STUDY OF A CLINICAL CASE OF OTOCEPHALY: CAUSE, CONTROL AND CONSEQUENCES

Abstract

Otocephaly is a very rare and always mortal congenital malformation, that shows the displacement ventromedial of the ears (sinotia), mandibular aplasia (agnasia), absence of the tongue (aglosia) and microstomia. In this article, after a review of the studies achievements about the theme in the scientific medical, perinatal and obstetric literature, we show a clinical case in the Perinatology unit of the Hospital “Prince Lara” from the Puerto Cabello city, in Venezuela, of a fetus of a teen mother, sixteen years old, pregnant for 32 weeks, primigest, without pathological antecedents but no perinatal control. The pacient was refers with polidramnios to Perinatal Unit. A study ecographic has been made and this shows that there are few aspects that determines to the doctors to make a therapeutic amniodrainage to protect the mother´s life. The necrosic study and autopsy confirms the prenatal finds. The attention to the case shows the utility of the bidimensional ecography for the valoration of the cervico fetal facial pathology and the necessity of a good prenatal control as condition of a good and opportune diagnostic that allows a genetic advice adequate to the situation that always goes to mortal conclusion.

Keywords: otocephaly, sinotia, agnatia, microstomia.

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