «fetal face». Featuring: a peculiar facies, short forearms and hypoplastic external genitalia. It is an inherited disease, extremely rare, reported an incidence of one per 500 000 live births. Its etiology is autosomal dominant mutation WNT-5A gene (3p14.3) and autosomal recessive gene is altered ROR2 (9q22); the latter is characterized by more marked semiological data and skeletal alterations. Its clinical features include: macrocephaly, moderate short stature, braquimelia mesomelic, genital hypoplasia, clinodactyly of the fifth finger, transverse palmar crease. At present stomatological triangular mouth, micrognathia, hypoplastic facial middle arched palate, gingival hyperplasia, cleft lip and palate, bifid uvula, large tongue, short lingual frenulum, malposition and dental crowding. The prognosis is generally good but the severity depends on the present complications such as heart or lung, as you determine the life expectancy of the patient. It is a congenital entity for which there is no specific treatment. We report a case of Robinow syndrome treated at the Dental Specialty Center.

REFERENCES

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