In the Interest of Accuracy
Facts about Clefting
1. What causes a cleft?
A cleft is caused in utero, when the tissues of the obicularis muscle (the tire-shaped muscle that forms the lips) and/or the tissues of the palate fail to fuse. This occurs in the fourth (lip) to the eighth (palate) weeks of gestation. The events that trigger this failure to fuse can be many and are often not known. Most clefting is the result of a multifactorial cause. Genetics is often involved, but is not always the only factor. There is usually a genetic factor and an environmental factor, even if it is the only cleft ever found in a particular family. Some of the environmental factors include, but are not limited to the following: animal resource vitamin A, over the counter cold medicines, cigarette smoke or second-hand smoke, environmental pollutants, recreational drugs or alcohol, too little folic acid, etc. Most times a cleft is already determined before a woman knows she is pregnant. Oftentimes it is not what the mother does while pregnant that contributes to clefting, but what affects the pre-gestational womb environment in the month or so prior to conception.
2. What is the proper, acceptable terminology for cleft?
The proper term is “cleft lip”, “cleft palate”, or “cleft lip and palate.” An archaic and offensive term would be “harelip”.
3. How many kinds of clefts are there?
There are many different types of clefting. The most common would be classified as either unilateral (one side) or bilateral (both sides.) Either the lip or the palate or both can be unilateral or bilaterally cleft. Clefts can also be complete or incomplete (bisecting all of the tissue of that structure or some of the tissue of that structure. They can also be submucous (affecting underlying tissue while surface tissue is intact.)
A Unilateral Cleft Lip would mean that the tissue is split under one nostril from the lip to the base of the nostril, and through the alveolar ridge to the incisive foramen. (This structure is called the “primary palate” and, if fully cleft, constitutes a complete unilateral cleft lip.)
A Unilateral Cleft Palate is cleft from behind the incisive foramen (the place where the gums and the palate meet, going from mostly vertical to mostly horizontal) and all the way through both the soft and hard palate, through the uvula. (This structure is called the “secondary palate” and if fully cleft, constitutes a complete cleft palate.) A unilateral cleft palate is unattached to the Vomer bone on one side only.
A Bilateral Cleft lip is split just as a unilateral, but from beneath both nostrils. Often the premaxilla is flipped upward and protrudes.
A Bilateral Cleft Palate is split just as a unilateral cleft palate is, but it is not attached to the Vomer bone on either side.
A submucous cleft usually involves the soft palate, but can also involve the lip and/or the hard palate. If it involves the soft palate there may be feeding issues and there may be speech issues. If it involves only the hard palate, there may be no issues. If it involves the lip, there will be what appears to be a cleft repair scar at birth. Surgery can minimize this scar.
Tessier Clefts are a more significant form of clefting. A Tessier cleft can also be called a “cleft face”. They are very rare and they involve soft tissue other than the lip and palate, and they involved bony structures other than the alveolar ridge.
To see various types of clefting, go to http://www.widesmiles.org/gallery/
4. Can a cleft be detected before birth?
Yes. A cleft can be detected by ultrasound in as early as the 16th week of pregnancy. However, many times, a normal ultrasound may miss a cleft, especially if it is not expected. A high level, or level two, ultrasound is more accurate in detecting a cleft, while a 3D ultrasound is the most accurate of all. Many clefts are discovered through ultrasound today, allowing parents to prepare for the birth of their cleft-affected child. To see examples of sonograms showing cleft, go to http://www.widesmiles.org/gallery/sonogram/
5. What are the issues that a person with cleft will have to deal with in life?
The first issue will be feeding. There are special bottles made to feed a child with a cleft, but many families find them difficult to obtain. Mom often has to deal with the real loss of breastfeeding as well, since most babies born with a cleft palate will be unable to nurse at the breast.
A child with a cleft will also face a number of surgeries. While the minimum is probably three, if both lip and palate are cleft (lip repair, palate repair and bone graft), some have had as many as a dozen or more.
There may be speech issues involved with clefting. While speech issues are not as pronounced as they once were, a child with a cleft can still experience nasality in the voice and some articulation errors that will need to be corrected through speech therapy, or through secondary palate repair.
Children with cleft often have non-functioning eustacian tubes, resulting in recurring ear infections. With myringotomy tubes, this is often a minor consideration, but many of these kids end up getting several sets of tubes and many ear infections along the way. In generations past, these recurring ear infections often led to hearing loss.
Children with cleft may have some cleft-specific dental issues, such as supernumerary, ectopic, rotated and missing teeth. It is not uncommon for a child with a cleft to have poor enamaling on teeth, which then leads to more carries.
Children with cleft will need extensive orthodontia. They will most likely need to have their palates expanded in order to bring the palatal segments in proper alignment prior to bone graft. They will also need their teeth straightened, as the cleft disrupts the natural placement of teeth. Some will have missing tooth buds and will need those teeth to be replaced artificially.
Some children with cleft experience psychological issues if they endure a lot of teasing and ostracism from their peers. Self esteem can be an issue, though parents and professionals are much more aware of an responsive to those needs now. Many children with cleft who do endure teasing are able to rise above this cruel form of hurt and become stronger people as a result. Others carry emotional scars that far outdistance the scars left by surgery.
The important thing to note is, even with the above issues, a child born with a cleft is mostly a normal, healthy child who has a few more issues than most kids. The family faces each issue one at a time and they do not overwhelm that child’s life experience.
6. Are most children who have a cleft mentally retarded or learning disabled?
No. Some may be, but that would not be as a result of having been born with a cleft. A cleft is simply the failure of the obicularis muscle and/or the tissues of the palate to fuse. There are some syndromes that may include both developmental delays and clefting, but those are a minority of cleft cases. There are also syndromes that include clefting and other issues, but NOT developmental delays. Still, the overwhelming majority of clefts are non-syndromic. They are simply a matter of a failure of certain tissues to fuse prenatally.
7. How many people are born with a cleft?
World-wide, the number is one in seven hundred. That makes clefting the fourth most common birth defect, and the first most common craniofacial birth defect. However, the number is not equally distributed among races. Among Asian, Native American and Hispanic peoples, the rate of clefting is one in five hundred. Among those of European ancestry the rate is one in seven hundred. Among those whose roots reach back to Africa, the rate is only one in one thousand.
8. What is the probability of having a child with a cleft?
The probability for the general population for giving birth to a child with a cleft, when there has been no clefting in that family’s background is 0.14%. When there is a first degree relative (mother, father, sibling, self, child), the probability jumps to 3 – 7%. If the cleft is the result of a syndrome that is autosomal dominant, then the probability jumps to an astounding 50%. So, basically, depending on various factors, the probability can be anywhere from 0.14 – 50%. Only a geneticist can give a family an accurate probability rate, and then, only after a thorough genetic study.
9. Where can I find more accurate information about clefting?
Go to http://www.widesmiles.org/ for the most accurate, lay-friendly information about clefting. You might also contact the Cleft Palate Foundation at 1-800-24-CLEFT.
10. I want to contact persons for interview. Can I get a list of names and contact information from you?
Yes. You may interview me, Joanne Green. I am mom to three children born with cleft and the founding director of Wide Smiles. You can also contact me for a list of families who have agreed to be interviewed. The list is annotated so that you can choose according to your needs. To find the list, please write me at JoSmiles@aol.com.