Sometimes children are born with malformations, whether or not detected before birth. This is the case of une little girl born with two mouths, as the doctors who followed her report, in the BMJ. In the third trimester of pregnancy, ultrasound revealed a mass on the right side of the jaw of the fetus. The doctors following the mother then thought that it was a cyst, a teratoma or a fibrous dysplosia. Only, at birth, it turned out that it was none of that. It was aboutdiprosopy, or cranofacial duplication. This congenital disease causes certain parts of the face to be duplicated.
A girl born with two languages and two mouths
The child was born at term, without complications during pregnancy. At birth, a second mouth was observed on the right side of his face, measuring between 1 and 2 cm. This second mouth had an oral cavity, a tongue, and “little teeth”, explain those who followed her. The second language reproduced the same movements in synchronization with the main language when it fed. “At a follow-up appointment at 2 weeks of age, the newborn was healthy, was eating well and was gaining weight without oral incompetence “, say the doctors.
The little girl was operated
At the age of 6 months, the surgeons operated on the little girl to remove her second mouth, without further treatment being necessary. Six months after the operation, the sutures were perfectly healed. The child was feeding normally, and only a small swelling was visible on the bottom of his face. However, she still couldn’t move the right corner of her mouth, probably because a nerve had been cut during the intervention, explain the doctors.
Diprosopia, an extremely rare malformation
In their report, the doctors who followed the girl explain that only about 35 cases of children born with two mouths have been reported in the medical literature since 1900. Disprosopia is therefore an extremely rare malformation. This congenital malformation does not concern that the mouth, other parts, or even the whole face, can be duplicated. This phenomenon is due to an abnormal activity of the SHH protein, responsible among other things for the width of the facial features, which is essential in the cranofacial arrangement when the future child develops. In extreme cases like this, some parts of the face are doubled.
The case of this child is out of the ordinary for two reasons. Not only is cranofacial duplication a rare congenital disease, but babies are rare to survive. Most infants are born stillborn, or survive for up to a few hours. The fact that this little girl survived and was able to be operated on is therefore extraordinary.
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