What is macrostomy?

Macrostomy, also known as Tessier No, 7 and a transverse cleft of the face, is the genetic deformity of the skin, muscle and tissue surrounding the mouth. This craniophial abnormality is present at birth and best treated shortly after, since this type of cleft can seriously delay the development of speech and language. While macrostomy is rare, it is usually present in a genetic disease known as syndrome and spectacular macrostomy (AMS). It represents one of every 200 face clefts and is usually the most obvious when a child laughs or crying, because the missing muscles and tissue show up as the face stretches. The most common treatment of this deformity is myoplasty or plastic muscle surgery and other correctional facial procedures that are most successful if they are carried out shortly after birth. Other than aesthetic purposes, remedial surgery for macrostomia needs to learn the child to speak correctly and in more serious cases to be able to eat.

The most common form of this craniophial cleft is lateral macrostomy, which is when only one side of the mouth and face is affected. From the side clefts of the face, most occur on the left side of the face. An even more rare form of macrostomy is a bilateral cleft of the face that affects both sides of the mouth and face. This can often get the baby to see that it has a mask or has two faces. These cases require remedial surgery as quickly as possible because children with bilateral macrostomy often have extreme food problems or cannot eat at all.

This craniophial abnormality is often part of larger AMS. This genetic disorder is extremely rare and includes deformities of head, face, abdominal walls, nipples, fingers, genitalia and skin. Together with side or bilateral facial cleft, children with AMS often have few lids, eyelashes and eyebrows. While corrective surgery is used to treat AMS for both face clefts and other abnormalities, the chances of mental lipArdation with AMS is extremely high.

While the appearance concerns patients with macrostomy, the subsequent speech delay and learning disabilities associated with these delays are the biggest problem of doctors and parents. Surgery is not guaranteed to remedy the problem, but in most cases it dramatically improves macrostomy. Therefore, remedial surgery performed at the closest possible time is the best treatment for these patients and provides their best chance of normal growth and development.