Macrostomy: photo, ablefaron-macrostomy syndrome

Macrostomy (macrostomia; macro- + Greek. stoma mouth) is a congenital deformation of the oral gap due to a transverse cleft of the face, it is a defect in the soft tissues of the corners of the mouth and cheeks in babies up to one year old. Sometimes the cleft reaches the earlobe and is accompanied by a malfunction of the facial muscles from the damaged side. Macrostomy is often accompanied by congenital malformations of the auricles, for example, a child may have obstruction of one ear.

What are the reasons

Macrostomy in most cases is not considered a separate disease, but is a significant part of other syndromes (most often hemichephalic microsomia).

Macrostomy is not a genetic disorder. There were cases when one of the born twins with a mouth gap was all right, while the other had a macrostomy. If the disease was a hereditary defect, then both twins were born with such facial deformities. The disease appears accidentally under the influence of negative factors during the carrying of a child. They have nothing to do with the chromosomal abnormality of the fetus. Scientists have not been able to identify the exact causes of the development of the disease.

It is important to distinguish macrostomy from ablepharon-macrostomy syndrome. This is a genetic disorder in which various anomalies of the head and face (craniofacial region), skin, fingers and even genitals develop.

In some, the ablepharon-macrostomy syndrome is accompanied by problems in the development of the nipples and abdominal wall, a delay in the development of the tongue, and sometimes mental retardation. The video shows the story of one boy with such a pathology:

How to identify a defect: symptoms

In addition to an external defect with a macrostomy on a child's face, irritation may appear as a result increased salivationbecause the mouth is open all the time.

The fundamental signs of a macrostomy:

• deformation of the oral gap;
• increased salivation;
• pathology, deformation of the mouth corners;
• "big mouth";
• auricle pathology.

Macrostomy in the photo:

Ablepharon-macrostomy syndrome:

How is the treatment carried out?

The entire course of treatment of the disease is aimed at suppressing some of its signs and manifestations.

Macrostomy can only be cured by surgery.

During therapy, doctors pay great attention to the condition of the mouth, it is important to prevent tissue infection, since this will significantly aggravate the condition of the little patient.

With macrostomy, plastic surgery is indicated, which is very important to carry out in the first 10 years of a child's life, otherwise the rehabilitation process and the prognosis will not be very good.

Surgical intervention is contraindicated in acute leukemia, with bone marrow pathologies and with a weakened patient's body. After a course of rehabilitation, the patient is often prescribed prosthetics front teeth, this can be called the final stages of treatment.

The operation is performed on children over a year old. After excision of the scar-altered edges of the gap, the soft tissues are sutured in layers. Particular attention is paid to the formation of the angle of the mouth.

Treatment of transverse facial clefts can be performed in the first year of life. After surgery, the child is able to eat normally. It is very important to keep the baby calm. He should not scream, because wide opening of the mouth brings pain and in itself provokes the baby to scream, and this has a bad effect on the healing process.

The goal of treating ablepharon-macrostomy syndrome is to eliminate some of the symptoms. Commonly used treatments have good results. For example, prior to initiating a broader therapy, appropriate eye drops and / or other adjuvant techniques can be used to prevent or relieve eye irritation and their dryness.

Sometimes with the help of plastic and reconstructive surgery it is possible to correct some malformations (anomalies of the eyelids, mouth and / or ears, malformations of the fingers, certain skin disorders, etc.). Other methods of treatment are only symptomatic and supportive.

Preventive measures

There is no way that could completely eliminate the risk of disease. But during pregnancy, you can follow some recommendations that reduce the risk of developing a macrostomy:

1. Monitor your blood sugar regularly. If possible, follow a diet in which light carbohydrates will be excluded.
2. Normalize a woman's body weight during pregnancy planning.

During the period of gestation, it is necessary to lead a correct lifestyle.